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American College of Physicians- Minnesota Chapter Annual Abstract Competition
Minneapolis Convention Center
November 2, 2012
Clinical Vignette
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Saranya
Balasubramaniam, MD
Warsame, Rahma MD;
Duggirala, Murali MD
Mathew Bartlett
Mark Nyman MD, FACP
Shedding Light on Voriconazole-Induced Phototoxicity
A 37 year old woman was initially hospitalized at another institution for a 6 month
history of nausea, epigastric pain, and a 50 pound weight loss. An upper endoscopy
showed a perforated ulcer for which she underwent partial gastrectomy with Roux-en-
Y anastomosis. Pathology of the ulcer revealed necrotizing granulomas with rare
degenerative fungal hyphae of unknown type concerning for Aspergillus. She was
started on Voriconazole 200 mg to be taken by mouth twice daily for 6 weeks.
Approximately one week after starting voriconazole she noted large, tense blisters on
her back, feet and hands. Physical exam was significant for an 8 x 10 cm tense bulla on
the dorsum of her right foot and a 6 x 6 cm tense bulla on the dorsum of her left foot.
They were fluid-filled and without surrounding erythema. Her skin was noted to be
diffusely hyperpigmented and further questioning revealed that she frequented tanning
salons. She had hypopigmented scars at sites of previously ruptured bullae on her back
and the dorsum of her hands. Laboratory testing demonstrated a mildly elevated CRP at
10.7 mg/L. Other laboratory testing was unrevealing including bullous pemphigoid
antibodies (BP-180 and BP-230), serum and urine porphyrins, as well as vasculitis and
autoantibody screening. Fluid cultures from the bullae were negative for bacterial,
fungal or viral growth. Review of the skin biopsy (performed at the outside hospital)
showed intraepidermal bulla with spongiosis, intracellular edema, and vacuolar
dermatitis consistent with a phototoxic drug reaction. A periodic acid-Schiff stain did
not show microorganisms. In consultation with dermatology, our patient was diagnosed
with voriconazole induced phototoxicity in the setting of ultraviolet tanning bed
exposure. Given that she had already stopped taking voriconazole, patient education
was the next step taken in her treatment. She was counseled regarding the avoidance
of sunlight and artificial tanning beds with future use of this medication. DISCUSSION
Voriconazole is a second generation triazole antifungal that is associated with
photosensitivity. Bullous-type photosensitivity as presented in this case is rarely
reported in the literature. Onset of voriconazole-induced phototoxicity can vary from
weeks to months depending on the degree of ultraviolet exposure. There is a paucity of
literature available on the incidence of voriconazole-induced phototoxicity with respect
to duration of therapy or intensity of ultraviolet exposure. Diagnosis is based on history
and clinical appearance of the eruption. Appropriate management is to discontinue
voriconazole if the clinical situation permits. Though infrequently reported, bullous-type
phototoxicty must be considered when bullae appear in the context of voriconazole use
and concomitant ultraviolet exposure. Given the common use of voriconazole, patients
should be educated to avoid sunlight and artificial tanning beds during therapy.
Pancytopenia in a patient reluctant to receiving blood transfusions
We report a case of pancytopenia, highlighting the initial approach, as well as the
management of one its most common causes. Briefly, we discuss the challenges
provided with patients refusing blood products, and the role of non-transfusion
therapies of aplastic anemia. Case A 20 year old Atheist male, from a Jehovah’s
Witnesses family was admitted with a 4 week history progressive light-headedness,
fatigue and an outside diagnosis of pancytopenia. He had otherwise been healthy,
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Fiona He, MD
except for a self limiting flu-like illness two months prior and was not taking any
medications or herbal supplements. He had been abstinent for the last 5 months, but
previously had consumed 8-10 units of alcohol a week. There was no history of tobacco
or illicit drug use. He reported one sexual partner over the last year and had no history
of sexually transmitted diseases. There was no history of toxic exposures or recent
travel, nor was there any family history of hematological disorders. Examination
revealed marked pallor of the palmar creases and conjunctiva. There was no
lymphadenopathy or hepatosplenomegaly. Auscultation revealed a grade 3/6, midsystolic
flow murmur loudest on inspiration, and located over the left sternal border
with no radiation. Workup discovered severe pancytopenia with an inappropriately
low corrected reticulocyte count. An extensive search for an underlying etiology,
including nutritional deficiencies, infectious and hemolytic disorders, as well as heavy
metal exposures, returned negative. A unilateral bone marrow biopsy showed a
hypocellular bone marrow with scant amounts normal appearing precursor cells, a
finding consistent with a diagnosis of aplastic anemia. Due to the family’s religious
beliefs, the patient was reluctant to receiving blood products. During his workup, the
patient was placed on neutropenic precautions and was initiated on prophylactic
antimicrobials. A contingency plan was set up in case he developed an infection or
spontaneous hemorrhage. After multiple discussions, the patient elected for packed red
cell and platelet transfusions with subsequent immunosuppressive therapy. Discussion
The differential for pancytopenia is broad and warrants an extensive work-up. Aplastic
anemia is a rare, but serious condition that results in pancytopenia. A bone marrow
biopsy is an essential test for diagnosing aplastic anemia and generally reveals an
absence or marked decrease in normal hematopoietic elements. Initial therapy includes
blood product support. Definitive therapy entails the use of immunosuppressive
therapy or, in appropriate candidates, hematopoietic stem cell transplants. Acute
infections, bleeding and severe anemia are complications of pancytopenia and require
immediate management. As the response to immunosuppressive therapy is delayed
over many months, it is unlikely that a patient would survive without access to blood
and platelet transfusions. While individuals may request non-transfusional therapies,
such as colony stimulating factors, there is no evidence suggesting a benefit of their use
in aplastic anemia.
Diabetes Insipidus as presentation of Acute Myeloid Leukemia
Introduction: Diabetes Insipidus (DI) is classified as either central or nephrogenic. Most
cases of central DI are idiopathic, but central DI may occur due to infiltrative diseases,
neurosurgical procedures, or trauma. The main presenting symptoms are polyuria and
polydipsia due to lack of ADH release and compensatory thirst to replace urinary water
losses. Case Presentation: A 53 year old M with no significant past medical history
presented with polydipsia and polyuria. 3 months prior to admission, the patient woke
up in the middle of the night with extreme thirst. From that point, he drank around 10
large glasses of water per day to keep up with his thirst demands. He also urinated very
frequently and woke up every 1-2 hours at night to drink or urinate. Diabetes Mellitus
was suspected, and he was prescribed Metformin for Hgb A1C of 6.2. Two months later,
he presented to the Emergency Department for new dyspnea on exertion, fatigue, easy
bruising and a 20 lb weight loss. He was anemic and thrombocytopenic. Bone marrow
biopsy showed hypercellular marrow with 16% blasts - consistent with Myelodysplastic
Syndrome with refractory anemia and excess blasts 2 (MDS-RAEB2)/AML. Subsequent
MRI showed loss of pituitary hyperintensity on T1 weighted images characteristic of
central diabetes insipidus. He was admitted for chemotherapy induction with Idarubicin
and Cytarabine. Na was 147 on admission. Serum osmolality was 302. Urine osmolality
was low at 78. Vasopressin was low at

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Hope Pogemiller, MD
Mahsa Abassi
Meera Sridharan, MD
Onelis Quirindongo-
Cedeno
absent without known pituitary pathology 10-15% of the time. Its absence is most
commonly noted with pituitary microadenoma, but can also be seen with central
diabetes inspidus. It is often inferred that DI is secondary to CNS infiltration of
leukemia, leading to the addition of intrathecal chemotherapy. Histopathological
analysis done on a small group of patients with DI and AML showed that 5 of 10 had
leukemic infiltration. Other findings were fibrosis, thrombosis, and necrosis of the
pituitary.
An Ancient Rash
Dermatologic manifestations in refugee populations may be challenging for the primary
care physician. Leprosy continues to affect millions of people worldwide including here
in the United States of America. A 32 year old female Burmese refugee presented to
her primary refugee screening exam with complaints of a red rash over her right
forearm. She described the rash as present for about 3 years, expanding, intermittently
pruritic, but without pain, loss of sensation, or ulceration. A refugee health screening
was negative for HIV, Syphilis, Chlamydia, Gonorrhea, and Hepatitis C. She had
immunity to Hepatitis A and B due to prior disease. TB-Quantiferon Gold was negative.
She had no past medical history and prior to her arrival, she had never seen a doctor.
Physical exam revealed a 3 x 7 cm annular macular lesion with a faint erythematous
border and central clearing on the right dorsal forearm. There was a half centimeter
hyperpigmented lesion centered in the middle of the rash and attributed to a burn
years ago. On her first visit, she was prescribed an over the counter fungal ointment for
presumptive tinea corporis. On follow up, she described minimal improvement in her
rash. With the fungal ointment, she did note her rash to be less erythematous and
raised. She was able to recall a close acquaintance in the refugee camp having a similar
rash. She was referred to dermatology for further work up. The dermatologist noted a
very subtle annular macule on her right forearm with a 5 mm faint pink rim with central
clearing. Examination with a Wood''s light did not reveal any pigment loss. Sensation to
light touch was faintly diminished at the site of the rash. She did not have any similar
lesions or depigmentation on the rest of her skin exam. A skin biopsy was performed
and pathology revealed acid fast organisms present, consistent with a clinical
impression of indeterminate leprosy. She was started on Rifampin and Dapsone for the
treatment of Mycobactrium Lepare. This case illustrates the need for a wider
differential when presented with dermatologic concerns in refugee populations. A
thorough history and physical as well as awareness of disease prevalence by region is
crucial for accurate diagnosis.
An Uncommon Cause of an Enlarging Scrotal Mass
Initial evaluation of a patient presenting with a scrotal mass involves a comprehensive
physical exam that will help determine if the scrotal mass is secondary to the swelling of
testis, spermatic cord, epididymis, or skin. The presence of pain, texture of the mass,
and transillumination can further help determine the etiology of the swelling. Case
Presentation: A 65 year old male presented to clinic for evaluation of a painless left
scrotal bulge. He first noticed the mass eighteen months ago and was diagnosed by an
outside facility with a variococele. The patient requested a second opinion as the
painless lump continued to grow. Initial examination demonstrated a 12-cm
inguinal/scrotal mass that transilluminated. The testicles could not be separated from
the scrotal tissue. Ultrasound of the scrotum was performed and revealed a large, fatty
appearing mass involving the left hemiscrotum. Urology was consulted and CT
abdomen-pelvis was performed and demonstrated the presence of a 16-x-9-x16-cm
soft tissue mass in the left inguinal canal consistent with liposarcoma of the cord. The
patient underwent left scrotal exploration, left radical orchiectomy and excision of
liposarcoma of the spermatic cord. Pathology specimens demonstrated an ‘18.5-x-16.5-
x-8.5-cm encapsulated, pale yellow, lobulated adipose tissue that was homogenous
except for a small focus of hemorrhage and fat necrosis.’ Final pathological diagnosis
was well differentiated liposarcoma (FNCLCC Grade 1). Discussion: Initial physical
examination of this patient was suggestive of a variococele by texture and hydrocele by
transillumination. Because of this discrepancy and continued growth of the mass,
further imaging was undertaken. Ultrasound and CT followed by surgery elucidated the
final diagnosis of a liposarcoma invading into the spermatic cord. Scrotal masses can
be testicular or paratesticular (epididymis, spermatic cord and fascia). Paratesticular
masses are usually benign and primarily arise from the spermatic cord. Although
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Ruth Bates, MD
Rahma Warsame,
Saranya
Balasubramaniam, Matt
Thoendel, Morgan
Medlock
Benjamin Bick, MD
Jason H. Szostek, MD;
Thomas F. Mangan, MD
liposarcomas are the second most common soft tissue tumor in adults, presentation
within the spermatic cord is extremely rare. Only 100 cases of malignant lesions within
the spermatic cord have been documented in the world. Most patients present in their
50s or 60s. Staging of liposarcomas is based on histological grade and presence of
metastasis. In this patient, no presence of metastasis was identified. The
recommended treatment of choice is surgery. Retroperitoneal lymph node dissection is
not indicated unless there is evidence of tumor invasion. If appropriate tumor resection
cannot occur, then radiation should be used because of relative resistance to
chemotherapy. Follow up of up to ten years is recommended. The patient described
here had no signs of reoccurrence at one year follow-up. This case, demonstrating the
invasion of a common cancer into an uncommon area, highlights the importance of a
methodological approach when working up any patient presenting with a scrotal
swelling.
Recurrent Oral Ulcerations
Severe recurrent oral ulcers can significantly affect a patient’s quality of life and
compels many to seek medical assistance. Unfortunately, the differential is broad,
often making precise diagnosis a challenge. Etiologies may be local or systemic;
traumatic, infectious, inflammatory, nutritional and even iatrogenic. One such case
was Mr. CO, a 22 y/o who presented with a two week history of extensive oral
ulcerations causing poor oral intake and dramatic weight loss. This was his third such
episode in a year and each episode (lasting over a month) had resulted in 5-7 kg of
unintentional weight loss in addition to successive loss of employment. Each episode
began as a single pale ulcer followed by multiple shallow ulcerations involving his entire
hard/soft palate, tongue, lips and gums. These shallow, irregular, often hemorrhagic
ulcerations resulted in such severe odynophagia that he was unable to swallow his
saliva. Review of systems was otherwise negative. He denied any recent illness, sick
contacts or participation in high-risk sexual behaviors. He took no daily medications
and was otherwise healthy. Prior work-up had been negative for evidence of antinuclear
antibodies, rheumatoid factor, HIV, HSV, candida, syphilis, gonorrhea or
chlamydia. EGD and colonoscopy were normal. A prior oral biopsy showed an
“ulcerative neutrophilic process without signs of vasculitis” and non-specific direct
immunofluorescence. Selectively repeated testing was again unremarkable, but repeat
oral biopsy showed lichenoid reaction consistent with erythema multiforme.
Erythema multiforme (EM) represents a spectrum of muco-cutaneous disorders
thought to be secondary to immune complex deposition. Manifestations range from
self-limited and rarely recurrent EM minor to life-threatening conditions such as
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Mr. CO was diagnosed
with an exclusively oral variant of EM major which is known to cause protracted,
recurrent but often self-resolving episodes of oral ulceration. While many cases are
idiopathic, HSV and mycoplasma pneumonia are known triggers. In the case of Mr. CO,
no such trigger was identified, but a short course of systemic prednisone was advised
for symptomatic relief. Because there is no definitive test for erythema multiforme,
Mr. CO illustrated the complexity involved in making this diagnosis. Clinically, oral EM is
distinctive for its widespread and often hemorrhagic mucosal lesions particularly
involving the lips. This often results in a swollen, ulcerated and crusted appearance. To
a large extent, however, oral EM is a diagnosis of exclusion and more common causes
of oral lesions such as viral infection, inflammatory bowel disease and medications must
first be ruled out. Though the results of direct immunofluorescence are often nonspecific
in oral EM, it is useful to exclude other immunological conditions such as
mucous membrane pemphigoid and pemphigus vulgaris.
Hey Stomach, What’s Your Kryptonite?
A 29 year old Hispanic male presented with a 2 year history of recurrent 1-week
episodes of severe nausea and vomiting with epigastric abdominal pain. These
episodes would typically occur early in the morning and last for several hours at a time.
Compulsory hot showers for up to an hour at a time provided some relief. He
presented to the emergency department several times and had normal abdominal x-
rays, normal esophagogastroduodenoscopy, unremarkable lab evaluation, and normal
urine drug screens including tetrahydrocannabinol (THC). On further questioning, he
admitted to smoking substances such as K2 and Kryptonite, which both contain a
compound similar to THC, up to 2 or 3 times per week over the past several years. After
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Wojciech Kraszkiewicz,
MD
Mahsa Abassi
David Miranda, MD
Yader Sandoval, MD;
Woubeshet Ayenew,
MD
previously smoking cannabis several years ago, he switched to K2 since it gave him a
similar sensation and was accessible at local gas stations and over the internet. He was
advised to discontinue these products, and his symptoms resolved within 2 months.
Described since 2004, cannabinoid hyperemesis (CH) is a form of cyclic nausea and
vomiting characterized by long-term, weekly cannabis use, cyclic nausea and vomiting,
resolution with cessation of cannabis, relief of symptoms with hot showers, and
abdominal pain. To date, there have been no reports of CH caused by synthetic
cannabinoid products. Products containing synthetic cannabinoids have been available
since 2004, often referred to as herbal products providing “legal” highs. Worldwide
legislation has tried to outlaw such products; however changes in chemical structure
have allowed makers to circumvent these laws. Furthermore, the metabolites excreted
in the urine require different assays for detection than used in standard drug screening
tests. These products often contain chemicals from the JWH family, which are potent
agonists of the cannabinoid 1 receptor, the same receptor by which THC produces its
psychoactive effects. Likewise, they often will produce similar adverse effects.
Reported short term effects after inhalation or consumption of synthetic cannabinoids
includes vomiting, coronary ischemia, and severe psychosis resulting in suicide
attempts. Reported long term effects include cognitive impairment and a dependence
syndrome. We believe that this case is the first reported case of cannabinoid
hyperemesis syndrome caused by a compound containing a synthetic cannabinoid. This
syndrome has been well described in patients who use cannabis, but may go
unrecognized in patients using products such as K2 and Kryponite. A normal urine drug
screen may point physicians away from this syndrome, and patients may not report use
if they believe they are using herbal products. Physicians should therefore have a high
index of suspicion for synthetic cannabinoid hyperemesis syndrome in patients who
present with classic symptoms despite a negative urine drug screen and lack of
cannabis use.
Something on Brain
Toxoplasmic Encephalitis is a relatively common disease process in HIV,
immunosuppressed patients who have less than 100 CD4 cells/mm3. Early
identification, treatment and prophylaxis is paramount to decreasing morbidity and
mortality associated with the disease. A 51 y/o Native American man with a history of
schizoaffective disorder, depression and Hepatitis C was brought into the hospital by his
case manager for 3 weeks of self neglect, isolationism, increased depression, and poor
hygiene. He was admitted to the mental health ward for mood stabilization and
reinstating of his medications. During his stay in the ward he was noted to have
episodes of confusion and socially inappropriate behaviors, such as, urinating on the
floor of his room. His vitals were stable. Physical exam was within normal limits. A
neurologic exam was nonfocal and despite periodic confusion he was noted to be alert
and oriented. An MRI was ordered and he was found to have a peripherally enhancing
28.3 x 29.6 x 23.2 mm mass in the cortical and subcortical parenchyma of the right
middle and inferior frontal gyri with surrounding edema and midline shift. Radiologic
impression was consistent with mass concerning for a primary CNS neoplasm or
metastasis. A CT of the chest, abdomen and pelvis was unremarkable. Neurosurgery
consultation was obtained and surgery was planned for resection. Due to several risk
factors including multiple sexual partners, drug and alcohol abuse, and homelessness,
an HIV test was sent at initiation of work up and returned positive at this time. Due to
his absolute CD4 count being only 7 cells/mm3, he was started on MAI and Toxoplasma
prophylaxis. He proceeded to surgery while all other laboratory studies were pending.
Preliminary pathology impression of the mass did not show any evidence for neoplasm.
At this time toxoplasma IgG returned positive. He was started on treatment for
toxoplasmic encephalitis. Final pathology results were positive for necrotizing cerebritis
with toxoplasma. HIV patients with CD4 counts less than 100 cells/mm3 and are found
to have a brain lesion should be presumptively started on treatment for toxoplasma
encephalitis. Neurosurgery should only be pursued if there is treatment failure.
“Boiling Water Through My Body: An Unusual Cause of Syncope in a Non-English
Speaking Patient¨.
More than 45 million people in the US speak a language other than English for routine
communication. - Non-English speaking patients represent a significant part of the
population treated at metropolitan county hospitals such as Hennepin County Medical
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Rahma Warsame, MD
Thomas Jaeger
Center. - Language barrier may be a risk factor for increased misdiagnosis in the non-
English speaking patient. Syncope accounts for 3-5% of ER visits and up to 3% of
hospital admissions. - Around 50% of patients are discharged without a clear diagnosis.
We present a challenging and unusual cause of syncope in which reaching a diagnosis
was complicated due to language barriers. Case Description An 81 year old non-English
speaking Hmong male with a history of hypertension and previous stroke was admitted
to our hospital for syncope. There were 3 syncopal events over the month prior to
admission. Physical exam was unremarkable and initial ECG showed a normal sinus
rhythm with left anterior fascicular block and right bundle branch block without
ischemic changes. Transthoracic echocardiogram had no major abnormalities and
cardiac biomarkers were negative. Throughout his initial course telemetry was
unrevealing. On the day in which discharge was planned, in light of unremarkable work
up he demonstrated intermittent complete heart block on telemetry. The onset of one
such episode was captured on ECG. Through the interpreter, the patient
communicated that each time the telemetry monitor alarmed he felt “boiling water
through my body”. Emergent transcutaneous pacing was instituted and subsequently
patient had a permanent pacemaker implanted for symptomatic paroxysmal AV block.
He was discharged to home without complications and he did not have recurrence of
his uniquely described symptoms. Discussion Paroxysmal atrioventricular block is a
poorly defined clinical entity characterized by abrupt and unexpected change from
1:1atrioventricular conduction to complete heart block, leading to syncope and
potential sudden cardiac death. Although no established predictors for paroxysmal AVB
exist, evidence of distal conduction disease at baseline is often present, with RBBB
being the most common finding. Prompt recognition of paroxysmal AVB is essential
because asystole with potential SCD may be preventable with permanent pacemaker
implantation. Non-English speaking patients are commonly hospitalized for evaluation
of syncope. Often workup of syncope may not determine the cause and a language
barrier increases the challenge in diagnosis. Thorough evaluation guided by the history
of presentation is necessary to reach the proper diagnosis and treatment.
CONCLUSIONS 1. Differentiation between the often benign and reversible vagal AV
block from paroxysmal AV block is critical. The hemodynamic consequences of
paroxysmal AV block are preventable by permanent pacemaker insertion. 2.
Management of non-English speaking patients is challenging, and thorough history and
examination with help from competent medical interpreters is essential to provide
optimal medical care.
Not Your Typical Headache
A 45 year old woman with no significant past medical history presented to the primary
care clinic with complaints of a persistent unilateral headache. It was a left sided,
throbbing headache that woke her up from sleep and persisted for three days. She
rated the pain as constant, 10/10 at its worst and progressively worsening. The
headache was exacerbated by minimal movement of her head to the left, without
alleviating factors. She had used alternating Ibuprofen 800 mg with 1000 mg of Tylenol
every four hours with minimal relief. There were no visual changes, nausea, vomiting,
fevers, chills, weakness, paraesthesias, dizziness, vertigo, ataxia or neck pain. She had
no head trauma nor performed heavy weight lifting or strenuous work outs. No prior
history of significant headaches. She had been seen in two urgent care clinics for similar
symptoms over the past week, and was prescribed Naproxen and rest. Physical exam
was remarkable for a toxic appearing patient and mild tenderness along the posterior
neck. Complete neurological exam was normal. Labs revealed a normal CBC and
electrolyte panel. Due to the ongoing and persist nature of her symptoms, magnetic
resonance imaging (MRI) and angiography were obtained. This demonstrated an acute
right vertebral artery dissection, with no involvement of the carotid arteries. She was
referred to Neurology and prescribed three months of 325 mg of aspirin. For pain relief
toprimate was used, and significantly reduced her symptoms. Repeat imaging three
months later showed complete resolution of the dissection and she returned to her
healthy baseline. Vertebral artery dissection is an uncommon cause of headaches and
stroke with an incidence of 1-1.5 per 100,000. It can become very challenging for
clinicians to determine which patients that present only with headaches require
additional imaging for diagnosis. Warning signs for headaches include the following:
new headache in patients older than 50, abnormal neurologic findings (papilledema,
diplopia, and visual changes), trauma, awakening from deep sleep, systemic symptoms
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Alexander
Vengerovsky, MD
Elfriede Agyemang,
M.D., William Ward,
M.D., Mark Nyman,
M.D.
Jonathan Alpern, MD
(fevers, malaise, or weight loss), and increased intensity with coughing, sneezing or
exertion. When these signs are present it is warranted to obtain imaging, specifically
MRI.
Obstructive jaundice associated with malignancy: An uncommon presentation of non-
Hodgkin’s lymphoma.
Acute obstructive jaundice is a rare presentation of primary non-Hodgkin’s lymphoma
of the common bile duct, although it can be seen in a number of malignancies and
usually results from primary biliary disease or metastatic spread of other
abdominopelvic malignancy. Radiological imaging could mimic cholangiocarcinoma. It is
important to pursue complete work in order to initiate appropriate therapy to reduce
significant morbidity and mortality. We report an example of a rare presentation of
diffuse large B cell lymphoma (DLBCL). A 73 year-old woman presented with a one
week history of right upper quadrant abdominal pain, malaise, dark orange coloration
of her urine, and pallor of her stools. Physical exam showed mild mucosal icterus and
cutaneous jaundice, as well as mild right upper quadrant tenderness. Laboratory
evaluation showed direct hyperbilirubinemia, mild elevation in alkaline phosphatase,
and elevation of liver enzymes to > 3 times ULN. Right upper quadrant ultrasound
showed intra- and extra-hepatic biliary ductal dilation and a 2.0 x 1.7 cm hypoechoic,
avascular mass in the region of the distal common bile duct. Appearance of the mass on
abdominal CT scan with contrast was suspicious for cholangiocarcinoma. In addition,
there were a number of hypo-enhancing lesions identified in the spleen and an
incidental finding of a duodenal ulcer. Endoscopic retrograde
cholangiopancreatography with biliary stenting identified a distal common bile duct
stricture and confirmed duodenal ulcer. Histopathology of the stricture biopsy was
negative for malignancy; however, the biopsy of the ulcer base revealed DLBCL. The
patient’s obstructive symptoms improved with biliary stenting within one day. A staging
PET/CT showed increased FDG uptake in the distal common bile duct, lymph nodes
above and below the diaphragm, and diffusely in the spleen. Bone marrow biopsy
revealed no marrow involvement. The patient was diagnosed with stage III DLBCL. She
was started on chemotherapy with rituximab, cyclophosphamide, doxorubicin,
vincristine, and prednisone. She had complete response as determined by repeat
PET/CT after two cycles of chemotherapy. There are very few case reports of NHL
presenting as acute obstructive jaundice in adults. Obstructive jaundice can be the
initial presentation of potentially curable malignancy, although it can mimic
malignancies such as cholangiocarcinoma which has poor prognosis. A complete workup
and timely therapeutic intervention reduces significant morbidity and mortality.
A silent transformation: A case for early screening and recognition
A 65 year old Ethiopian woman with a history of diabetes and rheumatoid arthritis on
chronic prednisone, presented with two weeks of nausea and vomiting and abdominal
pain. She denied fevers or chills. On physical examination, the patient was hypotensive
to 87/55 and tachycardic, which persisted despite receiving 5 liters of intravenous
fluids. Her abdominal exam was notable for hypoactive bowel sounds and tenderness
diffusely. A central line was placed, she was started on pressors, and admitted to the
intensive care unit. A CT scan of the abdomen revealed a distended gallbladder and
colonic thickening. The differential diagnosis at that time included CMV, Inflammatory
Bowel Disease, Rheumatoid arthritis, less likely Clostridium difficile. She was placed on
broad spectrum antibiotics for empiric coverage of organisms originating from the GI
tract. Blood cultures on day 2 of the hospitalization grew gram negative rods. The GI
team was consulted and a flexible sigmoidoscopy with biopsies from the descending
and sigmoid colon were obtained. This revealed nematodes with a strong suspicion for
strongyloides. A stool ova and parasites culture was obtained and this read: “chock full
of parasites”. The infectious disease team was consulted and after discussion with a
travel medicine specialist, it was felt that disseminated strongyloides was most likely.
Strongyloides serology was sent and this returned positive. The patient was initially
treated with 7 days of ivermectin. The patient has since been seen in travel clinic and
has been back to the hospital again for persistent joint pains. She was restarted on
ivermectin twice due to the initial concern that she was not adequately treated and
after the re-initiation of prednisone for debilitating joint pains. This case is informative
for many reasons. This patient developed the strongyloides hyperinfection syndrome
which continues to occur and be misdiagnosed in immigrants and refugees who are not
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Kate Birkenkamp, MD
Konstantinos Siontis, Jay
Jin, Shahnaz Ajani,
Christopher Farmer
Heidi Erickson, MD
adequately screened for strongyloides stercoralis. Many immigrants and refugees living
in the United States originate from countries that are endemic to strongyloides. When
patients with latent strongyloides become immunocompromised, the parasite
transforms into a more virulent organism-- a process still not entirely understood.
Mortality rates from the strongyloides hyperinfection syndrome approach 80% and
therefore Ivermectin, which has a low side effect profile, should be given empirically in
situations where laboratory tests results will take time to return. In order to prevent
this fatal complication, patients need to be screened and testing confirmed prior to the
initiation of immunosuppressive medications. In addition, a high index of suspicion is
needed for the strongyloides hyperinfection syndrome in immigrants and refugees
presenting with gram negative rod sepsis.
Mind the nodes: Diffuse Large B-cell Lymphoma masquerading as chronic respiratory
infection
Introduction: Diffuse large B-cell lymphoma (DLBCL) frequently manifests as an
enlarging nodal mass and systemic “B” symptoms. Extranodal involvement can lead to
variable presentations that may mask or delay diagnosis. We present the case of a
patient with chronic respiratory symptoms and pulmonary nodular infiltrates leading to
an extensive infectious work-up two years prior to final diagnosis. Case: A 64-year-old
male presented to his healthcare provider with fever, chills, right upper quadrant
abdominal pain, and acute-on-chronic dyspnea. He initially developed dyspnea and dry
cough two years prior to presentation while on a hunting trip, which recurred while
hunting a year later and persisted for the next 5 months. He was treated for pneumonia
with levofloxacin and prednisone followed by azithromycin and prednisone without
significant improvement. A few months prior to presentation, a chest CT demonstrated
mild hilar adenopathy and bilateral nodular pulmonary infiltrates. He underwent
bronchoscopy with BAL sampling and biopsies as well as VATS, all of which were nondiagnostic.
He was continued on prednisone for suspected atypical fungal infection with
initial improvement followed by progressive worsening of his respiratory symptoms.
Two weeks prior to presentation he was evaluated for jaundice and acute epigastric
pain and was found to have an obstructive pancreatic mass for which he underwent
ERCP and stent placement with subsequent worsening of abdominal pain and chills,
concerning for cholangitis. A pancreatic biopsy was unrevealing. On admission to our
facility, he was febrile and required high-flow oxygen to maintain saturations >90%. A
repeat chest CT demonstrated worsening mediastinal and hilar lymphadenopathy as
well as bilateral ground-glass infiltrates. Transbronchoscopic needle aspiration of the
subcarinal lymph nodes yielded pathology concerning for lymphoma and the BAL was
positive for Pneumocystis jiroveci. His respiratory symptoms improved with Bactrim. A
PET-CT scan revealed extensive FDG-avid lymphadenopathy above and below the
diaphragm. Subsequent paratracheal and subcarinal lymph node biopsies were
consistent with diffuse large B-cell lymphoma. Discussion: This case illustrates an
uncommon presentation of DLBCL that resulted in delayed recognition and onset of
appropriate therapy. DLBCL is common among the lymphoproliferative malignancies,
representing approximately 30% of all non-Hodgkin’s lymphoma cases. Extranodal
involvement, as seen in our patient, is documented in approximately 40% of cases. This
patient’s respiratory symptoms along with bilateral pulmonary infiltrates focused
investigations on an infectious etiology, despite evidence of mediastinal adenopathy.
His course was further complicated by PCP acquired while on steroid therapy without
prophylaxis, and extraluminal biliary tree obstruction, presumably due to a lymphoid
mass. This case highlights the importance of ruling out malignancy in an older patient
with mediastinal adenopathy and constitutional symptoms. Selection of appropriate
biopsy sites is also important for timely diagnosis, as this patient underwent at least
three biopsies and VATS before diagnosis.
Darling’s Disease: Disseminated Histoplasmosis in a Renal Transplant Recipient
Introduction Samuel Darling first described histoplasmosis at the beginning of the 21st
century. Histoplasma capsulatum is a dimorphic fungus found in soil contaminated with
bird or bat droppings. It is the most common endemic fungal infection seen in humans.
T-lymphocytes are crucial in limiting the extent of the disease. Case Description A 67
year old male with history of end stage renal disease secondary to type 1 diabetes
status-post deceased donor renal transplant presented with fever, chills, diarrhea,
leukopenia, thrombocytopenia and hyponatremia. A broad infectious work-up was
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Gayatri Acharya, MD
pursued. Initial chest X-ray was clear. Serum CMV PCR negative. On hospital day #5,
patient developed acute hypoxemic respiratory failure with bilateral pulmonary
infiltrates, refractory septic shock, pancytopenia, DIC, transaminitis and acute kidney
injury. He was endotracheally intubated and started on broad-spectrum antimicrobial
therapy. His immunosuppression was stopped. On day #9, patient’s urine and serum
histoplasma antigen (Ag) returned above the limit of quantification at which time
liposomal amphotericin B was started and all other antimicrobials were discontinued.
Histoplasma was subsequently cultured from fungal blood cultures and bronchoalveolar
lavage washings. CSF histoplasma Ag returned positive, however at a much lower titer.
Patient was extubated on day #16. He developed a drug rash on amphotericin B and
was transitioned to oral itraconazole. His renal function recovered, blood counts
improved and transaminases trended down. He was restarted on prednisone for
immunosuppression; mycophenolate was not resumed. He was discharged to a
rehabilitation facility on day #25. Patient has since returned home and is followed
closely by infectious disease. He remains on itraconazole with periodic measurements
of urine and serum histoplasma Ag to monitor progress of treatment. Discussion The
incidence of disseminated histoplasmosis is greatest among immunocompromised
individuals, especially those with HIV. The incidence in solid organ transplant recipients
is low, 1 case per 1000 transplant-person-years, and is mostly seen in renal and liver
transplant recipients.1 Disseminated infection may occur as a primary infection or
reactivation of latent infection. The diagnosis of disseminated histoplasmosis requires
a high index of suspicion. Serum and urine Ag detection is useful in
immunocompromised individuals when antibody production is often impaired.
Detection rates in disseminated disease are high; antigenuria is present in 92% and
antigenemia in nearly 100%.2 Cross reactivity is seen with blastomycosis and
coccidioidomycosis. Untreated acute disseminated disease is fatal over 2-12 weeks.
Treatment includes liposomal amphotericin B for severe disseminated and CNS disease
and itraconazole for mild to moderate disease and step-down therapy.3 Urine Ag levels
can be used to monitor response to treatment. References 1. Cuellar-Rodriquez, et al.
Clin Infect Dis. 2009;49(5):710-716. 2. Hage, et al. Clin Infect Dis. 2011;53:448. 3.
Wheat, et al. Clin Infect Dis. 2007;45:807.
Atypical Presentation of Erdheim-Chester Disease
Erdheim-Chester disease is a non-Langerhans histiocytosis which presents most
commonly with multifocal osteosclerotic lesions of the long bones. Presentation of
Erdheim-Chester disease without long bone involvement is quite rare. Case: A 50 year
old male presents from an outside hospital for further evaluation of possible systemic
inflammatory syndrome. Approximately six months prior to admission he began to have
fatigue, chest pain, shortness of breath, as well as abdominal pain, nausea, and
vomiting. He presented to the outside hospital and after a CT was performed of the
chest, abdomen, and pelvis he was found to have a large pericardial effusion, and
subsequent bilateral pleural effusions. A VATS was performed and fluid analysis showed
acute on chronic inflammation with atypical cells. On this admission, he presented with
new ascites, worsening abdominal pain, nausea, vomiting, loss of appetite, and acute
kidney injury. His ascites had been worsening for approximately 1-2 weeks, leading to
weight gain of 25 pounds. In addition, he had a leukocytosis, anemia, and elevated LFTs.
CT performed at outside hospital prior to transfer showed symmetrical hyperdense and
hyperenhancing soft tissue around the kidneys, with associated soft tissue thickening
around the aorta as well as the proximal iliac arteries. This perinephric and periaortic
rind raised concern for Erdheim-Chester disease. The patient denied any symptoms of
joint or bone pain. With the diagnosis of Erdheim-Chester raised, a bone scan was
performed. Results of the scan showed no definite medullary changes to support a
diagnosis of Erdheim-Chester disease. One zone of sclerosis and periosteal reaction was
found in the mid right femur, but this was thought to be a post-traumatic deformity
relating to an injury sustained by the patient after a fall while hiking in October 2011. To
further evaluate for Erdheim-Chester, a biopsy of the perinephric rind was taken. Biospy
revealed sheets of foamy macrophages, and immunophenotypic findings were CD68
positive and CD1A negative, consistent with a diagnosis of Erdheim-Chester disease.
Discussion: The most common presentation of Erdheim-Chester involves osteosclerotic,
xanthomatous or xanthogranulomatous infiltration of the long bones with foamy
macrophages. Patients typically present with associated juxta-articular pain. A small
subset of these patients may be asymptomatic, however bone scans will reveal the
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Fateh Baxerbachi, MD
Sherry-Ann Brown, MD
Eugene Scharf, MD;
Miguel Lalama, MD;
Anna Svatikova MD,
PhD; Sorin Pislaru, MD,
PhD
lesions. When the disease involves the retroperitoneum, it will present with rind-like
fibrosis surround organs and vessels, often causing organ dysfunction. Biopsy will
demonstrate foamy histiocytes, with lack of vasculitis or plasma cells. While cardiac
involvement does present with moderate frequency in Erdheim-Chester patients,
pleural involvement and symptomatic retroperitoneal involvement with fibrosis are less
common. One should consider pursuing biopsy of suspicious rind-like fibrosis, even in
the absence of bone findings, when symptoms are significant and vessel or organ
function is compromised.
Uncomfortably Numb: Systemic Toxicity Following Ropivacaine Continuous Nerve Block
Ropivacaine has been shown to be a safe long acting local anesthetic, with less cardio
and neurotoxicity than other local anesthetics. One report documented infusion of 4-6
mL/hr of ropivacaine 0.2% for six days without any adverse events. Another report
estimated the incidence of ropivacaine-induced toxic events to be 6-8 per 1,000,000
patients. ON-Q C-bloc is a continuous peripheral nerve block system that slowly infuses
local anesthetic near a nerve for pain relief. A 57 year old man with infiltrative
cardiomyopathy, pulmonary hypertension, type II diabetes mellitus, and end-stagerenal-disease
on peritoneal-dialysis was admitted for an ischemic right lower extremity
and underwent right above-knee-amputation. Post-operatively, he was started on a
right femoral catheter with infusion of 0.2% ropivacaine at 7 mL/hr through an I-Flow
ON-Q Pump. After two days, he was noted to be more lethargic and confused
throughout the day. He was given oxycodone and gabapentin. Later, he was noted to
have lethargy and bradycardia to the 40s. Physical exam revealed hypotension and
muscle tremors. Telemetry demonstrated sinus bradycardia alternating with junctional
rhythm and sinus pauses. On further questioning, he was found to have dysgeusia and
tinnitus. Ropivacaine toxicity was suspected and he was started on the lipid infusion
protocol. A total of 1125 ml of lipids was given. Initially, his bradycardia improved and
his blood pressure remained stable. However, due to his multiple comorbidities
(including renal failure), ropivacaine was difficult to eliminate from his circulation. A
trial of plasmapheresis was not successful, and later he worsened and expired.
Ropivacaine toxicity is rare and less common than other amide anaesthetics such as
bupivacaine. However, recommendations for maximum doses of local anesthetics are
not always evidence based. In August 2012, the FDA has issued a recall on the I-Flow
ON-Q Pump with ONDEMAND bolus button because of overdose risk. Infusion of lipid
emulsion has been shown in animal models to reverse cardiac toxicity through a
combination of reduced tissue binding by re-established equilibrium in a plasma lipid
phase and a beneficial energetic-metabolic effect. Case reports have suggested its
clinical efficacy in humans.
Oh Deer, Tick-Tock Heart Block
Lyme disease, a bacterial infection of the spirochete Borrelia burgdorferi, can affect the
myocardium, pericardium, and conduction system. Cardiac manifestations of Lyme
normally present weeks to months after infection in the second phase (early
disseminated phase). The most common presentation is atrioventricular block (leading
occasionally to syncope when high-grade block is present ), but myopericarditis can also
be seen. Chronic Lyme carditis has been reported to cause cardiomyopathy and can be
fatal. Diagnosis is confirmed by serology. Treatment consists of antimicrobial therapy
and prognosis is good with expected full resolution of conduction block and
inflammation. A 67 year old man from Wisconsin with past medical history significant
for hypertension, hyperlipidemia, and coronary artery disease, presented in July with
syncope. He endorsed generalized weakness, fatigue, malaise, fevers, chills, night
sweats, front-temporal headaches, and neck pain. He denied cough, dyspnea,
orthopnea, paroxysmal nocturnal dyspnea, lower extremity edema, chest discomfort,
palpitations, photophobia, arthralgias, nausea, or vomiting. Some of his symptoms
began four months prior to presentation and progressively worsened to the day of
admission. ECG on admission showed normal sinus rhythm with new-onset first-degree
AV block. On lab testing, his Troponin T was 0.24, 0.28, 0.31 ng/mL, and D-dimer >2000
ng/dL. He was initially managed as possible acute coronary syndrome, with intravenous
heparin and aspirin. A chest computed tomography scan showed no pulmonary
embolus. A transthoracic echocardiogram (TTE) showed normal ventricular size and
function with an ejection fraction (EF) of 60%. A dobutamine stress echocardiogram
was negative for myocardial ischemia. Chest x-ray, urinalysis, and blood and urine
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Ian Cheng-Yi Chang,
MD
Helina Kassahun, MD,
Uma Valeti, MD
cultures were unremarkable. The patient developed non-tender non-pruritic multifocal
progressive circular macular erythematous skin lesions on the lower extremities. The
lesions resembled early-onset erythema chronica migrans (ECM), albeit not the classic
target lesions. Lab testing yielded elevated C-reactive protein, erythrocyte
sedimentation rate, AST, lymphocytopenia, neutrophilia, and a positive Lyme
disease/Borrelia ELISA with confirmatory IgM Western blot. A cardiac MRI showed
epicardial delayed myocardial enhancement and hypokinesis, highly suggestive of
myocarditis. Telemetry revealed a transient second-degree Mobitz type I AV block. He
was diagnosed with Lyme Carditis. Due to cardiac involvement, he was treated with 6
weeks of oral doxycycline 100 mg twice daily. Two weeks after discharge, a follow-up
ECG showed resolution of heart block. A follow-up TTE was unremarkable, and skin
lesions and symptoms resolved. This patient’s presentation was typical for the time of
year in a Lyme endemic area. It is certainly possible that the patient might have missed
rashes preceding his admission. The likelihood of this is supported by the positive ELISA
and Western blot anti-Borrelia antibodies that usually appear 2-4 weeks after an ECM
rash. Lyme carditis should be considered in patients from rural areas who present with
pre-syncope or syncope.
Magnetic Resonance Imaging of Cardiac Tumors: A Case Report and Series From a
Tertiary Medical Center
Cardiac tumors are rare, with estimated prevalence of 0.002–0.3% at autopsy.
Symptoms and prognosis of cardiac tumors depend on size, location, and intrinsic
aggressiveness. Cardiac magnetic resonance (CMR) imaging is non-invasive and offers
unsurpassed soft-tissue characterization. CMR imaging facilitates accurate diagnosis,
prognosis, and treatment planning. We report an unusual case of primary cardiac
lymphoma, and a series of 32 patients with cardiac masses who underwent CMR
evaluation at our hospital within the last five years. Case Presentation A 55-year-old,
previously healthy woman with progressive chest pressure, exertional dyspnea,
orthopnea, palpitations, fatigue, and leg edema for four weeks was referred with a
cardiac mass. Physical exam revealed a distended internal jugular vein, irregular heart
sounds, ascites, and lower-leg edema. Right-sided heart failure from obstruction by the
cardiac tumor was clinically diagnosed. Initial labs showed a white blood count of 9.4
/mm3, with 79.9% neutrophils and 12.8% lymphocytes. CMR imaging confirmed a large
heterogeneous mass with necrosis and severe edema filling the right atrium and
ventricle. Encasing the aorta, right coronary artery, and pulmonary arteries, this mass
also caused tricuspid valve dysfunction. These features precluded tumor resection, and
the patient was admitted for high-risk cardiac transplantation. Given the risk of tumor
seeding, cardiac biopsy was initially discouraged. However, CMR imaging identified a
path via the inferior vena cava for safe biopsy, which diagnosed a diffuse large B-cell
lymphoma. The patient was successfully treated with chemotherapy. Case Series We
reviewed 32 CMR studies performed from January 2007-August 2012 for cardiac masses
in symptomatic patients, and masses diagnosed incidentally by other imaging
modalities. Made by histopathology or clinical evidence, final diagnoses included
primary benign tumor (N=4, 12.5%), primary malignant tumor (N=3, 9.4%), metastasis
(N=7, 21.9%), thrombus (N=9, 28.1%), and pseudotumor (N=9, 28.1%). Imaging features
commonly seen in malignant tumors include tissue heterogeneity, large size (> 5 cm),
involvement of more than one cardiac chamber and adjacent structures, and presence
of contrast enhancement. In addition, we identified a previously unreported pattern of
tumor perfusion and contrast enhancement in aggressive malignant tumors; this
enhancement gradient could represent varying degrees of vascularity and tumor
necrosis. Tumors and thrombi could be differentiated by thrombus correlates:
characteristic locations, adjacent scarred myocardium, and lack of contrast
enhancement. Discussion CMR imaging of cardiac masses is invaluable for diagnosis,
prognosis, and patient management. In our case report, it was instrumental for
avoiding a high-risk transplant and instead pursuing accurate diagnosis and successful
treatment. In many instances, CMR imaging serves as the final diagnostic arbiter before
histological confirmation. Structural information on great-vessel involvement, coronary
arteries, and valves is important for treatment planning. Physicians should be aware of
CMR imaging''s utility for cardiac masses, and consider ordering CMR studies when
clinically indicated.
RV4- Greg Solheid, MD Penny for your thoughts? Copper deficiency anemia masquerading as myelodysplastic
11

159 Anya Jamrozy, MD syndrome in a gastric bypass patient.
The number of bariatric surgeries in the US has increased dramatically over the past
decade, with an annual rate of approximately 200,000 adults. As a consequence, the
frequency of previously uncommon micronutrient deficiencies has been rising at an
accelerated rate. Therefore, an unexplained hematologic abnormality should prompt a
thorough nutritional evaluation in any patient with a history of bariatric surgery. Case
Presentation: This patient is a 59-year-old female with a history of Roux-en-Y gastric
bypass for morbid obesity in 2002, chronic vitamin B12 deficiency, and history of
alcohol abuse (no current alcohol use) with stigmata of alcoholic liver disease
demonstrated by elevated INR and chronic thrombocytopenia. The patient initially
presented with acute anemia (Hgb 7.1, MCV 107); laboratory evaluation revealed
normal vitamin B12 level (>2000) and folate levels, total bilirubin of 4.0 (mostly
indirect), elevated reticulocyte count (3.5%), and decreased haptoglobin. Ferritin was
elevated at 1142 with an STFR of 7.2 and a STFR:ferritin index of 1.41 – not suggestive
of iron deficiency. Patient was evaluated by gastroenterology given the acuity of her
anemia; both an upper endoscopy and colonoscopy were performed with no significant
findings. Given negative GI evaluation in conjunction with her chronic alcoholic
thrombocytopenia and mild leukopenia (WBC 4.7), there was concern for underlying
infiltrating bone marrow process. Bone marrow biopsy demonstrated normocellular
bone marrow with 5% ringed sideroblasts; there were no findings consistent with
myelodysplasia. Given elevated ferritin and increased iron storage, the patient was
screened for hereditary hemochromatosis and was heterozygous for the Hfe gene (HLA-
H). (Heterozygous individuals are not at risk for developing hereditary
hemochromatosis). Urine and serum protein electrophoresis demonstrated no
monoclonal proteins. A nutritional evaluation was completed which demonstrated
markedly low copper and zinc levels. At this time consensus among clinicians was that
pancytopenia was directly related to her copper deficiency as a consequence of
malabsorption from bariatric surgery. Patient started on oral copper replacement (6mg
x2 weeks, 4mg x2weeks, and 2mg x4weeks); within six weeks of initiation of
replacement therapy, the patient had resolution of her leukopenia and anemia with
modest improvement in her chronic thrombocytopenia. Discussion: This case
illustrates the need for clinicians to include a nutritional assessment when evaluating a
patient with any hematologic abnormality. Bariatric surgeries are one of the most
common causes of acquired copper deficiency; however, the clinical manifestations are
often offset by many years following the surgery and make this diagnosis challenging.
Copper plays a significant role in hematopoiesis and thus should be one of the first
micronutrients evaluated in the setting of explained pancytopenia
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TAVR to the Rescue: A New Option for the High Risk Patient
Aortic Stenosis is a degenerative valvular disease that worsens over time and occurs at
younger age in patients who have a congenital bicuspid valve. The prognosis of the
disease is worsened when patients start becoming symptomatic. Presented is a case of
severe aortic stenosis resulting in a decrease in left ventricular function in a patient
with a complicated medical history. A 43 year old female with a past medical history
significant for systemic lupus erythematosus with biopsy proven lupus nephritis, anti
phospholipid syndrome, and a bicuspid aortic valve presented to an academic hospital's
Emergency Department with shortness of breath, orthopnea, pretrial edema and a 10
pound weight gain over the past 12 days. In the ED, she was noted to have increased
work of breathing along with orthopnea, two plus pretibial edema, and a III/VI systolic
ejection murmur heart best at the right upper sternal border. Subsequently an
echocardiogram was done which showed that the patient had decompensated systolic
heart failure with an EF of 30% and severe aortic stenosis. Her hospital course was
complicated by her heart failure being refractory to medical management, renal failure
resulting in dialysis, and labs consistent with a lupus flare. She was deemed to not be a
surgical candidate for aortic valve replacement. She subsequently was deemed a
candidate for transcatheter aortic valve replacement (TAVR). This was done
successfully and she was discharged home with improvement of symptoms.
Symptomatic patients with aortic stenosis require intervention when refractory to
medical management. This usually requires surgical intervention but in high risk
patients who are not deemed to be a surgical candidate the options are limited. TAVR
is a new alternative therapy for medically complex patients.
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Kimberly Carter, MD
Eva M. Carmona
Porquera, MD, PhD
THDCC and Blair D.
Westerly, MD
Daniel Chan, MD
Christopher A. Aakre,
Rebecca C. Adair, Jason
A. Post, Siva S. Ketha
Chronic Sinusitis, Cough and Hemoptysis
Early recognition of the constellation of symptoms associated with granulomatosis with
polyangiitis will lead to prompt treatment and resolution of symptoms. Case
Description: A 69 year-old woman with a seven year history of chronic cough and
sinusitis presented with four weeks of progressive dyspnea and hemoptysis. She
endorsed fevers, night sweats, chills, weight loss of fifteen pounds, and hearing loss
over the past month. She stated that her symptoms began after a flood, which led to
mold exposure. Recent travel included the southwestern United States but no
international travel. Physical examination was remarkable for clear lungs, nasal
crusting, and middle ear effusions. Chest radiograph revealed multiple bilateral nodular
opacities, some of them with cavitation. Computed tomography scan of the chest
showed numerous bilateral cavitary pulmonary nodules and masses. Laboratory workup
was significant for positive cytoplasmic- type ANCA (c-ANCA) and anti-protease 3
(PR3 antibody). Urinalysis showed hematuria. Blood and sputum cultures were
negative for infection, and she was ruled out for tuberculosis. HIV and fungal serologies
were also negative. A bronchoscopy was performed and showed necrotizing vasculitis
without granulomatous formation. Bronchioalveolar lavage washings were negative for
infection or malignancy. A clinical diagnosis of granulomatosis with polyangiitis was
made. She was treated with high-dose steroids with improvement in symptoms. She
was discharged with plans to initiate immunosuppressive therapy with Rituximab.
Discussion: This is a classical presentation of granulomatosis with polyangiitis (GPA),
which is described as a systemic small vessel vasculitis characterized by frequent
involvement of the upper and lower respiratory tract. The disease most commonly
occurs in white adults, and patients may present with constitutional symptoms
including fever, fatigue, arthralgias, poor appetite, and weight loss. The presence of c-
ANCA with PR3 antibody is observed in the majority of patients with GPA. Other clinical
criteria include nasal or oral inflammation, abnormal chest radiograph showing nodules
or cavities, microscopic hematuria with or without red cell casts, or granulomatous
inflammation on biopsy of an artery or perivascular area. Two or more of these criteria
are very sensitive and specific for GPA. Early recognition of this disease will result in
prompt treatment and resolution of symptoms. Classic treatment involves high-dose
steroids and cyclophosphamide, but recent studies have shown that Rituximab offers
similar efficacy with less cytotoxic side-effects.
What’s eating my leg, or is it what I’m eating? An unusual presentation of diarrhea and
a chronic leg wound
A 70-year old male presents with a week-long course of severe non-bloody watery
diarrhea with associated dehydration, hypotension, nausea, and vomiting and is
discovered on admission to also have had a 6-month course of chronic outpatient
wound debridement for a large poorly-healing right lower extremity ulceration. He
states that his bowel movements prior to his current presentation have been normal.
He denies any food intolerance, constitutional symptoms, and unintentional weight
loss. His last colonoscopy was within the past year which was normal. His leg wound
initially presented six months prior as a red bump on his right lower leg that began to
rapidly enlarge and ulcerate over the course of one month. Initially this wound was
presumed to be associated with an insect bite or infection but failed to respond to
antibiotics within the first month. Vascular studies of the lower extremities were
normal. Over the next five months because of poor wound healing, multiple biopsies
were obtained which demonstrated nonspecific findings, negative fungal cultures, and
the concern for pyoderma gangrenosum was raised. The patient was started
empirically for three weeks on prednisone and mesalamine but discontinued a week
prior to presentation to our institution. On presentation the patient was
hemodynamically stable and had responded to volume repletion, he was afebrile and
demonstrated no leukocytosis. Laboratory evaluation was significant for ESR elevated
at 75 although a connective tissue cascade was negative. He was found to be Vitamin D
and K deficient. A comprehensive stool evaluation for C. Diff, enteric pathogens, and
parasites were all negative. A colonoscopy was performed which revealed no gross
changes however there was some granularity noted in the transverse colon.
Microscopically this area revealed mild active chronic colitis which was also seen in
random biopsies throughout the colon as well as the terminal ileum. A CT-enterogram
showed some subtle narrowing of the terminal ileum that could suggest inflammation.
Gastroenterology interpreted these findings to suggest Crohn’s disease that had
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Ryan Fabrizius, MD
somewhat responded to his previous three weeks of treatment. Dermatology agreed
that his leg ulceration was likely pyoderma gangrenosum associated with his
inflammatory bowel disease. The patient was discharged on mesalamine and
prednisone. At a six-week post-hospital followup we learned that his diarrhea had
resolved within a week of restarting mesalamine and prednisone and currently at four
months post initiation of treatment his leg ulceration is slowly demonstrating healing.
He continues on mesalamine. Pyoderma gangrenosum is a rare inflammatory
ulceration usually of the lower extremities affecting 3-10 individuals per one million
people annually. It is a manifestation of systemic inflammatory disorders and as in our
case may be the primary initial manifestation of underlying inflammatory bowel
disease.
Histomplasmosis in an Immunocompetent Patient
A 37 year old female with past medical history of obesity status post gastric bypass is
admitted to the hospital after being seen in clinic with painless jaundice. Her
transaminases were elevated, as were bilirubin and alkaline phosphatase. Abdominal
ultrasound obtained shortly after admission was concerning for a pancreatic head mass.
CT confirmed a 5 x 4.2 x 3.7 cm mass in the pancreatic head with common bile duct and
portal vein compression. Due to altered anatomy from gastric bypass, an EUS was not
attempted. Given the size of the mass and biliary obstruction it was determine surgical
resection was indicated regardless of the pathology. Pathology obtained from the
surgical specimen was consistent histoplasmosis, the patient was in no way
immunosuppressed. The patient is currently on a year long course of voriconazole.
Pancreatic histoplasmosis in an immunocompetent patient without pulmonary
symptoms is exceedingly rare. This case highlights the important fact that patients with
competent immune systems may still fall prey to infections classically defined as
"opportunisitic infections" in immunosuppressed patients.
Ear Pain to Brain: A Devastating Complication of an Ambulatory Disease
Acute Otitis Media is a common ambulatory disease commonly following a viral upper
respiratory infection, caused predominantly by S. pneumoniae, H. influenzae, M.
Catarrhalis, and respiratory viruses. Though the natural history of the disease is typically
uncomplicated, resolving with or without intervention, there are several possible
complications from this focus of infection. Bacterial meningitis is one of the most feared
sequelae. Though the cause of bacterial meningitis, in general, is usually unknown, it is
the most common intracranial complication of suppurative otitis media. Additionally,
otitis may be the direct cause of bacterial meningitis in up to 18% of people with acute
bacterial meningitis. Presented is a case of severe bacterial meningitis heralded by 3
days of acute otitis media. A 49 year old female with a past medical history significant
for migraines presented to an academic hospital’s Emergency Department with aphasia
starting that morning. She recently had three days of upper respiratory tract type
symptoms, along with right sided otalgia and otorrhea. She had been started on
cefuroxime by her primary care doctor two days previously without improvement in her
symptoms. When she awoke, her husband noticed that she was not responding to
questions, despite appearing awake and alert. Upon arrival to the Emergency
Department, she was found to have aphasia, left gaze preference, tachycardia and
hypotension, with mean arterial pressure in the 40s. Her physical examination was also
significant for red, bulging right tympanic membrane with blood in the auditory canal
and depressed consciousness. With intracranial hemorrhage and stroke leading the
differential diagnosis, a CT angiogram of the head and neck was performed, and found
to be normal. A lumbar puncture was performed, yielding 1002 RBCs, 4124 WBCs (84%
PMN), protein of 371, and glucose

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Badr Al-Bawardy, MD
Emmaneul Gorospe MD
MPH
Michelle Carlson, MD
are at risk as well. This case demonstrates one of the major complications of otitis
media. It also reviews the appropriate and timely management of acute bacterial
meningitis, a skill that should be possessed by general internists in every setting.
Refractory Ascites in a 51 Year Old Man
A 51 y.o. male was referred for recurrent ascites. He initially complained of progressive
lower extremity paresthesias for over a year. His paresthesias involved both feet with
associated foot drop. He was diagnosed with a demyelinating neuropathy at another
medical center. After 2 years, he developed recurrent ascites which did not respond to
diuretics. He was undergoing large volume paracenteses every two weeks. He does not
have any history of liver disease, heart failure, alcohol abuse, or other metabolic
conditions. He also developed easy fatigability, erectile dysfunction and a few
hemangiomas on his chest wall. He appeared chronically-ill with tense ascites,
peripheral edema and decreased deep tendon reflexes with peripheral sensory loss.
CBC, liver chemistries, creatine and coagulation panel were normal. TSH was elevated
at 12.3 mIU/L (normal range 0.3-5.0 mIU/L) and testosterone was 24 ng/dL (normal
range 240-950 ng/dL). Viral hepatitis, CMV, HSV, HIV and Syphilis serologies were all
negative. The ascites had a total WBC of 66 mm3 (75% mononuclear cells, 23%
lymphocytes and 2% neutrophils). Serum-ascites-albumin gradient was 0.9 g/dl with 2.6
g/dL total protein. No malignant cells were found. Fungal cultures, AFB stains and
bacterial cultures were negative. Abdominal ultrasound and CT scan revealed a normal
liver, biliary tract, spleen, hepatic, and portal venous systems. An echocardiogram did
not reveal signs of pulmonary hypertension, congestive heart failure, or IVC
obstruction. His skeletal survey showed sclerotic bone lesions of the hips. A bone
marrow biopsy revealed plasma cell proliferation. The patient was diagnosed with
POEMS syndrome and treated with bevacizumab. The ascites resolved after treatment
and the patient no longer required large volume paracenteses. Discussion POEMS
syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin
changes) is a rare syndrome that manifests with a combination of plasma cell
proliferative disorder, polyneuropathy, and multi-organ involvement. The patient had
neuropathy, hypothyroidism, hypogonadism/erectile dysfunction, plasma cell
proliferation with sclerotic bone lesions and new hemangiomas on the chest. In
POEMS syndrome, the presence of recurrent ascites has been documented since the
first published case series by Bardwick et al. in 1980. The formation of ascites is
attributed to diffuse capillary leak. The reason for this increased microvascular
permeability is still unknown but may be related to the combination of increased
vascular endothelial growth factors and monoclonal paraproteinemia. Direct or
functional damage of the vascular endothelium leads to fluid and protein loss from the
vascular space and accumulation in the peritoneum. The management of ascites in
POEMS syndrome is mostly supportive with judicious use of diuretics, dietary salt
restriction and regular paracentesis.
Delayed Diagnosis of Tension Pneumothorax in the Intensive Care Unit
Some diagnoses in medicine should not be missed: ST-elevation myocardial infarction,
pulmonary embolus, aortic dissection, tension pneumothorax (PTX), cardiac
tamponade. Unfortunately, these can be difficult to spot in the intensive care unit (ICU),
as the symptoms we usually rely on to trigger further investigation are obscured. In
such cases, high clinical suspicion is required. CASE: A 58-year-old male with colon
cancer metastatic to the lungs and liver was admitted to the ICU for acute respiratory
failure due to post-obstructive pneumonia. One evening, he was noted to be
increasingly agitated. This was thought to be due to turning with cares, and he received
a bolus of propofol. He became hypotensive to 65/45 mmHg and tachycardic to 135
bpm, with concomitant elevation in peak pressures to 60 cmH2O and decrease in SpO2
to 90% on 100% FiO2. Exam showed decreased breath sounds on the right and tracheal
deviation to the left. Bedside ultrasound showed a left-sided PTX, confirmed by chest
radiography (CXR). Needle thoracostomy was performed, and a pigtail catheter was
placed. Repeat CXR showed decreased mediastinal shift with large residual PTX, so a
large-bore chest tube was placed with good expansion of the lung. Both chest tubes
continued to leak large amounts of air, and the patient continued to be hypoxic to 88-
91% on 100% FiO2, with arterial blood gas showing pH 7.3, pCO2 49 mmHg, pO2 61
mmHg, and HCO3 23 mmol/L. Flexible bronchoscopy showed a large ulcerated lesion in
the proximal right mainstem bronchus with a fistula opening, presumed to represent a
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Suparna Chandra, MD
Faisal Syed, M.D.,
Joseph J. Maleszewski,
M.D., Nandan Anavekar,
M.D. and Imran Syed,
M.D.
Lisa Daniels, MD
Jessica Geiger M.D., Erin
Holl M.D, George
Saffouri M.D., Safia
Ahmed, Ryan Hurt M.D.
bronchopleural fistula. The patient was transitioned to comfort cares, and died later
that night. DISCUSSION: Despite classic findings of tension PTX in this ventilated
patient, including hypoxia accompanied by hypotension, high ventilatory pressures,
decreased ipsilateral air entry, and tracheal deviation,1 treatment was delayed due to
housestaff inexperience. Risk factors for overlooking PTX in the ICU include mechanical
ventilation, altered mental status, atypical location of the PTX, and development of the
PTX at night.2 In cases with clear clinical indicators of tension PTX, immediate needle
thoracotomy is indicated, followed by chest tube placement.3 In patients where the
diagnosis is less clear, ultrasound is more accurate and more rapid than CXR in
detecting PTX.4 A pigtail catheter is a safe and effective option in many patients,
though in a case series about 25% of patients with PTX treated with pigtail catheter
subsequently required large bore chest tube placement,5 as in this patient.
REFERENCES 1. Leigh-Smith S, Harris T. Emerg Med J. 2005;22(1):8-16. 2. Kollef MH. Crit
Care Med. 1991;19(7):906-10. 3. Yarmus L, Feller-Kopman D. Chest. 2012;141(4):1098-
105. 4. Zhang M, et al. Crit Care. 2006;10(4):R112. 5. Lin YC, et al. Am J Emerg Med.
2010;28(4):466-71.
Hidden in the Heart: Cardiac MRI in Investigating in Metastatic Melanoma
A 53-year-old obese woman with history of atrial fibrillation presented with chest
tightness on exertion. Her history was significant for melanoma of her left heel, with
subsequently discovered metastatic disease involving pelvic lymph nodes and her scalp.
She complained of nonspecific symptoms including occasional burning chest pain as
well as at episodes of chest tightness associated with exertion and at rest for onemonth
duration. Initial laboratory testing, ECG, chest X-ray was normal. Initial,
transthoracic echocardiogram showed evidence of biatrial enlargement and grade 2/4
diastolic dysfunction of unknown etiology. Concurrently, as part of routine melanoma
surveillance, (18) F-fluorodeoxyglucose positron emission tomography (FDG PET) scan
showed increased left axillary uptake and possible mediastinal uptake. Cardiac
magnetic resonance imaging (cMRI) revealed a 3.5 x 4.5 cm enhancing mass within the
right AV groove corresponding to the area of increased activity seen on PET. The lesion
completely encased the right coronary artery but did not invade appear to invade the
adjacent myocardium but was not amenable to surgical resection. The patient died of
complications of her metastatic melanoma two years after presentation. Histologic
examination of a resected portion of the mass exhibited metastatic melanoma with
involvement of the subjacent myocardium. This case highlights the need to maintain
high clinical suspicion for metastatic disease as a possible cause of any cardiac
symptoms. Further, this case demonstrates the superiority of dedicated cardiac imaging
with MRI in centers with clinical availability versus conventional cardiac imaging and the
benefit of early usage in the work-up of patients with known diagnosis of melanoma
and cardiac symptoms.
Recurrent Biliary Sepsis, complicated with zygomycosis and candida glabrata
Classically cholangitis has been diagnosed based on the presence of Charcot''s triad,
Reynolds’s pentad, and more recently the Tokyo guidelines. However, no criteria look at
risk of fungal cholangitis specifically. Once diagnosed, patients are started on empiric
antibiotics. No definite antimicrobials are defined for cholangitis, but accepted empiric
antimicrobials cover for polymicrobial infections from enteric bacteria, where as
antifungals are rarely initiated empirically. Patients not responding to antibiotics in the
first twenty four hours are at increased risk of mortality associated with severe
cholangitis. Therefore patients at risk of fungal cholangitis should be identified initially
in order to start antifungal therapies empirically. An 81-year-old male, with a history
of significant shrapnel injury to his abdomen, presented with recurrent cholangitis. He
had previously undergone liver repair, partial bowel resection, colostomy, and
splenectomy for his shrapnel injuries, as well as a Billroth II procedure for recurrent
peptic ulcer disease. He later developed multiple episodes of pancreatitis. As a
consequence, he subsequently developed a common bile duct stricture and ultimately
cholangitis. After a few episodes of recurrent cholangitis, he had a transduodenal
sphincteroplasty without much improvement. The patient then underwent a left-sided
external-internal biliary drainage catheter placed 10 years prior. More recently, he has
had increasing episodes of cholangitis presenting with the third episode in two months.
He recently had been treated with cefepime, metronidazole and caspofungin. On
presentation, he was febrile, with chills and abdominal pain, and mucoid drainage from
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Aaron Chan, MD
Brian, Hanson, M.D.
biliary drain site. He was alert and oriented, with no jaundice on exam. A
cholangiogram revealed debris in bile ducts. Due to atypical bile duct debris and
mucoid discharge around the drain, cytology was sent and revealed zygomycosis.
Endoscopic retrograde cholangiopancreatography (ERCP) was then preformed that
showed some biliary sludge that was easily flushed. Fungal cultures showed Candida
glabrata and bacterial cultures revealed polymicrobial infection with Aeromonas,
enterococcus durans, proteus mirabilis, enterococcus faecalis and pseudocoma
aeruginosa. He was treated with cefepime, metronidazole and fluconazole to complete
a two-week course. This case highlights the risks associated with fungal cholangitis.
Although Zygomycosis and Candida glabrata are uncommon pathogens in an
immunocompetent, non-diabetic patient, he had other associated risk factors.
Zygomycosis has been associated with hospitalizations and caspofungin. Candida
Glabrata has also been associated with recurrent cholangitis and hospitalization. This
patient was recently on multiple antibiotics as well as caspofungin. He had been in and
out of the hospital with multiple cholangiograms and biliary drain exchanges. He had
previously undergone a sphincterotomy and had chronic biliary drainage from internalexternal
drain. This patient also presented with new mucoid drainage from his
previously placed biliary drain. All of these factors put our patient at increased risk of
fungal cholangitis.
Burkitt Lymphoma presenting as colorectal cancer in the undiagnosed HIV patient.
Burkitt Lymphoma is generally diagnosed in patients from endemic areas of Africa,
those that are immunodeficient, and in the rare sporadic adult case. The diagnosis may
be overlooked when a patient who is not known to be immunodeficient presents with
symptoms consistent with an alternative diagnosis. A 50 year old woman with a history
of HTN and cocaine abuse is seen in the clinic to establish care. She was found to have
microcytic anemia on screening and recommended she undergo colorectal screening.
She chose to proceed with Fecal Occult Blood Testing (FOBT) at that time and her
anemia work-up was otherwise unremarkable. One month later, prior to completing
FOBT, she presented to the emergency department with one week of abdominal pain.
An abdominal mass was palpable, and following ultrasound it was concerning for an
ovarian mass given the location and quality on imaging. During her admission she
endorsed hematochezia and constipation, along with symptoms of generalized fatigue,
weight loss, and depression. Gastroenterology was consulted and she underwent a
colonoscopy. The scope was unable to pass through an ulcerated, circumferential,
partially obstructing large mass of the transverse colon. On biopsy it was resulted as a
poorly differentiated adenocarcinoma with marked tumor necrosis. Two weeks later,
following a right hemicolectomy the mass was identified as Burkitt Lymphoma. She was
subsequently tested and found to be positive for HIV. This case illustrates the
importance of a patient’s risks and a complete history when assessing a clinical
presentation. Although Burkitt Lymphoma in an HIV patient is not a rare finding, the
lack of screening and knowledge of HIV diagnosis in her case delayed further evaluation
and treatment. Recognizing the importance of HIV screening and diagnosis in high risk
patients broadens the differential and prompts early aggressive treatment.
Acute Hepatitis and Pancreatitis: An Unusual Etiology.
Hepatitis and pancreatitis secondary to cytomegalovirus is infrequently described in the
literature regardless of immunologic status. We report a case of hepatitis and
pancreatitis secondary to cytomegalovirus in an immunocompetent patient. A
previously healthy 31-year-old male was seen in clinic for burning epigastric pain that
radiated to the back, fevers, chills, night sweats, nausea and emesis which started
shortly after a viral-prodrome. He was found to have elevated lipase and
aminotransferases and was admitted to the hospital with acute pancreatitis and
hepatitis. Magnetic resonance cholangiopancreatography, endoscopic ultrasound and
intraoperative cholangiogram were unremarkable. His triglycerides and calcium were
normal. He had not consumed alcohol for three weeks prior to admission and had no
history of alcohol dependence. He was not on medications known to cause pancreatitis
and had no abdominal trauma. He tested negative for autoimmune pancreatitis, Epstein
Barr virus and mumps. Antinuclear antibody, F-actin, mitochondrial M2 antibody,
Hepatitis A, B and C serologies and autoimmune hepatitis were negative. Infiltrative
diseases such as copper overload and hemochromatosis were excluded as well.
Eventually, cytomegalovirus (CMV) DNA polymerase chain reaction was positive
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Dennis Manning MD
Aditya Devalapalli, MD
indicating acute or reactivated infection. Subsequent liver biopsy histology was also
consistent with CMV infection. He was treated with seven days of ganciclovir but
continued to have elevation in lipase and transaminases despite subsequently negative
CMV testing. The patient was discharged on hospital day twenty-eight with the
diagnosis of CMV hepatitis and pancreatitis. Unfortunately, he continues to require
chronic pain management and a gastrojejunostomy tube for nutrition. CMV has been
reported to cause disease in a number of different organ systems in
immunocompromised patients but rarely in immunocompetent patients. It is unclear if
early diagnosis of viral hepatitis and pancreatitis would have altered this patient’s
clinical course. Though, it does emphasize that pancreatitis and hepatitis secondary to
CMV should be considered in the work-up of immunocompetent patients, especially in
the presence of a recent viral-like illness.
Takayasu arteritis: Demographic Restriction?
Takayasu arteritis, frequently known as the “pulseless disease,” is a chronic idiopathic
large vessel vasculitis that most commonly affects women and the Asian population.
Less frequently Central or South American populations will get the disease. Within this
population, Takayasu has been associated with exposure to mycobacterium
tuberculosis. A twenty four-year-old male presented with a ten-day history of fever,
chills, anorexia, progressive shortness of breath, and a nonproductive cough. Four
months prior to presentation, with minimal exertion he noticed increasing fatigue and
bilateral calf pain. Pertinent medical history included anxiety and tobacco use. Family
history was unremarkable. Extensive cardiac, pulmonary, rheumatologic, and infectious
disease work up was performed because of a broad differential. Physical examination
revealed an overweight male in respiratory distress and tachycardia. A thrill and bruit
were present over the right subclavian area. Extremities were warm; however, left
radial and femoral pulses were diminished. Pedal pulses were present with Doppler. A
III/VI systolic murmur was best heard at the right upper sternal border, and a II/VI
diastolic murmur was heard throughout the precordium. No cyanosis was present.
Laboratory evaluation was significant for elevated CRP, hypoalbuminemia, positive PPD,
and positive QuantiFERON-TB. Sedimentation rate and autoantibodies were negative. A
transthoracic echocardiogram revealed ascending aorta dilatation and mild aortic
regurgitation. To evaluate further, CTA of chest and abdomen was performed and
demonstrated circumferential wall thickening of thoracic aorta and aortic branch
arteries. Complete occlusion of the proximal left subclavian artery was noted as well as
stenosis of the right subclavian and bilateral common carotid arteries. An ascending
aortic aneurysm with type B dissection extending into the descending thoracic aorta
was noted. Pulmonary arteries were unremarkable. Given his symptoms, laboratory
evaluation, and imaging studies he was diagnosed with Takayasu arteritis, started on
steroids, and eventually transitioned to Mycophenolate Mofetil for long-term
management. His aortic dissection was treated medically and latent TB was treated
with isoniazid. This case demonstrates that is important to be cognizant that although
Takayasu’s arteritis is associated with Asian descent, it is important to remember the
prevalence is high in the Central and South American populations in association with
active or latent TB.
Actinomycosis of the Gallbladder as Unique Imitation of Malignancy
Actinomycosis is a rare, chronic granulomatous disease typically characterized by the
formation of dense fibrosis, draining sinus tracts, fistulas, and abscesses.
Actinomycetes are gram-positive filamentous anaerobic bacteria, with Actinomyces
israelii being the most common human pathogen. Actinomycosis of the gallbladder, in
particular, is a very rare disease and is difficult to diagnose pre-operatively owing to its
imitation of malignancy. A 60 year-old male with type 1 diabetes mellitus and diffuse
large B-cell lymphoma, status post autologous stem cell transplantation with ongoing
chemotherapy, was referred to a major medical center for a large, necrotic-appearing
gallbladder mass. He had an episode of obstructive jaundice first noticed seven months
prior to admission, felt secondary to extrinsic compression, for which he was treated
with rituximab and methylprednisolone along with plastic biliary stent placement. His
cholestasis and jaundice shortly improved, and he continued to receive regular
chemotherapy with bendamustine and rituximab. He then experienced low-grade
fevers, night sweats, chills, malaise, and mild colicky right upper quadrant abdominal
pain with associated nausea and loss of appetite for two weeks prior to admission. He
denied any emesis, diarrhea, constipation, hematochezia, or melena. He had
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abdominal ultrasound, computed tomography, and magnetic resonance
cholangiopancreatography performed which revealed a large 11 cm x 6 cm x 7 cm
heterogeneous gallbladder mass with multiple cystic dilatations and irregular wall
thickening. He was referred to a large tertiary medical center given the concern for
complicated cholecystitis or neoplasm. On physical examination, he appeared nontoxic
and remarkably well, with only mild scleral icterus, mild jaundice, and minimal
tenderness to palpation in the right upper abdominal quadrant. Laboratory studies
were notable for hemoglobin of 8.9 g/dL, alkaline phosphatase of 771 units/L, and
elevated total and direct bilirubin levels of 3.8 and 3.0 mg/dL, respectively. He
remained hemodynamically stable and afebrile throughout his hospitalization. He was
empirically managed with piperacillin/tazobactam intravenously and eventually had
ultrasound-guided percutaneous cholecystostomy tube placement. Frankly purulent
biliary fluid was encountered, which revealed many gram-positive bacilli on gram stain.
Endoscopic retrograde cholangiopancreatography with sphincterotomy and biliary stent
exchange was performed, with no stone retrieval. He was discharged on ertapenem
intravenously for a course of six weeks, with plans to undergo definitive
cholecystectomy afterwards. Final culture of cholecystostomy fluid collected on two
separate occasions revealed growth for Actinomyces meyeri. There are a limited
number of case reports of gallbladder actinomycosis as this condition is most often
mistaken for neoplasm and thus diagnosed post-operatively, but may usually be treated
with medical therapy alone. This case demonstrates the importance of identifying
actinomycosis in the differential diagnosis for a gallbladder mass with extensive
inflammatory infiltration across fascial planes and multiple cystic compartments that
can often mimic malignancy.
The Howler: Did We Do That?
Adverse effects to previous therapies should be considered in complicated patients.
Risks and benefits should be considered in every treatment decision. Case: A 62-yearold
male with a history of depression, PTSD, GERD, hepatitis C status-post antiviral
therapy, and two past-year episodes of rhabdomyolysis presented with a 5 week history
of progressive weakness, myalgias, and persistent nausea. His last case of
rhabdomyolysis occurred 8 months prior to presentation, and attributed to medicationinduced
serotonin syndrome. Twelve weeks prior to admission, he killed a coyote with
his bare hands. Three weeks later, he went to Urgent Care for an unrelated concern
with no systemic symptoms. A rabies vaccine series was started. Six weeks prior, he
treated wood with spray-on Sikken (dipropylene glycol methyl-ester) without a
protective mask. Five weeks prior, he developed weakness in his back such that he
started the day upright and ended the day almost crawling. The weakness generalized,
and he developed myalgias most prominent in his calves and shoulders. He then
developed progressively dry eyes, dry mouth, night sweats, and nausea resulting in
non-bloody emesis. On admission, he endorsed burning substernal pain partially
resolved with antacids, constipation, and a dental cavity. His denies recent drug or
alcohol use. He denied fever, chills, rhinorrhea, respiratory or urinary symptoms,
diarrhea, joint pains or swelling, or skin changes. No recent travel. His last colonoscopy
was normal 3 years ago. He took cetirizine, chlorthalidone, potassium chloride,
bupropion, and zolpidem. His vital signs were normal throughout his admission. His
exam was significant for normal eyes, diffuse abdominal muscle tenderness, 4+/5
strength in upper and lower extremities though he fatigued very quickly, no
fasciculations, 2+ reflexes, dysmetric heel to shin test, normal finger nose finger, and a
positive Romberg test. He had a shuffling, unstable gait. He also had tender calves and
shoulders. His skin was normal except for healed hand and eyebrow lacerations. His
creatine kinase, CRP, ESR, thiamine, B12, RPR, copper, ANA, TSH, free T4, and morning
cortisol were normal. His AST was 114 and ALT 267, which both normalized quickly. His
basic metabolic panel was unremarkable. Hepatitis C, HIV, CMV, and EBV tests were
negative. Urine drug test was negative. C3, C4, and cryoglobulins are pending. Cervical
spine and brain MRI showed a few small old cerebellar infarcts. A chest radiograph was
clear. Ultrasound revealed hepatosteatosis. An EMG was normal. Discussion: A wide
differential was considered in this patient; however, serum sickness is the leading
diagnosis in the absence of a confirmatory test. Conclusion: All therapeutics can have
adverse effects. Moreover, adverse effects from recent therapies should be considered
when appropriate in the differential diagnosis of a complicated patient.
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Bryan Svobodny, MD
Brandt Esplin, MD
Joleen Hubbard, MD.
Adult Congestive Heart Failure exacerbation, a common presentation with an
uncommon underlying culprit: Partial Anomalous Pulmonary Venous Return
Partial Anomalous Pulmonary Venous Return is a rare congenital defect, with an overall
estimated incidence between .4-.7%. The malformation underlying Partial Anomalous
Pulmonary Venous Return involves the aberrant attachment of one of the pulmonary
veins to either systemic venous circulatory vessels or the Right atrium, instead of its
normal communication with the left atrium. This malformation is much less severe than
Total Anomalous Pulmonary Venous Return where all of the pulmonary veins are in
abnormal communication. The later malformation presents in early childhood, and
often requires surgical intervention within the first year of life. The later can often
times be asymptomatic through out life, or may present later in adulthood with
dyspnea on exertion, arrhythmias, or less commonly Right Heart Failure secondary to
pulmonary hypertension Case Presentation: A 48 year old Spanish male with no known
past medical history presents with two months of progressive shortness of breath, now
present at rest, fatigue, and two weeks of bilateral lower extremity edema. Initial work
up for heart failure was remarkable for an elevated Brain Natriuretic Peptide, and Chest
Xray remarkable for pulmonary edema. Due to additional lab values revealing
polycythemia and hypercapnia, Obstructive Sleep Apnea versus pulmonary embolism
became the working diagnosis due to evidence of biventricular heart failure, prior to
further imaging evaluation. The patient was initially treated with Lasix in the emergency
room, and later Nitroglycerin in the Medical Intensive Care Unit, while remaining on
Bilevel Positive Airway Pressure. D-Dimer on admission was 364 and CT pulmonary
Angiogram was ordered to rule out pulmonary Embolism. On the second day of
admission, a transthoracic echocardiogram was completed to evaluate the morphology
of the heart and its valves. Imaging exam findings were remarkable for partial
anomalous pulmonary venous return and a1.7 by 3.4 cm sinus venosus type atrial
septal defect. In regards to the anomalous venous connection, the right anterior
inferior pulmonary vein was attached to the superior vena cava. Patient was also noted
to have a left to right atrial shunt via the previously noted ASD. Previous therapies to
treat heart failure were continued and Sildenafil was started to combat Pulmonary
Hypertension. Prior to discharge a follow up appointment was scheduled with Adult
Congenital Heart disease specialists. Discussion: Congestive Heart Failure
decompensation is one of the most common causes for hospital admission, and is one
of the the leading causes of morbidity and mortality in the United States. Although not
a common culprit, congenital malformations like Partial Anomalous Pulmonary Venous
Return can be debilitating and sometimes deadly. This coupled with a track record of
decreased morbidity and mortality, make it an important diagnosis not to miss.
Isolated Cerebellar Metastasis: An Unusual Initial Manifestation of Right-Sided Colon
Cancer
Isolated Cerebellar Metastasis: An Unusual Initial Manifestation of Right-Sided Colon
Cancer Brandt L. Esplin, MD, PhD; Joleen Hubbard, MD. Mayo School of Graduate
Medical Education, Department of Internal Medicine; Mayo Clinic Division of Medical
Oncology, Rochester, MN. Intracranial metastasis of colon cancer is a rare occurrence
in an otherwise common malignancy. If encountered, the primary colonic site is usually
distal, left-sided (sigmoid/rectal), and in the setting of advanced disease with
concurrent metastases involving the liver and/or lung. A 55 year old female with no
significant past medical history presented to her local physician for a 2-week history of
right hand incoordination and gait instability. An MRI of her brain revealed an extraaxial-appearing
cerebellar region tumor, which was resected. Pathology revealed a
metastatic poorly-differentiated carcinoma. Immunohistochemical stains were positive
for AE1/AE3, CDX2, CK7, CK20, and CAM5, consistent with a non-neuronal,
gastrointestinal origin. A CT of her chest, abdomen, and pelvis revealed a 4.6 x 4.2 x
2.7-cm centrally necrotic mass along the medial aspect of the cecum and ascending
colon, concerning for a primary colon malignancy. No liver or lung metastases were
observed. A subsequent colonoscopy revealed a lesion in the ascending colon and a
biopsy confirmed an intermediate-grade adenocarcinoma. She had no prior screening
colonoscopy. Her postoperative course was uncomplicated without residual neurologic
deficits, and she denied any other symptoms, including weight loss, appetite change,
nausea, fatigue, abdominal pain, weakness, fevers, chills, or night sweats. She was
referred to Medical and Radiation Oncology for further management of her metastatic
colon cancer. Palliative systemic therapy following a three week course of whole-brain
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David Ewart, MD
radiation therapy was recommended. This case demonstrates a rare, but unfavorable
manifestation of right-sided colon cancer, whose initial presentation included an
isolated symptomatic intracranial metastasis without liver or other metastatic
involvement. Importantly, this experience highlights the necessity of maintaining a
broad differential diagnosis when determining the primary source of metastatic cancer,
as well as the value of executing timely cancer screening in adults.
Intracardiac histiocytic sarcoma presenting with subdural hematoma
Left atrial masses can pose a diagnostic dilemma as access to this chamber is more
difficult transvenously than the other three chambers of the heart. A 60-year old
woman with history of cardiac transplantation secondary to cardiomyopathy, atrial
fibrillation status post ablation and permanent pacemaker, hypertension and stage III
chronic kidney disease initially presented with a subdural hematoma,
thrombocytopenia and leukocytosis. Her thrombocytopenia and leukocytosis were
worked up one month previously and thought to be secondary to acute infection. She
was transferred to our facility for persistent platelet requirements to prevent
progression of subdural hematoma. Upon transfer she had progressive dyspnea
eventually requiring intubation. CT of the chest was done to evaluate for pulmonary
embolus and lung fields, revealing incidental left atrial mass of unknown etiology. The
mass was confirmed by transesophageal echocardiogram. The mass was felt to be
either tumor, thrombus or infectious but due to the location and patient’s
coagulopathy, we were unable to obtain tissue. Hematology was consulted for the
blood dyscrasias and bone marrow biopsy performed revealing what appeared to be
metastatic adenocarcinoma of unknown origin, unfortunately core biopsy sample was
of poor adequacy. PET/CT scan was performed with hypermetabolic regions appearing
in multiple boney areas including 1st rib and vertebrae, left atrial mass and right
adrenal mass. MRI was unable to be performed as patient had permanent pacemaker.
During the course of her stay, the patient required intermittent platelet drip and
multiple packs of platelets daily to maintain levels > 20,000 (with goal >50,000). Her
leukocytosis persisted throughout admission (in 30,000 range). Neurosurgery was
consulted and followed the patient throughout her stay to assist with management of
the subdural hematoma. She was able to be extubated briefly midway through her
admission but again had progressive tachypnea requiring same-day reintubation. She
failed pressure support trials on subsequent days. Right adrenal mass was subsequently
biopsied revealing histiocytic sarcoma. Further head CTs revealed progressive edema so
hypertonic saline was infused to maintain serum sodium 150-155mmol/L.
Unfortunately she later had concerning neurologic findings on physical exam and head
CT eventually revealed uncal herniation. Following family conference the patient was
placed on comfort cares, passing away shortly after. Autopsy was granted revealing
histiocytic sarcoma present as an intracardiac mass as well as multiple other locations.
This case represents an atypical mass of unknown etiology with multiple factors
precluding access to tissue. Persistent investigation revealing other potential sites of
metastasis allowed for a more feasible tissue biopsy site and eventual diagnosis of this
rare presentation of an intracardiac mass.
Back Pain: Worst-Case Scenario
Systemic lupus erythematosus (SLE) is an autoimmune disease with clinical
heterogeneity. This necessitates a panel of 11 diagnostic criteria associated with the
diffuse manifestations of the disease, 4 of which must be met for the diagnosis. Despite
a 10 year survival rate of 70% from the time of diagnosis, I describe a patient with a
flagrant history of immune dysregulation and a T-cell myeloproliferative disorder who
presented with rapidly progressive lupus myelitis necessitating a brain biopsy for
diagnosis. Case Presentation: A 70 year old female with a past medical history of
recurrent idiopathic thrombocytopenic purpura (ITP), status post splenectomy, and a T-
cell myeloproliferative disorder, discovered on bone marrow biopsy during a workup
for autoimmune pancytopenia, who presented with two to three weeks of progressive
back pain, weakness and numbness in the left leg, urinary retention, and confusion. Her
review of systems was otherwise positive only for a rash on her nose and cheeks that
occurred in the sunlight and the tips of her fingers getting white when in cold
environments. She had a distant history of smoking and a daughter and grandson both
have ITP. Her physical exam was remarkable only for confusion and mixed upper and
lower motor neuron signs. MRI’s of her brain and spinal cord showed findings
consistent with longitudinal myelitis. Autoimmune antibody panel, including ANA, was
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Shaina Rozell, MD,
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Eric Steinbrueck, MD
initially negative; ANA later became positive 1:40 with a speckled pattern. Lumbar
puncture was unrevealing but brain biopsy was eventually obtained and revealed
features consistent with lymphomatoid granulomatosis. She initially improved with high
dose corticosteroids but five months after diagnosis the patient expired due to sepsis
while receiving chemotherapy for relapsing disease. Discussion: This patient had a
longstanding history of immune dysregulation with severe clinical consequences. The
sensitivity and specificity of the 1982 SLE criteria are both 96%. Even prior to her
neurologic symptoms, she met the criteria for SLE with a hematologic, immunologic,
and renal disorder with a malar rash. However, the clinical overlap between
hematologic and autoimmune pathologic processes makes each of these symptoms
individually very insensitive in a patient with multiple potential disorders. In order to
attain the proper diagnosis and treat accordingly, clinicians must sometimes go to great
lengths, even if their efforts are ultimately futile.
Disorders of Arterial Dilatation and Obliteration in the Same Patient
A 73-year-old female presents with a six month history of worsening dyspnea on
exertion, orthopnea, abdominal distention, and lower extremity edema. Her past
medical history is significant for Hereditary Hemorrhagic Telangiectasia (HHT)
complicated by multiple pulmonary arteriovenous malformations (AVM) requiring
surgical resection and embolization, and by severe, transfusion dependent irondeficiency
anemia due to recurrent epistaxis and gastrointestinal bleeding. Laboratory
evaluation revealed a hemoglobin of 5.8. She was transfused and underwent upper
endoscopy with cauterization of multiple AVMs, and was initiated on aggressive diuresis
for volume-overload. An ECHO showed a Left Ventricular Ejection Fraction of 54%, but
also showed severe Tricuspid Regurgitation, increased Right Ventricular Systolic
Pressure, a dilated IVC without collapse, and hypokinesis of the apex, all of which had
worsened significantly since a prior ECHO. Right heart catheterization showed
pulmonary pressures of 54/23 with a mean of 38 and a wedge of 14, consistent with
pulmonary hypertension. Following administration of nitric oxide, her wedge pressure
remained unchanged and her mean pulmonary pressure decreased slightly.
Subsequently, she was started on Sildenafil and Carvedilol with significant
improvement. She was discharged with continued follow-up at the pulmonary
hypertension clinic. HHT is an autosomal dominant vascular dysplasia that classically
leads to recurrent epistaxis and gastrointestinal blood loss in addition to arteriovenous
malformations in the pulmonary, hepatic, and cerebral circulation. Pulmonary
hypertension, diagnosed by a right heart catheterization showing mean arterial
pressure greater than 25, is not an infrequent cause of right-sided heart failure in these
patients, but it, on the other hand, is a condition characterized by vasoconstriction,
excess cellular proliferation, and thrombosis that ultimately leads to increased vascular
resistance in the pulmonary circulation. It seems paradoxical that diseases leading to
both pulmonary arterial dilation and obstruction can regularly occur together. Genetic
analyses, however, have shown that the underlying mutations in both conditions are
directly related to the signalling pathway of the TGF-B superfamily. Initial treatment of
pulmonary hypertension consists initially of calcium channel blocker if a good response
to nitric oxide is observed during right heart catheterization, or a PDE-5 inhibitor if not.
In refractory or severe cases, prostacyclin analogues, which are direct vasodilators of
the pulmonary circulation, are used. This case illustrates the connection between these
two seemingly disparate conditions, and shows the importance of vigilance in these
patients, as effective treatments for pulmonary hypertension are available.
Valproic Acid induced Hyperammonemic Encephalopathy
Valproic acid can cause high ammonia levels that may actually induce
hyperammonemic encephalopathy regardless of a patient’s underlying liver status and
Valproic acid blood levels. Its mechanism of action is theorized to be inhibition of
carbamoyl phosphate synthetase I, the first enzyme in the urea cycle and thus inhibits
ammonia excretion, causing a metabolic encephalopathy. A 30 year old man with
previous traumatic brain injury recently started on Valproic acid for recurrent
headaches presented to the emergency department for agitation, difficulty
concentrating, and confusion. Earlier that day, he had gone to lay down complaining of
nausea. He awoke extremely disoriented, hitting his head violently against a wall
repeatedly. He proceeded to run out of his house and down along train tracks wildly.
His father called the police who found the patient combative and screaming
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Bradley Anderson, MD
Singh, Jaswinder; Jafri,
Syed; Cates, Joe;
Connor, Michael; Levy,
Michael
incoherently. He was finally subdued and brought in after receiving Ketamine in the
field. In the emergency room, he was found to have an elevated ammonia level of 205
umol/L. His Valproic acid level was in the low end of the therapeutic range 74.4 mcg/ml.
He required intubation for airway security. The patient was treated with carnitine
infusion, and his hyperammonemia quickly resolved after peaking at 394 umol/L. He did
develop some mild cerebral edema, necessitating hypertonic saline treatment. After
acute treatment, the patient still maintained difficulty with attention and poor
concentration and required rehabilitation. This case emphasizes the need for an
internist to consider Valproic acid as a possible cause of encephalopathy in a patient
presenting with altered mental status, especially in an age group where toxic ingestions
are frequently found to be initiators of acute mental status changes. Additionally, the
number of neuropsychiatric comorbidities in patients receiving Valproic acid can
especially limit the diagnostic workup and help overlook this medication’s role of
inducing a metabolic encephalopathy.
Aspiration Exasperation: Suspected tumor seeding following EUS-guided FNA of a celiac
lymph node
Endoscopic ultrasound-guided fine needle aspiration (EUS FNA) is an important tool for
evaluating pancreatic cancer due to the enhanced staging accuracy and ability to obtain
a tissue diagnosis. Tumor seeding after EUS-FNA is believed to be rare and only five
case reports have been described. A 51-year-old gentleman with new-onset jaundice,
15-lb weight loss and elevated serum CA 19-9 measuring 150 mg/dL, presented for EUS
after an abdominal CT demonstrated a large pancreatic head mass. On endoscopic
examination, the esophagus and stomach were normal-appearing. EUS revealed a large
5.0 x 2.6-cm pancreatic head mass with invasion of the portal vein and SMV confluence.
Additionally, a 6-mm lymph node was noted in the celiac axis. Ten FNA passes were
obtained from the pancreatic mass along with three samples from the celiac lymph
node. Cytology of the pancreatic mass was interpreted as positive for adenocarcinoma
and the celiac lymph node cytology was interpreted as suspicious, but not diagnostic of
malignancy. On the basis of EUS-FNA the tumor was staged as T3N1M0 and
chemotherapy and radiation therapy was initiated. Approximately 2 weeks into
chemoradiation therapy, the patient noted progressive nausea, vomiting and epigastric
pain. An upper endoscopy was performed revealing mild duodenitis and moderate
radiation-induced antral gastropathy. Moderate esophagitis and a linear ulceration
were also noted. Endoscopic ultrasound of the GE junction ulcer revealed a 1-cm
hypoechoic region that involved the submucosa with extension into the mucosa and
muscularis propria suggesting metastatic disease. EUS FNA of the hypoechoic region
confirmed the presence of adenocarcinoma. While the cytology of the celiac lymph
node in this case was reported as ''suspicious'' but not diagnostic of metastasis, positive
cytology of the described linear hypoechoic lesion and its limitation to the tract used for
FNA suggest that tumor seeding occurred. An additional primary gastroesophageal
tumor is made less likely given the extension of the tumor from the muscularis propria
into the mucosa with normal appearing mucosa surrounding the lesion. The possibility
of cytological contamination from the pancreatic mass is also unlikely given that lymph
node samples were collected before those from the pancreatic mass. Little
standardization exists to guide EUS-FNA of lymph nodes. As in this case, smaller lymph
nodes (

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Theodore Hanson, MD
Ryan Kelly, MD
a viral load of 785,000. He had been noncompliant with his antiretroviral therapy since
2007 and had been refusing medical care due to lack of insurance despite worsening
health. Patient was found to be in acute liver failure with elevated bilirubin, alkaline
phosphatase, and INR. CT demonstrated extensive intra-abdominal lymphadenopathy
with hepatosplenomegaly. Liver biopsy was performed demonstrating diffuse nonnecrotizing
granulomas containing acid-fast bacilli most consistent with an atypical
Mycobacterium species. During hospital admission, blood culture came returned with
growth of MAI. Patient was initiated on clarithromycin, ethambutol, and rifabutin.
After an extended hospital course, during which he was diagnosed with EBV-associated
large B-cell lymphoma infiltrative to the gastrointestinal tract and HIV dementia, patient
was discharged to a nursing home with Infectious Disease follow-up to initiate
antiretroviral therapy in two weeks Outcome: Patient returned to hospital
approximately one month post-discharge for fevers in the setting of worsening
pancytopenia. As bone marrow infiltrate on by either lymphoma or MAI were deemed
as the likely culprits, bone marrow biopsy was performed, demonstrating a hypocellular
marrow with granulomas containing acid-fast organisms presumed to be MAI. Patient
was discharged to hospice and died shortly after discharge. Discussion: From 1980 to
1981, the first case reports presenting homosexual or drug-abusing young people with
rare infections were appearing in medical literature. Approximately one year later in
1982, an article in JAMA presented five case reports where disseminated MAI was
found on postmortem autopsy in patients who had died from rare infections and
cancers. The general undertone of these case reports has connotations of both strain
and apprehension, as clinicians are perplexed by their young patients dying from a
mysterious epidemic. With both the discovery and increasing availability of
antiretroviral medications, the developed world has seen rapid decline of the incidence
of AIDS-defining illnesses such as disseminated MAI. For a new generation of physicians
who are contemplating the prospect of the “end of AIDS” worldwide, it is cases like
these that serve as a reminder of where the medical field has come and the trajectory
to which it is heading.
Left Ventricular Non-Compaction Presenting as Atrial Flutter
Left ventricular non-compaction (LVNC) is a condition of altered myocardial structure.
It can be loosely defined as myocardium with prominent trabeculae and intertrabecular
recesses that results in a myocardium with two layers: a compacted layer and a noncompacted
layer. As such, there is continuity between the left ventricular cavity and
the deep intratrabecular recesses. This condition is associated with high morbidity and
mortality rates. In spite of this prognosis, medical management can result in secondary
prevention of complications. A 32-year-old, otherwise healthy man presented to the
emergency department with palpitations. Prior to presentation, he consumed two
caffeinated beverages. He normally does not consume caffeine, and was otherwise
asymptomatic. The initial electrocardiogram showed an atrial tachyarrhythmia, most
consistent with atrial flutter with two-to-one conduction. He spontaneously converted
to sinus rhythm. On subsequent physical exam, the patient had normal S1 and S2 heart
sounds without murmur or gallop. Transthoracic echocardiogram was performed as
part of routine evaluation of the structure and function of the heart in the setting of
new onset atrial tachyarrhythmia. The echocardiogram showed left ventricular
enlargement with normal wall thickness, and increased trabeculations about the apex
concerning for LVNC. Doppler showed continuity between the ventricular cavity and
the trabecular recesses. The patient remained asymptomatic during hospitalization,
and was discharged with instructions for follow-up with a cardiologist. LVNC
commonly presents as chest pain, dyspnea, or atrial fibrillation. This case emphasizes
the importance of echocardiography in patients who present with supraventricular
tachyarrhythmias and are otherwise healthy without a history of heart disease. Timely
diagnosis of LVNC allows for prevention of complications. Management includes annual
Holter monitoring and anticoagulation as there are high rates of atrial and ventricular
arrhythmias and thromboembolic events in this patient population. Additionally,
patients with LVNC need to be counseled to refrain from competitive endurance sports
and weight lifting because of risk of sudden cardiac death from arrhythmia.
Putting the “E” in Environmental Exposure, A Case of Eosinophilic Pneumonia
Allergic reactions to environmental chemicals are quite common. Lung injury, both
acute and chronic, is one of the more common types of environmental injuries. The
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Eduardo Soto, MD
Yader Sandoval, MD;
Woubeshet Ayenew,
MD
amount of possible environmental toxins that can affect patients can be overwhelming.
Avoiding injury can often be as simple as taking preventative measures, such as wearing
eye gear and masks, but sometimes is more complicated. This case will illustrate a life
threatening reaction to a common environmental compound that may have been
prevented with some precautionary measures. Case: A 38 year old male presented to
his local emergency department with worsening shortness of breath and subjective
fevers that began acutely 2 days prior to presentation. The day symptoms started, he
was in his usual state of health, and spent the day repairing his deck. This involved
cutting red cedar and being exposed to red cedar dust, without a mask. One hour after
doing this, symptoms began. Shortness of breath progressively worsened, leading to
the presentation. In the emergency room he was febrile to 104 and hypoxic, and he
required intubation. The patient continued to be hypoxic even while intubated, leading
to the patient's transfer by air to a University Hospital. On arrival to the University
hospital, his ventilator settings were changed to ARDS settings, with some
improvement in his oxygen saturation. Initial workup included imaging, sputum and
blood cultures. Initial treatment included empiric broad spectrum antibiotics and
antifungals. A bronchoscopy was performed, demonstrating a negative gram stain but
34% Eosinophilia seen in the BAL. On the third day of admission, after no improvement
on empiric anti-microbials, he was initiated on high dose steroids for suspected
eosinophilic pneumonia, with rapid improvement in inflammatory markers, and his
FiO2 needs were able to be weaned over several days. The patient was extubated on
hospital day 9, and subsequently did well, on a steroid taper. Discussion: Occupational
exposures to cedar and pine woods have been demonstrated to cause both acute and
chronic lung disease. The substance suspected to cause the allergic reaction in red
cedar pine, plicatic acid, occurs via a hypersensitivity reaction, and is suspected to have
led to the eosinophilic pneumonia in this patient. Although injuries to dust sometimes
can be prevented by a simple “Dust Mask,” this type of mask doesn’t prevent injury
from particles smaller than 5 microns; and often the use of a fresh air supply respirator
is necessary to provide true “protection.” For the general public, the most importance
learning point is that once one sees dust, it’s time to take a break. Second, wood is not
simply “wood,” and some types of lumber come with more risks than others.
"Syncope: A Too Fast or Too Slow Dilemma."
Syncope is the abrupt and transient loss of consciousness associated with the absence
of postural tone. Cardiac syncope represents almost one third of the cases and around
15% of these are secondary to dysrhythmias. We present a case of syncope where
initial tachydysrhythmia masked the underlying conduction disease, which then
surfaced with clinically unstable bradydysrhythmia. Case Description A 70 year old
male presented to our hospital with exertional syncope. He had developed dizziness
and lightheadedness leading up to the syncope. Physical exam revealed an irregular
rhythm with otherwise normal cardiovascular exam. ECG on arrival showed atrial
fibrillation (AF) with rapid ventricular response (RVR), right-bundle branch block (RBBB)
and left-anterior fascicular block (LAFB). Use of AV nodal blocking agents was
entertained, but was deferred since he remained only minimally symptomatic and his
tachycardia was noted to gradually resolve without intervention. The avoidance of AV
nodal blocking agents proved to be prudent in the ensuing hours when he was noted to
have AF with slow ventricular response. The next day, AF resolved spontaneously. The
sinus rhythm unmasked significant infra-nodal conduction disease in the form of
trifascicular block - 1st degree AV block in addition to RBBB and LAFB. Later in the day
he deteriorated while walking bedside and developed complete heart block with escape
heart rate of ~ 20 bpm. His associated presyncope and altered mental status
responded promptly to atropine and transcutaneous pacing. A dual-chamber
permanent pacemaker was placed urgently and he had no recurrence of his presenting
symptoms. Discussion Cardiogenic syncope is common and dysrhythmias represent
around 15 percent of the cases encountered. Dysrhythmias may be either FAST (ex: VT
and SVT) or SLOW (ex: sick-sinus syndrome, high-grade AV block). This case
demonstrates a unique presentation of syncope and the challenge of identifying
tachydysrhythmia or bradydysrhythmia as the cause of the event. While AF with RVR
may have been the initial rhythm owing to his fall, once the acute stress resolved, the
spontaneous return to sinus rhythm revealed the other extreme of the dysrhythmia
spectrum - trifascicular block. It will appear that his fall while climbing the stairs was a
result of complete heart block. Conclusions • In cases of dysrhythmia related
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Selome Gezahegn, MD
Benji Mathews, MD
cardiogenic syncope, it’s fundamental to determine if event is related to a fast or slow
rhythm since treatment of each will be very different. • Bifascicular block is not
infrequent on routine ECG and does not raise alarm. However the presence of first
degree AV block in this setting defines the more concerning pattern of trifascicular
block. • Trifascicular block is an ominous electrocardiographic change which can be
seen in association with syncope. It suggests infra-nodal disease that may progress to
complete AV block.
MPO-ANCA Positive Pauci-Immune Rapidly Progressive Glomerulonephritis
MPO-ANCA Positive Pauci-Immune Rapidly Progressive Glomerulonephritis. Selome G.
Gezahegn MD.,Benji Mathews MD. Introduction: Rapidly progressive
glomerulonephritis (RPGN) is a clinical syndrome where a rapid decline in kidney
function is seen in the context of glomerular inflammation. Myeloperoxidase
antineutrophil cytoplasmic antibodies (MPO-ANCA) positive RPGN is a common cause
of RPGN and includes pathologic entities such as Granumomatosis with Polyangitis
(previously known as Wegener’s granulomatosis) and microscopic polyangiitis. Case
Presentation: We present a case of a 59 year old female with serologically and clinically
quiescent systemic lupus erythematosus (on Hydroxylchloroquine) and well controlled
hypertension who initially presented with two months of intermittent hematuria. Her
physical exam was unremarkable. Work up revealed normocytic anemia (hemoglobin
9.9 g/dL) and acute kidney injury with serum creatinine level of 2.79 mg/dL, which was
significantly elevated from a previously normal baseline. Urinalysis revealed mild
proteinuria. Hematuria was confirmed on urine microscopy (190 red blood cells per
high power field) but there was no evidence of red blood cell casts. Renal function
failed to improve with rehydration therapy. Due to a concern for lupus nephritis renal
biopsy was performed showing crescentic, necrotizing glomerulonephritis, which was
pauci-immune on immunofluorescence staining. Computed tomography scan of the
sinuses done as part of her work up was significant for thickening of the paranasal sinus
walls associated with bony erosions. Patient was later found to have a high serum MPO-
ANC titer confirming the diagnosis of MPO-ANCA positive pauci-Immune RPGN. The
patient was started on treatment with high dose steroids and cyclophosphamide. She
was discharged with prednisone 60mg daily and will have close follow-up with
rheumatology and nephrology. Discussion: Pauci-immune RPGN is the common cause
of RPGN and can occur with or without the presence of ANCA. The term refers to the
scarcity of immune complex deposits in immunofluorescence staining for
glomerulonephritis. Majority of patients have MPO-ANCA vasculitis mostly affecting
small vessels and has preponderance to the respiratory tract and kidneys. Our patient
had classic features of RPGN. Although she has history of systemic lupus, kidney biopsy
results were did not show evidence of lupus nephritis. The presence of MPO-ANCA
positive, pauci-immune necrotizing glomerulonephritis together with destructive
changes on sinus CT strongly suggest the diagnosis of Granumomatosis with Polyangitis
(Wegener’s granulomatosis).
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Evan Hardegree, MD
Robert Albright, DO
Drug Effects and Double Checks
Common causes of decompensated heart failure include myocardial ischemia, salt
ingestion, and medication noncompliance. However, there are less common
precipitants which should be considered. Hypothyroidism may be difficult to distinguish
from typical causes of heart failure, making it essential to consider this condition in the
differential diagnosis. Case Presentation: A 90-year-old man presented with 4 weeks of
progressive fatigue, weight gain and lower extremity edema. His history included
chronic heart failure (LVEF 15%) with a dual-chamber pacemaker for cardiac
resynchronization; paroxysmal atrial fibrillation and frequent ventricular ectopy
requiring initiation of amiodarone 6 months prior; and stage IV chronic kidney disease.
He reported medication and dietary compliance and denied anginal symptoms. His vital
signs were normal but his weight had increased by 4.6 kg. Examination was consistent
with decompensated heart failure, with jugular venous distension, inspiratory crackles
in the lung bases, and anasarca. The skin was dry and the thyroid exam was
unremarkable. An ECG and cardiac biomarkers ruled out acute myocardial infarction.
His serum creatinine was 3.7 mg/dL, significantly worse than his baseline of 2.3 mg/dL.
An echocardiogram revealed an LVEF of 12% with global hypokinesis and a moderatesized
concentric pericardial effusion. With diuresis, his pulmonary, abdominal and
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Livia Hegerova, MD
Sunil Mankad
Shauna Morrow, MD
peripheral edema improved, and his serum creatinine decreased to 3.2 mg/dL.
However, he remained severely fatigued. Given this and the new pericardial effusion,
serum thyroid studies were obtained, revealing a thyroid-stimulating hormone (TSH) of
187 mIU/L (normal 0.3 to 5.0), free thyroxine of 0.4 ng/dL (normal 0.8 to 1.8), and
negative anti-thyroperoxidase antibodies. He was diagnosed with amiodarone-induced
hypothyroidism and started on levothyroxine. His fatigue and fluid status improved
gradually over the next 4 days and he was dismissed from the hospital. Discussion:
Amiodarone may cause thyroid dysfunction (hyper- or hypothyroidism) due to its high
iodine content as well as direct toxic effects on thyroid parenchyma. Hypothyroidism
typically develops 6-12 months into therapy, and while classic symptoms of
hypothyroidism are seen (fatigue, weight gain, and cold intolerance), goiter is rare. In
elderly patients with chronic heart or renal failure, such symptoms may be overlooked.
Furthermore, hypothyroidism causes direct cardiovascular effects, including reduced
contractility, impaired diastolic relaxation, and increased peripheral vascular resistance.
Clinical findings may include pericardial effusion, nonpitting edema, QT prolongation
and ventricular ectopy. Cardiorenal syndrome refers to the pathophysiologic
interactions in combined cardiac and renal failure, which is marked by reduced cardiac
output and renin-angiotensin-aldosterone system activation, with resultant salt and
water retention, vasoconstriction, increased afterload, renal venous congestion, and
reduced renal perfusion. In summary, this case highlights the complex multisystem
pathophysiology of cardiorenal syndrome, illustrates a potential side effect of
amiodarone, and underscores the importance of a broad differential diagnosis and
thorough workup.
Sex, Drugs and Endocarditis
Infective tricuspid valve endocarditis is a rare life-threatening complication of
intravenous drug use, but an even more rare complication of pregnancy. The calculated
maternal and fetal mortality is high, estimated at 22.1% and 14.7% respectively.
Literature is limited on the management of this condition; however, with persistent
lesions delivery should be considered. Case Presentation: A 27 year old primigravida
was transferred to our facility at 18 weeks for evaluation of two weeks of fevers, chills
and generalized myalgias. Her past medical history was notable for polysubstance use
including alcohol and intravenous drug use. A review of systems was negative for
abdominal pain, diarrhea, urinary symptoms or travel exposures. On physical
examination she had a loud holosystolic murmur, gravid uterus, congestive
hepatomegaly, and lower extremity edema. Admission labs were significant for
leukocytosis of 25 x109/L, marked anemia and thrombocytopenia. Blood cultures at
outside hospital were positive for Staphylococcus aureus. She remained febrile,
tachycardic, and a transthoracic echocardiogram revealed the presence of a large (32 x
18 mm), highly mobile tricuspid valve vegetation and severe tricuspid regurgitation.
She was started on vancomycin and nafcillin, which was narrowed to naficillin when
sensitivities returned with MSSA. During her evaluation, the patient had complaints of
shortness of breath and pleuritic chest pain. A chest x-ray revealed round patchy
bilateral infiltrates consistent with septic pulmonary emboli and small pleural effusions.
Despite successful sterilization of blood (repetitive cultures negative), the patient
continued to spike fevers. Thoracentesis revealed microbial presence, requiring chest
tube placement for empyema. Given the patient’s active drug use at the time of
hospitalization and her clinical stabilization with antibiotics, conservative non-surgical
management with 4-6 weeks of appropriate antibiotic therapy was advocated.
Discussion: Right-sided lesions account for only 5-10% of cases of infective
endocarditis. Surgical vegectomy is reserved for evidence of persistent bacteremia,
refractory heart failure, recurrent embolic events despite antibiotics, or complications
such as mycotic pulmonary aneurysm or abscess formation. However, in a pregnant
patient with persistent symptomatic lesions, delivery should be considered without
regard for fetal maturity given the high fetal mortality rates. In the absence of
pregnancy, surgery can be considered for very large vegetations > 10-20 mm although
the risk of pulmonary and peripheral embolization decreases significantly after 2 weeks
of antibiotics.
AIDS Masquerading as Dermatomyositis: Underscoring the Importance of Sexual History
The sexual history continues to be one of the most unreliably obtained components of
the medical interview in primary care settings, leading to lost opportunities for
appropriate STI (sexually transmitted illness) screening as well as counseling regarding
27

safe sexual practices. In the following case, obtaining a routine sexual history may have
prevented a delay in diagnosis of HIV and subsequent AIDS. Case Presentation: A 53-
year old man with longstanding diagnosis of amyopathic dermatomyositis was admitted
to the hospital for evaluation and treatment of steroid unresponsive interstitial lung
disease. On admission, the patient demonstrated significant hypoxia (oxygen
saturation 74% on room air) with peripheral cyanosis and moderate respiratory
distress. The patient was admitted to the ICU for respiratory support with CPAP
(continuous positive airway pressure) and intravenous steroids. CXR revealed diffuse
ground glass opacities thought to be consistent with interstitial pulmonary disease.
However, given the patient''s deteriorating respiratory status, ongoing weight loss, and
lack of laboratory support of the diagnosis of dematomyositis (negative ANA, ENA and
anti-Jo 1), an alternative etiology was suspected. Sexual history taking revealed that
patient had several sexual encounters with male partners 5 years prior. HIV screening
was positive and CD4 count was 9/cu mm. The patient was empirically treated for
Pneumocystis pneumonia, which was later confirmed by biopsy obtained during
bronchoscopy. The patient was also found to have CMV viremia and was treated with
valganciclovir. With appropriate management, his condition improved and he was
discharged from the hospital. HAART (highly active antiretroviral therapy) was initiated
and patient''s CD4 count rebounded. Discussion: This case illustrates the importance
of obtaining a sexual history in all patients, including those perceived to be low risk.
This patient was a husband and a father, which may have contributed to failure to
obtain a sexual history. Recent studies indicate that the sexual history is often omitted
in the primary care setting; only 35% of PCPs (primary care providers) state that they
often (75% of the time) or always take a sexual history (1). Physicians cite several
factors, including embarrassment and time constraints, as reasons for excluding
discussion of sexual health. An adequate sexual history should include documentation
of sexual orientation, previous and current sexual partners, type of intercourse, and use
of barrier protection. The sexual history is an underutilized and cost-effective means to
early detection of STIs as well as a gateway to discussion of safe sexual practices and
behavioral risk reduction. 1. Nusbaum MRH, Hamilton CD. The Proactive Sexual
History. Am Fam Phys 2002; 66: 1705-12.
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Kathryn Gillen, MD
John Higgins, MD
Evan L. Hardegree, MD,
A Tough Diagnosis to Swallow
This is a case of a 60* year old female with no significant past medical history, but very
strong smoking and alcohol use history, who presented with weight loss and
odynophagia. She was eventually found to have esophageal histoplasmosis. The
importance of this case is that, although she had no significant risk factors for HIV in her
history, with such a rare diagnosis seen mostly in immunocompromised patients, it is
important to screen for HIV. Patient is a sixty year old female who presented to the
emergency department with progressive dysphagia, odynophagia, weakness and weight
loss that had been going on for almost one year. Exam revealed a cachectic female in
no apparent distress. Cardiac and pulmonary exam was unremarkable, abdominal
exam was only significant for a palpable liver, four centimeters below the costal margin.
In the emergency department, due to her profound weight loss (1/3 of her body
weight), a CT of her chest, abdomen and pelvis was done to evaluate for malignancy.
Chest CT revealed a 3.7x1.5 cm R lung mass. Labs drawn in the emergency department
revealed hyponatremia, hypokalemia and hypomagnesemia. Due to her continued
complaints of odynophagia and sore throat, an EGD was done and was scheduled on
the same day as the chest mass biopsy. The EGD results returned first and showed
esophageal histoplasmosis. This is a disease most commonly seen in
immunocompromised patients. Although our patient did not have any significant HIV
risk factors, we decided to screen for HIV due to this rare diagnosis. On the day of
discharge, the HIV screening test and the chest CT biopsy both returned. The good
news, the chest biopsy showed only fibrosis, no evidence of malignancy. The bad
news... our patient was HIV positive. Six months later, our patient is doing well. She
has started on HAART and her CD4 count has improved tremendously. She has started
to gain weight. With the treatment of the esophageal histoplasmosis, her dysphagia
and odynophagia were improving.
The First Cut is the Deepest: Stress Cardiomyopathy Presenting as Respiratory Distress
During an Outpatient Procedure
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Bekele Afessa, MD
Erin Holl, MD
Jessica Geiger M.D.,
George Saffouri M.D.,
Lisa Daniels M.D., Ryan
Hurt M.D. Ph.D.
Stress cardiomyopathy is a form of acute reversible cardiac dysfunction of uncertain
pathogenesis. This disorder mimics myocardial ischemia, making diagnosis difficult. A
59-year-old woman was undergoing induction of anesthesia before an outpatient chin
liposuction procedure. She received hydrocodone, diazepam and inhaled lidocaine
before the procedure. Immediately following the first incision, she developed acute
respiratory distress, requiring intubation, despite administration of flumazenil and
naloxone. She was transferred to the medical intensive care unit (ICU), where physical
examination revealed agitation, tachycardia, and foaming secretions in the
endotracheal tube. A chest radiograph showed diffuse pulmonary infiltrates consistent
with flash pulmonary edema. Electrocardiography demonstrated sinus tachycardia with
prominent Q-waves and ST elevation in the anterior leads concerning for anteroseptal
myocardial infarct. An echocardiogram was performed, showing apical akinesis,
preserved motion in the basal segments, and a left ventricular ejection fraction (LVEF)
of 20%. She subsequently developed atrial flutter with hypotension, and her initial
troponin T was elevated at 0.15 ng/mL (N=

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Yoel Korenfeld, MD
Marcus Salmen, MD
discharged on methimazole, metoprolol, and quetiapine as needed for agitation.
Follow up with Endocrinology was arranged for monitoring of thyroid hormone and
consideration of radioactive iodine therapy. Discussion: Graves’ Disease presenting
with thyrotoxic psychosis is an established, but rare clinical picture. Without the
presence of physical manifestations such as heat intolerance, sweating, tremor, weight
loss, palpitations, and gastrointestinal symptoms, this diagnosis could easily be
overlooked. Psychiatric presentations of GD range from depression to mania, and
include schizophreniform disorder, paranoia and delirium. This case illustrates the
importance of maintaining a broad differential for common presentations, such as
delirium, so that diagnosis and therapy can be initiated early to prevent life threatening
complications. Additionally, this case demonstrates the importance of ultrasound as an
adjunct in diagnosis of GD.
If no asthma and no COPD, what could wheezing be? An interesting condition long
missed due to premature closure and anchoring heuristic
Anchoring heuristic and premature closure are well described phenomena that leads to
diagnostic errors. Our patient had a long history of chronic obstructive pulmonary
disease (COPD) despite never having been exposed to risk factors for this
condition. Case: A 72 year old man with history of diabetes mellitus type 2,
hypertension, hyperlipidemia, stage 3 chronic kidney disease and long standing COPD
who was admitted to the hospital after he was found unconscious with severe
hypoglycemia. Insulin was recently added to his oral sulfonylurea regimen. Initial
management with glucose, decreased insulin dosage and discontinuation of
concomitant sulfonylurea resolved his hypoglycemia. His history of COPD was
questionable as he never smoked and had no other risk factors. Review of systems
revealed severe chronic constipation with overflow diarrhea. On exam he was found to
be on moderate respiratory distress despite normal oxygen saturation; he had severe
bilateral wheezing, irregularly irregular heart sounds, jugular venous distention and
lower extremity edema. He also was noted to easily flush. Initial laboratories were
significant for acute elevation of creatinine from baseline of 1.8 to 2.4 mg/dL and
urinary tract infection with hematuria, pyuria and moderate leukocyte esterase. Chest
X-ray and head tomogram were unremarkable. A renal ultrasound was obtained as part
of his work-up for acute on chronic kidney injury. Serendipitously, four liver lesions
were observed. The patient never had colonoscopy in the past as he repeatedly refused
it. Colonoscopy revealed a 2 cm frond-like lesion in the descending colon; the biopsy
showed an adenocarcinoma of the colon; a second, 3 cm fungating and partially
obstructing lesion was observed in the terminal ileum; biopsy was non-diagnostic.
Ultrasound guided liver biopsy showed a gastrointestinal neuroendocrine metastatic
tumor. Elevated (5)-HIAA at 617 mg in a 24 hour urine collection (normal

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Malli Ahmed, MD
Hannah Lichtsinn
a platelet count of 40 k/cmm. Upon urgent admission to the Obstetrics service, she was
afebrile and without significant physical exam findings, but she reported daily chills and
a recent history of partially-treated malaria. Initially, a broad differential of her
thrombocytopenia was entertained, including gestational thrombocytopenia, preeclampsia,
HEELP, thrombotic thrombocytopenic purpura, ITP, hemolytic anemia,
malaria, sepsis, and DIC. Additional labs revealed a mild anemia, an elevated
creatinine, an elevated lactate dehydrogenase, and a urinalysis showing small amount
of blood. Peripheral smear demonstrated parasitemia consistent with malaria, and she
was immediately begun on atovoquone-proguanil combination. The same day, she
developed significant rigors associated with recurrent late decelerations on fetal heart
tracing, leading to an urgent Caesarian section. She delivered a baby boy of 6lbs 3oz
with thick meconium, and was transfused 1 unit of packed red blood cells and 6 units of
platelets intra-operatively. Her recovery was complicated by mild pulmonary edema
which resolved with diuresis on the second post-operative day, acute blood loss anemia
requiring an additional blood transfusion, and an episode of hypoglycemia. In
consultation with multiple specialty services, her thrombocytopenia, anemia, acute
kidney injury, and hypoglycemia were all determined to be secondary to a mixed
infection of both Plasmodium falciparum and Plasmodium malariae. This was
consistent with pathologic examination of her placenta revealing evidence of both
chronic & active malaria infection. She completed a three-day course of atovoquoneproguanil,
began a fourteen-day course of primaquine, and was discharged home on
the fourth post-operative day with oral iron and folate therapy. She was seen at sixweeks
for follow-up with complete resolution of her anemia and thrombocytopenia,
and reported she and her infant were doing well before transferring her care to a
different facility. Discussion: This case illustrates the broad differential that must be
considered when thrombocytopenia is encountered in a pregnant immigrant, and
demonstrates an uncommonly seen mixed malaria infection. It serves to remind us that
in pregnant patients, malaria must be swiftly recognized and carefully managed to
avoid a wide-range of maternal and fetal complications.
Potato Salad With a Side of Red Herring
West Nile virus is a rare but serious cause of morbidity and mortality, particularly in
older populations. A 73-year old man with history of GERD and a repaired inguinal
hernia presented to the emergency room in early September with four days of malaise
including headache, diffuse body aches and weakness associated with vomiting and
diarrhea. These symptoms started after a church picnic. He reported that three other
people who attended the picnic had been ill with “flu-like” symptoms that had since
improved. He was admitted to the hospital for work-up and treatment. Hospital
course: On hospital day one he was febrile without leukocytosis. Physical exam revealed
a well-appearing man with generalized weakness, resolving erythematous rash on the
chest, and minimal abdominal pain without organomegally. His presentation appeared
most consistent with a possibly food-borne viral or bacterial gastroenteritis. He was
treated with IVF resuscitation while stool and blood cultures were ordered. On hospital
day two he remained febrile with new leukocytosis. He was started on ciprofloxacin and
metronidazole for likely bacterial gastroenteritis. By the morning of hospital day three
his diarrhea had resolved however he remained febrile with diffuse weakness. By that
afternoon he had developed worsening headache with neck pain and photophobia.
Kernig’s sign was positive. CSF demonstrated lymphocytic pleocytosis and a peripheral
smear showed plasma cells. Antibiotics were discontinued with a new diagnosis of viral
meningitis. During the subsequent two days he had resolution of photophobia but
progressive weakness of his upper and lower extremities associated with cloudy
thinking and bilateral hearing loss. He had no respiratory compromise and by hospital
day six he demonstrated improvement in strength and balance. On hospital day seven
West Nile IgM and IgG, drawn on hospital day three, returned highly positive. All other
serum and CSF infectious studies returned negative. Discussion: This case is of
particular interest because it highlights the difficulty of early diagnosis of West Nile
virus even in the season of peak incidence of late summer and early fall. The
confounding factor for this patient was the report of sick contacts after an outdoor
potluck meal which appropriately led clinicians to consider food borne illnesses as likely
mechanisms of disease. It is possible that the patient's sick contacts had an unrelated
illness, though, it is certainly possible that they also had West Nile fever. West Nile virus
can manifest as West Nile Fever with symptoms consistent with our patient’s initial
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Patrick Hagen, MD
Haleigh James, MD
Siu-Hin Wan, M.D and
Mark Wylam, M.D.
presentation and can progress to neuroinvasive disease with meningitis, encephalitis or
meningoencephalitis. Lymphocytic pleocytosis of CSF is characteristic of West Nile
encephalitis. Plasma cells on peripheral blood smear have also been described.
Currently there are no evidence based treatments for West Nile virus beyond
supportive care.
Isolated Myeloid Sarcoma: A Great Disease Mimicker
Myeloid Sarcoma is a rare disease entity with variable presentation making prompt
diagnoses challenging. A 60 year old otherwise healthy male presented to clinic with
vague abdominal pain of one months duration. Initial workup was unremarkable with
the exception of a hardened umbilicus on physical exam. The patient was referred to
surgery for possible umbilical hernia but instead was started on a course of oral
antibiotics for presumed cellulitis. However, the abdominal pain intensified and
abdominal CT was subsequently obtained showing abnormal peritoneal deposits and
possible liver lesions. The patient was referred for colonoscopy but prior to his
appointment was admitted for again worsening abdominal pain Repeat abdominal CT
showed peritoneal carcinomatoses and initial omental biopsy showed likely
granulocytic sarcoma. In order to make a definitive diagnoses plus properly stage and
prognosticate, he ultimately required laparoscopic omental biopsy, bone marrow
biopsy, umbilical core biopsy, and inguinal lymph node FNA. Laboratory workup was
largely unremarkable with WBC of 5,600 , Hgb of 15.5 g/dL, and platelets of 281,000.
Basic metabolic panel and LFTs were unremarkable save a Cr of 1.38 mg/dL. Uric acid
was within normal limits but LDH was elevated to 1541 U/L (normal range 325-750).
PSA, Immunoglobulins, SPEP, HIV, HBV, HCV were all negative or unremarkable.
Further workup included a brain MRI and Echocardiogram both without significant
findings. Ultimately, a PET-CT showed advanced metastatic disease throughout the
chest, abdomen, and pelvis including a 7.7 x 6.1 cm mass in the root of the mesentery
invading the adjacent small bowel. Patient was re-admitted for induction therapy with
idarubicin and cytarabine. A final diagnosis of myeloid sarcoma was made based on
presence of myeloid cell markers from the omental and umbilical biopsies as well as the
absence of T or B cell markers. Interestingly, despite the advanced disease, bone
marrow biopsies showed normal cellular lineages, iron studies, morphology, and
cytogenetics. Prior to induction, lumbar puncture showed no blasts or abnormal cells
but thoracentesis of bilateral pleural effusions returned blasts with similar cell markers
and molecular abnormalities as described above. The patient has responded well to
treatment and other than pancytopenia has had a relatively uncomplicated course thus
far. This case demonstrates the challenge of making a prompt diagnosis of granulocytic
sarcoma, especially when isolated and not in the context of AML. Granulocytic sarcoma
can present in numerous anatomical locations with case reports describing breast,
orbital, skin, nasal, gastrointestinal, bladder, and soft tissue. Even when suspected
based on imaging and clinical history, workup can be involved and confirmation based
on morphology, cytogenetics, and flow cytometry challenging. Importantly, moving
quickly to diagnosis and initiation of treatment is critical as this disease can be rapidly
progressive and transform into AML at any time.
Abdominal pain, fatigue, and syncope - signs of cardiac tamponade?
Cardiac tamponade is a life-threatening clinical diagnosis that can be easily missed if a
complete physical examination or the proper studies are not performed in a timely
manner. When a pericardial effusion accumulates suddenly, pressure is exerted on the
heart, leading to hemodynamic compromise. Symptomatology may vary tremendously
from patient to patient; therefore the clinician must have a high clinical suspicion in
those presenting with evidence of cardiogenic shock. Case: A 32 year-old woman with a
history of pulmonary embolism and right atrial mass status post right atrial
thrombectomy and left pulmonary artery embolectomy 2 weeks ago presents with
epigastric pain, fatigue, and syncope. She has been on warfarin for the past month and
has an INR of 4.2. On exam, she has normal heart sounds, mild epigastric tenderness,
cool clammy extremities, and bilateral lower extremity edema. AST, ALT, GGT, and
alkaline phosphatase are 1457, 1025, 423, and 431, respectively. Abdominal ultrasound
obtained due to concern for Budd-Chiari syndrome reveals no thrombus, but does show
abnormal blood flow in the splenic, portal, and hepatic veins with periodic cessation of
forward flow, correlating with respiration. Echocardiogram reveals a large posterior
pericardial effusion with evidence of tamponade physiology. Given the effusion’s
location, pericardiocentesis is initially deferred. Overnight, the patient develops
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Kyle McKenzie, MD
Jackson J. Liang, DO;
Alexandra P. Wolanskyj,
MD
Dan Pease, MD
Douglas Rausch, MD
hypotension to 50/40 mmHg. Emergent pericardiocentesis is performed, draining
bloody fluid. Her hemodynamic status improves and she is transferred in stable
condition to the medical floor. Discussion: This case illustrates the variety with which
cardiac tamponade can present. Although "Beck''s triad" of hypotension, jugular
venous distention, and muffled heart sounds is thought of as the classic presentation,
all three of these signs are present only in minority of patients with this condition. In
fact, none of these were initially present in the patient described above. While fatigue,
epigastric pain, and elevated LFTs are not commonly associated with tamponade, they
are quite characteristic of this diagnosis when it is instigated by cardiac surgery at least
7 days prior. Originally described in the 1960s, delayed post-operative cardiac
tamponade is poorly recognized due to its low incidence and vague symptomatology.
Post-operative anticoagulation increases its risk, which is consistent with our patient''s
INR of 4.2. Patients typically present with vague symptoms and signs including fatigue,
malaise, epigastric or sternal pain, mild dyspnea on exertion, and abnormal LFTs. They
may also have fever, leukocytosis, oliguria, and prerenal azotemia. The clues pointing
toward tamponade causing compromised heart function in our patient included her
cool clammy extremities and her abnormal splenic, portal, and hepatic blood flow
varying with respiration. She developed hypotension when her pericardial effusion
created so much pressure around her heart that it was not able to pump effectively,
leading to cardiogenic shock. Pericardiocentesis was life-saving.
A 74-year-old man with fevers, facial pain, and neutropenia
Agranulocytosis, or failure of bone marrow to produce granulocytes, including
neutrophils, has a broad differential diagnosis and can be life threatening. Etiologies
include primary bone marrow disorders as well as, autoimmune, infectious and toxic
causes. Drug effect must be considered and careful review of medication and
supplement history is essential to determine the cause for the agranulocytosis and
target management approach accordingly. Clinicians should be mindful of drug
associated toxicity which may be acute or delayed, and recognize the variability in onset
and recovery. Case Presentation: A 74-year-old man presented to his local emergency
department complaining of left-sided jaw and facial pain, fevers, and chills for three
days. He had a history of chronic lymphocytic leukemia (CLL) and had been treated
with pentostatin, cyclophosphamide, and rituximab one year prior. Past medical
history also included type 2 diabetes and coronary artery disease. Recently he had
been relatively healthy with the exception of pneumonia one month prior which was
treated with several courses of antibiotics but could not recall which ones. On arrival to
the hospital, physical exam revealed an elderly nontoxic appearing man, febrile to
38.9°C with other vital signs normal. There was significant tenderness of his left
maxillary sinus and mandible. The remainder of his exam was normal. Lab results
were: 1.0 Leukocytes with an absolute neutrophil count (ANC) of zero, hemoglobin 10.7
g/dL, and platelet count 205,000/microL. Bone marrow biopsy demonstrated 30%
involvement by scattered interstitial lymphoid aggregates, normal erythropoiesis and
megakaryopoiesis, and absent granulocytic and monocytic precursors. CT scan of the
sinuses revealed near-complete opacification of the maxillary sinuses, maxillary
infundibula, and ethmoid air cells bilaterally. He was diagnosed with acute sinusitis and
treated empirically with intravenous antibiotics and antifungals. Further workup
revealed a negative antineutrophil antibody, and minimal residual disease of his CLL on
flow cytometry. Infectious and rheumatologic workups were negative. Careful review
of outside records revealed he had received two 10 day courses of
trimethoprim/sulfamethoxazole (TMP-SMX), and this was ultimately thought to be the
causative agent. Filgrastim injections were initiated and will continue until ANC
recovers to 1000. Bacterial sinusitis treatment was continued with
amoxicillin/clavulanic acid, until ANC recovery. Discussion: This case demonstrates a
rare, yet severe hematologic side effect of a commonly used antibiotic. Drug induced
agranulocytosis can occur quickly or in a delayed fashion, by direct toxicity on myeloid
precursors or by immune mediated destruction of neutrophils. Factors leading to poor
outcomes in agranulocytosis include age over 65 years, ANC

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Alexander Boucher, MD
Anne Blaes, MD
Naomi Fennell, MD
sore throat, and difficulty swallowing. A CT of the neck revealed a right peritonsillar
abscess. ENT performed an I+D, complicated by prolonged bleeding. At thas time a CBC
was ordered and revealed pancytopenia, with Hgb 9.1 g/dL, Plts

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Peter Hurley, MD
Anne Blaes, MD;
Michael Linden MD
fever, edema, hypoxia and respiratory distress with infiltrates on chest imaging, and
hypotension. Differentiation syndrome usually occurs 1-4 weeks after induction with
all-trans retinoic acid or arsenic trioxide and is potentially fatal if not recognized and
treated appropriately. Case Presentation: A 63 year old woman with no significant past
medical history presented with multiple bruises after sustaining a mechanical fall in her
garage. Her exam was remarkable only for bruising. Laboratory evaluation revealed a
white blood count of 1.5, hemoglobin of 9, and platelet count of 17. A CXR showed
mild cardiomegaly and clear lung fields. A peripheral smear was done showing 63%
blast cells and pathagnomonic “faggot” cells. Patient was diagnosed with acute
promyelocytic anemia and was started on all-trans retinoic acid. She had a bone
marrow biopsy confirming APL and showing hypercellular bone marrow (85% cellular)
with 75% of overall cellularity occupied by blasts. FISH showed t(15;17), confirming the
diagnosis of APL. On day 9 of treatment with ATRA, patient had a WBC of 0.9, Hg of 9.7,
and platelet count of 35. She received a transfusion of packed red blood cells and
platelets. Eighteen hours after the transfusion she developed fever, hypoxia, edema,
and confusion. The cross-cover resident was paged. Patient was transferred to the ICU
and a CT of the chest was ordered. The CT scan of her chest showed diffuse bilateral
ground glass opacities. The differential diagnosis for patient’s symptoms included
differentiation syndrome, acute respiratory distress syndrome, pulmonary hemorrhage,
hospital acquired pneumonia, transfusion related lung injury, and pulmonary edema.
Patient was treated with IV decadron with rapid clinical improvement. Discussion:
Differentiation syndrome is not a common diagnosis and could have been easily missed
as it includes a constellation of findings shared with many other illnesses. Because this
patient had received a blood transfusion the day that she developed differentiation
syndrome, TRALI was on the differential diagnosis. If she had been treated for TRALI
with supportive care, she likely would have worsened. Given her leukopenia, her
symptoms could also have been mistaken for systemic inflammatory response
syndrome with respiratory distress, which would not be treated with steroids.
Fortunately, this patient was treated appropriately with steroids and had rapid
improvement in her symptoms. This case demonstrates the importance of good
documentation in notes. Because the possibility of differentiation syndrome was
documented in the oncology note, the cross-cover resident was able to read about the
syndrome, recognize that this was the likely diagnosis, and treat appropriately with
steroids.
Burkitt’s Lymphoma in Pregnancy: Two Cases of Successful Treatment and Continued
Fertility
Burkitt’s Lymphoma (BL) is a rare but aggressive malignancy that most commonly
effects people during their reproductive years. We present two cases of pregnancy that
are complicated by BL that demonstrate the challenge of diagnosing and managing
aggressive disease during pregnancy. Case 1 is a 30 year old previously healthy female
who was 26 weeks pregnant who presented with 2 weeks of anemia and bilateral
breast enlargement. Core biopsy of her breast was positive for BL. She was in tumorlysis
at the time of presentation. She began treatment with multi-agent
chemotherapy. The next day she went into pre-term labor and a caesarian-section was
performed which successfully delivered a healthy baby girl. She finished the full course
of chemotherapy achieving complete remission. Both the patient and daughter are
alive and well 2 years later. She continues to menstruate on an every 28 day cycle. Case
2 is a 23 year old female with a history of HIV who was 14 weeks pregnant who
presented with 6 weeks of back pain and lower extremity neurologic deficits. MRI of
her spine showed an epidural mass at T5-T7. Biopsy of this mass was positive for BL.
She began treatment with multi-agent chemotherapy. Unfortunately she had a
miscarriage. The patient received grief counseling. She attained complete remission
with chemotherapy. Two years following the completion of her chemotherapy, she is
pregnant. These cases illustrate that BL can occur during pregnancy. It is important to
recognize when a pregnant patient presents with both physical and laboratory
abnormalities that are beyond those expected with physiologic changes of pregnancy
because malignancy may be the cause of such complaints. BL is fatal within weeks if
untreated thus prompt diagnosis is critical. Treatment of BL includes multi-agent
chemotherapy that can cause infertility. We present two cases in which pregnant
women underwent treatment for BL and maintained fertility suggesting that pregnancy
may provide protection from chemotherapy related infertility.
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Christopher Janish, MD
Brummond, Alissa M.D.;
McDonald, Furman
M.D.
Hayan Jouni, MD
Steven L. Driver, M.D.,
and Joerg Herrmann,
M.D.
Don''t Stop The Beat: A Novel Cause of Recurrent PEA Arrest
Case Presentation: 85 year old female with history of HTN, CKD stage 3, and breast
cancer treated with anastrazole transferred from outside ED for recurrent episodes of
syncope, intermittent diarrhea, and 30lb weight loss. In the 6 months prior, she had 4
episodes of PEA arrest, some lasting greater than 20 minutes, and subsequently
underwent placement of a permanent pacemaker. Extensive cardiac work up including
angiography and echocardiogram was normal. Immediately upon transfer to our facility,
she began to feel flushed and light-headed. She was tachycardic and profoundly
hypotensive, and her mental status deteriorated rapidly to unresponsiveness. She
developed PEA arrest; EKG showed appropriate pacemaker function with 1:1
conduction. CPR was initiated with return of circulation within 2 minutes. She was
transferred to the CCU. Basic labs revealed chronic macrocytic anemia, mild
leukocytosis with mild peripheral eosinophilia, and serial troponins were negative.
Several hours later, she again developed hypotension with diffuse flushing, devolving
into PEA arrest requiring intermittent CPR and multiple doses of epinephrine. Bedside
echocardiogram was normal except a moderate pericardial effusion; tamponade
physiology was absent. Extensive laboratory work up was initiated to look for carcinoid
syndrome, pheochromocytoma, medullary thyroid carcinoma, VIPoma, and systemic
mastocytosis. CT scan of the chest, abdomen, and pelvis revealed multiple mixed lytic
and sclerotic foci throughout axial skeleton compatible with metastases. Cetirizine and
ranitidine were started with concern for systemic mastocytosis and iliac crest bone
marrow biopsy was performed. Tryptase and urine N-methyl histamine returned
markedly elevated and urine beta prostaglandin F2 alpha returned near ULN. Bone
marrow biopsy revealed numerous foci of spindled mast cell infiltrates involving 30% of
bone marrow cellularity consistent with aggressive systemic mastocytosis. KIT gene
mutation analysis was positive for the KIT D816V mutation. She was subsequently
placed on cetirizine, ranitidine, cromolyn sodium, montelukast, and aspirin. She had no
recurrence of her symptoms, and the rest of her hospitalization was unremarkable. In
follow up, she is strictly avoiding histamine-containing foods, and has had no further
episodes on this regimen. Discussion: Systemic mastocytosis (SM) is a
myeloproliferative neoplasm characterized by monoclonal proliferation of mast cells.
SM is a rare condition with an incidence of less than 10 cases per 1 million people;
common presenting symptoms include urticaria pigmentosa, abdominal pain, diarrhea,
and anaphylaxis. Aggressive systemic mastocytosis (ASM) is the least common of
several subclasses of SM, comprising only 3% of all new diagnoses. SM is associated
with mutations in the c-kit gene, with KIT D816V point mutation being the most
common. Treatment for ASM involves antihistamines, leukotriene antagonists, mast cell
stabilizers, aspirin, and, in some cases, cytoreductive therapy. This case is a novel
presentation of a rare disorder, but in patients with symptoms of histamine excess,
ASM should be considered.
A Classic Case of Amyloid Cardiomyopathy
A 55 year-old-female with a past history of hypertension, dyslipidemia, and carpal
tunnel syndrome had slowly progressive fatigue, weight loss, early satiety, and diarrhea
over a 12-month period. She then developed several syncopal episodes attributed to
orthostatic hypotension and one episode of cardiorespiratory arrest secondary to
ventricular tachycardia. She was successfully resuscitated and was transferred to our
hospital for further assessment. Evaluation included an electrocardiogram which was
significant for peripheral low voltage QRS and pseudo-anteroseptal-infarction pattern
(Figure available). Transthoracic echocardiogram showed an ejection fraction of 50%
and a constellation of findings strongly suggestive of amyloidosis (Several figures
available). Abdominal fat aspirate demonstrated amyloid deposition with Congo red
staining; submucosal amyloid deposition was also identified in a gastric biopsy obtained
one year earlier at the time of an upper endoscopy (Figures available). The amyloid was
confirmed to be of AL-type (lambda-light chain type by mass spectroscopy). A bone
marrow biopsy revealed slight marrow plasmacytosis (5-10%) without definite
immunoglobulin light chain restriction. Serum and urine free light chains were elevated.
Serum NT-pro-BNP was 20,977 pg/mL and serum cardiac troponin-T was 0.12 ng/mL.
Elevation of these cardiac biomarkers indicates poor outcomes in patients with primary
systemic amyloidosis. Unfortunately, she was ineligible for combined stem cell and
cardiac transplant given her multi-organ involvement with amyloidosis. She was started
on Prednisone and Melphalan, and an implantable cardioverter defibrillator was placed
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Katherine Janssen, MD
Gregory Kaufman, MD
Joanne E. Yi, MD; David
R. DeLone, MD; Allen J.
Aksamit, MD; Mark R.
Litzow, MD
for secondary prevention. Beta-blocker and ACE inhibitor therapy were re-initiated but
require careful titration in these patients. - Discussion: Low voltage QRS and pseudoanteroseptal-infarction
pattern on ECG along with thickened, echo-bright myocardium
on echocardiography should always raise the suspicion for an infiltrative process such
as amyloidosis. Elevation of serum NT-pro-BNP and cardiac troponin is associated with
poor outcomes in patients with primary systemic amyloidosis. Reduced longitudinal
peak systolic myocardial strain on echocardiography is also associated with adverse
outcomes in patients with primary AL amyloidosis.
A Curious Case of Stimulant Toxicity
The use of medications for controlling ADHD is a common practice. In college students,
the use of energy supplements continues to grow and combining these is likely a
frequent occurrence. CASE: An 18 year old college football player presented to the
emergency room with progressive shortness of breath and intermittent chest pain
lasting for one day. The patient reports staying up all of the previous night to work on a
project. The next day he took his regular dose of Lisdexamfetamine and a 5-hour
energy due to staying up the previous night. He went to football practice and took
another 5-hour energy. At football practice, the patient became weak and nauseated,
and was unable to complete practice. He became short of breath, and it worsened
throughout the afternoon. His roommate called the paramedics when his shortness of
breath and chest pain. In the ED the patient was tachypneic to 27 breaths per minute,
but maintaining appropriate oxygen saturations. He appeared to be in significant
respiratory distress. CT scan was negative for PE. His EKG was concerning for
progressive T wave inversion in the lateral precordial leads and ST elevation in the
anterolateral leads. His second troponin came back elevated at 0.049. Due to his
ongoing chest pain, the patient was urgently taken to the catheterization laboratory.
His coronary arteries were free of clot. Cardiac ECHO and MRI were significant for
hypertrophy consistent with athletic conditioning. After one evening in the hospital,
the patient’s chest pain resolved, his EKG normalized, his troponin peaked, and he was
discharged. DISCUSSION: This patient demonstrates the potential consequences of
stimulant toxicity; lisdexamfetamine and over the counter energy supplements can
result in severe symptoms and laboratory findings mimicking STEMI. Caution should be
used when simultaneously taking multiple stimulants.
A Rare Infectious Neurologic Complication Following Allogeneic Hematopoietic Cell
Transplantation (HCT)
Learning Objectives 1. Recognize the presentation of Progressive Multifocal
Leukoencephalopathy (PML) in the post HCT setting. 2. Understand PML may present
following peripheral count recovery, in the absence of lymphopenia. 3. Review the
diagnostic limitations of cerebrospinal fluid (CSF) analysis in confirming the diagnosis of
PML. Case Description A 65-year-old man with a history of high-risk pre-B ALL, initially
treated with induction chemotherapy on a standard modified BFM regimen in
combination with rituximab, underwent an allogeneic, HLA sibling matched, HCT with
reduced intensity conditioning consisting of fludarabine and melphalan. Initial posttransplant
complications included coagulase negative staphylococci bacteremia,
enterococcus faecalis infection in the L5-S1 disk space, CMV viremia, as well as
gastrointestinal and skin GVHD. Bone marrow biopsy on day +109 post-transplant
revealed no evidence of leukemia and confirmed donor engraftment. On day +318, a
complete blood count revealed an absolute lymphocyte count of 2.19 k/uL (normal 0.70
– 4.70 k/uL). The patient’s wife reported on day +329 that he was weak and his lower
extremities were stiff and “hard to bend.” Neurological examination revealed masked
facies, marked bradykinesia, rigidity of both lower extremities, and bilateral Babinski
signs. He was unable to ambulate. MRI of the lumbosacral spine was negative for
potential cause or recurrence of bacterial infection. MRI of the brain revealed T2/FLAIR
hyperintensity in the left corona radiata and centrum semiovale, extending to the
subcortical arcuate fibers, suggestive of inflammatory disease. Examination of the CSF
was unrevealing, with only a slightly elevated protein of 42 mg/dL. Specifically, PCR for
HSV, VZV, CMV, HHV-6, EBV, and JCV were negative. Brain biopsy was performed to
confirm the diagnosis. Light microscopy revealed marked gliosis and smudged nuclei in
the left frontal lobe. In Situ Hybridization (ISH) studies performed on paraffinembedded
tissue were positive for JCV DNA and the diagnosis of PML was confirmed.
The patient quickly progressed, developing quadriparesis and the inability to swallow.
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Daniel Kebed, MD
Jennifer Hsu AB, Mark L.
Wieland MD
Brian McChesney, MD
Pursuant to discussions with the patient and his family, comfort care measures were
instituted and no additional therapy was attempted. The patient died on day + 362,
approximately one month after symptom onset. Discussion PML is an important entity
to include on the differential diagnosis of HCT patients presenting with
neurodegenerative symptoms. To our knowledge, and after a review of the adult
English literature, there have been only four other tissue confirmed cases of PML after
allogeneic HCT. PCR for JCV DNA in the CSF has limited sensitivity, and when in doubt,
ISH studies for JCV DNA from a brain biopsy should be considered for diagnostic
confirmation. As this case demonstrates, profound lymphopenia may not be present at
symptom onset, and this should not be reason to exclude PML from diagnostic
consideration.
Disseminated Nocardiosis
A 72 year old Caucasian woman was admitted to the hospital directly from an
outpatient bronchoscopy suite for acute onset of fever, cough and tachycardia
following transtracheal biopsies of a right upper lung mass. Prior to admission she
described six months of progressive fatigue, chills, functional decline, and 40 pounds of
unintentional weight loss. Review of systems was otherwise unremarkable. Past
medical history was significant for polymyalgia rheumatica on chronic glucocorticoid
therapy, chronic kidney disease , hypertension, and type 2 diabetes. After fluid
resuscitation, she remained hemodynamically stable throughout the hospitalization
with initial intermittent low-grade fever. Physical examination was pertinent for a
tender 3x5 cm fluctuant mass associated with the right deltoid muscle, fluctuance and
tenderness of the right gastrocnemius muscle, and a 1x1 cm tender nodule associated
with the left parotid gland. The cardiopulmonary, abdominal, and lymphatic
examinations were unremarkable. Admission laboratory evaluation was remarkable
for a normocytic anemia, mild neutrophilic leukocytosis, elevated sedimentation rate
(82 mg/dL), elevated creatinine (2.4 mg/dL), and elevated hemoglobin A1C (8.3%).
Prior CT of the chest showed a large right upper lung mass with multiple bilateral
pulmonary nodules and mediastinal lymphadenopathy. Gram stains of fluid aspirated
from the left deltoid and left leg demonstrated branching gram positive bacilli. Cultures
of this fluid and of tissue from the lung and parotid gland grew nocardia farcinica. She
underwent surgical debridement of the deltoid abscess and needle drainage of the left
lower extremity and parotid abscesses. Antimicrobial therapy included trimethoprimsulfamethoxazole
(TMP-SMX), imipenem and moxifloxacin for disseminated
nocardiosis. The hospital course was complicated by TMP-SMX induced hyperkalemia
that responded to dose adjustment. She was discharged on a prolonged antimicrobial
course. At outpatient follow-up, her symptoms had largely resolved and there was no
evidence of new metastatic infection. Nocardiosis is a rare bacterial opportunistic
infection that predominantly afflicts patients with compromised immune systems.
Common sites of infection include the lungs, central nervous system, and soft tissue.
Our patient had disseminated nocardiosis, which typically carries a poor prognosis.
TMP-SMX is the backbone of antimicrobial therapy for nocardiosis; treatment duration
may be up to 12 months.
The Fragile Red Blood Cell: Hemolysis in Pernicious Anemia
Vitamin B12 deficiency is a relatively common disease that has many different signs and
symptoms. It is important to remember that left untreated vitamin B12 deficiency can
cause significant morbidity and mortality. This relatively extreme case demonstrates
the numerous clinical signs and symptoms of severe and prolonged B12 deficiency. DO
is a 54 year old man who presented to the Emergency Department after progressive
jaundice and “cola colored” urine the week preceding admission. He noted that for the
past several months he had increasing lower extremity edema and exertional dyspnea
only able to walk about a block before needing to rest. He also noted weakness,
numbness, and tingling in his hands. He has a medical history of hepatitis C. He was
not taking any medications. He denied any history of drug or alcohol abuse. He was
not on any special dietary restriction. On physical exam, patient was notably jaundiced
and scleral icterus was present. He had 3+ pitting edema in bilateral lower extremities.
Systolic flow murmur was present. Pin-prick, light-touch and vibratory sensation were
diminished to bilateral mid-shin and mid-forearm. Admission CBC was notable for
Hemoglobin of 3.0 with an MCV of 130, WBC 1.5, and platelet count of 80,000. His liver
function tests were notable for increased bilirubin, the majority of which was indirect,
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Peter Lund, MD
Maya Kessler, MD
John Bundrick, MD;
Janelle Gooden, MD
and normal transaminase levels. He had an elevated d-dimer, fibrinogen 150,
haptoglobin less than 20, and LDH of 3735. His peripheral smear showed marked
macrocytic anemia with occasional hyper-segmented neutrophils. His vitamin B12 level
was 81 (lower limit of normal is 150). Patient was initially admitted to the Medical ICU.
He received 2 units of red blood cells carefully monitoring for any signs of volume
overload. He then received IM vitamin B12 replacement daily while in the hospital. His
potassium was closely monitored for any sign of hypokalemia. After 4 days, he was
discharged with the plan for weekly B12 injections. His CBC at a 2 month follow up
appointment normalized. He had a normal EGD. Intrinsic factor binding protein was
positive. The main learning point for this case is to recognize that pernicious anemia
leading to vitamin B12 deficiency can present with a range of symptoms, which can be
quite severe, one of which is jaundice secondary to intramedullary red cell hemolysis.
Sexy Diarrhea: Giardia lamblia presenting as a sexually transmitted infection
There are many commonly recognized sexually transmitted infections (STI). Of the
protozoal infections, Trichomonas vaginalis is most frequently in the differential. Other
protozoa including Giardia lamblia and Entamoeba histolytica must also be considered,
especially in men who have sex with men. A 66-year-old homosexual male with a
history of atrial fibrillation, hypertension, diabetes mellitus, and obstructive sleep
apnea presented to clinic with five day history of watery diarrhea occurring after eating.
No fevers, chills, nausea, vomiting, melena, hematochezia, or abdominal pain. No sick
contacts but week previously ate food at the state fair. No recent travel, healthcare
exposure, or antibiotic use. No recent camping trips and no contact with children in
daycare. Vitals were within normal limits and abdominal exam was unremarkable. Basic
metabolic panel and complete blood count were unremarkable. Symptoms and
duration appeared consistent with viral gastroenteritis with plan to return if symptoms
persisted. The patient returned to clinic two days later with increased frequency of
stools. Stools remained liquid and green occurring after any oral intake. He had no
fever, nausea, vomiting, abdominal pain, or orthostasis. Stated symptoms were similar
to infection with Shigella he had many years previously. Tachycardia with unremarkable
abdominal exam. Upon further review of social history, patient stated he was sexually
active and was having anal intercourse. This information raised the question of fecaloral
transmission of protozoal disease. Labs were negative for Clostridium dificcile,
Treponema pallidum, HIV, Salmonella, Campylobacter, Shigella and Escherichia coli.
Giardia lamblia trophozoites were seen in ova and parasite exam and Giardia antigen
was positive. He was treated with metronidazole for seven day course with complete
resolution of diarrhea. Given no other clear exposure and recent intercourse, most
likely mode of acquisition was sexual transmission. Review of this case demonstrates
the importance of considering the full social history when developing a differential
diagnosis. While Neisseria gonorrhoeae, Trichomonas vaginalis, Chlamydia trachomatis
are common STIs, other possible infections can be sexually transmitted. Giardia lamblia
should be considered when acute diarrhea is seen, especially in men who have sex with
men.
A Low Cost Approach to Diagnosing a Multi-system Problem
A cachectic, jaundiced 56 year-old man presented with worsening of chronic postprandial
vomiting. He had been hospitalized several times over the past 5 years for
similar symptoms. His co-morbid conditions included hypertension, COPD, and chronic
neck and back pain. His medications were amlodipine, albuterol, tiotropium,
fluticisone, and sulindac. His prior work-up was extensive with several EGDs, ERCPs,
and CT scans of the abdomen and pelvis, revealing only biliary microlithiasis. Hospital
records showed multiple admissions for chronic vomiting, hyperbilirubinemia, and
acute kidney injury. During previous hospitalizations his symptoms and laboratory
abnormalities had rapidly improved with seemingly minimal interventions other than IV
fluids. On admission his vital signs were normal. Physical exam was notable for
cachexia and jaundice. Total bilirubin was elevated at 2.7 mg/dl; direct bilirubin was 1.7
mg/dl. His alkaline phosphatase, AST, ALT, amylase, and lipase were within normal
limits. Gamma-glutamyltransferase was elevated at 195 IU/L. Creatinine was elevated
at 1.5 mg/dl. Urinalysis showed pyuria, hematuria, and proteinuria. Electrolytes and
ABG revealed a non-gap metabolic acidosis, and urine chemistries were consistent with
a type 1 renal tubular acidosis. He had a normocytic anemia with normal iron studies
and no evidence of hemolysis. His reticultocyte percent was inappropriately normal.
Abdominal ultrasound and EGD were normal. Systemic infiltrative processes were
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Catalina Norman, MD
considered. Sjogren Syndrome was ruled out as he did not have dry eyes or dry mouth
and serologies were negative. Amyloidosis was considered, but his SPEP, UPEP, and
light chains were normal, and gastric biopsies were negative. As cholestatic jaundice
was his most specific finding, this abnormality was used to anchor further investigation.
Primary biliary cirrhosis was considered, but anti-mitochondrial antibodies were
negative. Medications were considered. A quick search of PubMed revealed sulindac
had been associated with cholestatic jaundice. Sulindac had also been associated with
interstitial nephritis and marrow suppression. It was not reported to cause chronic
vomiting, but it seemed the likely culprit in this case. Temporally he had begun taking
sulindac shortly before his symptoms began. His sulindac had been held at admission
and by hospital day 3 he was clinically improved with resolution of vomiting and
normalization of laboratory values, except anemia. His sulindac had also been held
during previous admissions, fitting with his history of improvement during previous
hospitalizations. There was no need for a medication re-challenge, as this had already
been done inadvertently several times. This case demonstrates the importance of
diagnostic reasoning as a high value low cost alternative to extensive testing. It also
demonstrates an important clinical pattern that should trigger consideration of a
medication reaction — symptoms that resolve during hospitalization only to recur at
discharge. Further, it highlights the importance of keeping medications in the
differential of multi-system reactions.
Weather the Thyroid Storm: An Overlooked Cause of Altered Mental State
Introduction: Thyroid storm (TS) is a rare but potentially life-threatening condition
characterized by exaggerated clinical signs of hyperthyroidism. Case Report: A sixtyeight
year old female with a past medical history of duodenal perforation status-post
omental patch repair was brought to the emergency department after the emergency
services had been notified by the patient’s attorney, whom she had called forty times
that day. She denied any complaints, but believed that she was in the 18th century and
being held as part of a conspiracy. On arrival, vital signs revealed a heart rate of 144
beats/minute, blood pressure of 121/71 mmHg, respiratory rate of 25-breaths/ minute
and temperature of 37.6’C. Examination, including thyroid palpation was unremarkable
asides from tachycardia, agitation and severe confusion (disorientation to time, place
and person). Laboratory testing revealed a normal complete blood count, renal
function and liver enzymes. Thyroid function testing revealed markedly suppressed
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Hannah Lichtsinn, MD
Josh Overgaard, MD
Peter Lund, MD
Hiding in plain sight: A late diagnosis of a rare metabolic disease
Inborn errors of metabolism are rare and can easily be misdiagnosed as hypoxic
ischemic encephalopathy in early childhood, leading to significant morbidity and
mortality in later life. History: A 43-year old man presented with one day of nausea and
vomiting with left sided weakness. Head CT was within normal limits and MRI was not
successful secondary to patient movement. He was eventually intubated in the ED
secondary to progressive lethargy. Past Medical History was significant for “mild
cerebral palsy”, seizure disorder with onset at age 30 with grand mal seizure, and lupus
anticoagulant syndrome with one DVT. His medications were limited to carbamazepine
and warfarin. Hospital Course: On hospital day two his ammonia was elevated to 160.
Carbamazepine was discontinued and lactulose initiated. On hospital day four his
ammonia had risen to 336 and an EEG demonstrated seizures. Complete metabolic
evaluation revealed normal serum arginine and citrulline with elevated serum ornithine
and elevated urine homocitrulline consistent with a diagnosis of Hyperammonemia-
Hyperonithernemia-Homocitrullinuria (HHH) syndrome. He received treatment with
hemodialysis, low protein TPN, sodium phenylacetate/sodium benzoate and sodium
phenylbutyrate with complete medical recovery and only minor cognitive deficits at
three-month follow-up. Additional History: On further questioning, it was found that
the patient had a lifelong history of chronic emesis as well as protein avoidance. His
family history was significant for one sister with cognitive delay attributed to cerebral
palsy and another sister with chronic emesis and protein avoidance. He has two healthy
siblings. This patient and one of his sisters went on to have identification of a novel
nonsense mutation in the SLC25A15 gene, characteristic of HHH syndrome. Discussion:
HHH is a rare metabolic disease caused by an autosomal recessively inherited mutation
in the SLC25A15 gene. This mutation produces a defective ORNT1 transporter resulting
in ineffective transport of ornithine and citrulline across the mitochondrial membrane
in the urea cycle. HHH can lead to life threatening hyperammonemia if not recognized
and treated. It generally presents in early childhood with protein food intolerance,
vomiting, lethargy, coma, seizures, progressive spastic paraparesis and mental
retardation. This constellation of symptoms, as in this patient, can be subtle and easily
misdiagnosed as cerebral palsy secondary to hypoxic ischemic encephalopathy. Though
rare, inborn errors of metabolism should be considered in children with postnatal onset
of encephalopathy or spastic paraparesis with unremarkable birth history, positive
family history of neurocognitive deficits or in any child with elevated ammonia without
evidence of liver dysfunction.
The Near Lethal Back Massage
Introduction: A review of the predictive value of musculoskeletal tenderness in risk
stratification of chest pain. Case Presentation: A 94 year old female with significant
history of hypertension, hyperlipidemia, and left bundle branch block (LBBB) presented
to the ED with 24 hrs of crampy left posterior shoulder pain radiating down the left arm
that was exacerbated by lying down and alleviated by massage. She could recall no
injury. There were no associated symptoms. Vital signs were normal and physical
examination revealed a tenderness over the left superior scapula. Her initial EKG
revealed an unchanged LBBB. A troponin I was negative. She was given nitroglycerine
which completely alleviated her pain. She was admitted for a cardiac rule out and
placed on a nitroglycerine infusion. Her enzymes remained negative overnight however
she did have an episode of chest pain that was relieved with increased nitroglycerin
infusion rate. In the morning a myocardial perfusion study was ordered. Repeat
enzymes, however, were positive that afternoon and the patient was taken urgently to
percutaneous coronary angiography. She was found to have 90% lesions to the
proximal LAD, ramus, and proximal RCA. These were stented and the patient
underwent routine post-PCI care. Her shoulder pain resolved and she had no further
episodes in hospital or on cardiology follow up two months later. Discussion: In this
case, it might have been easy to disregard the pain as musculoskeletal pain with the
clear description and initial negative or nondiagnostic studies. By this examiner’s
experience, reproducible chest wall or other musculoskeletal-type pain is commonly
taught by residents and medical staff as being a strong negative predictor of visceral
thoracic disease such as myocardial ischemia or PE. In actuality, tenderness and even
reproducibility are not specific enough to rule out MI or PE. Although the negative
likelihood of ACS with reproducible chest pain is about 0.4, in a patient like this one
with multiple risk factors, pretest probability remains quite high. Interestingly, the
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Tossapol
Kerdsirichairat, MD
John Lake, Rajeev
Attam, Mustafa Arain,
Martin Freeman
Alexis Leal, MD
Sanjay Kalra, M.D.
accuracy for predicting PE with reproducible pain is much worse and is reported to have
sensitivity and specificity of 17% and 79% respectively.
Pan-mesenteric thrombosis causing portal biliopathy
Portal biliopathy is a term used to describe biliary ductal and gallbladder wall
abnormalities seen in patients with portal hypertension. These changes are
predominantly seen in patients with extrahepatic portal vein obstruction (EHPVO).
Intra-abdominal sepsis is the common cause in childhood while hypercoagulable state,
myeoproliferative disorders, and underlying cirrhosis are common causes in adults. We
report a case of portal biliopathy diagnosed and treated endoscopically in our
institution. A 35-year-old male with a past medical history significant for celiac sprue
and idiopathic portal hypertension requiring splenectomy and splenorenal shunt during
childhood, chronic portal vein thrombosis, was found to have elevated alkaline
phosphatase of 1,015 U/L on annual check-up without jaundice or pruritus. Physical
exam showed no signs of chronic liver disease or lymphadenopathy. Other liver
enzymes were mildly elevated with AST of 222 U/L, ALT of 286 U/L, and total bilirubin of
1.5 mg/dL. His liver tests had previously been normal. He underwent magnetic
resonance cholangiopancreatography (MRCP), which showed intrahepatic biliary
dilatation with area of stricturing near the confluence of the right and left hepatic
ducts, and mesenteric edema with lymphadenopathy in the portocaval region,
retroperitoneum, and mesentery. Transabdominal ultrasonography with doppler
showed cirrhosis, slow flow within portal vein with cavernous transformation, and
sludge within a dilated gallbladder. Investigations for the etiology of cirrhosis, and a
possibility of hypercoagulable state and myeloproliferative disorders were all
unremarkable. Endoscopic ultrasonography (EUS) with doppler was limited due to the
extensive venous collateral formation but revealed no mass at the stricture. He further
underwent endoscopic retrograde cholangiopancreatography (ERCP) showing a highgrade
stricture in the distal bile duct, which was treated with temporary biliary stenting.
Postoperatively, he had significant improvement in the liver enzyme levels. He
remained asymptomatic at a two-year follow-up visit. This case highlights the approach
to an uncommon cause of biliary obstruction. Portal biliopathy is more common and
pronounced in patients with EHPVO than in patients with idiopathic portal hypertension
or cirrhosis. For patients with EHPVO, who develop biliary symptoms, abnormal alkaline
phosphatase activity, or dilated bile ducts, MRCP is the initial investigation of choice.
Patients with evidence of biliary obstruction by MRCP should undergo EUS with doppler
to delinate the etiology of the obstruction. If portal bilopathy is confirmed, the
treatment of choice is porto-caval shunting. ERCP with biliary stent placement is the
treatment of choice in patients who are not candidates for the porto-caval shunting.
Cessation is Key: A Case of Pulmonary Langerhans’ Cell Histiocytosis (PLCH)
Langerhans’ cell histiocytosis (LCH) is a rare condition that affects adults, primarily
between ages 20 to 40. The majority present with single organ pulmonary involvement,
often with abnormal imaging studies that may be accompanied by cough and/or
dyspnea. A sixty-six year-old female presented to her local physician for persistent
cough with clear sputum, mild hoarseness and progressive dyspnea. She denied fevers
or night sweats but complained of general malaise and forty pound weight loss over 3
months. She had smoked for thirty five years and had occasional bouts of bronchitis
during previous winters. She was initially treated with antibiotics, but when symptoms
persisted, a chest CT scan was performed which showed innumerable nodular lesions
with predominance within the upper lung fields. A PET scan showed sufficient FDG
uptake that surgical lung biopsy was performed. Rather than the expected carcinoma,
pathology suggested Langerhans’ cell histiocytosis. She quit smoking the day of her
biopsy and no active treatment was offered. Her cough progressively improved and
became mild and intermittent with clear sputum, although she continued to have
dyspnea on exertion. Pulmonary function tests (PFTs) showed mild airflow obstruction
and reduced diffusing capacity (50% predicted) but with little improvement over 6
months. A chest CT done 5 months after diagnosis showed significant resolution of the
nodular lesions with modest centrilobular emphysema remaining. Due to continued
dyspnea, she underwent cardiac evaluation with echocardiogram and coronary
angiogram, which were unremarkable. An overnight oximetry led to a polysomnogram
and initiation of CPAP with improvement in her daytime fatigue. Due to continued
dyspnea, treatment with prednisone was initiated and when little improvement was
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Jackson Liang, MD
Kalkidan Bishu MD,
Nandan Anavekar
MBBCh
John Meyerhofer, MD
achieved, she was referred to our facility for further evaluation. Upon presentation,
she continued to have mild clear sputum production with occasional cough and
dyspnea on exertion. She had been significantly inactive since diagnosis and had
regained all of the previously lost weight. Physical exam was significant only for obesity
(BMI 37.9). A repeat chest CT and PFTs revealed continued improvement with her
diffusing capacity now at 73% predicted. ECG, echocardiogram, overnight oximetry (on
CPAP) and baseline laboratory studies were normal. Review of the outside pathology
confirmed pulmonary Langerhans’ cell histiocytosis with background bronchiolitis. The
patient’s dyspnea was felt to be secondary to deconditioning and obesity, and she was
advised a rigorous weight loss and exercise program coupled with continued tobacco
abstinence. The pathogenesis of LCH is unknown, but nearly all cases of pulmonary
involvement occur in current or previous smokers. This case is unique in that it
illustrates the presentation of an infrequent illness and the remarkable benefit of
smoking cessation on disease regression. The bulk of her improvement occurred prior
to corticosteroid therapy which likely contributed only side-effects rather than benefit.
Infective Endocarditis with Septic Embolism Causing Acute Stroke- Successful Solitaire
Thrombectomy
INTRODUCTION: Ischemic stroke due to septic embolism is one of the most feared
complications of infective endocarditis (IE). The optimal treatment of stroke in this
setting has not been well defined. CASE: A 70-year-old woman was brought to the
emergency room after being found down by her son in her home. On arrival, she was
febrile, hypotensive, tachycardic, and tachypneic. She was admitted to the intensive
care unit, fluid resuscitated, and started empirically on intravenous antibiotics. Her
blood pressure stabilized and she was transferred to the floor, where multiple sets of
blood cultures grew streptococcus Agalactiae. Transesophageal echocardiogram
demonstrated a 2cm mobile mass on the atrial surface of her posterior mitral valve
leaflet, consistent with vegetation due to IE. She was transferred via ambulance to our
tertiary hospital for further management. En route to our hospital, she developed
acute right-sided weakness and expressive difficulty. Upon arrival, she was seen
immediately by a neurologist and her NIH Stroke Scale score was determined to be 24.
Physical exam demonstrated aphasia, right hemiparesis, right sensory loss, and a right
Babinski sign. She became drowsy and developed respiratory distress, prompting
intubation. Head CT without contrast revealed no evidence of intracranial hemorrhage.
CT Angiogram revealed complete abrupt occlusion of a large anterior left M2 segment
by a presumed septic embolus. CT Perfusion imaging demonstrated the presence of
salvageable penumbra in the left MCA distribution. She underwent emergent
mechanical thrombectomy. Full recanalization was achieved with successful
thrombectomy using the Solitaire retrievable stent. There were no procedural
complications and post-procedure CT scan revealed no evidence of hemorrhage.
Within hours, her right hemiparesis improved and after one day she could move all
extremities against gravity. By day four, she exhibited no residual signs of her acute
stroke. Due to her poor baseline functional status, both her family and the
cardiovascular surgions decided she was a poor surgical candidate. She was discharged
to a skilled nursing facility to continue rehabilitation and complete her course of
intravenous antibiotics. DISCUSSION: The most common cause of intracranial
hemorrhage in patients with septic emboli is hemorrhagic transformation of the
ischemic infarct. IV tPA may potentiate the risk for hemorrhagic conversion in these
patients. The Solitaire Flow Restoration device is an intracranial stent that self-expands
when deployed in an occluded vessel and allows for withdrawal of the thrombus once
unfolded. We describe the second documented case of successful revascularization of
ischemic stroke due to septic embolism using the Solitaire device. One should consider
transferring patients with symptoms of acute ischemic stroke in the setting of IE to a
facility with the capability to perform endovascular mechanical revascularization, as it
may be associated with lower risk of intracranial hemorrhage than IV tPA.
A ''B'' cell dilemma: Common Variable Immune Deficiency and non-Hodgkin Lymphoma
Common variable immune deficiency (CVID) is an immune disorder caused by a lack of
B cell differentiation. Diagnostic findings include a reduced serum IgG with low levels of
IgA and/or IgM and minimal response to vaccinations. Characteristically these patients
have recurrent sinus or pulmonary infections, autoimmune disease, and an increased
risk of malignancy, particularly non-Hodgkin lymphomas (NHL). A 62 year-old male was
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Beth Thielen, MD
referred for an evaluation of immune deficiency following treatment of a right femur
large B-cell NHL. He reported a childhood history of multiple sinus infections. Most
notably he had a severe episode of pneumonia which required hospitalization for 19
days at the age of 20. Workup during this pneumonia was significant for low
immunoglobulin levels. Serum electrophoresis performed in his 50s due to continued
infections again showed complete absence of gammaglobulin. He then started monthly
intravenous immunoglobulin replacement. At the age of 62 he had sudden onset of
right hip and leg pain after he stood up from the toilet and quickly turned to put the
seat down. He sought care at an emergency room and was diagnosed with pathologic
fracture of the right femur. He underwent internal fixation and pathology from the
retrieved bone fragments showed CD20+ large B-cell NHL. Further evaluation showed
no other foci of lymphoma. He was noted to have normal IgG and undetectable levels
of IgA and IgM at the time of this fracture. He achieved complete remission after 4
cycles of R-CHOP (rituximab, cyclophosphamide, doxorubacin, vincristine, prednisone)
and localized femur radiation therapy. Immunologic evaluation revealed unremarkable
nasal, oropharynx, pulmonary, cardiac, and abdominal physical exams. Decreased
strength was noted in the right leg quadriceps and hamstrings as well as decreased
right patellar tendon reflex. Immune deficiency testing at this time showed zero CD19
B-cells, normal total IgG and IgG subclasses, near-absent IgA, undetectable IgM, and
normal IgE. Flow cytometry was performed to evaluate for Bruton’s tyrosine kinase
(BTK) protein which showed present protein expression. Gene sequencing was
performed with normal BTK gene results. However, rituximab, a CD20 monoclonal
antibody, is known to cause suppression of CD-20 B cells of variable duration. Thus the
differential between Bruton’s agammaglobulinema versus CVID was shown to be CVID.
Rituximab therapy may have complicated a clear correlation between the cause of the
findings of his B cell and immunoglobulins levels. This case increases awareness of the
correlation between CVID and an increased risk of malignancy. Further, patients should
be evaluated for a history concerning for immune deficiency when presenting with NHL.
If the history is suggestive of immune deficiency, patients should undergo workup prior
to treatment of NHL given that immune testing results could be complicated by prior
treatment with rituximab.
An atypical cause of cholecystitis
Acalculous cholecystitis represents a minority of all cases of acute cholecystitis, and
many of these cases develop in patients who are critically ill. However, in this case it
presented in a previously healthy individual. The patient is an 18-year-old Guatemalan
girl with no personal or family history of biliary disease who had recently returned to
Minnesota after spending her summer vacation in her home village. Nine days after her
arrival in Minnesota she developed fever, headache, myalgias and sore throat. At the
same time she also began having intermittent nausea and non-bloody, non-bilious
emesis and noted a transient rash on her abdomen that looked like “bug bites.” She
felt better after several days of symptoms and then began having many small, loose
stools without blood or mucus and worsening epigastric and right upper quadrant
abdominal pain. She presented to a clinic, where her exam was remarkable for normal
vital signs and mild diffuse abdominal pain. Laboratory testing performed at that visit
was notable for mild leukopenia with neutrophil predominance, mild
thrombocytopenia, moderately elevated liver enzymes and a negative rapid strep test.
Blood culture was also sent and grew Gram-negative rods within 10 hours. Because of
the positive blood cultures, the patient was sent to the emergency room and admitted
for further management. On admission she had normal vital signs but was noted to
have a new 2/6 systolic murmur at LUSB as well as more pronounced epigastric and
RUQ tenderness. Laboratory analysis revealed the previously described abnormalities
as well as mild anemia, markedly elevated alkaline phosphatase and mildly elevated
total bilirubin. Abdominal ultrasound was performed and revealed gallbladder sludge,
wall thickening to 8mm, and a normal common bile duct. She was evaluated by the
surgical team, who did not think cholecystectomy was indicated, and the patient
improved with antibiotic therapy alone. The organisms from the blood were ultimately
identified as Salmonella O Group D, which includes Salmonella typhi. Although
acalculous cholecystitis is often found in patients who are seriously ill, it has also been
described as a complication of infections with several bacterial species including
Salmonella, Leptospira, Coxiella and Vibrio. Interestingly, the complication this patient
had--acalculous cholecystitis as a consequence of Salmonella infection-- is rarely
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Luis Lomeli, MD
John E. Eaton
Jasmine Riviere
Marcelin, MD
Stefan Cordes, MD
Dennis Manning, MD
described in adults but is much more common in the pediatric literature. Adults with
acalculous cholecystitis are often referred for cholecystectomy due to the concern for
gangrene and perforation with this form of cholecystitis. However, cases series from
the pediatric literature indicate that medical management without cholecystectomy has
good outcomes. Although there are fewer instances of this presentation in adult
patients, there are case reports suggesting that adults can also be managed nonsurgically
with complete resolution of gallbladder inflammation on follow-up imaging.
The Not So Benign Hepatic Hemangioma
Liver hemangiomas are the most common benign tumors of the liver, with an estimated
prevalence of 5-20% (1). When hemangiomas grow to more than 5 cm in size, they are
called giant hemangiomas. More rarely, a condition called hemangiomatosis may occur
when there is involvement of innumerable hemangiomas. Little is known about the
etiology, prevalence, and natural history of hemangiomatosis. We report a patient with
a giant hemangioma and hepatic hemangiomatosis complicated by hemorrhagic shock
and compression of the inferior vena cava (IVC). A 60 year old female presented to an
outside hospital due to abdominal distension, fatigue, and lower extremity edema.
Physical exam was significant for tachycardia, hepatomegaly, ascites, and lower
extremity edema extending to her groin. Labs showed a new normocytic anemia
(hemoglobin of 8.2). An abdominal paracentesis collected 2 liters of bloody fluid. CT
scan of the abdomen was positive for hepatomegaly with one large (5 cm) hemangioma
and innumerable smaller hemangiomas. The patient underwent angiography, which
confirmed the diagnosis. She subsequently received a coil embolization of her right
hepatic artery. Initially, her symptoms improved. One month later, she developed
recurrent abdominal distension and severe lower extremity edema. A repeat CT scan
showed a newly developed compression of the inferior vena cava and medial renal
veins (Picture 1). Venogram then confirmed the near total occlusion of the entire IVC
with infrarenal thrombosis (Picture 2). She had recurrent intra-abdominal hemorrhage
and a hepatic angiogram with coil embolization was repeated (Picture 3). Unfortunately
she was not a candidate for a liver transplantation or IVC stent. During her
hospitalization she acutely decompensated requiring cardiopulmonary resuscitation
and intubation. Ultimately, comfort care measures were instituted and the patient
expired. The natural history of hemangiomatosis is poorly understood and there are
no current guidelines on its treatment. This case illustrates the potentially severe
complications of giant hepatic hemangiomas and hemangiomatosis, which include mass
effect, Kasabach-Merritt syndrome, hypothyroidism, high output cardiac failure,
hemorrhage, and death. In contrast to our patient, most hemangiomas are
asymptomatic and have a low risk of morbidity and mortality. They most often occur in
women in between their third and fifth decade. Most common symptoms include right
upper quadrant pain and discomfort. However, care must be taken to fully evaluate the
patient with hepatic hemangiomas as other causes of abdominal pain have been found
in up to 50% of cases. While the preferred treatment is surgical enucleation, these
options may not be feasible in patients with multiple hemangiomas or
hemangiomatosis. As in our patient, coil embolization by interventional radiology can
be considered, and treatment should be considered in symptomatic patients. Continued
research is needed to better understand appropriate management of
hemangiomatosis.
Acute disseminated histoplasmosis after adalimumab (Humira) therapy for psoriatic
arthritis
Introduction: Disseminated histoplasmosis is a serious complication of tumor necrosis
factor alpha (TNF-α) antagonist therapy used for management of various
autoimmune disorders. Serious infections in moderately to severely
immunocompromised patients may have atypical presentations, leading to delay in
diagnosis. Patients on biologic agents with systemic complaints should be evaluated for
granulomatous infections and treated promptly to avoid adverse events. Case
Description: A 60 year old male from Iowa with a 14 year history of psoriatic arthritis
treated with adalimumab (Humira) and methotrexate was admitted to a local hospital
for evaluation of fever and headache. Two weeks before admission he experienced
generalized malaise, myalgias, recurrent evening chills, rigors and fevers up to 102ºF,
followed by a disabling headache and photophobia. He received supportive IV fluids for
a diagnosis of non-specific viral illness. He was discharged, and subsequently re-
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Anya Jamrozy, MD
hospitalized after developing a non-productive cough, nausea and vomiting. Workup
revealed peripheral lymphocytosis, diffuse bibasilar pulmonary infiltrates and periportal
lymphadenopathy on abdominal computerized tomography (CT) scan. Acyclovir,
doxycycline and ceftriaxone were initiated without improvement and he was
transferred to our facility for further evaluation. On arrival the patient was afebrile, in
hypoxic respiratory distress (respiratory rate of 20/min and SpO2 80% on 4L nasal
cannula) with abdominal tenderness and worsened headache. Adalimumab and
methotrexate were discontinued. A chest CT scan demonstrated numerous bilateral
upper lobe patchy opacities with prominent hilar and mediastinal lymphadenopathy.
Right upper quadrant ultrasound revealed calculous cholecystitis and repeat lumbar
puncture revealed 10 lymphocytes without organisms. Bronchoalveolar lavage revealed
yeast and voriconazole was added. Later, Histoplasma serology and culture were
positive and voriconazole was switched to amphotericin B. His fever, cough and hypoxia
subsequently resolved, with improvement of his headache. He was discharged on longterm
itraconazole. Three months later, repeat chest CT scan revealed near complete
resolution the infiltrates. He was advised not to restart adalimumab but consider
methotrexate or prednisone for psoriatic arthritis flares. Discussion: Psoriatic
arthritis occurs in approximately 30% of patients diagnosed with psoriasis. Early
management strategies were limited to methotrexate, but more recently include TNF-
α antagonists. Granulomatous infections are a risk with use of TNF-α
antagonists, most frequently infliximab and etanercept. Adalimumab is the only biologic
agent approved for monotherapy, but patients like ours on combination therapy have
greater risk of infection. This case highlights the importance of recognizing the risk of
serious fungal infection in patients on less-commonly associated biologic agents and the
significant benefit of early initiation of antifungal therapy.
Brugada Syndrome Unmasked by Hypercalcemia Secondary to Primary
Hyperparathyroidism
Brugada syndrome (BS) is a rare cardiac abnormality, with high risk for ventricular
arrhythmias and sudden cardiac death. Diagnostic criteria currently include EKG
changes in conjunction with an episode of ventricular fibrillation or ventricular
tachycardia, family history of sudden cardiac death, unexplained syncope or similar EKG
changes in family members (Wilde, et al). Medications, such as flecainide, have been
used diagnostically to unmask BS EKG changes and other medications and conditions,
including hypercalcemia, have also been shown to provoke BS EKG changes. A 47-
year-old man, with a history of hyperlipidemia, presented for evaluation of syncope and
described a one month history of palpitations. The initial EKG showed normal sinus
rhythm (NSR), short QT (QT 320 / QTc 359) and ST elevation (V2-V6). Serial EKGs in the
ED showed classic “coved” ST elevation in precordial leads (V1-V3), consistent with BS.
Admission vital signs were within normal limits and serial cardiac enzymes were normal.
Serum studies showed an elevated Ca at 12.3 mg/dL (Mg 1.9 meq/L, K 4.6 meq/L). CXR
was normal and TTE demonstrated an EF of 55%. CT coronary angiogram showed no
coronary artery disease. Electrophysiology study showed induced crista terminalis
tachycardia and ablation was unsuccessful due to proximity of the phrenic nerve; ICD
was placed without complication. Hypercalcemia workup was consistent with primary
hyperparathyroidism (Ca12.3, PTH 265, PO4 2.6) and the patient received hydration, IV
pamidronate and cinacalcet 30 mg BID with calcium level dropping from 12.3 to 11.2.
US thyroid detected two nodules posterior to the right lobe of the thyroid gland,
suspicious for parathyroid adenomas. Radionucleotide parathyroid scan did not identify
a parathyroid adenoma. Right inferior parathyroidectomy was performed during a
subsequent hospitalization, with appropriate fall in PTH intraoperatively. Pathology
was consistent with parathyroid adenoma. Subsequent PCR DNA sequencing for
cardiac sodium channel gene mutation (SCN5A) was without abnormalities and family
screening cardiac evaluations for Brugada Syndrome were negative. A mutation in the
SCN5A gene, which sequences for the alpha subunit of the cardiac sodium channel, has
been associated with BS but was only found in 18-30% of cases (Priori, Et al). This
channel mutation results in decreased sodium inflow and subsequently decreased
duration of normal action potentials, proposed as the etiology of ventricular
arrhythmia. Additional genetic abnormalities, including calcium channel mutations,
have been associated with BS EKG changes and short QT (Antzelevitch et al). Drugs that
have been shown to induce Brugada-like EKG changes include cardiac sodium and
calcium channel blockers, beta blockers, tricyclics, SSRIs and cocaine intoxication;
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Adam Foss, MD
Christopher Martin, MD
although medication-induced EKG changes and clinical significance remains
controversial. In this case, hypercalcemia is the proposed provoking factor for BS EKG
changes, as primary hyperparathyroidism was diagnosed concurrently with Brugada
Syndrome.
Unfolding the Diagnosis
Introduction: The differential diagnosis of progressive neurodegenerative disorders is
extensive. When evaluating these disorders it is important to rule out reversible causes
and to consider rare disorders in the differential. Diagnostic algorithms, when
available, should be used. Case: A 73-year-old gentleman presented with progressive
gait discoordination, ataxia, speech disturbances, and cognitive decline. He had been
discharged to a nursing home 11 days prior to admission with a diagnosis of failure to
thrive. The patient was reportedly ‘normal’ and independent with activities of daily
living two months prior to admission. His past medical history included HIV, bipolar
disorder, cognitive disorder NOS, essential tremor, prostate cancer, obstructive sleep
apnea, and chronic kidney disease. HAART therapy was discontinued by the patient one
month prior to admission, but he continued to take risperidone, fluoxetine, tamsulosin,
and simvastatin. His vital signs were notable for systolic hypertension. Neurologic
examination showed a somnolent patient who could follow one-step commands and
was oriented to person only. Pronounced bradylalia was noted. Bilateral upper
extremity ataxia and broad-based gait with titubation were observed but strength was
normal. He was also noted to have an exaggerated startle response with myoclonic like
jerks. Routine laboratory studies were within normal limits and CD4 count was 203. A
lumbar puncture revealed pleocytosis with 24 nucleated cells per cubic mm (100%
lymphocytes), elevated protein, negative gram stain, negative cultures, negative viral
serologies (HSV, VZV, JC Virus, EBV, CMV, Cryptococcus), and a protein 14-3-3 of 1.6
ng/mL (Normal < 1.5). Brain MRI revealed abnormal signal in the left posterior cortex
‘consistent with encephalitis’ with ‘prion disease or HIV encephalitis… less likely, given
the distribution.’ EEG revealed focal slowing over the left temporal and parietal regions
without ‘periodic complex discharges… to suggest prion disease.’ Cancer screening was
up-to-date. MRI findings were consistent with neither CNS lymphoma nor progressive
multifocal leukoencephalopathy (PML). Despite a mild elevation in the 14-3-3 protein,
MRI and EEG findings were not consistent with classic CJD. CSF pleocytosis and
elevated protein findings occur in less than 1% of CJD cases. A diagnosis of viral
encephalitis was made superimposed on possible underlying HIV dementia. The
patient’s condition rapidly deteriorated and he died three weeks later. Autopsy
revealed spongiform changes consistent with Creutzfeldt-Jakob disease, a prion
disease. In this case, a very rare cause of neurological decline was discovered on
autopsy in a patient with underlying medical conditions that broaden the differential
diagnosis for neurological decline. Eliminating rare diagnoses prematurely due to a
non-classic presentation is a diagnostic error that can be avoided by using diagnostic
algorithms.
Thrombotic thrombocytopenic purpura with associated posterior reversible
encephalopathy syndrome in a 16 yr old female; a rare clinical presentation.
Thrombotic thrombocytopenic purpura is a relatively rare condition with the classic
pentad of microangiopathic hemolytic anemia, thrombocytopenia, fever, renal failure,
and neurologic symptoms. This case is a 16-year-old female with end stage renal
disease secondary to lupus nephritis who presented with new onset seizures, anemia
with hemoglobin of 7.9 g/dL, and thrombocytopenia with platelets of 49,000. Lactate
dehydrogenase was elevated at 2189 U/L with haptoglobin less than 6 mg/dL. A
peripheral smear was done showing schistocytes Due to new onset seizures, patient
underwent an MRI of the brain showing findings consistent with posterior reversible
encephalopathy syndrome. This case is a rare presentation of thrombotic
thrombocytopenic purpura and is confounded by the patient having associated
posterior reversible encephalopathy syndrome. A review to the approach to patients
with suspected thrombotic thrombocytopenic purpura will be discussed.
A salty affect, lower extremity swelling, ecchymoses, purpura and anemia map a
diagnosis of vitamin C deficiency.
Introduction: Scurvy is a disease that has been repeatedly forgotten and rediscovered
throughout recorded history. It presents with a constellation of symptoms including
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Adam May, MD
Lori Blauwet, MD
malaise, lethargy, edema, bleeding of the gums, purpura, ecchymoses and corkscrew
hairs w/ hemorrhagic halo. Case Presentation: A 61 year old male without significant
past medical history was admitted to the medicine service with lower extremity
purpura and ecchymoses chiefly on the posterior thigh/buttocks and right leg swelling.
Social history was significant for being unmarried, legal blindness and a 200 pack-year
smoking history. The patient was easily irritable and frequently requested to leave the
hospital. Basic labs were remarkable only for anemia-HgB 7.4. A lower extremity
ultrasound was performed showing a questionable non-occlusive DVT of the right
popliteal vein. This presentation prompted a significant hematologic, oncologic and
rheumatologic work up, including pan-imaging, which did not yield significant findings.
Throughout a seven-day hospital course, the patient’s physical exam and anemia
completely resolved. Despite an alarming presentation, the patient was deemed fit for
close follow-up as an outpatient with the Heme-Onc and Medicine clinics. On they day
of discharge, a dermatology consult was requested for skin biopsy. Corkscrew hairs
with hemorrhagic halos were noted, and an ascorbic acid level was ordered which came
back abnormally low. Upon this news, nursing staff reported there had been repeated
requests for orange juice throughout the hospitalization. Further discussion with the
patient revealed he consumed only simple canned meats; never fresh fruits or
vegetables. He was discharged on oral vitamin C supplementation along with
Coumadin therapy and has been closely followed for the past 3 months without further
sequalae. Discussion: Scurvy presents similarly to more serious disorders such as deep
venous thrombosis, rheumatologic diseases and malignancies. Monotonous/peculiar
diets, smoking, mal-absorption syndromes and patients on hemodialysis should raise
suspicion for vitamin C deficiency. Early diagnostic consideration can significantly lower
medical costs and work-up. Overall 14% of males and 10% of females are vitamin C
deficient according to a NAHE survey and elderly men living alone appear to be most at
risk. Despite this, there have been only 57 deaths due to scurvy in the US reported
between 1979 and 2005 according to the CDC.
Asymptomatic Bicuspid Aortic Valve: The Ejection Click and Murmur that Got Away
Congenital bicuspid aortic valve is the most common congenital heart disease,
occurring in 1% to 2% of the population. More than 1/3 of patients with this anomaly
will eventually develop serious complications including valvular dysfunction, heart
failure, or aortopathy. Given this risk, patients with asymptomatic disease must be
identified and closely followed with noninvasive cardiac imaging. A 51-year-old male
with a past medical history of aortic coarctation (excision and re-anastomosis in 1968),
documented childhood ejection click and murmur, and avoidance of medical care for
greater than 30 years presented with a chief complaint of shortness of breath. His
symptoms began more than one year prior with a mild decline in exercise capacity. Five
to six weeks prior to presentation, he developed progressive dyspnea at rest,
orthopnea, paroxysmal nocturnal dyspnea, and marked bilateral leg swelling. He
presented to our Emergency Department, where he was found to be in respiratory
distress with diminished oxygen saturation, signs of volume overload, ECG with marked
amplitude in his lateral leads, elevated BNP, and chest x-ray remarkable for bilateral
infiltrates and widened mediastinum. Aggressive diuresis was initiated and he was
admitted to a Cardiology service. Cardiac exam was notable for diminished and delayed
carotid upstrokes, laterally displaced PMI, soft S2, harsh 3/6 late-peaking systolic
crescendo-decrescendo murmur loudest at the base with radiation to both carotids and
a soft, high-pitched early diastolic decrescendo murmur loudest at the left sternal
border. Transthoracic echocardiography revealed a bicuspid aortic valve with severe
regurgitation and stenosis. Other echocardiographic findings included left ventricular
chamber enlargement, global myocardial hypokinesis, reduced left ventricular ejection
fraction (33%) and an enlarged aortic root and ascending aortic aneurysm. Chest CT
angiogram confirmed the ascending aortic aneurysm (60 mm) with extension into the
distal ascending aorta. The patient was subsequently evaluated by the Cardiovascular
Surgery service. He then underwent successful replacement of his aortic valve, aortic
root, and ascending aorta with a mechanical composite valve-graft conduit. Following
surgery, he had persistent left ventricular enlargement with diminished ejection
fraction. Long-term heart failure therapies were initiated and regular follow-up in the
Heart Failure clinic was arranged. This case illustrates the potential long-term
consequences of an initially asymptomatic bicuspid aortic valve. Patients with this
congenital anomaly have a propensity for eventual critical valve stenosis, valvular
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Eric Nelsen, MD
Abinash Virk
Anthony Accurso, MD
Daniel DeSimone, M.D.,
Robert Lohr, M.D., John
Eaton, M.D., David
Katzka, M.D., Taofic
Mounajjed, M.
insufficiency, irreversible heart failure, and aortopathy. The AHA/ACA guidelines
recommend serial transthoracic echocardiograms at 1 or 2 year intervals depending on
the patient’s cardiac and aortic root anatomy.
Sudden Onset Blindness with Sphenoid Sinusitis
INTRODUCTION Isolated sphenoid sinusitis can have a wide range of presentation.
Presentations can range anywhere from headache to complete blindness. We present
an unusual case of an invasive sphenoid fungal sinusitis presenting as sudden onset
blindness. CASE A 64 year old female with past medical history of transfusion
dependent pure red cell aplasia, secondary hemochromatosis, ITP on steroids, chronic
ulcer on her left leg and poorly controlled DM type 2 presented to an outside facility
with worsening of a 2 month history of waxing and waning altered mental status. On
admission at the outside facility, she was found to have disorientation with no focal
signs and leukocytosis of 18.4. She was started on vancomycin and meropenem. CT of
head was negative for acute process; however there was evidence of sphenoid mucosal
thickening. CT of her leg showed an ulcer with soft tissue inflammation without
osteomyelitis. On hospital day two she developed complete right eye paralysis and
blindness. MRI of head and orbits showed possible pseudotumor of the orbit and
evidence of sphenoid sinusitis. Outside hospital was concerned for Tolosa-Hunt
syndrome, thus steroids were increased. Due to continued clinical deterioration she
was transferred to our facility. On admission to our hospital she was unable to
participate in the majority of examination because of altered mental status. On
physical exam, her left pupil was round and reactive without conjunctival icterus or
pallor. Her right pupil was 4 mm fixed and not reactive to light. There was mild
periorbital swelling without erythema involving the eyelid. She had complete ptosis of
right eye. Infectious disease was consulted because of concern for an invasive fungal
infection. Liposomal amphotericin B was started at 5 mg/kg the evening of admission.
ENT emergently took her for sphenoid debridement. Surgical findings reported an
invasive fungal ball which was debrided, cultures and pathology were sent. Given her
risk factors for mucormycosis, AmBisome was increased to 10 mg/kg and parenteral
caspofungin with nasal amphotericin irrigations were started. Cultures and pathology
confirmed Aspergillus fumigatus. AmBisome was lowered to 5 mg/kg due to renal
impairment. The patient continued deteriorating and was eventually changed to
comfort-care. She expired on hospital day 5. CONCLUSION Isolated sphenoid sinusitis is
rare because of decreased nasal airflow due to the anatomic location of the sphenoid
sinuses. Symptoms usually include headache, retro-orbital pain, diplopia,
exophthalmos and rarely blindness. Our patient’s history of hemochromatosis, chronic
steroid use and uncontrolled DM were high risk factors for invasive fungal infections
including aspergillosis and mucormycosis. Invasive fungal infections should be
considered early in the differential diagnosis in immunocompromised patients with
isolated sphenoid sinusitis and new onset blindness. This disease is associated with high
mortality. They should be managed urgently with antifungals and surgery.
Opportunistic Esophageal Infection in a Patient with HIV and Adequate CD4 Count
Case Presentation: A 48-year-old, HIV-positive female presented to General Internal
Medicine clinic with chief complaint of epigastric pain. The patient was referred to the
Internal Medicine service from the emergency department after presenting to the ED
two days prior with similar symptoms. During the patient''s visit to the General Internal
Medicine clinic, physicians were concerned for a gastric or esophageal ulcer, and the
patient was referred for endoscopy. The differential diagnosis included esophagitis
secondary to opportunistic infections such as Candida, CMV, and HSV. However, given
that the patient’s most recent CD4+ T-cell count was 640 cells/µL, the team felt that
opportunistic infection was fairly unlikely. Endoscopy revealed a large ulcer at the
gastro-esophageal junction with 75% of the circumference involved. Numerous
scattered ulcers with exudates were noted between the GE junction and the midesophagus.
In the proximal esophagus, multiple white exudates were noted to be
visually consistent with Candida esophagitis. Biopsies and staining reviewed by the
Pathology department were positive for HSV esophagitis. The patient was started on
treatment for Candida esophagitis with fluconazole, loading dose of 400 mg and 200 mg
per day thereafter for fourteen days. A 28-day course of omeprazole was also
recommended. Within a few days, the patient reported tremendous improvement in
her symptoms. The Infectious Disease department felt that the patient’s esophageal
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Calla Brown, MD
Michael Aylward, MD
disease was not a progression of her HIV. However, the ID service was concerned that
the patient’s presentation may have been precipitated by a reaction between intraarticular
triamcinolone injections and ritonavir/atazanavir. ID believed that the HIV
medications may have decreased the clearance of triamcinolone and contributed to her
immunodeficiency. The ID service recommended continuation of the fluconazole and
omeprazole but did not recommend anti-viral treatment of the HSV. Because the
patient had a repeat CD4+ T-cell count of 745 cells/µL and was experiencing rapid
clinical improvement, the ID service believed that the patient’s immune-system was
adequate for neutralization of the HSV infection without medical intervention.
Discussion: CD4+ T-cell monitoring is the standard of care for patients with HIV, and a
commonly-accepted goal CD4+ T-cell count is >500 cells/µL. Esophagitis due to
Candida, HSV, or CMV infection is normally considered to be an AIDS-defining illness.
Patients are at especially increased risk of such opportunistic esophageal infections
when CD4+ T-cell counts are

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Axel Grothey, MD
two weeks of right upper quadrant abdominal pain, low-grade fevers, nausea and
vomiting five weeks after undergoing laparoscopic appendectomy. She had a past
medical history significant for three previous pregnancies resulting in cesarean section
deliveries, and left upper and lower extremity injuries secondary to torture six years
ago; she had lived in the United States for eighteen months. On physical examination
she was tachycardic, mildly hypotensive, and had significant abdominal distension and
severe tenderness to light palpation in the RUQ area, with no guarding or rigidity.
Cardiovascular, pulmonary, and bimanual examination were otherwise non-remarkable,
and she was not jaundiced. Urine pregnancy test was negative and urinalysis was
unremarkable. A non-contrast CT scan obtained in the emergency department,
compared with pre-operative CT imaging, demonstrated a new 4.3 by 2.5 cm ovalshaped
cystic-appearing liver lesion in the right lobe, suggestive of abscess. She was
discharged with instructions to follow-up with general surgery. She presented again to
our clinic one week later. As she had further clinical deterioration as evidenced by
increasing pain and anorexia, in addition to a physical examination that was consistent
with her previous aforementioned examination, she was admitted to the university
hospital internal medicine service for further workup and evaluation. Laboratory
evaluation on admission was significant for mild anemia, a mildly elevated alkaline
phosphotase, and a lack of leukocytosis. Repeat CT imaging showed persistence of the
liver lesion and a right-sided pleural effusion. She underwent CT-guided core liver
biopsy, and culture grew Bacteroides fragilis and light growth of an anaerobic gramnegative
bacillus that could not be typed due to failure to thrive. She was discharged
home on an oral antibiotic regimen of metronidazole 500 mg four times daily and
ciprofloxacin 500 mg twice daily, to be continued for fourteen days. She began
treatment five and a half weeks from the onset of symptoms, with resolution of her
fevers but with mild persistent abdominal pain. Liver abscess is a known complication
of appendectomy, and may be more common in laparoscopic appendectomy as
compared to open procedures. Presentation can vary from insidious onset of clinical
symptoms, including fever, abdominal pain, and vomiting, to fulminate hepatic failure
and sepsis. However, her presentation was complicated by delays in diagnosis and
effective treatment. She had many risk factors for delayed definitive treatment,
including limited English-language ability, her status as a refugee secondary to torture,
and lack of medical insurance. Although delay of treatment did not result in a
catastrophic outcome in this case, delayed treatment of a liver abscess could lead to
rupture of the abscess, sepsis, or death.
Delirium presenting as the only clinical manifestation of adrenal insufficiency
Case A 62 year old male with metastatic melanoma was admitted for pain crisis and
fevers. On hospital day 5, he developed a prolonged, hypokinetic delirium characterized
by agitation, lethargy, and hallucinations. Physical exam was unremarkable. His
creatinine was 6.0 mg/dL (baseline 1.0 mg/dL). His calcium was mildly elevated at 11.0
mg/dL. TSH was 10.1 with normal thyroxine. Blood and urine cultures at admission
were positive for E. coli, but all additional cultures were negative. Neuroimaging of the
head was negative for acute pathology or metastatic lesions. A biopsy of the
malignancy did not reveal superinfection. He continued to spike fevers on broad
spectrum antibiotics without clinical improvement. Interrogation of his intrathecal pain
pump revealed normal function. He was started on thyroid replacement. His renal
failure resolved without intervention. His delirium persisted and discussions were
initiated with family regarding transitioning to comfort cares. However, less common
etiologies were considered, and he was screened for adrenal insufficiency. A morning
cortisol level was depressed at 1.0 mcg/dL. ACTH was low at 6.8 pg/mL. Cosyntropin
stimulation test was consistent with adrenal insufficiency. He was started on
hydrocortisone and his delirium resolved. Discussion Delirium is an epidemic in
hospitalized patients. Not only is it common in the hospitalized patient, it is associated
with adverse outcomes and increased healthcare costs. Unfortunately, up to one-third
of patients will not experience resolution of their delirium at the time of discharge.1,2
Psychiatric manifestations of adrenal insufficiency have been described for some time.3
However, adrenal insufficiency manifesting purely as delirium has not been described
outside of rare case reports.4,5 While this patient’s adrenal insufficiency was likely
secondary to ipilimumab, a novel monoclonal antibody used in the treatment of
melanoma, given the prevalence of glucocorticoid therapy in hospitalized patients,
screening for adrenal insufficiency should be considered in any patient with persistent
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Rachael Krob, MD
delirium. References 1. Siddiqi, N, House, AO, Holmes, JD. Occurrence and outcome of
delirium in medical in-patients: a systematic literature review. Age and Ageing 2006; 35:
350–364 2. Cole, MG, Ciampi, A, Belzile, E, Zhong, L. Persistent delirium in older hospital
patients: a systematic review of frequency and prognosis. Age and Ageing 2009; 38: 19–
26 3. Hoyle, L, Kramer, SI. The psychiatric manifestations of endocrine disease.
Advances in internal medicine. 1984; 29: 413-415 4. Fang, VS, Jaspan, JB. Delirium and
neuromuscular symptoms in an elderly man with isolated corticotroph-deficiency
syndrome completely reversed with glucocorticoid replacement. J Clin Endocr and
Metab. 1989; 69: 1073-1077. 5. Pavlovic, A, Sivakumar, V. Hypoadrenalism presenting
as a range of mental disorders. BMJ Case Reports. 2011; 1-4
Diagnosis of Histoplasmosis Mediastinitis in Patient with Chief Complaint of Severe
Headache
Introduction: Histoplasmosis is the most prevalent endemic mycosis in the United
States, routinely seen in the Mississippi and Ohio River Valleys. It is an infection that is
usually asymptomatic, only occasionally resulting in clinical significance. However,
failure to recognize this disease in patients with unclear underlying inflammatory
process can lead to significant morbidity, especially when immunosuppressive therapy
is being considered. Case Presentation: 66 year old male with past medical history
significant for vertebral basilar insufficiency, chronic kidney disease, renal artery
stenosis status post stenting, presented with a debilitating headache. Patient also
complained of a mild, non-productive cough with some associated chest pain of one
week in duration. His outpatient medication list was significant for clopidogrel and
warfarin. On exam, he was afebrile and vitals were stable. He was neurologically intact
with no palpable pulsating temporal artery, and lungs were clear to auscultate.
Laboratory evaluation was significant for WBC of 22K with 81% PMN. Head CT was
negative for acute pathology; subsequent MRI was significant for chronic infarctions. A
CXR was negative for acute infiltrates. Pan culture, including blood culture and urine
culture were negative. For the persisting severe headache, patient was treated with
valproate acid and opioids for possible diagnosis of status migrainosus, with little
improvement. Concern for CNS vasculitis led to workup that revealed elevated ESR (75
mm/hr) and CRP (26.4 mg/dL), and normal ANA, P-ANCA and C-ANCA; a non-specific
variant of CNS vasculitis was considered. With persistent mild cough, a chest CT was
obtained to rule out occult infectious etiologies prior to initiating empiric steroids. This
revealed severe gas mediastinitis, and patient was immediately started empiric
antibiotics. Despite treatment, patient’s leukocytosis and cough persisted; his headache
did improve with valproate acid. A fungal culture obtained prior to patient’s discharge
was positive for Histoplasmosis IgG and 1:8 complement-fixation. Patient was put on 5
months therapy of Itraconazole and this subsequently resolved his respiratory
symptoms and leukocytosis. A follow up chest CT showed complete resolution of
mediastintis. Discussion: Histoplasmosis mediastinitis is not a common diagnosis to
arrive at, especially in a patient with very minimal respiratory symptoms. When empiric
treatment for a non-infectious inflammatory process is considered, it is essential to rule
out all possible underlying infectious etiologies, as delay in diagnosis compounded by
immunosuppressive therapy could be catastrophic and lead to significant morbidity and
mortality.
A Case of IRIS in a young man with AIDS and multiple co-infections
Patient Case: ET is a 24yo Filipino male with a recent past medical history of
hospitalization for left-sided neck mass. He was diagnosed with AIDS, with a CD4+ T cell
count of 6 cells/mm3 and viral load of 676,000 copies/mL. He was also found to have
scrofula, pulmonary tuberculosis (MTb), Pneumocystis jirovecii pneumonia (PCP), and
Giardia lamblia infection. He was initially started on standard quadruple therapy for
MTb including isoniazid (INH), rifampin, ethambutol, and pyrazinamide (PZA). Prior to
admission he had intermittently taken levofloxacin for treatment of the neck swelling.
Additionally, he was initiated on trimethoprim-sulfamethoxazole (TMP-SMZ) for PCP
treatment, metronidazole for Giardia treatment, and azithromycin for Mycobacterium
avium-intracellulare (MAI) prophylaxis. Two weeks after initiation of MTb treatment,
antiretroviral therapy (ART) was begun with emtricitabine, tenofovir, and raltegravir.
The raltegravir dose was adjusted for rifampin interaction. Ten days after discharge, 16
days after starting ART, he presented to the HIV-clinic with recurrence of fever,
increased pain and size of scrofula, tachycardia, and recurrence of diarrhea. He was
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Taiwo Ngwa, MD
Mark A. Sandefur,
Joshua R. Menke and
Mark L. Wieland
Krishna Patel, MD
Kyle McKenzie, MD,
Larry Baddour, MD
readmitted and, after thorough evaluation for other cause of worsening symptoms,
diagnosed with immune reconstitution inflammatory syndrome (IRIS). He was found to
have Bowenoid papulosis of the penis and an oral herpes simplex virus ulcer, which
were treated with cryotherapy. Repeat viral load was 5320 copies/mL. CT neck showed
reduction in cervical adenitis size. Results from MTb cultures from previous admission
resulted, revealing isolated isoniazid resistance. Isoniazid was discontinued, with
continuation of the other three anti-Tb medication as well as prior to admission ART.
Seven days after readmission, high-dose prednisone was initiated for treatment of IRIS,
as the patient continued to have high fevers. He showed substantial clinical
improvement and was discharged about a month after readmission. Conclusion: This
case exemplifies the risks and benefits of early initiation of ART in patients with
profound immunosuppression. In patients with HIV with substantial
immunosuppression, early initiation of ART is key to preventing increased morbidity
and mortality. However, early initiation of ART has been correlated with an increased
incidence of IRIS, likely due to lack of suppression of a newly fortified CD4+ population
in the presence of opportunistic infection (OI) antigens. When patients recently started
on ART present with worsening symptoms, it is important for the general internist to
rule out etiologies other than IRIS, including drug reaction, bacterial superinfection,
drug resistance, and inadequate treatment due to nonadherence or insufficient
absorption. While identification of IRIS is important, it is rarely fatal. Thus, despite the
risk of IRIS with ART initiation, the mortality reduction from early ART is currently
thought to outweigh the risks.
Calciphylaxis in a Patient with Normal Kidney and Parathyroid Function
A 77-year-old female presented to our institution with painful skin lesions of her
abdomen and lower extremities. Symptoms began with tender swelling on the left side
of her abdomen that progressed in severity and distribution over six months. There
were no associated symptoms and review of systems was unremarkable. There were
no recent medication changes or environmental exposures. Past medical history
included type-2 diabetes, atrial fibrillation (on warfarin), and peptic ulcer disease.
Physical examination revealed an obese female in significant pain with movement of
affected areas. Skin exam showed subcutaneous firm induration throughout her lower
abdomen and lower extremities. There was a 4 x 8 cm necrotic ulcer across the lower
abdomen and a 4 x 6 cm ulcer on the right posterior calf with overlying eschar and
surrounding retiform purpura. The remainder of the exam was unremarkable, including
normal lower extremity pulses. Laboratory studies were remarkable for a normal
creatinine, slightly below normal calcium and phosphorus levels and a normal
parathyroid hormone level. A serologic vasculitis workup, including MPO and PR3, was
negative. Abdominal x-ray did not show calcifications of skin. Tibial/fibular x-rays
revealed serpentine calcifications in a predominantly vascular distribution. CT of the
chest/abdomen/pelvis and serologic paraneoplastic panel did not suggest associated
malignancy. Excisional biopsy of her left medial thigh lesion revealed subcutaneous
vascular calcifications consistent with calciphylaxis. Warfarin was discontinued, and
treatment was initiated with intravenous bisphosphonates and hyperbaric oxygen. The
patient did not improve, treatment was discontinued, and palliative care was
intensified. Calciphylaxis is a rare and devastating condition manifested by medial
calcification of the arterioles that leads to ischemia and subcutaneous necrosis. It is
seen almost exclusively in patients with end-stage renal disease who are hemodialysis
dependent or who have recently received a renal transplant. Among patients without
kidney disease, primary hyperparathyroidism is the most consistent risk factor. In this
case, we describe the much less common manifestation of calciphylaxis in a patient
with normal kidney and parathyroid function. There are many treatments for
calciphylaxis described in case series, none of which consistently impact the grim
prognosis.
Know that Nocardia Can Cause Noxious Effects
Nocardia species have the ability to cause localized or systemic disease. Nocardia is
typically regarded as an opportunistic infection; however, one-third of patients are
immunocompetant. Therefore a thorough history, physical examination, and broad
differential are necessary for diagnosis. A seventy-six-year-old female from
Minnesota, who winters in Arizona, presented for evaluation of fatigue, intermittent
fevers, increasing dyspnea, and nonproductive cough which began nine months ago
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Juliana Perez Botero,
MD
Aditya Devalapalli, Elie F
Berbari
with decreased energy and fatigue. An extensive work up was performed and was
significant for an elevated ESR and CRP, positive p-ANCA, and negative evaluation for
coccidiomycosis. Given her symptoms and work up, there was concern for a vasculitic
process. Prednisone 20 mg daily was initiated and was titrated to 30 mg daily without
Pneumocystis prophylaxis. Her symptoms improved with systemic steroids, but when
decreased, returned. Her clinical course was complicated by normocytic anemia and
acute kidney injury thought to be secondary to vasculitis. Pertinent medical history
included atrial fibrillation, diabetes mellitus, polymyalgia rheumatica, obstructive sleep
apnea, tobacco use, and sulfa allergy. At the time of evaluation she was febrile and
tachycardic. Physical examination was notable for an ill appearing female, irregularly
irregular rhythm, normal lung examination, a 2 cm mobile anterior cervical lymph node,
swelling in left parotid gland, and a soft tissue mass in her right deltoid. Chest x-ray
showed scattered nodular infiltrates in the right mid lung with focal consolidation in the
right upper lobe (RUL). Differential diagnosis was less concerning for vasculitis and
more concerning for infectious or malignant etiology. A chest CT was obtained and
confirmed a large mass in the RUL with multiple bilateral pulmonary nodules and
mediastinal lymphadenopathy. PET CT demonstrated FDG uptake in the RUL mass,
several lung nodules, mediastinal lymph nodes, and a right deltoid mass. She had no
known trauma to the arm and was up to date on cancer screenings. The deltoid mass
was aspirated and bronchoscopy was performed. Gram stain demonstrated gram
positive branching bacilli, and cultures later confirmed the presence of Nocardia
farcinica. She was initially treated with imipenem, linezolid, and minocycline given her
sulfa allergy. A graded sulfa challenge was initiated, tolerated, and she was discharged
on trimethoprim-sulfamethoxazole (TMP-SMX), imipenem, and moxifloxacin with
outpatient follow up. Several factors make the diagnosis of Nocardia difficult including
lack of specific clinical and radiographic characteristics, slow growth of cultures,
availability of molecular tests, and overgrowth of other microorganisms. Lack of clinical
trials complicates optimal treatment and duration of therapy; however, there is some in
vitro data to support individualized therapy for complicated infection dependent on the
immune status of the patient. Sulfonamide, especially TMP-SMX, is considered the drug
of choice. Additionally, when TMP-SMX is given as Pneumocystis prophylaxis, it
decreases the risk of nocardial infection.
EBV POSITIVE PRIMARY CNS LYMPHOMA IN A PATIENT WITH AUTOIMMUNE
CEREBELLAR ATAXIA TREATED WITH CELLCEPT
Epstein Barr virus (EBV) associated lymphoproliferative disorders are a known
complication of chronic immunosuppression. This complication occurs in organ
transplant recipients or HIV infected patients. We describe a case of EBV positive B cell
lymphoma in a non-transplant immunosuppressed patient receiving mofetil
mycophenolate (MM) therapy. A 64 year old female with a history of autoimmune
cerebellar ataxia diagnosed in 2004, presented on September 2012 with a 7 month
history of nausea, vomiting and diplopia. She had been receiving MM therapy since
2005, occasionally requiring steroid pulses to treat exacerbations. Brain MRI performed
4 months prior to her presentation revealed small vessel degenerative changes.
Cerebrospinal fluid (CSF) analysis revealed 149 cells/mcL with 96% lymphocytes. Fungal
smear of CSF revealed the presence of yeast. Blood and CSF cultures, fungal antigens
and serology including cryptococcus were all negative. She received a course of
Liposomal amphotericinB and 5-fluorocytosine followed by fluconazole for presumed
cryptococcal meningitis. Simultaneously, the dose of MM was reduced. In the ensuing 3
months, her nausea and diplopia worsened and she developed bilateral occipital
headache and increased ataxia. A repeat MRI performed during the current admission,
revealed multiple nodular enhancing bilateral frontotemporal lesions. CSF examination
showed 17 total nucleated cells/mcL with a predominance of lymphocytes and normal
flow cytometry; CSF fungal antigens (cryptococcus, histoplasma), and PCR (toxoplasma,
HSV and JC virus) were negative. The patient’s CSF EBV PCR was positive; raising the
suspicion of EBV associated lymphoproliferative disorder. PET-CT showed FDG avidity of
the new CNS lesions. Stereotactic brain biopsy was performed. Histopathologic
examination confirmed the presence of EBV positive B-cell lymphoproliferative
disorder. She was started on chemotherapy with gradual symptom resolution. Epstein
Barr virus (EBV) associated primary CNS lymphoma is uncommon in non-transplant
recipients and HIV negative patients. MM is used as a steroid sparring agent in
autoimmune diseases due to its T cell predominant inhibitory effect. Intrinsic to its
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Kara Roberts, MD
Peter Lund, MD
Nadeem Khan, MD
mechanism of action and as illustrated by this case, there may be an increased
predisposition in patients receiving long term therapy. This diagnosis should be
suspected in immunosuppressed patients with neurologic symptoms and ring
enhancing lesions on neuroimaging studies.
Renal Cell Carcinoma Complicated by Pulmonary Tumor Embolism
HPI A 53 y.o. man without significant past medical history presented to his PCP with
abdominal fullness, constipation and a second episode of hematuria. Abdominal CT
showed a large right renal mass that extended into his IVC, consistent with renal cell
carcinoma. Nephrectomy was scheduled a week later. Prior to the nephrectomy, he
experienced sudden onset of severe chest pain and shortness of breath. His wife found
him essentially unresponsive and activated EMS. CT PE study showed a large saddle
pulmonary embolus located at the main pulmonary artery bifurcation. He was
hemodynamically unstable and required intubation and pressor support. Interventional
radiology attempted catheter directed thrombolytics and mechanical thrombectomy,
but determined that the embolus was not clot, but was instead a tumor embolus. TEE
showed significant right-sided strain and a small PFO with a right to left shunt. He went
emergently to the operating room and underwent an open pulmonary embolectomy.
Hemodynamics improved and he was weaned off pressors and extubated. Off sedation,
he was noted to have persistent decreased LOC. Head CT showed an evolving, several
day old infarct in the area of inferior branch of the left MCA, with some degree of
associated edema and shift. This was felt to be due to paradoxical tumor embolus from
his renal cell carcinoma, through the PFO due to high right-sided pressures and the
right-to-left shunt. Following a brief stay in acute rehab, he was readmitted to undergo
radical nephrectomy and embolectomy. While on the operating room table, he
experienced cardiac arrest and was pronounced dead after attempted resuscitation.
Discussion Renal cell carcinoma (RCC) has an incidence of about 60,000 cases annually
in the United States. It accounts for about 2% of adult cancers worldwide. The classic
triad of RCC includes flank pain, hematuria and abdominal mass. This triad is present in
only about 15% of cases. Vascular invasion is fairly common in RCC, with tumor
embolus occurring in 4-10% of cases. Presence of tumor extending into the renal vein
and beyond tends to carry a poor prognosis. Prognosis can be determined based on
how far into the IVC the tumor extends. When pieces of tumor embolize to the
pulmonary vasculature, it presents clinically identical to pulmonary thrombo-embolus.
Both situations present equally with dyspnea, pleuritic or non-pleuritic chest pain and
increased heart rate and respiratory rate. An additional risk to these is patients is the
presence of a persistent PFO . Once the tumor embolizes to the lungs, right-sided heart
pressure can increase dramatically such that a right-to-left shunt can occur through a
PFO and increase the risk of arterial embolization and ischemic stroke.
250 lbs of weight loss with a pill.
Introduction: Obesity is an epidemic in modern day and age. With its ever increasing
rates and impacts on the already burdened health care systems, it makes the role of
physicians even more important in preventing and if possible treating reversible causes
of obesity. Ascites is a common complication of end stage liver disease. Description:
35 years old gentlemen with past medical history of erbs palsy, obesity with a BMI of >
60 and a direct inguinal hernia, who presented with a dull constant right sided non
radiating chest pain for a week, after he had a mechanical fall down while climbing
stairs. During evaluation, he was found to have considerable ascites. He did have a
moderate drinking history of alcohol, but denied having any other stigmata of liver
disease. Initial paracentesis ( 9 liters) showed bloody ascites which was exudative (
protein > 4.5 gm ) and with a SAAG of < 1.1. based on age and low risk factors
evaluated Autoimmune ( AMA, ANA, anti-trypsin, Iron studies, ceruplasmin ), infectious
( TB, hepatitis A, B, C, HIV, CMV, Fungal and bacterial ), environmental ( alcohol, drugs,
medications ), metabolic ( Thyroid, endocrine, renal, hepatic ), Malignancy (omental,
Liver, bone, testicular, lung, abdominal and lymphoma ) work up was done in detail
which was all negative. He underwent liver biopsy which did not reveal any signs of
cirrhosis or liver disease. He underwent cardiac catheterization which revealed normal
right sided filling pressures. he was started on Aldosterone antagonist and diuretic that
helped him diurese > 3 Liters daily. He was discharged with close follow up and weekly
BMP labs for prevention of over diuresis. His Renal functions remained with in normal
limits. His weights continued to decrease from 520 lbs to 400 on discharge and then to
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Ryan Koene, MD
Erin Lorence, MD
Tarek Hamieh
330 and 250 in 2 months period. He was seen by urology as outpatient and finally had
laparoscopic repair of hernia which surgeons had been previously declining due to his
obesity. He has been following up in primary clinic regularly and feels considerable
more energetic since losing 250 lbs of extra weight. Discussion: Ascites is a common
problem. Common causes include cirrhosis ( 81%), cancer ( 10%), heart failure ( 3%), TB
( 2%), dialysis ( 2 %), pancreatic disease ( 1 %), others ( 1%). It is important to realize
that it could be a reversible cause, if common causes and illnesses are ruled out. He
underwent numerous investigational procedures and all the etiologies were ruled out
one at a time. Conclusion: Ascites is a troublesome and late stage disease, most
often associated with ESLD. Young patient should be evaluated in detail for better long
term survival and improving quality of life.
Patient with Adrenal Gland Enlargement
A 53-year-old immunocompetent man presented to clinic with persistent nausea,
anorexia, weight loss (~30 kg) and chronic back pain. An X-ray of the back showed
possible vertebral compression fractures, and subsequent CT scan of the lumbar spine
revealed bilateral adrenal gland enlargement (> 5.5 cm). Malignancy was suspected, but
no focal uptake was seen on bone scan. Chest radiograph was also unremarkable. Six
weeks later, a dedicated adrenal CT showed non-enhancing adrenal enlargement. He
was subsequently recognized to have adrenal insufficiency and was started on
hydrocortisone replacement therapy. Eventually, a CT-guided fine needle aspiration
biopsy (FNAB) of the left adrenal gland confirmed histoplasmosis. The diagnosis of
adrenal histoplasmosis was made nearly five months after the initial finding of adrenal
gland enlargement, despite a known history of disseminated histoplasmosis (previously
diagnosed with colonic and laryngeal biopsies). The patient resumed treatment with
itraconazole 200 mg twice daily for one year. Fludrocortisone was started in addition to
hydrocortisone. While adrenal destruction and adrenal hypofunction due to
Histoplasma capsulatum is well described, it remains an uncommon diagnosis. The
radiographic appearance of glands destroyed by histoplasmosis or other infections is
large (> 4.5 cm), while in autoimmune adrenalitis the glands are often reduced in size.
Metastatic (and rarely primary) cancer can also yield adrenal enlargement, but does not
demonstrate a normal morphologic contour as often seen with infection. Due to the
mass-like appearance of adrenal histoplasmosis, it is often misdiagnosed as
tuberculosis, lymphoma, or malignancy. As a result, adrenal hypofunction may be a
cause of death.
HLH: A rare case with a common problem
Hemophagocytic lymphohistiocytosis (HLH) is a severe, potentially fatal condition
characterized by high spiking fevers, hepatosplenomegaly, pancytopenia, coagulopathy
and the presence of phagocytosis of hematopoietic cells on bone marrow biopsy.
Acquired forms are often triggered by infections, malignancies, toxins or rheumatic
diseases. We present a 44 year old Vietnamese male with a past medical history of
hypertension and untreated chronic tophaceous gout who presented to the hospital
with one week of intermittent fevers, abdominal pain, nausea, diarrhea and bleeding
gums. Additional history revealed that he previously took an herbal remedy, likely
containing local mushrooms, for his gout. Physical exam was positive for fever,1/6
systolic ejection murmur, splenomegaly, tophaceous gout on right elbow and knee.
Initial lab values were significant for: pancytopenia elevated ferritin and triglycerides
and initial viral studies were negative. Peripheral smear showed microcytic anemia and
thrombocytopenia. Abdominal/pelvis CT showed moderate splenomegaly without
adenopathy. A bone marrow biopsy showed hemophagocytosis. The diagnosis of HLH
was made. The etiology was initially assumed to be undetected viral infection; however,
toxins or rheumatic disease were also potential causes. He was scheduled to start the
standard chemotherapy/steroid regimen; however this regimen’s efficacy was unknown
if the etiology were toxin or rheumatic disease. Given negative labs for infection and no
specific treatment or antidote for toxins, rheumatology was consulted for possible
tophaceous gout as the etiology of HLH. It was hypothesized that the patient had long
standing tophaceous gout lead to a cytokine storm resulting in HLH. Given that chronic
gout is defined by IL-1 activity, an anti-IL-1 (anakinra) was initiated as treatment.
Additionally, he was treated with high dose steroids. His laboratory values returned to
normal and he was discharged home. This case illustrates the consequence of
considering all etiologies of a particular diagnosis, especially when the treatment
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Megha Prasad, MD
Andreas S. Beutler, MD
Jason Puckett, MD
Luke Hafdal M.D.; Flavia
Rosado M.D.; Maria
Dioverti Prono, M.D.; M.
Rizwan Sohail, M.D.
depends on the cause. Although HLH is rare, the importance of obtaining a
comprehensive history and maintaining a broad differential can be applied to general
internal medicine.
A 48 year old lady with chest pain, tachycardia, shortness of breath and flushing
Case Our patient is a 48 year old lady who presents with 10-15 episodes per day of
flushing , chest pain, shortness of breath and palpitations. Our patient’s past medical
history is significant for breast cancer diagnosed at age 42, for which she has received
bilateral mastectomy, with positive margins and negative lymph nodes, as well as
adjuvant chemotherapy. She had recently been diagnosed with recurrent breast
cancer, and had failed paclitaxel. She was evaluated with an EKG which showed sinus
tachycardia and premature atrial complexes. Transthoracic echocardiography
demonstrated normal left ventricular size and function, no valvular abnormalities, and
possible superior vena caval obstruction. CT Venogram of the chest was performed.
which showed deep venous occlusive thrombus of the left subclavian vein and
nonocclusive thrombus of the left internal jugular vein. No flow was identified in the
brachiocephalic vein. Large anterior chest mass was also identified which was FDG avid
on PET/CT. The patient emergently underwent balloon angiography and stent
placement in the mid-upper superior vena cava. She was then initiated on
anticoagulation and seen by radiation oncology who initiated radiation therapy in an
attempt to shrink our patient’s anterior mediastinal mass. Discussion SVC syndrome is
a life-threatening condition caused by obstruction of the superior vena cava, either by
invasion or external compression, thrombus formation or both. Malignancy is the cause
of 60-85% of cases of SVC syndrome. Our patient presented with nonspecific
symptoms including tachycardia, flushing and dyspnea. Her presenting symptoms,
especially in the setting of her malignancy, prompted echocardiography and then chest
CT which confirmed the diagnosis of superior vena cava syndrome secondary to a
hypermetabolic anterior mediastinal mass. Dyspnea is one of the most common
presenting symptoms of SVC syndrome, however due to the nonspecific nature of this
complaint, the diagnosis of SVC syndrome can often be missed. Patients with SVC
syndrome most commonly report facial swelling and fullness especially when leaning
forward. Our patient did not experience either of these symptoms, rather simply
reported “flushing.” In a patient with a known history of malignancy, one should be
careful not to miss SVC syndrome as a diagnosis, and realize that there can be a wide
spectrum of presenting symptoms. The patient was managed appropriately with
anticoagulation for her thrombus, stent placement, and radiation therapy. Our case
highlights the importance of high suspicion for SVC syndrome in the setting of
malignancy, the need for appropriate imaging to confirm the diagnosis, and the need
for immediate management.
Acute Disseminated Histoplasmosis Associated with Hemophagocytosis
Background We present an interesting case of acute disseminated histoplasmosis in an
immunocompromised patient that was complicated by hemophagocytic syndrome.
Case: A 55-year old female presented to emergency department (ED) with 7-days
history of increasing fatigue and worsening dyspnea over the last 24 hours associated
with non-productive cough. She had long-standing history of myasthenia gravis and she
was on immunosuppressive therapy with Mycophenolate mofetil for two years. She
was not on other medications. She did not report fever or chills at initial presentation.
Patient was tachycardic (heart rate in the 120’s) and hypoxic (SaO2 in the low 90’s on 2L
of supplemental O2) in ED. Physical examination demonstrated cervical
lymphadenopathy but was otherwise unremarkable. Her labs were remarkable for
pancytopenia (hemoglobin 8.8g/dl, leukocytes 2,500 per µl, and platelets 43,000 per
µl). Liver function tests were normal and her renal function was unchanged from
baseline. Computer tomography of the chest, abdomen and pelvis demonstrated
bilateral ground glass infiltrates and diffuse lymphadenopathy. She was admitted to
the critical care unit where she was intubated for respiratory distress. A bronchoscopy
was performed and airways were normal. Initial bronchoalveolar lavage (BAL) fluid was
positive for Aspergillus antigen and she was empirically started on Voriconazole. The
diffuse lymphadenopathy paired with pancytopenia raised concerns of a malignancy
and a bone marrow biopsy was performed that demonstrated diffuse involvement with
Histoplasma and evidence of hemophagocytosis. At that time, the BAL PCR for
Histoplasma capsulatum was reported strongly positive. Therefore, patient was
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Evaluating Hypertension and Hypokalemia
Hypertension and hypokalemia are commonly encountered patient scenarios. When
present in the same patient, one must consider if they are linked by common
pathophysiology. A thirty-three-year-old male with no past medical history
presented to the emergency department with a two-day history of progressive bilateral
lower extremity weakness. At the time of presentation, he was unable to ambulate
secondary to severe weakness. He was taking no medications. Examination showed a
systolic blood pressure greater than 200 mm Hg, bilateral lower extremity weakness
and hypoactive reflexes. Laboratory evaluation revealed a potassium of 1.3 mEq/L (3.6-
5.2 mEq/L) and bicarbonate of 29 mmol/L (22-29 mmol/L). ECG showed first degree AV
block and anterolateral nonspecific t-wave changes. The differential included:
renovascular disease, primary hyperaldosteronism, Cushing syndrome, and Liddle’s
syndrome. Retroperitoneal ultrasound with Doppler examination of the renal arteries
showed normal kidneys without significant renal artery stenosis. Plasma cortisol was
normal. Urine potassium excretion was 30 mmol/24 hr. Plasma renin activity (PRA)
was suppressed at

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Aaron Graumann, MD
goal of treatment is prevention of systemic embolic events through anticoagulation. If
there is evidence of a possible foci of abnormal electrical activity, radiofrequency
ablation is an option for achieving rhythm control. Case description: 71 year old
woman with a history of DM type 2, HTN, recurrent atrial arrhythmias, PVD, cirrhosis
and OSA. Had a lumpectomy and radiation therapy to her right breast due to breast
cancer. Has been cardioverted multiple times in the past and NSR was temporarily
achieved however converted back into atrial flutter/fib. Given her persistent rhythm
abnormality she was on diltiazem 180 mg for rate control and coumadin for
anticoagulation, remained tachycardic and there was concern for arrhythmia induced
cardiomyopathy. Underwent an EP study on 8/21/2012 which found that her atrial
flutter was originating from the left side of her heart, ablation was not attempted at
that time. After this underwent DC cardioversion with return of NSR. Developed
respiratory distress and was noted to have pulmonary edema in the recovery room
requiring BiPAP. Transthoracic echocardiogram showed decreased left ventricular
ejection fraction of 33% with wall motion abnormalities not consistent with any
coronary artery distribution highly suggestive of non Tako-Tsubo stress
cardiomyopathy. Repeat TTE showed further decrease in her ejection fraction in the
15% to 20% range, with contractile basal segments and wall motion abnormalities now
located in the mid to distal segments of the heart which was consistent with Tako-
Tsubo type stress cardiomyopathy. She was diuresed with intravenous lasix and loaded
with digoxin for rate control and to improve cardiac contractility. She had another DC
cardioversion and was started on amiodarone, coreg, lisinopril and lasix. Coumadin was
also continued for anticoagulation. After this the patient remained in NSR with
improvement of her cardiac function evidenced by a TTE performed on 9/4 with no
evidence of wall motion abnormalities and an EF of 74%. Discussion: Procedural stress
can be a trigger for stress induced cardiomyopathy, DC cardioversion has been
associated with this complication. This can cause a clinical picture consistent with
congestive heart failure and should be managed accordingly. With adequate support it
is possible for cardiac function to return to normal. This case illustrates the importance
of maintaining a high degree of suspicion for this complication after performing a DC
cardioversion and how heart function can be completely recovered with adequate
treatment.
Subtle Symptoms to Comatose: Adult Onset of Inborn Error of Metabolism
Adult presentation of inborn errors of metabolism is associated with significant
morbidity and mortality. Many physicians that care for adults are not familiar with
presentation and management of inborn errors of metabolism. A 38 year old man was
found unconscious at home. He was normal when seen by his wife earlier in the day. He
had been initiated on prednisone for an asthma exacerbation. In the months preceding
presentation, he exhibited episodic altered mentation and decreased level of
consciousness, fatigue, headache and loss of fine motor function of his hands. MRI,
lumbar puncture and EEG were performed but did not reveal an underlying cause. Liver
transaminases were mildly elevated with concurrent mild ammonia elevation. This
prompted a gastroenterology referral, in which steatohepatitis was found on abdominal
CT. These findings, along with a family history of Reye-like syndrome in his brother
prompted a diagnosis of Reye-like syndrome. Upon presentation, there was seizure-like
activity of his upper extremities. He was intubated for airway protection and loaded
with fosphenytoin. His physical exam revealed a nonresponsive male on propofol
sedation with extensor posturing of the upper extremities, no response to pain, and
normal cough and corneal reflexes. Head CT revealed subtle cerebral edema.
Intracranial pressure was initially 18. EEG revealed a diffuse encephalopathy without
eleptiform activity. Laboratory work-up revealed mild elevation in ALT to 49 U/L with
AST normal at 25 U/L. Serum ammonia was markedly elevated at 485 micromol/L.
Dexamethasone and mannitol were initiated for elevated intracranial pressure.
Hemodialysis was instituted for hyperammonemia. 10% dextrose infusion, sodium
phenylacetate and sodium benzoate, and arginine were started due to concern for a
urea cycle disorder. Genetic testing confirmed the diagnosis of ornithine
transcarbamylase, or OTC, deficiency. With the above therapies, the patient’s
laboratory abnormalities slowly returned to normal and after several weeks the
patient’s encephalopathy began clearing. He was discharged to a comprehensive
rehabilitation program after a hospital stay of nearly one month. At that time, it was
unclear if he would be able to live independently due to damage from cerebral edema
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Thomas Maust, MD
David Griffin, MD
Meredith Reisenauer,
MD
Jackson J. Liang, DO,
Henry J. Schultz, MD
MACP
at presentation. This case highlights a common adult presentation of a urea cycle
disorder. The most frequent symptoms are neurological, with episodic altered
mentation or decreased level of consciousness being the most common initial
symptoms or signs. Metabolic stressors leading to presentation are illness, surgery,
pregnancy, change in diet or glucocorticoid use. Internists should consider the diagnosis
of a urea cycle disorder with patients presenting with decreased level of consciousness
and hyperammonemia as well as a patient with mild aminotransferase elevations and
marked ammonia elevations.
Acute goiter enlargement in the setting of stable hypothyroidism
Primary lymphoma of the thyroid, although rare, should always be considered in the
differential diagnosis of patients with thyroid nodules, goiter, and carcinomas. The
rarity of this disease and the resulting prognosis and treatment makes consideration
and early diagnosis important. A 66 year old male with a past medical history of
hyperlipidemia, hypothyroidism, tobacco use, and intractable simple partial epilepsy,
presented to a new primary care physician, with a several week history of a rapid
enlarging anterior neck mass, desiring endocrinology consultation. Upon further
history, the patient reported a known diagnosis of hypothyroidism for approximately
five years from a previous physician. The patient was on a stable dose of levothyroxine
for the past three years with serum thyroid function tests showing euthyroidism. Given
the enlarging neck mass, the aforementioned physician obtained several thyroid
ultrasounds over the course of a couple months showing an enlarged thyroid with both
lobes measuring approximately 9-10 cm in diameter. Subsequent endocrinology
consultation recommended otolaryngology consultation for possible thyroidectomy
with the working diagnosis of a likely multi-site papillary tumor. Prior to the
otolaryngology consultation, the patient reported a further enlargement of the mass
over several days with dysphagia, hoarseness, and difficulty with secretions. CT imaging
obtained at the emergency department showed obstruction of the proximal esophagus
and compression of the trachea. The patient was transferred to a higher level of care
facility and was admitted. Flexible laryngoscopy showed anterior pressure on the
arytenoids with a patent airway. Thyroidectomy was again suggested. Fine needle
aspiration of the thyroid returned positive for malignancy favoring large cell lymphoma
with flow cytometry showing a few small lymphocytes and expression of pan B cell
markers. The patient was discharged with a follow up appointment for an incisional
biopsy. This subsequently returned positive for large B cell lymphoma. A
multidisciplinary conference concluded thyroidectomy was not in the best interest of
the patient, but rather an oncology consultation for likely chemotherapy. Subsequently,
six cycles of R-CHOP was recommended followed by possible involved field radiation.
The patient tolerated the initial chemotherapy with minimal side effects and the size of
the mass decreased by approximately 50 percent after the first cycle and resolution of
his symptoms after the second cycle. The patient is currently tolerating ongoing
chemotherapy with minimal side effects. This case illustrates the rare and intriguing
rapid enlargement of a goiter in the setting of stable hypothyroidism. Given the rarity of
extranodal lymphomas arising from the thyroid and the treatment options it is
important to keep this in the working differential of the individual presenting with
complaints of a rapidly enlarging neck mass. Prompt recognition of this diagnosis is
important with regards to treatment and its possible connections to other well-known
thyroid conditions.
More than Skin Deep: The Importance of Investigating Rashes
Introduction: Dermatologic conditions may be the first manifestation of serious
systemic disease in otherwise asymptomatic individuals. Internists will often treat skin
conditions with topical corticosteroids or antifungals without further investigation and
miss an opportunity to detect treatable diseases early in their progression. Case
Presentation: A healthy 43-year-old African American male presented with a 10-week
history of rash on his left lateral forearm. The lesions started on the skin at the site of a
soft tissue injury from a motor vehicle accident 16 years earlier. He denied any other
complaints. Physical examination revealed violaceous, raised, shiny papular lesions
overlying a soft lipomatous mass on the left lateral forearm. Similar-appearing lesions
were seen in the malar region of the face, sparing the nose, which he had not noticed
until it was brought to his attention. Upon detailed inquiry of the patient’s social
history, he admitted that he had used intravenous heroin until two years prior. His last
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sexual activity was three months ago, after which his female partner told him she
tested positive for an unknown sexually transmitted infection. Due to concern for
possible HIV-related Kaposi’s sarcoma or secondary syphilis, a complete sexually
transmitted infection panel was ordered. Testing for HIV, Hepatitis B, syphilis,
chlamydia, and gonorrhea was negative. However, Hepatitis C Antibody was reactive,
and the reflex quantitative HCV RNA was elevated at 284,000 IU/mL. A presumptive
diagnosis of lichen planus associated with Hepatitis C was confirmed by punch biopsy
and dermatopathology. The patient was educated about the viral illness and informed
of the necessity of informing prior sexual partners of his disease. He subsequently was
seen in hepatology clinic to discuss initiation of antiviral treatment. Discussion: Lichen
planus is an inflammatory dermatologic condition associated with Hepatitis C infection.
Anti-HCV antibodies can be found in 10-40% of patients with lichen planus. Lesions are
classically described as violaceous, polygonal, flat-topped, shiny papules and plaques
with an overlaying white, lace-like pattern known as Wickham’s striae. Lichen planus
may also involve the nails, scalp, and mucosal surfaces. The rash may exhibit the
Koebner Phenomenon, appearing at areas of previous skin trauma, as in our patient.
Lichen planus has also been associated with other systemic diseases such as primary
biliary cirrhosis, dermatomyositis, inflammatory bowel disease, and myasthenia gravis.
This case demonstrates the importance of searching for underlying systemic illnesses in
the presence of cutaneous lesions so that early treatment can be initiated. In this case,
a simple arm rash brought an otherwise asymptomatic patient to seek healthcare
advice which led to the acquisition of a complete social history. This resulted in early
detection of Hepatitis C, education to prevent the spread of disease, and early
treatment to avoid the development of cirrhosis and hepatocellular carcinoma.
When Diamonds are not a Girl’s Best Friend: A Case of Late Onset Primary Hyperoxaluria
When Diamonds are not a Girl’s Best Friend: A Case of Late Onset Primary
Hyperoxaluria Danielle G Haselby, G2, Department of Medicine Hennepin County
Medical Center, Minneapolis, MN Primary hyperoxaluria is a rare autosomal recessive
inborn error of metabolism that typically presents in childhood or adolescence.
However there are case reports of this disease presenting in adulthood. Although this
condition is uncommon, clinical suspicion and early diagnosis are essential in order to
implement treatment to help prevent progression to renal failure and multisystem
organ failure. A 67-year old female with past medical history of breast cancer, IBS,
chronic fatigue, and depression/anxiety presented to clinic with 5-day history of nausea
and vomiting. Patient reported 3 episodes of nausea/vomiting that were non-bilious
and non-bloody, as well as decreased appetite, malaise, and LLQ abdominal pain. She
described the abdominal pain as having a burning quality and had been present for
approximately 1 month. As part of her work-up a basic metabolic panel was checked
which revealed that the patient was in renal failure with a SCr of 9.5 and BUN 93.
Patient was called and advised to report to the ED for further evaluation. The patient
was admitted for further work-up given her acute kidney injury as her SCr was known to
be normal 6 months prior. Patient also reported several months of dry mouth, frothy
urine, increasing fatigue, and 10-15 lbs. weight loss. A renal consult was obtained by
the primary service and a kidney biopsy was performed in addition to serologic work-up
including complements, FANA, ANCA, serum electrophoresis and immunofixation, anti-
GBM Ab, serum free light chains, hepatitis serologies, and HIV Ab. Renal US revealed
increased echogenicity consistent with chronic medical renal disease. The H&E light
microscopy from patient’s biopsy was consistent with oxalate nephropathy. Patient’s
serum oxalate level was elevated from normal at 96 micromol/L (reference range
normal up to 27 micromol/L). Patient was negative for G170R mutation, the most
common mutation of the alanine glyoxylate aminotransferase gene. Given the patient’s
renal dysfunction and uremic symptoms the decision to initiate renal replacement
therapy was made. The patient was discharged home and electively re-admitted 3 days
later for initiation of dialysis after placement of a tunneled permcath. To date, the
patient continues on conventional hemodialysis and has been placed on the kidney
transplant list. This case highlights a rare genetic mutation of the liver that manifested
initially with symptoms consistent with renal failure, which is common with primary
hyperoxaluria. Given primary hyperoxaluria is a rare autosomal recessive disease that
often progresses to ESRD and ultimately systemic oxalosis, accurate diagnosis and
initiation of treatment is essential to prevent progression.
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Joseph Messana, MD
Michael Newman, MD
Oana Dickinson,
Heather Thompson
Buum
Let’s Get to the ‘Heart’ of the Matter
Let’s Get to the ‘Heart’ of the Matter Joseph A. Messana, Resident, Department of
Medicine, University of Minnesota, Minneapolis Minnesota Patient VS is a 58 year old
female who had cirrhosis secondary to hepatitis C, HIV on HAART (abacavir, lamivudine,
raltegravir), and chronic kidney disease stage III. She is on tacrolimus and bactrim for
her liver transplant. Additionally, she has iron overload secondary to previous
transfusions and takes steroids for chronic adrenal insufficiency. She also had a history
of a cryptococcal lung abscess on fluconazole, and a recent history of restless leg
syndrome requiring ropinirole. She presented with weakness, shortness of breath,
vomiting, and worsening diarrhea. Two weeks prior to admission, she complained of
acute dehydration without any abdominal pain, myalgias, nor sweating. Four days after
this began, she had her first phlebotomy for iron overload, exacerbating her weakness.
The patient’s bowel movement frequency increased from 3-4 stools a day to 9 the day
prior to admission. The day of admission she had a mechanical fall without head
trauma. The amount of time she remained on the floor is questionable, as she
developed increasing confusion and altered mental status in recent days. In the
emergency room she had global weakness with 3/5 strength and no pain. Evaluation of
creatinine kinase revealed an elevation to 9,371. She was also found to have a CD4 of
93, and a UTI with a urinalysis revealing a white count of 66. Aggressive fluids of
normal saline were then initiated to treat rhabdomyolysis. Her fluconazole, bactrim,
ropirinole, raltegravir, and tacrolimus were all held. The patient’s CK decreased to
5,661 after 1.5 days, only to increase again to a maximum of 11,895. Despite
hyperphosphatemia, the patient did not have hyperkalemia, which is not expected in
rhabdomyolysis. Since three days of aggressive fluids did not yield a resolution in CK
elevation, further diagnoses were pursued. On closer physical examination, the patient
had rough and cracked lateral aspects of the fingers, peri-ungual changes, and motor
weakness in proximal muscles at the hip girdle and proximal lower extremities. A
diagnosis of dermatomyositis was pursued with anti-JO antibodies and aldolase. An
MRI of her pelvic girdle and hips was performed, revealing edema and no myonecrosis
nor inflammation. This could support a nonspecific myositis, inflammatory etiology, or
infectious etiology. Consideration for a biopsy began, along with a cluster of infectious
labs including adenovirus and toxoplasmosis. Soon, anti-JO antibodies returned
negative, and attention was brought again to her medications. Her lamivudine was
finally discontinued and her creatinine kinase normalized, supporting the final diagnosis
of lamivudine toxicity. This case emphasizes rhabdomyolysis is not always of a simple
etiology like a fall, and that all medications must be scrutinized for potential deleterious
effects.
Postpartum internal jugular vein thrombosis masquerading Lemierre''s syndrome
Hypercoagulability of pregnancy develops in the first trimester. The risk of thrombosis
rises throughout pregnancy and into the postpartum period. After delivery, the
thromboembolic risk is 84-fold greater when compared to non-pregnant women. The
incidence of venous thromboembolism declines quickly after delivery. When the risk
returns to baseline is not clear from current data. A 27 year-old female was referred to
our service describing a one day history of progressive right neck swelling, throat pain
radiating to her right arm, and dysphagia. One month prior, she underwent caesarian
section delivery. Post-surgery she developed an infected intraabdominal hematoma
that was treated with intravenous antibiotics and percutaneous drainage. On physical
exam she was febrile (38.8C), tachycardic, tachypnic and relatively hypotensive
(101/56mmHg). She was not in acute distress. The right anterior and lateral neck areas
were swollen and tender. The range of motion of the neck, mandible and right shoulder
was reduced due to severe pain. The oropharynx was free of erythema, ulcers, and
exudates. The lower abdomen had a healing surgical scar with local tenderness and
subcutaneous induration along the lower midline. Bowel sounds were present. Her past
medical history and pregnancy course were unremarkable. Her right internal jugular
vein had not previously been cannulated. There was no family history of blood clotting
disorders. Laboratory tests revealed leukocytosis and elevated inflammatory markers.
Chest radiography demonstrated a left basilar pleural effusion. Ultrasonography
demonstrated an acute appearing occlusive thrombus extending from the right internal
jugular to the innominate vein, and through the right subclavian, axillary, and basilic
veins to the level of antecubital vein. Lower extremity ultrasonography was negative for
deep venous thrombosis. Blood cultures were negative for growth. Treatment with
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Shaina Rozell, MD
Evan Hardegree, MD1,
Mark Lee1, MD, Mark
Enzler, MD2
George Saffouri, MD
Jessica Geiger M.D., Lisa
Daniels M.D., Erin Holl
M.D., Justin Peacock
Ph.D., Ryan Hurt M.D.
Ph.D.
antibiotics, anticoagulants, and analgesics was started. Over the following days the
patient remained afebrile and experienced improvement of her symptoms. This case
illustrates an unusual presentation of postpartum thrombosis associated with
persistent fevers. Although, the thrombus location is consistent with Lemierre''s
syndrome, the lack of oropharyngeal inflammation makes this diagnosis less likely.
Additionally, the patient never underwent cannulation of her internal jugular, which
makes local infection and locally provoked thrombosis less likely. In this patient,
provoked thrombosis of the internal jugular vein is associated with postpartum
hypercoagulability and infected intraabdominal hematoma. In our patient, postpartum
enoxaparin for subsequent deliveries was recommended. Risk factors such as
preeclamsia, immobility or recent surgery like cesarean delivery in this case, appear to
be important risk factors for postpartum patients. Determining which patients should
receive thromboprophylaxis remains a challenge. Without large randomized trials,
optimal protocols have yet to be developed.
A Pain in the Neck: A Dentist’s Worst Nightmare
Abstract: Here we describe a case of a retired dentist who had Viridans Group
Streptococcus associated Lemierre’s syndrome. Recognition of this constellation of
symptoms may lead to more timely treatment and better outcomes. Case
Presentation: A 75-year-old retired dentist presented to his primary physician with
fever, lethargy, increasing rigors, and left neck pain. Medical history was significant only
for a patent foramen ovale (PFO). Laboratory evaluation revealed a leukocytosis of
16,200, urinalysis revealed 4-10 WBCs and bacteria, and a chest radiograph revealed a 2
cm opacity in the right upper lobe. A CT of the chest was obtained and revealed
multiple masses in the right lung. He was empirically started on Levofloxacin. He
followed up three days later at which time blood cultures revealed growth of Viridans
Group Streptococcus. This constellation of findings in the presence of a PFO raised
concern for endocarditis with septic embolization. His vital signs were normal, with
pertinent physical findings revealing significant tenderness along the left posterior neck
musculature, without nuchal rigidity. Skin examination revealed no signs of distal
embolization. Extensive oral examination revealed no source of infection. A
transesophageal echocardiogram was performed, with no evidence of vegetation. His
neck pain continued to progress and due to concern for abscess, a MRI of the cervical
spine was obtained and revealed a left posterior myositis and a left external jugular vein
thrombus, which was confirmed by ultrasonography. The clinical scenario led to the
diagnosis of septic thrombophlebitis of the left external jugular vein, or Lemierre’s
syndrome, with septic emboli to the lungs secondary to Viridans Group Streptococcus.
He was started on a 4 week course of Ceftriaxone and made considerable
improvement. Discussion: Jugular vein suppurative thrombophlebitis (Lemierre
syndrome) is a rare form of septic thrombophlebitis with a reported incidence of one in
1 million people. Symptoms are variable but Lemierre syndrome should be suspected
in patients presenting with antecedent pharyngitis, pulmonary emboli and persistent
fevers despite antibiotics. The interval between the preceding pharyngitis and the onset
of thrombophlebitis is usually less than one week, although it can occur up to 2 weeks
later. The most commonly isolated organism is Fusobacterium necrophorum (81.7% of
cases), although other isolated organisms include Fusobacterium nucleatum, Eikenella
corrodens, Bacteroides species and anaerobic streptococci. As part of normal oral flora,
these organisms only become pathogenic during impaired host defenses, allowing for
invasion of the pharyngeal space and surrounding tissues. Following primary infection,
septic thrombophlebitis of the jugular vein develops from local invasion via peritonsillar
vasculature or lymphatics . Septic emboli may occur, most commonly affecting the
lungs (79.8%), joints (16.5%) and rarely the CNS. Timely diagnosis is essential, as
mortality ranges from 4% to 18%, even with prompt medical treatment.
Muscle Atrophy: Looks can be Deceiving
Introduction: Muscle Atrophy is frequently suspected in elderly, frail, and/or
malnourished individuals rather than those who are obese and well-fed. Serum
creatinine is known to be an unreliable surrogate of renal function in those afflicted
with muscle atrophy. Case Presentation: A 66 year old foreign man flew in for inpatient
treatment of chronic medical problems including extensive peripheral arterial disease
and type 2 diabetes mellitus. Initial exam was primarily notable for his significant
obesity with a BMI of 48 kg/m2. After stabilization on a general medicine ward, initial
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labs showed a normal electrolyte panel except for an elevated potassium of 5.7 with a
serum creatinine of 0.6, and poor diabetes control with a Hgb A1c of 8.8% and point of
care blood glucoses in the 300s. The hyperkalemia was attributed to his long overseas
travel without administration of his normal insulin. Later in his hospitalization, a serum
potassium of 6.2 was noted on morning labs. After intervention with IV insulin,
albuterol, and Kayexalate, the potassium returned to normal, and the stat
electrocardiogram (ECG) was unchanged and normal from admission. Looking back at
his labs, it was noted that the patient had run in the high-normal range of potassium
since presenting to the hospital. Brief review of his labs during his hospital stay showed
a persistently normal and unchanged serum creatinine, lowering suspicion of diabetic
nephropathy and prompting a discontinuation of his ACE inhibitor. Further history
revealed that he had been hydraulic-chair-bound for about seven years due to morbid
obesity. A serum cystatin c was drawn given the patient’s many years of being chairbound
with the thought of long-term muscle atrophy making the creatinine an
inaccurate glomerular filtration rate (GFR) surrogate. This value came back at 2.78,
corresponding to a GFR of 22.4. Further cystatin c values decreased to a steady level
corresponding with stage 3 chronic kidney disease (CKD), likely from diabetic
nephropathy. The patient was eventually discharged via a medical flight for ongoing
care in his home country after his hyperkalemia resolved and remained within normal
limits. Discussion: This case demonstrates the need to maintain a high clinical suspicion
for muscle atrophy in those who are bed or chair bound, regardless of BMI or
nutritional status. This may prompt use of cystatin c as the surrogate for GFR, given the
unreliability of serum creatinine in those with low muscle mass. Recognizing this early
can uncover underlying chronic kidney disease or acute kidney injury, two diagnoses
that can drastically change a patient’s management.
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MD
“Shock or not?” Importance of the physical exam.
Introduction Pericardial tamponade and septic shock are life-threatening emergencies
that can have a similar clinical presentation. This case illustrates the importance of the
physical exam in differentiating the two, in order to make a rapid, life-saving diagnosis.
Case description A 64 year-old female was admitted to the hospital with a 2 week
history of dyspnea on exertion associated with worsening pleuritic chest discomfort.
She also complained of a cough productive of “frothy” sputum. Remainder of review of
systems was positive only for bilateral leg swelling (right greater than left) that
preceded the shortness of breath by one week. On examination, she was tachypneic
and her chest was clear to auscultation bilaterally. Cardiovascular exam revealed an
irregularly irregular rhythm with a normal S1 and S2. The extremity exam was notable
for bilateral, 2+ pitting edema, (right greater than left). Labs revealed a leukocytosis
and elevated lactate, and plain radiograph of the chest demonstrated small bilateral
pleural effusions and a cardiac silhouette that was in the upper limit of normal for size.
Antimicrobial therapy with ceftriaxone and azithromycin was initiated. Over a 12 hour
period, she became increasingly hypotensive, tachycardic, tachypneic, and was
described as “restless.” She was aggressively resuscitated because of the concern for
septic shock. The lower extremity edema prompted evaluation with an echocardiogram
that demonstrated a massive pericardial effusion with echocardiographic signs of
pericardial tamponade. An emergent pericardiocentesis yielded 850mls of bloody
effusion that was sent for cytology. Her vitals improved and stabilized after
pericardiocentesis. Cytology returned positive for lung adenocarcinoma. A chest CT
then revealed a right apical mass with infiltration of the carina and trachea. Given the
advanced stage of the malignancy, the patient opted for hospice after discharge.
Discussion Both septic shock and pericardial tamponade can cause hypotension,
tachycardia and tachypnea; a good physical exam is required to differentiate the two.
Pericardial tamponade is characterized by an elevated jugular venous pressure,
diminished heart sounds, pulsus paradoxus >12 mmHg, kussmaul’s sign, cold clammy
extremities, and increased systemic venous resistance (though the classic signs and
symptoms of tamponade are seen in only 50% of all cases). Septic shock, on the other
hand, is characterized by warm extremities and decreased systemic venous resistance.
A high index of suspicion for tamponade is required to make an accurate diagnosis. This
case also illustrates the importance of considering other differential diagnoses
mimicking septic shock.
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Raghuwansh Sah, MD
Michael Wilson, MD;
Anjali Bhagra, MD
Bradley Salonen, MD
Mark Lee, MD
Prolonged fever with headache and lymphadenopathy
Prolonged fever with headache and lymphadenopathy. Raghuwansh P Sah, MBBS;
Michael Wilson, MD; Anjali Bhagra, MD Department of Internal medicine, Mayo Clinic,
Rochester, MN, USA. Presentation: A 23 year old female presented to her local ED with
severe occipital headache, nausea and fevers. CSF analysis showed normal cell count,
protein and glucose but initial gram stain read was gram positive cocci (cultures turned
out negative later). She was started on IV ceftriaxone and vancomycin, and given 2
doses of oral prednisone. After a brief period of response, her symptoms relapsed and
remained refractory despite 10 days of IV antibiotics. She presented to our facility at
this point with worsening of her symptoms. Rest of her history was unrevealing.
Assessment: On arrival, she was febrile (38.6 C) with stable vitals. Exam revealed thick
neck (BMI of 31 kg/m2) without meningeal signs. Initial tests included pancytopenia,
elevated CRP, LDH and unremarkable CSF. While undergoing further testing, IV
antibiotics were continued with addition of acyclovir. She developed oliguric renal
failure on day 3 with an unremarkable U/A. Extensive infectious and rheumatology
work-up was negative, and antibiotics were stopped. However, her fevers and
headache remained persistent. Diagnosis: MRI of the brain and neck was obtained to
rule out any abscess which revealed massive posterior cervical lymphadenopathy.
Axillary and inguinal lymphadenopathy was seen on CT scan. Excisional biopsy of the
deep cervical lymph node was performed which was negative for malignancy but
revealed histiocytic necrotizing lymphadenitis suggesting a diagnosis of Kikuchi-
Fujimoto Disease. Renal biopsy showed acute tubular necrosis. Management and
follow-up: Her symptoms resolved with supportive treatment by day 6, with
improvement in pancytopenia and renal function. She had two additional self limited
recurrences in the next three months. Currently she remains symptom free.
Discussion: Kikuchi disease is a rare, poorly understood condition affecting young
females. We wish to highlight the diagnostic challenge presented by this condition
which mimics more sinister and common causes such as lymphoma. These cases often
require prolonged, extensive work-up. Excisional lymph node biopsy is required for its
diagnosis. Lymphadenopathy is present in all patients; most commonly cervical nodes
(posterior triangle) are involved. Fever and headache are often present while a third
have rash. Aseptic meningitis is a rare presentation, which was not seen in our patient.
The disease has a benign, self-limited course in most patients with resolution of
symptoms in 1-4 months. Recurrences are rare. Conclusion: Kikuchi Disease is a rare,
benign disease presenting with fever and lymphadenopathy in a young female. Its
diagnosis can be challenging and often requires excisional biopsy while a delay in
diagnosis can lead to unnecessary and potentially harmful treatments as well as
financial and psychologic comorbidity.
The Curious Case of Chronic Swelling
Chronic extremity swelling is a common symptom for which it is often difficult to
diagnose and treat the underlying etiology. Common causes include deep venous
thrombosis (DVT), venous insufficiency, Baker’s cyst, and cellulitis. However, when
swelling continues to persist or causes limb compromise, one must consider malignant
causes such as sarcoma. We present a 49 year old gentleman with a past medical
history significant for unprovoked right leg DVTs (2000 & 2002) and pulmonary
embolism (2002) for which he takes chronic warfarin anticoagulation who presented to
a general internal medicine clinic for evaluation of left lower leg swelling. He reported
that the swelling started gradually around two years and reached it maximum size
within one month. Since that time, the swelling has been stable in size. The only
symptoms over the past two years had been intermittent foot drop and paresthesias
distal to the swelling. Physical examination revealed asymmetry of the lower legs with
the left being larger than the right due to localized swelling of the mid, anterior lower
leg. There were no associated skin changes. On palpation, a firm, discrete mass was
noted anteriorly and laterally without any associated edema. Strength and neurological
testing was grossly intact. An ultrasound with doppler demonstrated a heterogeneous
mass with areas of blood flow within the mass. MRI showed a very heterogeneous,
multilobulated mass within the anterior compartment of the calf that extended from
just below the knee to above the ankle. A core needle biopsy of the mass was
subsequently obtained, and the pathological examination revealed a myxoid/round cell
liposarcoma. FISH studies showed rearrangement of the DDIT3 gene at 12q13, which is
observed in myxoid/round cell liposarcoma. Staging of the sarcoma with a
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Lacy Schwarze, MD
Benjamin Van Vranken,
MD
Peter Lund
chest/abdomen/pelvis CT revealed metastases to the right chest wall, mesentery, left
and right buttocks. Sarcomas are rare tumors in adults that are often asymptomatic and
unrecognized until late in their course. This case highlights the importance of full
evaluation of painless masses that do not improve with time.
When a headache is more than just a migraine.
Introduction: Chronic headaches including migraine are common, but further imaging
should be obtained when there is a change in the typical pattern for a patient. Changes
can represent a more severe underlying process such as subarachnoid hemorrhage or
tumor. Case: Ms. C is a previously healthy, 29 yo female who presented to the ER with
symptoms of a severe bifrontal headache with onset approximately 6 hours prior. The
headache was associated with intractable nausea and vomiting, without photophobia,
and initial severity was typical. On presentation to the ER she was noted to be
neurologically intact, with an otherwise normal exam. Vitals were significant for a blood
pressure of 180/114. Only past medical history was a rare migraine (last 3 years ago)-
usually with photophobia and unilaterally, resolving with rest. No history of tobacco,
ethanol, drugs, or prescription medications. In the ER she was treated with Zofran, IVF,
and Dihydroergotamine. She was diagnosed with migraine, felt better, and was
discharged to home. Three days later she presented to primary care. She noted both
headache severity and nausea had worsened. She described the pain as bilateral, and
noted the pains worsened with bending over and coughing. Pain did not worsen with
lying down. She denied photophobia. Again there was no neurologic deficit, but she
remained hypertensive. Because of atypical presentation imaging was pursued. An MRI
showed a small subarachnoid hemorrhage (SAH) and bilateral MCA aneurysms- one
clearly the source of the sentinel bleed. She was admitted, had one of the aneurysms
emergently coiled, and had later clipping of an additional two. She was discharged on 3
antihypertensives. Discussion: This patient presents with a fairly common issue- severe
headache in a patient with a history of migraine. However, several things about her
case lead to need for further imaging. Particularly, the initial presentation of headache
was atypical for her and worsened with specific positions. Studies have shown that the
yield is only 0.4% in all imaging obtained for patients referred with isolated,
nontraumatic headache. However, imaging is most like to be positive in thunderclap
headaches, headaches radiating to the neck, and temporal headaches in patients >55
yo; or in those with new headache who are immunocompromised, pregnant or have
cancer. Other red flags include: a change in the pattern of headache; new seizures or
headache with systemic illness; personality change; or symptoms that indicate
increased intracranial pressure- worsening in a.m. or with coughing, sneezing, and
straining; or any new abnormal neurological finding. Of note typically is SAH is
suspected CT is the preferred modality- but in a history of chronic headache with a
change MRI is preferred. CT is best in first 6 hours following sentinel bleed.
Nodosum’s Doppleganger, Erythema Induratum: A Case of Diagnostic and Therapeutic
Challenges
Erythema induratum is a form of panniculitis associated with tuberculosis (TB) and
Hepatitis C virus (HCV). This case describes how a specific diagnosis of panniculitis is
made and how it can reveal underlying medical illness. A 75 yo F with a history of HCV
and latent TB presented with painful skin lesions on her right leg. She reported 1 week
of a painful, firm, superficial nodule on her anterior right thigh. Over time, this grew in
size and became quite painful. She also had a smaller nodule just above her right knee
and two smaller nodules on her right calf posteriorly, which had appeared more
recently. She had lost 9 kg in the last 6 months. She had been treated for pneumonia in
the last month, but there was no longstanding history of cough or subjective fevers.
There was no history of hemoptysis, sore throat, arthritis, abdominal pain or diarrhea.
On examination, she was hemodynamically stable and afebrile. On the right lower limb,
an 8 cm, firm, tender, superficial nodule with surrounding erythema and warmth was
noted. Also, a small nodule above right knee and two small tender nodules over calf
were found. There were enlarged inguinal and femoral nodes on the right side. Two
skin biopsies were performed. One was sent for TB PCR, which was negative. This
sample was also sent for mycobacterial, tissue, and fungal cultures, all of which were
negative. The other skin biopsy was sent for histopathology, which demonstrated
dermal mixed granulomatous inflammation extending to subcutaneous fat in a lobular
distribution. Fungal and mycobacterial stains were negative. A CT chest demonstrated
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Rebecca Hanson, MD
Sagar Patel, MD
new nonspecific reticulonodular opacities in the left lower lobe greater than right lower
lobe, which did not clearly indicate reactivation of pulmonary TB. The patient was
discharged to home. The history and physical exam findings were most consistent with
erythema nodosum and erythema induratum. The biopsy favored erythema induratum.
A number of etiologies were considered, including TB, HCV, sarcoidosis, streptococcal
pharyngitis, and histoplasmosis. When caused by TB, it is not unusual for erythema
induratum of Banzin to be negative for mycobacterial culture, PCR and stains. This case
posed a unique challenge in decision making, given that erythema induratum in this
patient was most likely to have been caused by either TB or HCV. Choosing to treat the
HCV could result in worsening of the TB infection, given the immunosuppressive effects
of peg-interferon. The decision was made to start an antimicrobial trial against TB. She
will follow up in Infectious Disease clinic.
Hermetic Headache
Headache is a common symptom that is generally a benign condition. However,
headache in a solid organ transplant recipient can herald a more insidious disease
process and warrants a thorough work-up. A 29-year old man with a history of endstage
renal disease secondary to lupus nephritis, status post living donor transplant 6
months ago presented to an outside hospital with a headache and neck pain. Initial
conservative management failed and his headache persisted. Lumbar puncture was
performed and showed 15 white blood cells with 56% lymphocytes and 11% segmented
cells. Glucose was 54, protein 75. Gram stain was negative. CSF was negative for
numerous pathogens tested. MRI showed increased signal of right hippocampus and
meninges, consistent with meningitis or lupus vasculitis. He was diagnosed with aseptic
meningitis. His headache and neck pain resolved, and was discharged after a 5 day
hospital course. He again presented to the outside hospital 4 days later with recurring
headache. He now reports confusion, fevers, chills, nausea, vomiting, blurry vision and
photophobia. He was transferred to HCMC for further management. Repeat MRI on
admission showed severe mass effect in the right parietotemporal lobe with right uncal
herniation secondary to edema and hemorrhage. The differential was broad, including
opportunistic infections, lupus vasculitis, malignant process, or thrombosis. Expert
consultation was obtained from infectious disease, neurology, neurosurgery and
rheumatology. The intracranial lesion was felt to be too diffuse for a high yield
biopsy. He was started on high dose steroids and broad-spectrum anti-infective
agents. Multiple cultures of serum, urine, sputum, and CSF were unrevealing. The
patient gradually declined, developing altered mental status, left sided weakness,
homonomous hemianopsia, and slurred speech. Repeat head imaging showed stable
mass effect, edema and uncal herniation. On hospital day 28, a brain biopsy was
done. Results showed monomorphic B-cell post transplant lymphoproliferative
disorder. Given his low performance status, he was not a candidate for intensive
chemotherapy and started on rituximab. On hospital day 34, he developed respiratory
distress requiring intubation. On hospital day 35 he developed a dilated left
pupil. Imaging showed worsening uncal hernation and midline shift. Emergent
ventriculostomy was placed. Intracranial pressure was measured, and despite
aggressive induced hypernatremia, hyperventilation, sedation and paralysis, the
pressure ranged from 22-44. On hospital day 41 the family withdrew life support and
the patient died hours later. Headache in a solid organ transplant is a non-specific
symptom with a broad differential diagnosis. Clinicians should have a high suspicion for
post transplant lymphoproliferative disorder, especially in the first two years after
transplantation. Early tissue diagnosis is key for the rapidly progressive and highly fatal
central nervous system PTLD. Vasogenic edema, hemorrhage and the diffuse character
of this patients lesion made a swift diagnosis challenging.
Red-herrings: A case of delayed hemolytic transfusion reaction
Sagar S. Patel, M.D., Resident Physician, Department of Medicine, University of
Minnesota, Minneapolis, MN. Delayed hemolytic transfusion reactions occur days to
weeks after a blood transfusion. They are often due to an antibody response after reexposure
to a foreign red blood cell (RBC) antigen that has been previously
encountered. A 66-year-old female with a past medical history of right hip
replacement six years ago presented on post-operative-day ten from a left hip
replacement to the Emergency Department with complaints of dark-brown colored
urine and stool, fever, chills, and malaise. The patient reported receiving a blood
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transfusion for post-operative anemia. Her pain was well controlled and she was
ambulating without difficulty. She reported having daily chills the week prior to
admission, but felt febrile only the day prior to admission. Most concerning to her were
episodes of dark-brown urine and stool, she denied hematochezia or melena, easy
bruising or bleeding. She was on warfarin and aspirin since the surgery and chronically
on doxycycline for rosacea. She also reported consumption of “natural” black licorice
two days prior to admission. Her physical examination included left hip and digital
rectal examinations as well as an assessment for any signs of bleeding and was
unremarkable. SIRS was noted initially in the Emergency Department given her
presentation with hypertension, tachycardia, and fever and there was concern for
possibly infected hardware. She was started on broad-spectrum antibiotics and
intravenous fluids. Her hemoglobin dropped from 10.7 three days prior to admission to
9.3 on presentation. She was noted to have mild renal insufficiency and a urinary
analysis (UA) significant for “blood,” but no red blood cells and only granular casts were
seen. Upon transfer to the floor, rhabdomyolysis was ruled out with a negative CK. This
therefore suggested hemoglobinuria. LDH was found to be elevated at 2703 and
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Pedro J. Caraballo MD.
Eugene Scharf, MD
Miguel Lalama MD,
William Freeman MD
Acute Pancreatitis with a Normal Serum Amylase and Lipase
Introduction: Serum amylase and lipase are highly sensitive and specific markers for
the diagnosis of acute pancreatitis. Using a cut off of three times the upper limit off
normal, amylase has a sensitivity of 72-85% and a negative predictive value of 82-98%.
Lipase, was found to be 55-100% sensitive, with negative predictive values between 88-
100%. We report on an atypical case of acute pancreatitis in which the serum amylase
and lipase were normal. Case Presentation: A 74 year old male was admitted for fever,
abdominal pain, and confusion of two days of duration. His medical history was
remarkable for prostate cancer with bone metastases and a recent pathological
fracture. One month prior, his medications were changed to bicalutamide, tamoxifen,
and denosumab. His history also included COPD, hypertension, hyperlipidemia and
type 2 diabetes mellitus. He had no history of alcohol abuse, pancreatitis, or hepatitis.
He was tachycardic and hypotensive. Exam revealed bibasilar crackles, abdominal
distention, epigastric tenderness, and mild peripheral edema; it was otherwise
unremarkable. Laboratory tests showed no leukocytosis, hematocrit 25.1, hemoglobin
8.2, CRP 21, lactate 0.8, creatinine 5.85, BUN 88, bicarbonate 19, potassium 5.3, and
calcium 1.09 (reference range 1.10-1.30). Serum ethanol was not detected. Alkaline
phosphatase, LDH, triglycerides, and bilirubin were normal, AST 59 (reference range 10-
40) and ALT 21 (reference range 2-45). Serum amylase was 21 (reference range 20-
100) and lipase was 26 (reference range 7-60). An abdominal ultrasound showed no
evidence of hepatobiliary disease but did show ascites, pancreatic inflammation, and a
peripancreatic fluid collection. Abdominal CT without contrast showed findings similar
to the ultrasound with prominent fat stranding adjacent to the tail of the pancreas. He
was treated with intravenous fluids, broad spectrum antibiotics, and analgesics. His
hospital course was complicated by gram negative sepsis and care was withdrawn three
days after admission. Serum amylase and lipase remained normal on daily
measurements during his admission. Discussion: According to the American College of
Gastroenterology, the diagnosis of acute pancreatitis can be made with two of the
following three features: 1) abdominal pain that is characteristic of acute pancreatitis,
2) serum amylase and/or lipase at least three times the upper limit of normal, and 3)
findings of acute pancreatitis on CT scan. As demonstrated by this case, normal enzyme
levels do not exclude the diagnosis of acute pancreatitis and clinicians should be aware
of atypical presentations. References 1. Banks PA, Freeman ML. Practice guidelines in
acute pancreatitis. Am. J. Gastroenterol. 2006;101(10):2379–2400. 2. Al-Bahrani AZ,
Ammori BJ. Clinical laboratory assessment of acute pancreatitis. Clin. Chim. Acta.
2005;362(1-2):26–48.
To close or not to close? A 40 year old man with a patent foramen ovale now presents
with a new left parietal lobe infarct.
Background The prevalence of patent foramen ovale (PFO) has been shown to be about
25- 30% in the general population. The presence of a PFO has not been associated with
an increased risk in ischemic stroke. However in cryptogenic stroke (roughly 30-40% of
all ischemic strokes) the prevalence of PFO is increased. Clinical trials have not
established the efficacy of PFO closure in secondary stroke prevention. Case report A
40 year old right handed man with a history of right carotid terminus coiling presented
to an outside hospital with a chief complaint of headache and word finding difficulty.
The patient was observed with nonsensical speech, word finding difficulties but intact
comprehension. Physical exam was remarkable for left facial droop, right arm paresis,
and expressive aphasia. He was hypertensive with systolic blood pressure in the range
of 190 mmHg. A head CT was negative for acute infarct, hemorrhage, mass, or edema.
He was airlifted to our center where on arrival his facial droop had improved however
he was misspelling simple words. Routine labs were unremarkable except for a
potassium of 3.4 and creatinine of 1.8 (baseline is 1.5-1.7). Medical history included
hypertension, gout complicated by stage II CKD, hypercholesterolemia and stable
cerebrovascular aneurysms. The patient denies history of migraine, seizure, stroke,
smoking, and illicit drug use. Imaging with MRI with MRA was remarkable for an acute
left parietal lobe infarct at grey white junction and no new cerebrovascular lesion.
Transesophageal echocardiography was remarkable for a PFO with right to left
intracardiac shunt at rest which increased with Valsalva. Echocardiography did not
show intracardiac mass or thrombus. 24 hour Holter monitoring revealed normal sinus
rhythm. Cardiology was consulted to discuss management options. Ultimately the
patient elected for percutaneous closure of his PFO with a Gore Helex atrial septal
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occluder. Post procedure, he was placed on clopidogrel 75mg for 30 days and aspirin
325mg for 6 months. At discharge his neurological deficits had resolved. At one month
follow up repeat echocardiography was negative for intracardiac shunt or device
thrombus. Discussion The clinical management of PFO in the setting of a
cerebrovascular event requires deciding between percutaneous closure or conservative
medical management for secondary stroke prevention. Although multiple nonrandomized
observational studies have shown a benefit to closure, the randomized
controlled clinical trial CLOSURE I found no definite benefit in PFO closure versus
medical management in recurrent ischemic events. Furthermore, the placement of the
closure device has inherent risks including device thrombus, atrial fibrillation and
residual shunt. In the CLOSURE I trial, 5 of the 12 ischemic events in the treatment arm
(PFO closure) were believed to be device related. The management of PFO in
cryptogenic stroke remains controversial.
Did I have a heart attack Dr?
Myocarditis is an insidious disease that usually presents with a wide variety of clinical
features including asymptomatic patients, chest pain and symptomatic fulminant heart
failure.Usually caused by a wide variety of infectious pathogens (virus, bacteria and
fungi).Gold standard diagnosis includes an endomyocardial biopsy however it is a
moderately invasive dx test that is usually not performed.Dallas criteria became the
universal diagnostic tool for histologic diagnosis of myocarditis.However studies have
shown that the criteria probably underestimates the true incidence of myocarditis since
the degree of intraobserver variability is large.Given the lack of widely available, safe
and accurate non-invasive diagnostic tests the true incidence of myocarditis remains a
challenge.CASE DESCRIPTION:Mr. DEJ is a 65 y.o male with PMH including HTN who
presents to the ED with 3 day h/o intermittent substernal chest pressure, SOB, n/v.EKG
with no significant ischemic changes but troponin elevated at admission.Treated as
NSTEMI with heparin load and Plavix.On HD#0 patient became febrile – T max of
39.4.CXR normal, UA clean, no h/o diarrhea or abdominal pain.TTE showed lateral
WMA.Coronary angiogram on HD #1 demonstrated mild CAD but no evidence of
obstructive disease.Cardiac MR on HD# 2 showed normal LV function but no evidence
of WMA. Pt discharged on HD #3 on CAD risk factor modification meds(statin, beta
blocker and antihypertensive.)DISCUSSION:Chest pain currently accounts for 20 – 40 %
of ED admissions.In a sizeable fraction of patients presumed to have ACS, coronary
angiography fails to detect culprit lesions to explain troponin leak and EKG/TTE
findings.However, these patients are still treated as NSTEMIs and are discharged on
secondary preventive therapies.NSTEMI trials with biomarker positivity – frequency of
culprit free angiography ranges between 4.4 – 14%.Yet, elevated troponins in the
absence of significant obstructive CAD should warrant further research for other causes
of myocardial injury.Recent studies suggest that the majority of these patients may
actually have undiagnosed myocarditis; and the prevalence of this disease has
increased, as current troponins assays are ultra sensitive to mild trop leaks.It would be
reasonable to consider the financial cost and potential complications of lifelong CAD
therapy in patients that are mistakenly labeled as having a heart attack. CONCLUSIONS:
-Development of higher sensitivity troponin assays will lead to increase number of pt
with presumed ACS but culprit free angiography. -Introduction of cardiac MR into
management of trop elevations without coronary angiographic culprit CAD has
demonstrated that non coronary causes of acute myocardial injury – particularly
myocarditis are far more prevalent than previously thought. -Can cardiac MR be used to
reassess patients that were diagnosed with NSTEMI after discharge to accurately give
them the diagnosis of “heart attack”? -Can cardiac MR give further important clinical
information that could contribute to further tailoring of post MI medical therapy?
Why you shouldn’t leave your friends face down in the dirt: Management of ARDS
secondary to Blastomycosis Pneumonia
In August, a 21 yo woman with treatment resistant pneumonia was transferred to a
regional hospital for pulmonology consult and management. Her symptoms had
started 2.5 weeks earlier with pleuritic chest pain and fever. She first presented to a
local ED, was diagnosed with pneumonia and treated with ceftriaxone, levofloxacin and
vicodin. Over the following two weeks she had six visits to seek care for worsening
symptoms. She was treated with ceftriaxone, azithromycin, an albuterol inhaler, and
more vicodin for pain. Despite some intermittent improvement in symptoms, she
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continued to have fevers and chills, productive cough, myalgias, and significant left
chest and back pain. A clinic chest x-ray 10 days after initial presentation showed
worsening pneumonia, and she was admitted to the hospital with pain, dyspnea, and
cough. Over the next 3 days, she continued to decompensate and required high flow
oxygen to maintain O2 sats >92% and narcotics for cough and pain. Bronchoscopy was
performed and broad budding yeast was identified in the lavage fluid, later identified as
Blastomyces dermatiditis. Thirty minutes after treatment with liposomal Amphotericin
B was initiated, the patient developed hypotension, dyspnea and hypoxemia requiring
BiPAP. Treatment was changed to oral itraconazole after this presumed allergic
reaction to amphotericin and over the next two days the patient’s respiratory status
declined even further. She was intubated for oxygenation, and treatment with
amphotericin B was resumed with premedication and fluid flushes pre and post
medication. On day 2 of amphotericin treatment, CXR was markedly worse with diffuse
bilateral pulmonary infiltrates, and a diagnosis of ARDS was made. After review of
guidelines and existing literature, corticosteroids treatment was initiated. The patient
continued to require high doses of sedation and close to maximal ventilatory support
but improved with decreased leukocytosis, no fevers, and eventually lessening
ventilator support until extubation on day 9 of treatment with amphotericin and methyl
prednisolone. Despite adequate antifungal treatment, there is a high mortality rate
associated with blastomycosis-associated ARDS. Occurring in

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Anjali Bhagra MBBS
Vanessa Smith, MD
Diana Dean, MD
Prednisone). The patient’s symptoms improved rather quickly, and serial transthoracic
echocardiograms showed significant shrinkage of the tumor, stable pericardial effusion,
and resolution of initial tricuspid valve involvement. Follow-up with oncology and
cardiology is ongoing. This case demonstrates the importance of tissue diagnosis in the
treatment of cardiac tumors as other clinical markers such as presentation and location
cannot provide definitive diagnosis. However, the case also illustrates the importance
of thoughtful discussion amongst clinicians working as a team to advocate for a patient.
Persistence Pays: Disseminated Histoplasmosis in an Immunocompromised Host
Histoplasmosis is usually a self-limited or even asymptomatic disease caused by a
dimorphic fungus found in the soil. Disseminated histoplasmosis commonly presents
with non-specific systemic symptoms that require a high index of suspicion for
diagnosis. A 70 year old male former smoker with seronegative inflammatory arthritis,
on methotrexate and etanercept, hypertension, and hyperlipidemia was admitted to his
local hospital for confusion, weakness, fever, and 1 to 2 months of progressive fatigue.
During the hospitalization, the patient developed acute renal insufficiency, elevated
transaminases, thrombocytopenia and was transferred to our institution for further
management. On presentation the patient was afebrile with a blood pressure of
106/64 and a respiratory rate of 20. On exam he was somnolent, using accessory
muscles during respiration, and had bilateral lower extremity weakness. Initial labs
showed platelets 63, creatinine 1.4 mg/dL, alkaline phosphatase 239, AST 175, and ALT
76. Following admission, the patient became increasingly tachypneic (RR 32) and
developed a new oxygen requirement. A chest CT was negative for pulmonary embolus
but revealed scattered bilateral hilar and mediastinal lymphadenopathy with a 2 x 4 cm
nodular mass along the right middle lobe segmental bronchi. The following day, the
patient became febrile to 38.7º C with a waxing and waning mental status. Further
evaluation with a head CT was negative for acute intracranial findings, and an
abdominal ultrasound was unrevealing for hepatobiliary pathology. A broad infectious
diseases work up was started including tick borne pathogens, viral and fungal
serologies, and urine antigen studies. Histoplasma and blastomyces serology antibodies
were positive but equivocal. Because of the positive findings on chest imaging and to
identify a definitive diagnosis, bronchoscopy with lavage and biopsies was performed.
Lymph node pathology showed necrotizing granulomas highly suggestive of a fungal
etiology, and the patient was started on liposomal amphotericin B. Over the next few
days, the patient decompensated with worsening mental status, fever, and
thrombocytopenia. A bone marrow biopsy was done and showed multiple granulomas
and Grocott methenamine silver stain demonstrated forms morphologically consistent
with histoplasma yeast. Finally, an elevated complement fixation titer and the presence
of an M band on immunodiffusion confirmed the diagnosis of disseminated
histoplasmosis. With continued antifungal therapy, the patient''s clinical condition
improved in addition to his liver function, kidney function, and thrombocytopenia. The
patient was ultimately dismissed after twelve days of hospitalization on oral
itraconazole for one year. Several weeks after dismissal, bronchial lavage cultures grew
histoplasmosis. This case demonstrates the diagnostic challenge of identifying
pathogens in an immunocompromised host and the utility of a multidisciplinary
evaluation. Disseminated histoplasmosis is a fatal disease in weeks to months and a
high index of suspicion is required to recognize the infection and initiate treatment in a
timely manner.
Chin-on-chest deformity: a disabling and preventable complication of osteoporosis
Introduction: Osteoporosis progresses asymptomatically, until complicated by fracture,
deformity, and painful disability. While routine screening is not recommended by the
World Health Organization for men, bone mineral density (BMD) should be obtained in
the setting of a low trauma fracture, radiographic osteopenia, loss of more than 1.5
inches in height, and in those with additional established risk factors (e.g.
hypogonadism). Some groups, such as the National Osteoporosis Foundation,
recommend screening in all men older than 70 years. Case description: A 69 year-old
man presented to Endocrinology clinic for management of osteoporosis. His past
medical history was significant for tobacco and alcohol dependence, and cardiovascular
disease. He had been wheelchair bound (10 years), and disabled secondary to
advanced spinal osteoporosis, complicated by prior vertebral compression and pelvic
fractures. His physical examination disclosed severe cervical kyphosis resulting in a
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Yader Sandoval, Gautam
Shroff
chin-on-chest deformity. There were no findings of increased neck flexor tone, neck
extensor weakness, or cervical myelopathy. His weight at the time of evaluation was
102 kilograms with a height of 178.5 centimeters (body mass index = 32). BMD of the
right femoral neck showed a T-score of -2.6, consistent with osteoporosis. Laboratory
evaluation showed a 25-hydroxyvitamin D level of 41 ng/mL (goal greater than 30),
serum calcium of 9.5 mg/dL (normal: 8.9-10.1), phosphorus of 4 mg/dL (normal: 2.5-
4.5), and alkaline phosphatase of 99 U/L (normal: 45-115). Thyroid stimulating
hormone and serum protein electrophoresis studies were normal. Orthopedic surgical
referral was recommended, and he underwent corrective cervical spine fusion.
Continued osteoporosis management with vitamin D and calcium supplementation,
with the addition of daily Teriparatide injections was recommended. Discussion:
Etiologies of the dropped-head syndrome may be easily distinguished on the basis of
the clinical examination demonstrating floppy (e.g. neck extensor myopathy), forced
(e.g. cervical dystonia), or fixed neck deformities (e.g. cervical spine deformity, as in our
case). Chin-on-chest deformity as a result of excessive cervical kyphosis secondary to
osteoporotic compression fractures is considered rare in modern times. Our patient
had multiple preventable risk factors for osteoporotic fractures, including tobacco and
alcohol dependence. Our case demonstrates the disabling functional consequences of
inadequately treated osteoporosis, and highlights the critical importance of
preventative management.
A Murmur in Pregnancy: Innocent Until Proven Guilty
Introduction The majority of pulmonary stenosis (PS) cases are asymptomatic, isolated
congenital defects with little effect on the probability of survival. Similarly, isolated PS
during pregnancy has minimal overall affect but severe PS has been shown to increase
labor and delivery risk. Consequently, balloon valvotomy may be indicated to reduce
labor and delivery risks. This case presents an asymptomatic pregnant woman with
incidental systolic murmur on exam found to have severe PS. We discuss clinical
indications for treatment and her management course. Case Description A previously
healthy 24 year old female was admitted with a threatened preterm labor at 25 weeks.
She underwent spontaneous vaginal delivery without complications. Postpartum
physical exam revealed the presence of a prominent harsh ejection systolic murmur,
best heard at the left-upper sternal border without associated click. She denied
dyspnea, orthopnea, paroxysmal nocturnal dyspnea, chest pain nor episodes of
cyanosis or squatting episodes. Electrocardiogram showed RVH. TTE revealed PS
(valvular and infundibular components) with systolic gradient of 68 mmHg, along with
RV enlargement and hypertrophy, and a left-to-right atrial shunt consistent with an
ASD. Ultimately her pregnancy and postpartum course were unremarkable. She was
referred to a congenital heart disease center for further evaluation. Discussion
Pulmonic stenosis (PS) is a common defect often found as an incidental isolated
congenital defect with a mild familial occurrence of 2%. Around 90% of cases of PS are
valvular, but it is critical to distinguish from sub- and supravalvular PS as management
may differ. The majority of cases follow a benign course, but severe or symptomatic PS
especially during pregnancy may benefit from balloon valvotomy. The idea behind this
treatment during pregnancy is that the strained right ventricle has an additional
gestational volume that compounds the load on the heart which may increase risk for
labor and delivery complications. It is possible that preterm labor occurred as a
consequence of her heart disease. Clinically suspected PS cases should begin work-up
with an ECG, which often demonstrates evidence of RVH followed by echocardiography
and rarely cardiac catheterization. Cardiac MR or TEE is often necessary to evaluate the
subvalvular region and identify coexisting congenital abnormalities. Conclusions
•Clinicians should differentiate an innocent flow from pathological murmur in
pregnancy. Auscultatory findings and presence of a click bear emphasis. •Isolated PS
requires close evaluation to ensure that coexisting congenital abnormalities are
excluded first. For example, PS is a commonly seen with Tetralogy of Fallot. •Further
management is contingent upon symptoms, severity and anatomical factors that can be
evaluated non-invasively using echocardiography and cardiac MRI. •Severe PS in
pregnancy may have increased risks during labor/delivery requiring balloon valvotomy.
•Isolated valvular PS can be approached by balloon valvotomy but sub-pulmonic
stenosis may necessitate surgical correction.
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Kevin Rank, MD
Sancta St. Cyr, MD
Deirdre Pachman, Tony
Chon
Pneumonia, a new face on an old friend.
INTRODUCTION: Pneumonia, be it hospital or community acquired, is a common
disease that nearly every medical professional will face. However, a casual relationship
with the disease will often allow more severe forms to go undertreated. CASE: A 71
year old woman with COPD presented to the hospital for a fourth admission with the
same complaints of fevers, chills, and a productive cough. During her previous
admissions she had been diagnosed with pneumonia and been treated with various IV
antibiotics, and released home on oral medications, only to return to the hospital with
the same symptoms. On her third admission the patient underwent bronchoscopy with
BAL which grew both Aspergillus and polymicrobial bacteria on cultures. She was seen
by the infectious disease specialists, started on broad spectrum antibiotics and
antifungals, and released home. Less than one week later she returned to the hospital
complaining of weakness and increasing fever, as high as 103 degrees. This being her
fourth admission, and in light of the patient’s continuing fevers despite antimicrobial
therapy, she underwent CT scan for a possible cryptogenic infection. On CT she was
found to have a large, fluid filled bullae, suspicious for bullitis. She was restarted on a
more intensive regimen of her home medication, and discharged. DISCUSSION: This
case illustrates the importance of maintaining vigilance when one is encounters a
common illness. Too often when confronted with a disease which we are comfortable
treating, there can be a tendency to go on "auto-pilot" and not do our due diligence,
and give the patient the thorough investigation needed.
Meth is (near) death: Sepsis and Meningitis from a decorative plant
Meth is (near) death: Sepsis and Meningitis from a decorative plant Kevin Rank,
University of Minnesota Department of Medicine Introduction/Case Description: A 56
year old male from Western Wisconsin was admitted to the ICU with concerns for
intraventricular hemorrhage and rhabdomyolysis. He had a past medical history of polysubstance
abuse and resultant seizure disorder and had been admitted multiple times
in the past for drug overdoses. His urine toxin screen was positive for alcohol and
methamphetamine. He was apparently found on his farm unresponsive and was
intubated and brought in. Collateral history was not available at that time. His vitals
were significant for mild fever, tachycardia and hypertension. His physical exam was
remarkable for sluggish, but responsive pupils and multiple abrasions covering nearly
his entire body, including deep scratches along his spinal column. Labs returned
remarkable for elevated CK, leukocytosis and acute renal failure. His CSF returned with
87 WBC (neutrophilic predominance) and xanthochromasia. Head CT revealed findings
consistent with hemorrhage. The patient was treated for intraventricular hemorrhage,
dehydration and drug withdrawal, but unfortunately continued to decline, becoming
more hypotensive, tachycardic and febrile. Eventually, blood cultures and CSF culture
returned positive for Leclercia adecarboxylata and pantoea agglomerans. Further
history was then obtained from the patient’s significant other and it was ascertained
that patient had been down in a patch of decorative saw grass for approximately 12-18
hours before he was discovered. He was immediately placed on Ceftriaxone and began
to improve steadily. He was ultimately discharged to TCU and now follows regularly in
clinic and continues to do well. He is no longer using meth and has been abstinent from
alcohol as well. Discussion/Learning points: Infection with Leclercia and Pantoea
species is uncommon and generally described in patients with significant underlying
immune compromise. Both organisms have also been described as colonizers of plant
species and aqueous environments. This case emphasizes (1) the importance of
obtaining a thorough clinical history and considering all potential contributing factors to
clinical presentation. It also describes (2) the rare phenomenon of sepsis and meningitis
from Leclercia adecarboxylata and Pantoea agglomerans, with even more rare CSF
involvement. Finally, (3) it is a firm reminder for clinician’s not to fall into the trap of
“early closure,” even when the clinical history and presentation seemingly present a
reasonable explanation for the patient’s symptoms.
All Skin and Stumbles: A case of Guillain Barré syndrome associated with varicella zoster
Case: A 76 year old male with a history of basal cell carcinoma, chronic kidney disease
and coronary artery disease presented to the emergency department after noticing
new tingling in his bilateral upper extremities. A week and a half prior, he began
complaining of a right-sided facial lesion. He was evaluated by dermatology as an
outpatient and was started on cephalexin then minocycline for concern of cellulitis.
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Elfriede Agyemang
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Despite being on antibiotics, his facial lesion became more erythematous and new
lesions appeared on his chest. Punch biopsies of the lesions on his face and chest
returned positive for varicella zoster. He was started on valacyclovir in addition to his
antibiotics. Four days later, he developed upper extremity paresthesias with worsening
facial rash. After presenting to the emergency department, he was admitted to the
general medicine service for concern of facial cellulitis. At presentation he was afebrile
with a normal white blood cell count. Varicella IgM and IgG were positive and HIV was
negative. Dermatology and Infectious diseases were consulted and determined that he
had a mild form of disseminated varicella zoster without any evidence of a
superimposed bacterial infection. His cephalexin and minocycline were stopped and his
dose of valacyclovir was increased to 1000 mg four times daily. The following day
while preparing to be discharged home, he developed new gait instability and
weakness. Neurologic exam demonstrated decreased reflexes in bilateral upper and
lower extremities, broad based gait and symmetric decreased strength in his arms and
legs. The next day, he no longer had triceps, brachioradialis, patellar or Achilles reflexes.
His respiratory status was monitored and remained unaffected. A lumbar puncture
revealed an elevated total protein level of 59 and a corrected nucleated cellularity of
approximately 25-30. CSF testing for Lyme and CMV were negative. Electromyography
showed a subacute, demyelinating, sensorimotor polyradiculoneuropathy consistent
with acute inflammatory demyelinating polyradiculopathy (AIDP). He received 5 days of
intravenous Immunoglobin with resulting arrest of his symptom progression. He then
participated in three weeks of inpatient rehabilitation. Two months after his initial
presentation, he had returned to his neurologic baseline Discussion: Varicella zoster
reactivation is known to cause neurologic complications including post-herpetic
neuralgia, neuropathy and encephalitis. Guillain Barré syndrome (GBS) is a group of
disorders, including AIDP, characterized by symmetric muscle weakness and decreased
to absent deep tendon reflexes. This is rare following herpes zoster infections, occurring
1% of the time compared to other precipitating infections. The latency period from skin
lesions to GBS presentation can be between 2 days and 2 months. In this case, it was 2
weeks. It is unknown if antiviral therapy can prevent the development of GBS, therefore
new neurologic changes associated with varicella zoster should raise concern of GBS.
An Un-“Well” ECG
Certain subtle changes on ECG that go unrecognized by many clinicians could represent
an underlying life-threatening cardiac pathology. We present a case of Wellens
Syndrome, which describes characteristic ECG findings associated with obstruction of
the proximal LAD. Identification is critical for helping prevent the development of an
anterior wall MI and death. A 58-year old woman with no prior cardiac history, a 6-pack
year smoking history, a history of untreated hypertension and hyperlipidemia,
presented to the emergency department with sudden onset substernal chest pain
without radiation. The pain developed suddenly at work where she provides janitorial
services and was initially relieved upon resting for several minutes, but returned upon
resuming activity. The pain was associated with dyspnea, diaphoresis, nausea, and
dizziness. On presentation, she received two sublingual nitroglycerine and aspirin which
resolved the pain entirely. Physical examination was unremarkable. Serial troponins
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anterior wall MI was only 8.5 days. Thus, recognizing the Wellens Syndrome ECG
pattern is critical for preventing the morbidity and mortality associated with an anterior
wall MI.
Sickle Cell Trait: Not Always A Benign Carrier Condition
A 24 year old male was admitted to the hospital with left upper quadrant pain. He had
been seen in the Emergency Department twice in the previous week for nausea,
vomiting and dehydration after binge drinking. CT done on the day of admission
showed heterogenous enhancement of the spleen and hemoperitoneum without free
air. Splenic infarct was suspected and the patient was treated with IV pain medications
and fluids. He was discharged four days later when pain had improved but no cause of
his left upper quadrant pain had been found. Five days later the patient’s left upper
quadrant pain increased and he began to be short of breath so he was readmitted. At
this admission his platelets had risen to 992,000. At this time the patient also had a
repeat abdominal CT which revealed increasing splenomegaly with resolving
hemoperitoneum and a left-sided pleural effusion. Splenic infarct was still on the
differential but infectious or infiltrative process was also highly considered. The patient
had an extensive workup for possible infectious, malignant and hematologic causes of
his splenomegaly. HIV, PPD, Jak2, RPR, bcr abl, FISH (looking for 9:22 translocation),
SPEP/IFE, lupus inhibitor, anticardiolipin ab, beta 2 GP antibody were all negative. CMV
IgG and EBV IgG were positive but both IgM were negative. Right upper quadrant
ultrasound was negative for hepatocellular carcinoma. B12 and folate were within
normal limits. Iron and transferrin were low with high reticulocyte count and ferritin
level. Peripheral smear showed slight normocytic anemia with very rare Howell-Jolly
bodies and marked thrombocytosis with rare atypical platelets. Hemoglobin
electrophoresis was consistent with hemoglobin S trait. The patient also had
thoracentesis showing exudative effusion which was consistent with a reactive pleural
effusion due to the splenic process. On hospital day #6 patient had acute worsening of
his abdominal pain. He had a repeat abdominal CT which was highly suspicious for
splenic abscess but on that same day abdominal ultrasound showed no abscess. On
hospital day #15 the patient was taken to the operating room for splenectomy. The
spleen was severely impacted and difficult to dissect out. Surgical pathology on the
spleen showed splenic infarction with focal areas of sickled red cells. This case
demonstrates a splenic infarct from sickled red cells due to dehydration after binge
drinking in a patient with sickle cell trait. The potential for life-threatening events in
patients with sickle cell trait has been described in the literature and is most often seen
in patients with hypoxia due to high elevation or with concomitant spleen or
hematologic disorders. This case illustrates the importance of keeping in mind the rare
but serious side-effects of this usually benign condition even in previously healthy
patients at sea level.
Hematochezia in a Patient with Metastatic Melanoma
Hematochezia is a common problem most internists encounter on a relatively routine
basis and the differential is usually straightforward. A less common cause of
hematochezia is immune-related colitis secondary to ipilimumab, a drug used to treat
metastatic melanoma. Knowledge of this side effect is important in diagnosing and
appropriately treating patients with hematochezia who take ipilimumab. A 73-year-old
man presented with two weeks of bloody diarrhea, cramping abdominal pain just prior
to stooling, and a 20 pound unintentional weight loss. He denied fevers, chills, nausea,
or vomiting. Pertinent past medical history included metastatic melanoma treated with
ipilimumab and arthritis for which he took NSAIDs. On exam he was afebrile and
hemodynamically stable. His abdomen was soft and nontender and hemorrhoids were
noted. Labs revealed a 3 gram drop in hemoglobin and no leukocytosis. Fecal
leukocytes were present and Clostridium difficile PCR, stool culture, and stool ova and
parasites were negative. Given that stool studies did not show infectious etiology, the
patient was started on high dose intravenous steroids to treat immune-related diarrhea
secondary to recent therapy with ipilimumab. Despite steroids, hematochezia
continued and on hospital day 3 a colonoscopy was done to exclude alternative
diagnoses such as cytomegalovirus. Endoscopy revealed edema, erythema, friability,
and deep ulcerations throughout the entire colon. Biopsies were taken with cold
forceps and microscopic pathology was indeed consistent with immune-related colitis.
Due to continuing hematochezia refractory to steroids, infliximab was given in
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Erin Scanlon, MD
Paul Swieciki, MD
Timothy Call MD
accordance with published guidelines used to treat immune-related adverse events
secondary to ipilimumab therapy. The patient’s symptoms quickly resolved and he was
transitioned to a slow oral steroid taper. Ipilimumab is a monoclonal human IgG1
antibody against CTLA-4 and indirectly mediates T-cell activation and antitumor
immune responses. It has shown to improve overall survival in patients with previously
treated, unresectable stage III or IV melanoma. Unfortunately, adverse effects due to
T-cell proliferation may occur, with diarrhea as the most common. Colonic toxicity may
become severe enough to cause perforation, need for colectomy, and even death.
Treatment consists of high dose intravenous steroids for hematochezia or diarrhea
severe enough to require hospitalization. If symptoms do not improve in 5 to 7 days,
infliximab 5mg/kg can be given once every two weeks until symptoms resolve. It is
important to be aware that immune-related colitis secondary to ipilimumab not only
exists but is a common side effect of the drug. Failure to initiate appropriate therapy
promptly could result in significant morbidity and even mortality.
Possible Cardiac Stent Allergy
Promus Everolimus-Eluting Coronary Stents are contraindicated in patients with known
hypersensitivity to everolimus, cobalt, nickel, chromium, and acrylic and fluoropolymers.
PROMUS stents contain a primer polymer (poly (n-butyl methacrylate) that
functions as an adhesion promoter between the bare metal and drug matrix layer.
While cardiac stent allergy is a rare occurrence, the allergic syndrome can present with
refractory angina, dyspnea, wheezing, facial flushing, and edema. A 51 year old male
with a history of multiple coronary interventions presented to his Cardiologist with
refractory angina despite maximal medical therapy including Prasugrel. Given
progressive “burning chest pain”, he eventually underwent an angiogram with
percutaneous cardiac intervention to the mid left anterior descending to the D4 branch
with a Boston Scientific PROMUS drug eluting stent. One week following placement of
the stent, he was hospitalized for chest pressure, bilateral hand swelling, and facial
flushing. Given his atypical symptom complex with no evidence of recurrent ischemia
on his extensive coronary imaging performed, concern that he could be experiencing a
medication side effect or hypersensitivity syndrome was considered. A non-expanded
Xience stent was taped to the patient’s skin to monitor for a local hypersensitivity
reaction. Thirty minutes following the administration, the patient began to experience
localized erythema around the stent placement, swelling of the bilateral hands, and
pruritis at the stent placement site. He did not experience anaphylactoid features
including hypotension or laryngeal edema, and lab work up was negative for
eosinophilia or lymphocytosis. He was continued on Prasugrel, and treatment was
initiated with a prednisone burst. Because the patient felt better with improvement in
the local reaction, and reduction chest pain, it was thought that he had experienced a
hypersensitivity to the polymer on the stent, not the metal, as the metal is not exposed
in a non-expanded stent. Within a month, however, the burning chest pain and
dyspnea returned, and did not resolve with an additional course of steroids and
antihistamines. A repeat angiogram showed micro-vessel disease that was not
amenable to intervention. He was hospitalized several days following the angiogram for
dyspnea and wheezing. He subsequently had improvement in his symptoms after he
discontinued Prasugrel and changed to Plavix. He no longer had burning chest pain and
had improved exercise tolerance to his baseline of being an avid biker. Although
allergic complications of coronary artery stents are rare, this case is an example of the
utility in a modified patch testing in a potential allergic reaction, and illustrates that
hypersensitivity reactions to medications can present in an atypical fashion.
How Low Can You Go?
Introduction: Drug induced lupus is an uncommon immunologic disorder of unclear
etiology with 15-30,000 new cases identified each year. Commonly implicated drugs
include procainamide, hydralazine, and minocycline. Most common symptoms include
arthralgia, myalgia, and arthritis. Onset is typically abrupt and the basis of therapy is
cessation of the offending agent. Classical laboratory abnormalities include abnormal
ANA and anti-histone antibody levels. Case Description: A 57 y/o Hispanic man,
originally from Guatemala, with a past medical history of adult acne (on minocycline)
presented for evaluation of an acute onset anemia. One month prior to admission, the
patient developed mild left sided abdominal pain which was treated symptomatically
with NSAIDs. Two weeks prior to admission, the patient described the insidious onset of
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Harmony Tyner, MD
Larry Baddour, MD
Johanna Bischof, MD
dizziness, fatigue, and high grade fevers. These symptoms were progressive and one
week prior to presentation he developed pale hands, scleral icterus, and brown urine
discoloration. On presentation to an outside facility it was noted he had a hemoglobin
5.4 g/dL, positive direct coombs, and evidence of hemolysis. Due to the inability to
obtain matched blood he was transferred to our facility for treatment. Upon admission,
the patient was fatigued with severe pallor, jaundice, and scleral icterus. Initial studies
noted hemoglobin 3 g/dL, haptoglobin

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Tiffany Shih, MD
Arkadiusz Dudek, MD,
PhD
Rena Singleton, MD
thrombotic purpura (TTP). Further evaluation during his hospital course demonstrated
an atrophic spleen with howell-jolly bodies on peripheral smear indicative of functional
asplenia. Perfusion imaging of his limbs was performed which demonstrated no blood
flow below his bilateral ankles and wrists, outlining the degree of necessary
amputation. His family struggled to contemplate his life after four-limb amputations, in
addition to the many surgeries he would need to address the necrotic tissue comprising
much of his face. While trying to determine a care plan, his course became complicated
by invasive aspergillosis, at which time his family decided to withdraw support. It was
not until after his death, more than 14 days after initial presentation, that final blood
culture results confirmed the diagnosis of Capnocytophaga canimorsus.
Capnocytophaga canimorsus septicemia must be considered in any patient presenting
with sepsis and DIC with or without the history of a dog bite. Definitive cultures may
take weeks to return, necessitating a high index of suspicion. Early antibiotics and
aggressive supportive cares are the mainstays of treatment, but their efficacy is often
hampered by late patient presentations due to the slow growing nature of the
bacterium. Even despite appropriate therapy, the results of invasive Capnocytophaga
infection can be devastating.
Capecitabine: A Novel Treatment for Refractory Extramammary Paget''s Disease
Introduction: Extramammary Paget''s Disease (EMPD) is a rare cutaneous
adenocarcinoma typically treated with topical medications, surgical excision, or laser
therapy. More specifically, imiquimod 5% cream applied 3 times weekly for 16 weeks
often will induce a complete resolution of EMPD. However, in patients with recurrent
EMPD, particularly in sensitive areas such as the perineal area, systemic
chemotherapies may be necessary to eradicate more aggressive forms of this disease.
Case Presentation: An 81 year old male with a history of recurrent EMPD involving the
scrotum and inguinal area and previously in remission for 3 years presents to his
dermatologist for a skin check. He has no complaints except that he has noticed new
pruritic patches on his penis and scrotum. His scrotum and inguinal fold reveal wellhealed
scars after multiple modes therapies including topical creams, photodynamic
therapy, and Mohs surgery without evidence of recurrence. However, there were new
scaly, hypopigmented patches on the left aspect of the penile shaft and erythema
involving the scrotum, consistent with EMPD. The patient was again offered topical 5-
flourouracil with methyl aminolevulinate photodynamic therapy. Unfortunately, he
experienced significant discomfort both during the laser procedure and postoperatively
and was therefore continued on topical medications alone and reevaluated 3 months
later. Disappointingly, his EMPD continued to spread and on examination, there were
diffuse erythematous plaques on the scrotum and new involvement in the inguinal
region. At this point, the patient has exhausted all standard therapies and has had
recurrent, refractory diseases for almost 20 years. He was therefore referred to
oncology for oral chemotherapy. Oral capecitabine, a chemotherapy agent shown to
decrease actinic keratosis, basal cell carcinoma, and squamous cell carcinoma of the
skin was offered to the patient at 500 mg twice a day for 14 days followed by 1 week
off. After 5 cycles, the areas involving EPMD began to visibly recede in the inguinal
areas bilaterally with still residual involvement of the scrotum. His capecitabine was
subsequently decreased, leading to recurrence involving the inguinal region. The
patient tolerated the medication well with no complaints with the exception of
occasional episodes of diarrhea. Discussion: This case demonstrates capecitabine, an
oral chemotherapy drug that is enzymatically converted to 5-flourouracil, as an
effective therapy for refractory and recurring EMPD. Although decreasing the
capecitabine led to recurrence of disease, a regimen of 2 weeks on and 1 week off was
able to induce recession of EMPD and possibly lead to remission with few side effects.
In the future, capecitabine may be an effective treatment option for not only recurrent
disease but sensitive areas where topical, surgical, and photodynamic therapy may
result in significant discomfort or irritation.
Pseudo-Brugada in the setting of extreme hyperkalemia from rhabdomyolysis
Introduction: Brugada Syndrome is an arrhythmia syndrome that presents on
electrocardiogram (EKG) as a particular pattern with pseudo-right bundle branch block
(RBBB) and ST elevations in leads V1-V3. There are three patterns of ST elevations that
are differentiated into separate types. It is important to recognize such EKG
presentations because Brugada Syndrome is associated with increased risk of sudden
79

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Vaibhav Vaidya, MD
Christopher DeSimone,
MD PhD, Associate;
Faisal Syed, MD, Fellow;
Samuel Asirvatham,
MD, Faculty.
cardiac death in patients with otherwise normal cardiac studies. Case description: A
40-year-old male with polysubstance abuse, schizoaffective disorder and bipolar
disorder presented with right lower extremity numbness and weakness after being
found unconscious for an unknown length of time. He reported using methadone and
marijuana and denied other drug or alcohol use. Outpatient medications were Abilify
and clonazepam. On exam, he was afebrile and mildly hypotensive. Regular rate and
rhythm without murmur was noted, and loss of deep and superficial peroneal, sural and
tibial nerve sensation and 0/5 right ankle flexion and extension were found. Laboratory
studies demonstrated WBC of 19.5k/ul, potassium of 8.2mmol/L, creatinine of
3.83mg/dL, a peak troponin of 11.480 and creatine kinase of 144,166U/L. Urine
toxicology was positive for marijuana and methadone. Initial EKG on admission
demonstrated a Type 1 Brugada pattern with an incomplete RBBB-like pattern and
coved ST elevations in V1 through V3. A 2010 EKG was later found and a possible
incomplete RBBB was noted. He received urgent hemodialysis and was also dialyzed
intermittently for anuric kidney injury. Diagnosis of myocardial pseudo-infarction in the
setting of hyperkalemia was made and ST changes on EKG resolved with improved
hyperkalemia. Echocardiogram demonstrated no wall motion abnormalities and normal
left and right ventricle size. Right lower extremity compartment syndrome was
managed conservatively and patient was ambulatory at discharge. Discussion:
Manifestation of Brugada on EKG is variable and at times transient in some genotypes.
Factors can affect degree of ST segment elevation, like adrenergic or vagal stimulation
and findings like RBBB are described in the literature as masking true Brugada. When
evaluating a patient for Brugada Syndrome or Type 1 Brugada pattern, possible
confounders must be considered, such as patients with pre-existing incomplete RBBB
morphology. New EKG findings in a such a patient, without knowledge of previously
noted RBBB, can make the patient virtually indistinguishable from a patient with true
Type 1 Brugada. Patients with Brugada pattern on EKG in the absence of clinical criteria
are considered to have Brugada pattern, not Brugada Syndrome. However,
spontaneous Type 1 Brugada pattern on EKG (which may be transient) is associated in
certain cases with increased risk for sudden cardiac death when present with certain
structural abnormalities. In patient cases such as the one described here, thorough
evaluation for confounders will help guide appropriate management.
Ventricular tachycardia originating in the left main coronary ostium – ablation and
anatomic correlates.
Introduction: Ventricular tachycardia can lead to palpitations, syncope, sudden cardiac
death and recurrent episodes can lead to tachycardia induced cardiomyopathy.
Catheter ablation is one of the first line treatments of recurrent ventricular tachycardia.
Origin in the coronary ostia is rare and represents a significant diagnostic and
therapeutic challenge. Case description: A 44 year old man with a past medical history
of pulmonary sarcoidosis presented for evaluation of fatigue and palpitations. A 12 lead
EKG revealed wide complex tachycardia with right bundle branch block morphology,
precordial concordance, inferior axis in leads II, III and aVF and negative deflections in
leads I, aVR and aVL, representing left ventricular outflow tract or pulmonary-aortic
root origin of ventricular tachycardia. A Holter study illustrated numerous PVCs and
runs of ventricular tachycardia, with the longest run lasting approximately 33 minutes.
Cardiac MRI revealed low-normal ejection fraction and a gallium scan did not have any
findings of cardiac sarcoidosis. Electrophysiological study was performed and there
were frequent premature ventricular contractions, complex ventricular ectopy and nonsustained
runs of ventricular tachycardia. The predominant morphology represented
more than 90% of all arrhythmic activity throughout the study and was similar to the
morphology detected previously. Left coronary cusp origin was suspected for the first
morphology and trans-septal access to the left atrium was obtained. Mapping within
the left coronary ostium itself demonstrated earliest onset of the arrhythmia, with 60
msec delay compared to the surface leads. Since ablation cannot be carried out within
or in close proximity to the coronary ostium, an aortic root isolation procedure was
carried out. Ventricular tachycardia could not be induced following isoproterenol
infusion. PVCs were lesser in number and density, but were still visible. The cardiac
venous system was mapped and a near perfect match to the 12 lead tracing was
obtained in a rightward branch of the anterior interventricular vein. This area was
empirically ablated and the patient was escorted to his room in stable condition. At 5
month follow up, patient did not have any symptoms of palpitations or fatigue off
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Marcia Venegas-Pont,
MD
Shaina Rozell MD,
MPH1, Samantha
Stoven MD2, Stephen
Hauser MD3
Hani Ahmed, MD
antiarrhythmic therapy. Holter monitoring did not reveal any runs of ventricular
tachycardia and revealed premature ventricular contractions that were significantly less
compared to before ablative therapy. Discussion: This case illustrates a rare site for
origin of ventricular tachycardia and the importance of awareness of anatomic
correlation of the site of origin of ventricular tachycardia. Ablation near the coronary
ostium is technically difficult and can lead to myocardial ischemia, if close to the ostium.
It is important to be aware that mapping an arrhythmia to the ostium may indicate
origin in areas close to the ostium that are otherwise difficult to map.
An Enigmatic Case of Chronic Diarrhea, Deciphered
A 67 year-old female presents with two years of persistent watery, nonbloody diarrhea,
6 to 7 times a day, associated with poor appetite and 70 pound weight loss. Medical
history is significant only for distal esophageal narrowing status post myotomy in the
1970s and cholecystectomy in the 2000s. Previous workup had included serologic
testing for celiac disease, infectious workup, and neuroendocrine markers all which had
been normal. Previous endoscopy with small bowel biopsy had been completely
normal, however stomach biopsies showed focal active chronic gastritis. Additionally,
she had been treated for possible small bowel overgrowth but diarrhea recurred soon
after treatment. Upon admission, laboratory evaluation revealed a creatinine of 1.2
(baseline 0.9). Infectious workup including Clostridium difficile and HIV test were
performed and negative. Fecal electrolytes were obtained with fecal osmotic gap
consistent with a secretory diarrhea. CT enterography was obtained and unremarkable.
Subsequently upper endoscopy demonstrated diffuse inflammation of the esophagus
with superficial ulcers involving the esophageal bulb and post bulbar duodenum. Biopsy
of the duodenum and terminal ileum revealed complete villous atrophy, thickened sub
epithelial collagen bands with mild intraepithelial lymphocytosis consistent with
collagenous sprue. She was initiated on oral budesonide as well as gluten free and
limited lactose diet with significant improvement on discharge. Collagenous sprue is an
extremely rare disorder, characterized by diarrhea and severe malabsorption
accompanied by histological findings of subepithelial collagen deposition and severe
villous atrophy of the small bowel mucosa. The term was first introduced by Weinstein
et al in 1970s, although descriptions of collagenous deposits dates back to a case
described in 1947. Its exact etiology remains under investigation with proposed links to
both celiac disease and refractory sprue. Clinically, the disease affects more women
than men with a female to male ratio of 2:1, with middle-aged to elderly persons mostly
affected. An increased prevalence of coexisting autoimmune conditions have been
reported, with a 2-fold to 12-fold higher observation that that reported in celiac
disease. Traditional thinking is that collagenous sprue is a complication of celiac
disease. Many patients however with collagenous sprue are unresponsive to a glutenfree
diet and lack serologic or clinical data that could have suggested celiac disease as
the likely etiology. The pathophysiology is not completely understood, with thoughts of
increased expression of fibrogenic genes, especially procollagen I and increased
eosinophils contributing to progression. Treatment includes initiation of a gluten-free
diet as well as immunomodulatory therapy. A clinical response was observed in 24
(80%) patients after treatment with combination of a gluten-free diet and
immunosuppressants, whereas 9 and 5 patients had histologic improvement and
complete remission, respectively.
Shock and End Organ Damage in the ICU: Is it Sepsis or something else?
Shock and End Organ Damage in the ICU: Is it Sepsis or something else? Hani Ahmed,
Department of Medicine, HCMC History: A 52 yo M without a significant PMH
presented with a two week history of fever and abdominal pain. His symptoms were
progressive and associated with night sweats, weight loss and jaundice. At the time of
presentation he was quite ill with a high grade fever, jaundice, nausea and vomiting. He
was admitted to the MICU with initial concern for acute cholangitis. This was
sufficiently ruled out and although a focus of infection was sought and never found, he
was empirically treated with broad spectrum anti-infectives. Despite this, the patient’s
clinical course progressively deteriorated with the development of multi organ failure.
He acquired pancytopenia, ARDS, hypotension requiring pressors and oliguric renal
failure requiring continuous dialysis. Physical exam: On presentation, the patient’s vitals
were BP 95/45, HR 148, RR 36, temp 40 C Pertinent findings included a mildly distended
diffusely tender abdomen with normal BS. Cardiovascular, chest and neurologic systems
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Doris Nguyen, MD
Karna Sundsted, MD
and Jason Szostek, MD
were normal. Work up: pertinent labs included a bicarb of 18, BUN 38, creatinine 1.9,
lactate 8.0, WBC 18 K, platelets 21 K, INR 1.4 Total bilirubin is 5.1, AST 245, ALT 143, Alk
phos 190. CT abdomen demonstrated prominent portacaval, porta hepatic, para-aortic
and inguinal lymphadenopathy with surrounding fat stranding compatible with
inflammatory changes as well as splenomegaly. Inguinal node biopsy returned positive
for ALK positive anaplastic lymphoma. Outcome: With the diagnosis confirmed, the
decision was made to begin treatment with CHOP chemotherapy while the patient was
still critically ill in the ICU, and before staging could be completed. Thereafter the
patient slowly recovered clinically and was transferred out of the MICU three weeks
after institution of chemotherapy. Discussion: Anaplastic large cell lymphomas are a
category of aggressive lymphomas further classified based on the presence or absence
of the ALK mutation. They can be primary (5 % of all non-Hodgkin’s lymphomas) or
secondary to anaplastic transformation from another lymphoma. The prognostically
worse ALK positive lymphomas often present with B symptoms (high fever, weight loss,
night sweats). Extranodal involvement is common with 10 % of cases involving the liver,
lungs and bone marrow. Uncommon clinical manifestations of this malignancy as
described in the literature include significant leukocytosis (reactive and rarely
leukemoid), hepatitis, ARDS, bone and muscle involvement. The presentation of multi
organ damage mimicking septic shock presumably as a result of overwhelming
inflammatory responses such as in our patient is very rare and not very well
characterized in the literature. In conclusion, when a patient is admitted to the ICU with
non infection related multi organ damage, consider aggressive lymphoma in the
differential diagnosis and obtain imaging as appropriate.
Sweet and Sour: Sweet''s Syndrome leading to Diagnosis of Streptococcal Abscess
A 74-year-old woman presented to clinic with a six-day history of chills, fatigue and
fevers up to 102°F. She denied ill contacts, bug bites, animal exposures, new
medications or recent travel. The day prior to presentation, she developed a painful,
nonpruritic rash on her bilateral buttocks but denied any other localizing symptoms.
Her past medical history was notable for stage IIIA endometrial adenocarcinoma
treated by total hysterectomy and bilateral oophorectomy and postoperative radiation
9 months prior to this appointment, as well as, herpes zoster of the right V1 trigeminal
nerve distribution 3 months prior. On exam, she had erythematous, violaceous,
blanching plaques on her bilateral buttocks. More anteriorly, there were multiple
erythematous plaques with several small vesicles. The remainder of her exam was
unremarkable. Laboratory studies were remarkable for a mild leukocytosis with
neutrophilia, an erythrocyte sedimentation rate of 95 mm/hr (0-29 mm/hr) and a c-
reactive protein of 320.8 mg/L (

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David Stoy, MD
Scott Reule, MD
Brianna Vaa, MD
Hemang Yadav
Rhabdomyolysis and Acute Renal Failure following Coxsackievirus Infection
INTRODUCTION: Viral infections are commonly associated with acute nonspecific
myalgia and benign myositis, especially in the pediatric population. More rarely in
adults, rhabdomyolysis with or without accompanying acute renal failure (ARF) is the
consequence of Enteroviral infection. CASE DESCRIPTION: A 19 year old African-
American female presented to her college''s health clinic for diffuse and progressive
mylagias. She had been training intensely as a cheerleader, and had originally attributed
the leg and arm aches to her cheerleading workouts in the setting of abnormally warm
autumn temperatures. She had been trying NSAIDs which had not helped her muscle
aches. She also reported fever and sore throat, both of which had resolved within the
past week. Over the past day, she noted worsening leg swelling and dark urine. Due to
elevated serum creatinine (Cr) on her clinic labs, she was sent to the emergency room
for acute renal failure. Additional history and physical in the ER revealed diffuse muscle
tenderness. Total CK was elevated to over 20,000. Additionally, her serum creatinine
was 3.5 She was hospitalized for rhabdomyolysis with acute renal failure. She
developed worsening muscle edema without evidence for compartment syndrome. The
patient became anuric for approximately 48 hours, but did not complain of uremic
symptoms or develop a pericardial friction rub. Nephrology consult service followed the
patient closely during her hospitalization, and despite evidence for hypervolemia they
decided against initiating hemodialysis. During her hospitalization, both serum CK and
Cr continued to climb, peaking at over 60,000 and 15 respectively). After eleven
hospital days, the patient''s renal function gradually improved. CK and Cr returned to
normal after seventeen days, and she was discharged back to college without lab
findings to suggest residual muscle or renal damage. Serological studies disclosed a
concurrent Coxsackie B4 virus infection. DISCUSSION: Although most Coxsackie B virus
infections are asymptomatic, physicians should be aware of the potential for
rhabdomyolysis along with its complication of acute renal failure. Viral serological
testing may reveal the diagnosis in a case of rhabdomyolysis after a viral prodrome.
1,25 (OH2)D3-mediated Hypercalcemia as a Presenting Feature of Diffuse Large B Cell
Lymphoma
Introduction: An 87 year old woman presented with progressive generalized weakness
of several weeks duration. Investigations revealed hypercalcemia of unclear etiology.
Case description: An 87 year old Caucasian woman presented to the Emergency Room
with a one-year history of progressive fatigue leading to functional decline and an
inability to care for herself. She also noted recent onset exertional dyspnea and lower
extremity swelling. Her medical history was significant for grade 3 diastolic heart failure,
chronic kidney disease, chronic anemia, and a remote history of breast and lung cancer
in remission. Notably, she had been hospitalized two months earlier with similar
symptoms. After an extensive workup, the only abnormality noted at that time was
hypercalcemia (highest level 13.5 mg/dL) and an elevated 1,25 (OH2)D3 of 12.5 pg/ml.
An initial body CT revealed possible omental nodularities, which had regressed
completely at the time of attempted CT-guided biopsy a week later. Remainder of her
workup was negative. She was instructed to limit her dietary intake of both calcium
and vitamin D, and hydrochlorothiazide was stopped. On this occasion, she was
admitted to the medical floor for further management. Examination revealed an elderly
woman with generalized weakness, lower extremity edema, and hyperreflexia. The
hemoglobin level was 9.7 g/dL, consistent with her history of chronic anemia. Serum
creatinine was elevated at 2.0 mg/dl. Total calcium was 13.5 mg/dL (normal 8.9-10.1
mg/dL) and 1,25 (OH2)D3 level was markedly elevated at 139 pg/ml (normal 18-78
pg/ml). Parathyroid hormone was appropriately suppressed at 7.1 pg/ml (normal 15-65
pg/ml) and parathyroid hormone-related peptide was normal. Evaluation for
paraproteinemia was unremarkable with a negative serum protein electrophoresis,
serum free light chains, immunofixation, and urine protein electrophoresis. Angiotensin
converting enzyme was normal. Mycobacterium tuberculosis screening by interferon
gamma release assay was negative, as was a fungal survey. Major causes of 1,25
(OH2)D3 mediated hypercalcemia had been excluded with the exception of lymphoma.
CT abdomen/pelvis was again performed and was reported as showing widespread
peritoneal carcinomatosis, new since two months prior. Bilateral pleural effusions were
present. CT-PET confirmed extensive neoplastic disease involving the diaphragm,
pleura, omentum, and mesentery. Cytology of the pleural fluid was negative but biopsy
of the omentum was positive for large B cell lymphoma. She was transferred to the
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Joshua Vogt, MD
Kyle Klarich, MD
Yader Sandoval, MD
David Williams, MD;
Steven R. Goldsmith,
MD.
Hematology service and is currently undergoing R-CHOP chemotherapy. Discussion:
Hypercalcemia mediated by elevated 1,25 (OH2)D3 has a limited differential diagnosis,
including either granulomatous disease (tuberculosis, sarcoidosis, or fungal disease) or
lymphoma. Nearly one half of all hypercalcemic patients with lymphoma present with
elevated concentrations of 1,25 (OH2)D3. In patients with hypervitaminosis 1,25
(OH2)D3 and hypercalcemia, a stepwise consideration of the limited differential
diagnosis can lead to prompt diagnosis and early treatment.
Constrictive Consideration: Late pericarditis in the post-pericardiotomy patient
Effusive-constrictive pericarditis is an uncommon condition which must be considered
in post-pericardiotomy patients, even months after surgery. A 58-year-old man
presented to his primary care physician complaining of worsening dyspnea on exertion
two months after emergency embolectomy for a saddle pulmonary embolus. He
denied any chest pain, but noticed a significant decrease in his exercise capacity that
progressed over the past three weeks. Previously able to exercise 20 minutes on his
treadmill, he now reported that he became severely short of breath after two minutes.
He denied rest symptoms but admitted orthopnea. He had recently been
subtherapeutic on Coumadin yet had an IVC filter in place. Examination revealed
elevated jugular venous pulsation (JVP) and asymmetric edema of the lower
extremities. Furosemide 20mg daily was initiated and a CT of the chest as well as
echocardiogram were ordered. CT chest did not show recurrent pulmonary embolus
but revealed a moderate pericardial effusion. Transthoracic echocardiogram showed an
echodense material as thick as 1.2cm in the pericardial space with minimal free
pericardial fluid. There was evidence for constrictive physiology but no right atrial or
ventricular collapse to suggest cardiac tamponade. Five days following initial
presentation, the patient’s symptoms continued to progress, and he presented to the
emergency room. Physical exam revealed elevated JVP with positive Kussmaul’s sign.
Heart sounds were distant without any murmurs or rub. Pulsus paradoxus was
11mmHg. Laboratory analysis revealed an erythrocyte sedimentation rate of 67mm/hr
and a C-reactive protein of 69.1mg/L. INR was therapeutic at 2.7. Repeat
echocardiogram showed unchanged constriction without tamponade. Given the
echodensity of the pericardial space, lack of free pericardial fluid, and elevated
inflammatory markers, there was concern for pericardial coagulum versus effusiveconstrictive
pericarditis. Therapy with Colchicine and Ibuprofen was initiated. A cardiac
MRI showed evidence of delayed pericardial enhancement consistent with active
inflammation in addition to an infero-apical pericardial effusion. The patient remained
stable without symptoms at rest and he was managed medically. Given the lack of chest
pain, Colchicine and Ibuprofen were stopped in favor of 40mg Prednisone daily for two
weeks with a two-week taper. The patient remained stable and was discharged home
with close follow up. This case highlights the importance of awareness of effusiveconstrictive
pericarditis as a cause of reduced exercise tolerance in the postpericardiotomy
patient even months after surgery. This condition is characterized by
inflammation of the visceral pericardium with multiple etiologies. Classically, right
atrial diastolic pressure remains elevated despite pericardiocentesis. Postpericardiotomy
patients usually have a fibrinous constrictive picture, but MRI in our
patient supports an inflammatory etiology. If symptoms do not resolve, the definitive
therapy is pericardiectomy. Post-pericardiotomy patients have many potential causes
for dyspnea and this case demonstrates an effective workup, diagnosis, and treatment
of effusive-constrictive pericarditis.
Appendicitis Presenting as Cardiac Tamponade
Introduction Appendicitis is one of the most common surgical emergencies and causes
of abdominal pain. Mortality rate for non-perforated appendicitis is 0.1% and for
perforated appendicitis at least 3%. Perforation is associated with peritonitis and
complications such as intra-abdominal abscesses. We present a patient with pyogenic
pericardial tamponade from contiguous spread from a pyogenic liver abscess, which
was suspected to be seeding from a ruptured appendix. Case Description A 52-year-old
male presented with profound hypotension and respiratory distress requiring emergent
intubation. Bedside ultrasound in the subcostal window revealed a large pericardial
effusion along with a liver mass. Computed tomography confirmed the large liver mass
protruding into the pericardial sac raising concern for fistula tracking into pericardium.
The patient rapidly developed clinical signs of pericardial tamponade. Emergent
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Jason H. Szostek, MD
Karna Sundsted, MD
Jason Szostek MD
parasternal pericardiocentesis drained 450cc of purulent fluid, resulting in dramatic
clinical improvement. Cultures of the fluid grew Streptococcus milleri and Bacteroides
fragilis. Source of infection causing the hepatic abscess and pyopericardium was subacute
ruptured appendicitis that had formed an appendiceal-colonic fistula, allowing
bacterial transmission through the portal system, reaching the liver and, via a suspected
fistula, into the pericardium. The patient survived the episode, but developed profound
constriction within weeks, necessitating pericardiectomy. He was subsequently
discharged home and is now well. Discussion Pyopericardium is a rare entity
associated with a high mortality. Therapy consists of draining purulent fluid in the
pericardium and administration of the appropriate antibiotics. In patients with
pericardial effusion and hemodynamic compromise consistent with cardiac tamponade,
urgent drainage should be performed. Constrictive pericarditis occurs when the fibrotic
pericardium impedes normal diastolic filling due to loss of elasticity and
pericardiectomy is the definitive treatment for patient with symptoms. Conclusions 1.
Appendicitis is a common surgical emergency which may be complicated by peritonitis
and intra-abdominal abscesses. 2. Liver abscess can be a complication of perforated
appendicitis and, although rare, as in this case may present with pyopericardium. 3.
Early recognition, antibiotics and drainage are the most important steps in the
management of pyopericardium.
Sagittal band rupture: A shared recreational hazard for boxers and kitten owners
A 59 year-old woman presented with pain and erythema over her left third
metacarpophalangeal (MCP) joint. She was well until the prior evening when her kitten
collided with the dorsal MCP joints of her left hand, which was in a clenched fist. She
did not sustain a cat bite or scratch. There was immediate pain over the left third MCP
joint, and, over the course of the next day, the pain worsened with associated erythema
and swelling. She reported extreme pain with movement of her left third finger at the
MCP joint; there was no further trauma to her left hand. Review of systems was
negative for spreading erythema, fevers, chills, or history of gout. On physical exam,
she was afebrile and generally well-appearing. A 2cm x 4cm area of dark erythema, with
surrounding ecchymosis, was noted over the left third MCP joint. No skin tears or
abrasions were noted at the site; however there was a clean 0.5cm abrasion noted at
the upper forearm without surrounding erythema. There was exquisite tenderness to
palpation over the left third MCP joint line with minimal range of motion and limited
muscle strength of the left hand due to pain. Otherwise, the examination was
unremarkable. Laboratory studies revealed normal leukocyte count (WBC 8.2x10^9/L)
and normal C-reactive protein (

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total hip arthroplasty in 1999 was admitted to our institution with chills, generalized
weakness and fevers. Five days prior to admission to our hospital, he was discharged
from an outside hospital on trimethoprim-sulfamethoxazole after demonstrating
persistent methicillin-sensitive staphylococcus aureus (MSSA) bacteremia. On
admission, physical exam revealed a 3/6 harsh systolic ejection murmur but was
otherwise unremarkable. Blood cultures grew MSSA within 12 hours. A TEE showed no
evidence of endocarditis. Because of persistent bacteremia, an indium scan was
performed, revealing increased uptake at the aortic valve, left hip and lumbar spine.
Based on clinical and aspirate evidence, the left hip was felt not to be infected, but an
MRI spine confirmed osteomyelitis at L3/L4. The next day, a screening
electrocardiogram showed CHB with a junctional escape rhythm. The patient remained
asymptomatic, and a subsequent cardiac CT did not show perivalvular abscess or
extension. Because of his symptomatic aortic stenosis and IE, the patient was offered
surgical consultation but declined. At discharge he remained asymptomatic with a
junctional escape rhythm, and it was decided to consider permanent pacemaker
placement following further antibiotic administration. Unfortunately, the patient
expired one month after discharge. We report the case of a patient who developed
CHB from MSSA aortic valve IE without radiographic evidence of perivalvular extension.
CHB is often the result of perivalvular extension or edema of adjacent conductive
cardiac tissue. Although rare, conduction abnormalities can also be a complication of IE
without this extension. Aortic valve IE more commonly leads to CHB because of its
proximity to the left and right bundle branches. By contrast, mitral valve infections are
more likely to cause low grade atrioventricular blocks or supraventricular arrhythmias.
TEE is the most common diagnostic tool, but when negative and clinical suspicion is
high, the use of alternate diagnostic modalities, including cardiac CT and indium scan,
may yield the diagnosis. Development of conduction abnormalities during native valve
endocarditis is a poor prognostic sign, especially those that persist after antibiotic
treatment. Although there is no consensus, some advocate valve replacement,
particularly in cases where aortic valve involvement produces conduction abnormalities
that persist greater than one week.
Pheochromocytoma in von Hippel-Lindau Disease: Positive 123I-MIBG with
indeterminate CT.
Introduction: Pheochromocytoma is a rare tumor with an approximate incidence of 2-8
cases per million/year. This is a case presentation of pheochromocytoma associated
with von Hippel-Lindau (VHL) disease. Case presentation: A 53-year-old man with a
twenty-year history of hypertension was evaluated by his primary care physician for a
six-month history of intermittent dizzy spells and associated headaches. Physical exam
was significant only for hypertension. He was taking benazepril, hydrochlorothiazide,
diltiazem, and clonidine. Primary care physician was concerned of possible
pheochromocytoma with patient’s presenting symptoms and family history of
pheochromocytoma. Urine metanephrines were sent and returned mildly elevated.
Abdominal CT showed complex right kidney mass concerning for renal cell cancer (RCC),
simple kidney cyst, and indeterminate thickening of the left adrenal gland. Patient was
referred to endocrinology and urology for further management of possible
pheochromocytoma and RCC. Before biochemical testing was repeated by
endocrinology, his clonidine was stopped, since clonidine can interfere with result
interpretation. Plasma and urine metanephrines came back mildly elevated again.
Given inconclusive biochemical testing and no discrete nodules in abdominal CT, initial
suspicion for pheochromocytoma was low. Patient’s primary care physician was called
to schedule a sleep study given patient had symptoms of sleep apnea, which can be
associated with excessive catecholamine secretion and misinterpretation of
biochemical test results of pheochromocytoma. During this discussion, primary care
physician revealed that she also took care of patient’s sister, who had
pheochromocytoma and carried the diagnosis of von Hippel-Lindau disease, which is
inherited in an autosomal dominant pattern. Their father also had pheochromocytoma
and died from RCC. Patient was a poor historian, and he didn’t know these details of his
family history. This strong family history increased our suspicion for
pheochromocytoma, so 123I-MIBG scan was done, which showed nodular left adrenal
gland with abnormal MIBG uptake consistent with a left adrenal pheochromocytoma.
Genetic testing for VHL was also sent. Patient is now undergoing work-up for surgical
resection. Discussion: Pheochromocytomas are catecholamine-secreting tumors that
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Abdurrahman Hamadah,
MD
arise from chromaffin cells of the adrenal medulla and extra-adrenal sites. Up to 25% of
cases are hereditary. Prevalence of VHL disease is approximately 1 in 35,000 individuals,
and approximately 50% of VHL patients develop pheochromocytoma. The other clinical
features of VHL syndrome include retinal and CNS hemangioblastomas, renal cysts and
RCC, pancreatic cysts and islet cell tumors, endolymphatic sac tumors, and cysts and
cystadenomas of the epididymis and broad ligament. Abdominal CT or MRI is the initial
imaging modality of choice for localization of pheochromocytomas, with >90% and
93.3-100% sensitivity, respectively. In the setting of high clinical suspicion and
inconclusive CT/MRI or biochemical test results, functional imaging tests like 123I-MIBG
scan should be considered. This scan is highly specific (95-100%) but with a sensitivity of
77-90%.
When It Quacks Like a Duck... It’s Not Always a Duck: A Lesson in Unusual Clinical
Presentations Leading to Delayed Diagnosis
A 79 year old male presented to the medical ICU after being transferred from an
outside hospital for hypotension with a presumptive diagnosis of septic shock. The
patient had a past medical history significant for immature acute myeloid leukemia with
pancytopenia (wbc: 0.7, hemoglobin: 6.3, platelets: 16) which had been diagnosed 2
months prior and treated with chemotherapy. The patient recounts the story that he
had been feeling weak and tired all day, and later when he went to take the garbage
out he had a near-syncopal episode. He became profoundly dizzy, and was forced to
crawl back towards the house where his wife called 911. Upon arrival, he admitted to
having a little stomach ache all day, but was otherwise feeling fine. He was awake and
oriented with an otherwise normal physical exam except mild bradycardia (low 50’s).
No hypoxia or dyspnea. No JVD or peripheral edema. Extremities were warm with
strong distal pulses. EKG showed previously noted T-wave inversions in the inferior
leads, and right-sided EKG was negative for ST changes. CXR and blood/urine cultures
were obtained and he was started on broad spectrum antibiotics, however the patient
was requiring high doses of levophed and vasopressin despite aggressive volume
resuscitation. Several hours after arrival, the patient suddenly went from sinus rhythm
into what appeared on telemetry monitoring to be complete heart block. STAT 12 lead
EKG was unable to capture this, but showed 1st degree AV block (markedly prolonged
PR interval) and 2nd degree AV block with variable conduction. The patient remained
asymptomatic. Troponin drawn at the outside hospital was 0.07, repeat was 0.64. The
patient then returned to sinus rhythm with a regular rate. A cardiac echo was obtained
which showed EF 50-55% (unchanged from previous), but with mildly elevated
pulmonary artery pressure and a bouncing interventricular septum “highly suggestive of
pulmonary embolus”. Follow up troponin was now 7.5. A diagnostic dilemma ensued
about confirming possible PE with CT-Angiogram (patient had become anuric from
acute kidney injury secondary to the hypotensive insult) or proceeding with
anticoagulation (for either ACS or PE) in the setting of severe thrombocytopenia.
Heparin was started but the patient continued to be hemodynamically unstable so the
decision was made to proceed to the cath lab. The patient ended up having triple vessel
disease including 100% occlusion of the right coronary artery at the ostium and he was
subsequently transferred to the cardiac ICU following intervention with angioplasty and
stenting. This case again illustrates the highly variable way in which heart attacks can
present, particularly in the elderly. Furthermore, this case represents the importance of
maintaining a broad differential even when the diagnosis has seemingly been made
prior to their arrival in your hospital.
A Silent Presentation of a Serious Illness: Infective Endocarditis
Infective Endocarditis (IE) is often diagnosed clinically based on physical exam,
laboratory, and echocardiographic findings. Pathologic diagnosis can be used to confirm
the diagnosis in patients undergoing valvular replacement or repair. Rarely, IE can be
clinically silent and diagnosed based on pathology of valve tissue removed for non-IE
reasons. The management of this situation may not be straightforward. A 67-year-old
male presented to our institution with acute onset dyspnea on exertion. He has history
of asymptomatic mitral valve (MV) regurgitation for 20 years. A transthoracic
echocardiogram showed myxomatous changes of the MV leaflets with evidence of
ruptured chordae leading to severe regurgitation. Surgical repair was deemed
necessary. He had ongoing issues with his teeth and underwent cleaning and multiple
tooth extractions prior to surgery. He received a course of oral amoxicillin for his dental
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Luke Seaburg, MD
Megan Krause
Natalia Usoltseva, MD
Basma Ricaurte
work. His review of systems was unrevealing. His physical exam showed multiple absent
teeth without evidence of periodental disease, and a 2/6 holosystolic murmur at the
apex. Intraoperatively, he was found to have severe mitral regurgitation with torn
anterior leaflet chordae, and severe myxomatous changes. The MV was repaired by
placing new prosthetic chordae and posterior annuloplasty. He received perioperative
cefazolin for surgical prophylaxis. Surgery was uneventful. Subsequent histopathologic
examination of the valve tissue revealed active endocarditis with extensive involvement
along tendinous chordae, containing numerous pleomorphic organisms with variable
Gram staining. Based on these findings, he was diagnosed with definitive IE. Given the
preoperative indolent course, the likely organisms involved in this case would be of the
HACEK group and Abiotrophia species. His blood cultures obtained postoperatively
were negative. He was initiated on a six-week course of ceftriaxone. To attempt to
identify the organism, further molecular testing on valve tissue for 16S rRNA gene PCR
and PLEX-ID assay were submitted. The results of these tests are pending at the time of
submission of this abstract. This case highlights the fact that IE can be silent. The
diagnosis in this case is not made clinically and is incidental on valvular tissue
pathology. The management should be targeted towards organisms recovered on the
valve tissue. If no organisms are identified on the tissue, treatment as per culture
negative infective endocarditis published guidelines would be appropriate.
Community Acquired Vancomycin Intermediate-Susceptibility Staph Aureus (VISA)
Endocarditis Treated with Ceftaroline Prior to Valve Replacement
Vancomycin Intermediate-Susceptibility Staphylococcus aureus (VISA) is an emerging
pathogen which poses significant therapeutic challenges, especially in situations where
the treatment mainstay includes vancomycin. One of the newest agents approved for
treatment of VISA infections includes the expanded fourth generation cephalosporin,
ceftaroline. A 26-year-old otherwise healthy female with no history of IV drug use
presented from an outside hospital with severe right lower lobe pneumonia,
bacteremia and concern for endocarditis. She described one month of progressive
dyspnea on exertion as well as intermittent chills and low-grade fevers. A TEE had been
obtained by her local providers which was concerning for pulmonic valve endocarditis.
Outside blood cultures drawn at the time of transfer demonstrated VISA. Despite 2
weeks of treatment with linezolid prior to transfer, she remained febrile. Her physical
exam demonstrated reduced right sided breath sounds with positive egophony in the
base, as well as a normal S1 and S2 with normal P2 component and a grade IV/VI
systolic murmur heard best in the left upper sternal border with an overlying palpable
thrill. Additionally she had slight clubbing on exam. She described always having a left
upper sternal border thrill and low exercise tolerance as a child. During her
hospitalization she remained dyspneic on exertion, but hemodynamically stable with
oxygen saturations in the low 90s on room air. Given the presence of the normal P2,
clubbing on exam, and history of longstanding left upper sternal border thrill, a
congenital echocardiogram was obtained which demonstrated significant sub-valvular
pulmonic stenosis, an atrial septal defect with right to left shunt, elevated right sided
pressures, and a small pulmonic vegetation. Based on this, the diagnosis of congenital
heart disease with VISA endocarditis and pneumonia secondary to septic emboli was
made. Treatment was initiated with daptomycin, ceftaroline, rifampin, and synergistic
gentamicin. Rather than treating with two anti-VISA antibiotics, daptomycin was
ultimately discontinued, as it did not provide treatment for the pneumonia. After 1
week of treatment with ceftaroline, gentamicin, and rifampin, the patient stopped
experiencing fevers. She was discharged and completed 6 weeks of this regimen with
resolution of her pneumonia and no evidence of recurrence of endocarditis. She has
since received a pulmonic valve replacement and sub-pulmonic myotomy. This case
demonstrates the difficulty that vancomycin resistance poses in the treatment of
complex infections, and demonstrates one of very few cases in the literature when
ceftaroline was used to treat endocarditis.
Unusual case of acute respiratory failure and isolated hemidiaphragm elevation
Introduction Acute respiratory failure in myasthenia gravis together with marked
ocular, bulbar or limb weakness constitutes a “Myasthenic Crisis”. Myasthenic acute
respiratory failure in the absence of other muscle involvement is unusual and may be
unique when it presents with only unilateral hemi-diaphragm elevation. Case
presentation A 78 year old female with a history of diastolic heart failure, atrial
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Jessica Voight, MD
Dr. Gillian Grafton
fibrillation, essential hypertension, pulmonary embolism, diabetes mellitus type 2 and
myasthenia gravis with bulbar involvement presented with the acute onset of dyspnea.
She denied fever, chills, cough, chest pain, dysphagia, blurred vision or weakness. Her
medications included coumadin, furosemide, metformin, spironolactone,
pyridostigmine bromide and metoprolol. Temperature was 36.5 C, blood pressure
170/74 mmHg, pulse 78/minute, respirations 36/minute and SaO2 94%. She was in
moderate distress with staccato speech and using accessory muscles of respiration.
Breath sounds were decreased bilaterally with prolonged expiration, but no rales or
wheezes. Neurological examination showed normal mentation, motor strength (5/5),
sensation, reflexes, and cranial nerves II-XII. Because of impending respiratory failure,
elective intubation was performed. CXR demonstrated unilateral elevation of the left
hemi-diaphragm. Chest computed tomography with contrast showed atelectasis of the
left lower lobe with left hemidiaphragm elevation but without evidence of pulmonary
embolism. The following day CXR showed bilateral diaphragmatic elevation.
Interruption of phrenic nerve transmission due to myasthenic crisis was suspected
resulting in four courses of plasmapheresis, glucocorticoids and azathioprine. Her
respiratory status improved and she was successfully extubated. Final CXR showed
normal bilateral diaphragm positioning. Discussion Myasthenic crisis usually involves a
combination of respiratory failure together with ocular (ptosis, diplopia,
ophthalmoplegia), bulbar (dysphonia, dysarthria, dysphagia) and generalized (facial and
limb muscle) weakness. This results in need for assistance with feeding, respiratory
secretion clearance and supportive ventilation. Occasionally, respiratory insufficiency is
out of proportion to limb or bulbar weakness. Rarely, respiratory failure is the only
clinically overt manifestation. The causes of a unilateral raised diaphragm on CXR
include atelectasis, lobar collapse, partial lung resection, pulmonary infarction,
pneumonia or radiation fibrosis. Abdominal causes include dilated stomach or colon,
hepatomegaly, splenomegaly or subphrenic abcess. Pseudo-elevation results from a
sub-pulmonic pleural effusion, large pleural mass or diaphragmatic hernia. Intrinsic
hemi-diaphragm elevation is caused by interrupted phrenic nerve transmission resulting
from CNS lesions, invasion by malignant neoplasm, trauma, surgery or neuromuscular
disorders. Our patient presented unusually as myasthenic crisis with isolated
respiratory failure without ocular or bulbar involvement. Moreover, unilateral
diaphragm paralysis is far more common with a localized process than a neuromuscular
disease. Therefore, despite its possible uniqueness, unilateral hemi-diaphragm
elevation and isolated respiratory failure should include myasthenia gravis crisis in the
differential diagnosis
The “Dirt” on this Fun-Gi
Our patient is a 68 year-old male veteran with limited access to medical care, who
presented to the Emergency Department (ED) with the complaint of increasing
dyspnea. He had frequent cough productive of thick green sputum and began to
experience shortness of breath at rest. He was a one pack-per-day cigarette smoker for
the past forty years. He resided in the woods near a lake in northern Minnesota
without access to running water or electricity. Upon arrival to the ED he was found to
be tachypneic and with a neutrophilic leukocytosis. A chest CT scan revealed a large
mass occupying the right upper lobe and right hilum of the lung, which was encasing
the pulmonary artery branches, also with extensive bilateral adenopathy and volume
loss. The mass was read by Radiology to be highly suspicious for unresectable
carcinoma with lymphangitic spread to both lung fields and metastatic disease. Our
working diagnosis was a primary lung malignancy, but given that our patient met SIRS
criteria and with the extent of his pulmonary infiltrates, broad-spectrum antibiotic
therapy was also initiated. Pulmonology performed bronchoscopy with
bronchoalveolar lavage and endobronchial biopsy. Unfortunately, the biopsy was nondiagnostic
so the patient was scheduled for image-guided biopsy with Interventional
Radiology. Meanwhile, despite receiving antimicrobial therapy for presumed postobstructive
pneumonia, the patient’s oxygenation worsened significantly during
hospital days 1-5. On the morning of the scheduled IR biopsy, the patient’s
bronchoalveolar lavage culture began to grow Blastomycosis dermatitidis and
treatment with Amphotericin B was initiated for pulmonary blastomycosis. He had no
evidence on exam of disseminated Blastomycosis. Unfortunately, the patient’s
oxygenation continued to worsen and he was transferred to intermediate care. He
required continuous bi-level positive airway pressure and his chest x-ray illustrated
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Noah Goldfarb, MD
significant disease progression consistent with acute respiratory distress syndrome
(ARDS). On the basis of a series of two case reports published in the journal of CHEST,
2008, our patient was started on high-dose intravenous Methylprednisolone for
treatment of Blastomycosis-induced ARDS to decrease the inflammatory response.
Both of the patients from that series were intubated; however our patient managed to
narrowly escape endotracheal intubation. He completed fourteen days of
Amphotericin B and ten days of Methylprednisolone followed by a Prednisone taper; he
continues on a six-month course of Itraconazole. This case illustrates the importance of
ultimately making a diagnosis clinically, based primarily on history and physical exam,
rather than being mislead by a radiographic finding suggestive of another diagnosis.
Ascites, Adnexal Mass, and Cancer?
Tuberculosis is a rare cause of ascites in the United States, but is significantly more
common in immigrant populations. Far more common causes include liver disease and
carcinomatosis. The diagnosis is made even more challenging when the clinician is
faced with evidence (albeit perhaps erroneous) supporting an incorrect etiology. Ms. W
was a 33 year old Ethiopian woman who presented to the emergency department with
increasing fatigue, low energy, and general malaise for the last six months. Over the
previous three weeks, she had had increasing swelling of and pain in her abdomen.
Since the swelling began, she noted increased shortness of breath, at rest and with
activity, as well as pain in her abdomen with deep breathing. The shortness of breath
was worse sitting up rather than lying flat. She had decreased appetite, as well as
nausea and one episode of vomiting the day of admission, without blood. One week
prior she noted a single episode of blood in her stool and for the past week her stools
were ''yellowish, not dark enough''. She had also noted palpitations over the last
several weeks, occurring intermittently and often accompanied by abdominal pain. She
acknowledged skin changes as well, turning ‘yellow’ at times. Three days prior to
coming to the ED, she had sought care with her primary care physician, who performed
an abdominal/pelvic ultrasound which revealed a large right adnexal mass. After
admission, a diagnostic paracentesis was performed without complication, which
revealed a SAAG of less than 1.1, as well as a serum CA-125 of 123 U/mL. A battery of
other tests were sent at that time, including a Quantiferon Gold, viral hepatitis
serologies, and HIV. A transthoracic echocardiogram revealed hyperdynamic function
without valvular lesions or volume overload. An abdominal pelvic CT scan revealed a
right adnexal mass, moderate ascites, mesenteric lymphadenopathy and peritoneal
implants. Given the above results, the patient was discharged after a three day hospital
stay with an appointment to see Gyn/Onc outpatient as her picture was thought most
concerning for an ovarian malignancy. After discharge, her Quantiferon Gold test
returned positive, raising the suspicion for TB as the cause of her abdominal mass and
ascites. Abdominal fluid culture as well as peritoneal mass tissue biopsy confirmed
Mycobacterium tuberculosis, and the patient was started on a four drug anti-TB
regimen and follow-up care with infectious disease. This case illustrates the importance
of maintaining a wide differential diagnosis for a common problem, particularly in
immigrant populations. The available evidence and laboratory analyses pointed
strongly toward one cause, which turned out to be incorrect; thus a wide differential
may help avoid certain forms of diagnostic bias and error.
The Hidden Effusion
Case Description: A 69 year-old male smoker with a past medical history significant for
coronary artery disease presented with progressively worsening shortness of breath
with exertion over 4-6 weeks. He also complained of a cough and increased leg
swelling. He had been evaluated five weeks prior to admission for similar complaints, at
which time he was treated for a presumed COPD exacerbation with prednisone,
azithromycin and albuterol. On review of systems he noted a decreased appetite but no
change in weight. He denied fevers, chills, night sweats, chest pain or orthopnea. In
the ED, a chest x-ray was obtained and only demonstrated mild pulmonary congestion.
Initial work-up, including a CMP, CBC and EKG, was unremarkable. On examination,
the patient had normal cardiac sounds with no rub, no JVD, expiratory wheezes and
decreased breath sounds at the right lung base. A 1.5 cm hard, mobile, non-tender
lymph node was found in his left axilla. CT chest was obtained and revealed a large
pericardial effusion, moderate sized right pleural effusion and bilateral
axillary/mediastinal lymphadenopathy measuring up to 1.5 cm in diameter.
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Siu-Hin Wan, MD
Jackson Liang, Haleigh
James, Colin West
Echocardiogram revealed no pressure effects on the cardiac chambers but did show
mitral inflow respiratory variation consistent with early tamponade physiology.
Pericardiocentesis was performed. Bacterial, viral and mycobacterial cultures and stains
were negative. Cytology for malignant cells was also negative. Additional laboratory
tests including RF, ANA, ACE level, TSH, SPEP and a peripheral smear were all
unremarkable. The left axillary lymph node was finally biopsied and the tissue specimen
was read as consistent with the diagnosis of Angioimmunoblastic T-Cell Lymphoma
(AITL). Discussion: AITL is a rare peripheral T-cell lymphoma with an aberrant
proliferation of follicular helper T-cells (TFH). AITL makes up 1-2% of all cases of non-
Hodgkin’s lymphoma (NHL). It typically affects elderly adults in their sixth to seventh
decade of life. It classically presents with failry acutely with constitutional symptoms,
generalized lymphadenopathy (76%), splenomegally (35%), hepatomegally (26%) and a
morbilliform skin eruption (21%). While serous effusions are a common complication
of lymphomas, pericardial effusions are uncommon. In AITL effusions occur in 25-53%
of patients. In general, lymphoma-associated effusions tend to signify a poorer
prognosis, with increased disease relapse rates after chemotherapy and decreased
survival. Unfortunately, AITL itself portends a poor prognosis with a five-year overall
survival of only 32%. In patients who present with a malignant effusion, the diagnosis
of lymphoma can be difficult to make since the cytology of these malignant effusions is
often negative. While cytologic examination of the serous fluid may not always be
helpful, clinical clues, such as the absence of inflammatory signs and the presence of
tamponade can help predict a neoplastic pericardial effusion, as seen with our patient.
Graves’ Hyperthyroidism Presenting as Unintentional Weight Loss in a Middle-Aged
Man
Introduction: Involuntary weight loss may be from decreased energy intake or
derangements in metabolism resulting in increased energy expenditure.
Hyperthyroidism causes the metabolic rate to increase which may result in weight loss
and should be considered in all cases of unintentional weight loss. Case: A 48 year-old
gentleman with a history of esophageal reflux disease presented to his physician
complaining of unintentional weight loss. He was previously 240 pounds and over four
months, lost 30 pounds or 12.5% of his weight. His only complaints were increased
stress and nonradiating epigastric burning which was not associated with activity or
food. He denied nausea, constipation or diarrhea and did not have any changes in
bowel habits, appetite, or activity. He had smoked cigarettes for 10 years. Family
history was noncontributory. He was tachycardic with a pulse in the low 100s. He had
protrusion of his eyeballs and difficulty closing his eyelids. A fullness of the left neck
was palpated. He demonstrated trembling of the hands. The rest of the examination
was unremarkable. Given his history of esophageal reflux disease as well as epigastric
discomfort, an EGD was scheduled to evaluate for gastric ulceration or malignancy but
was not performed. Given his smoking history, a chest X-ray was ordered which
showed no signs of lung malignancy. Electrocardiogram revealed only sinus
tachycardia. Routine laboratories included an unremarkable CBC and normal renal and
hepatic function. Thyroid stimulating hormone (TSH) level was suppressed to less than
0.01 mIU/L (normal 0.3-5.0) and free thyroxine level was elevated to 2.5 ng/dL (normal
0.8-1.8). A radioactive iodine uptake scan demonstrated diffuse increase in uptake, and
a thyrotropin receptor antibody test was positive at 14 IU/L (normal

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Thomas Waterbury, MD
Adelaide Olson, MD
PhD, Associate,
Department of
Cardiovascular
Medicine, Mayo Clinic,
Rochester, MN
Sarah Yamaguch, MD
Pulmonary Embolism Presenting as Acute Coronary Syndrome
Introduction: Pulmonary embolism is a potentially life-threatening condition with
variable clinical presentations, making early diagnosis and treatment challenging.
Case Presentation: A 57-year-old gentleman with morbid obesity, sedentary lifestyle,
impaired fasting glucose, and obstructive sleep apnea was admitted from the
Emergency Department with a five day history of progressively worsening exertional
shortness of breath, as well as chest tightness and substernal pressure. Symptoms
were relieved with rest. He denied lower extremity pain or swelling, recent travel, or
recent surgery. He also denied tobacco use, recent aspirin use, or family history of early
coronary artery disease. On initial assessment, the patient was diaphoretic and ashen.
Vital signs were remarkable for a heart rate of 88 beats per minute, low oxygen
saturation of 84% on room air, and blood pressure of 118/63 mmHg. On exam, heart
was regular without murmurs or gallops, and lungs were clear to auscultation
bilaterally. No lower extremity edema or tenderness was present. Cardiac enzymes
were found to be mildly elevated on laboratory evaluation, and an electrocardiogram
showed sinus tachycardia with symmetric T wave inversions in the anterior leads. After
receiving sublingual nitroglycerin, he experienced complete resolution of his chest
pressure. Aspirin was administered, and an initial clinical diagnosis of non-ST-elevation
myocardial infarction was made. Therapy was initiated with supplemental oxygen,
intravenous heparin, low dose beta-blocker, aspirin, atorvastatin, and clopidogrel.
Coronary angiography revealed a normal right coronary artery with diffuse nonobstructive
atherosclerosis in the left main, left anterior descending, and circumflex
arteries. A transthoracic echocardiogram demonstrated right ventricular enlargement
with moderately reduced systolic function, elevated right ventricular systolic pressure
(46 mmHg), and mild tricuspid regurgitation. The left ventricle was D-shaped, and the
ejection fraction was normal (66%). These echocardiographic findings suggested right
ventricular overload, and pulmonary embolism was suspected. Intravenous heparin
was continued. An acute occlusive venous thrombosis within the left popliteal vein was
diagnosed by lower extremity ultrasound. Chest CT angiography demonstrated
multiple bilateral pulmonary emboli with nearly complete occlusion of the pulmonary
arteries. Laboratory workup for an underlying thrombophilia was negative. The
patient’s symptoms gradually improved, and he was discharged from the hospital on
oral anticoagulation therapy with concomitant low-molecular weight heparin for
bridging. Discussion: Pulmonary embolism is an important diagnostic consideration
when evaluating patients with acute onset chest pain. The most common ECG finding
in the setting of pulmonary embolism is sinus tachycardia. Findings on
echocardiography may suggest right ventricular strain. Chest CT angiography may
demonstrate large, multiple bilateral pulmonary emboli, as illustrated in this case. An
accurate and detailed medical history guided the selection of appropriate imaging
modality, which prompted the diagnosis of pulmonary embolism, a potentially lifethreatening
condition.
A Classic Case in a Non-Classic Patient
Malignant mesothelioma is almost solely associated with older male patients with
history of asbestos exposure. Consequently, this disease may not be considered in
persons who have a “classic” presentation but are not the “classic patient.” A
previously healthy 18 year old woman presented with a one month history of right
sided chest pain and dyspnea on exertion. There was no history of trauma and review
of systems was unremarkable. Chest CT revealed a large right pleural effusion with left
mediastinal shift and multiple pleural plaques. Additional history revealed one known
exposure to tuberculosis 2 years prior. Her mother was from El Salvador but had lived in
the US since age 14. The patient had never used tobacco. She underwent thoracentesis
with improvement in her symptoms. Cell count revealed 166 red blood cells, 1580
nucleated cells (12% polymorphonuclear cells, 77% lymphocytes, 10% macrophages,
and 1% mesothelial cells.) Pleural fluid analysis was consistent with an exudative pleural
effusion. Pleural fluid cytology was unrevealing with negative bacterial and fungal
cultures. Quantiferon gold was negative. At that time, chronic diseases such as
longstanding fungal infection or pleural endometriosis were considered. Ovarian cancer
was also considered as the patient had a large left ovarian cystic mass. She was
discharged but returned several days later with worsening symptoms and was found to
have a recurrent right pleural effusion. Lung biopsy and mechanical pleurodesis were
performed with video-assisted thoracic surgery. Several days following surgery,
92

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Marina Zakharova, MD
Jerrod Quarles, Chief
Resident, Aaron
Graumann, PGY-3, Dr.
Nacide Ercan-Fang,
Associate Residency Pr
Jorge Brenes Salazar,
MD
histopathology showed malignant mesothelioma, epithelial type, invasive in fibrous and
adipose tissue. Immunohistochemical staining was positive for calretinin and
cytokeratin 5/6 and negative for TTF-1 and CEA-p supporting the diagnosis of malignant
mesothelioma. The patient underwent right extrapleural pneumonectomy and
extended thoracotomy with resection of the diaphragm and pericardium with radical
lymphadenectomy and diaphragmatic and chest wall reconstruction. Fourteenmonths
after surgery she continues to recover well. This case illustrates the fact that despite a
very classic presentation, the correct diagnosis may not be considered if the patient
falls outside the typical demographic for a particular disease. This is a rare presentation
of a rare disease. However, it is vital to maintain a broad differential diagnosis while
investigations are still underway, and to aggressively pursue and confirm diagnosis
when faced with symptoms that seem out of place or unusual in a particular patient
demographic.
Two very rare autoimmune diseases manifesting in one individual.
Autoimmune disorders might involve any organ and tissue with different degrees of
severity and progression. Therefore it is very important and sometimes life-saving to
diagnose autoimmune disease in time. The following is a case of a previously healthy
gentleman presenting with a constellation of non-specific symptoms that progressed to
severe illness. A 35-year-old previously healthy caucasian male was initially seen in the
clinic because of weakness, loss of appetite, leg swelling, and severe back pain for 1
month. Physical exam was unrevealing for rash, bleeding or lymphadenopathy. CT scan
of the chest showed prevertebral mass extending from T1- T7. At that time, lymphoma
was suspected; therefore, a biopsy was performed. The patient was sent home with
very close follow up until the biopsy results were back. Two weeks later, he was
brought to the ED with acute respiratory failure, requiring intubation. The
bronchoscopy showed massive diffuse alveolar hemorrhage. Multiple granulomatous
pulmonary nodules, acute renal failure, and DVT of upper and lower extremities were
discovered on further work up. Treatment for presumed AAV with pulmonary- renal
syndrome was initiated. The patient received multiple transfusions of blood products,
high doses of steroids, and a course of plasmapheresis. He was successfully extubated
several days later. At that time, his renal function had not recover yet; therefore,
hemodialysis was initiated. Blood tests were positive for PR3 ANCA and extremely high
levels of serum IgG4. Kidney biopsy revealed pauci immune crescentic
glomerulonephritis. Fibro- inflammatory changes with proliferation of T- and B- cells
with no malignant cells were discovered on prevertebral mass biopsy, and staining
revealed multiple IgG4- positive cells. Diagnosis of AAV with pulmonary- renal
syndrome and concomitant IgG4- related sclerosing disease with paraspinal
pseudotumor/sclerosing mediastinitis was established. The patient continued to
improve with prednisone and cytoxan therapy, and he was discharged home from the
hospital. His renal function never recovered, and the patient has remained on
hemodialysis since. Diagnosis of autoimmune disorders is frequently delayed due to
variable clinical manifestations and non-specific symptoms. Our case illustrates how
failure to promptly diagnose ANCA-associated vasculitis (AAV) might lead to
development of severe pulmonary-renal syndrome with irreversible kidney damage or
even death. A second, superimposed autoimmune disorder like IgG4-related sclerosing
disease could make diagnosis of AAV even more difficult. Therefore, autoimmune
processes should be very high on the differential with similar presenting cases, and
premature closure should be avoided.
Smooth muscle gone wrong: recurrent Takotsubo cardiomyopathy in a postmenopausal
female with esophageal spasms
Smooth muscle gone wrong: recurrent transient apical cardiomyopathy (Tako-tsubo) in
a postmenopausal female with diffuse esophageal spasms Since its first description in
1990, transient apical cardiomyopathy (also known as Takot-subo syndrome) has been
recognized more frequently with the widespread use of echocardiography. A 66 year
old woman with a history of AV nodal ablation for atrial fibrillation and severe, diffuse
esophageal spams (requiring surgical myectomy) had been hospitalized multiple times
at a nearby institution with shortness of breath and chest pain. Most of her symptoms
had been attributed to manometry- confirmed esophageal spasms. During her first
admission, an echocardiogram demonstrated an ejection fraction of 25-30% with apical
anterior, inferior and lateral wall akinesis, compatible with stress cardiomyopathy. She
93

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Oana Dickinson, MD
David Benditt MD, Brian
Hanson MD, Alisa
Duran-Nelson MD
Nicole Gentile, MD
Amindra S. Arora, M.B.,
B.Chir
underwent a coronary angiogram 48 hours later with no evidence of obstructive
disease and normalization of left ventricular function. Coronary vasospasm was
suspected but no provocative testing was performed. She presented 6 months later to
our hospital complaining of severe chest pressure that started abruptly after she
became furious at her driver for being late. Her ECG on admission displayed a
ventricular paced rhythm, with new ST segment elevation in anterior precordial leads;
she was given aspirin and started on heparin infusion. Once intravenous infusion of
nitroglycerin was initiated, she had resolution of her index symptoms as well as her ECG
changes as compared to baseline. She had a minor elevation in her troponin levels.
Echocardiogram performed the next morning revealed an EF of 15% with apical
ballooning, consistent with stress cardiomyopathy. She received supportive
management and was started on long acting nitrates and calcium channel blockers with
no recurrence of symptoms. On admission day #5, her left ventricular function was
reassessed by echocardiogram and noted to be within normal limits. Recurrent
Takotsubo cardiomyopathy is rare, estimated to occur in less than 10% of all cases. The
underlying pathophysiology of transient apical cardiomyopathy involves endothelial
dysfunction with transient coronary vasospasm at the epicardial or arteriolar level.
There has been clear documentation of reciprocal stimulation between the esophagus
and coronary arteries, such that spasm in one organ is able to elicit a reflex arch on the
other one and potentially propagate a vicious cycle response. These types of patients
seem to have particular predisposition to abnormal smooth muscle function. Takotsubo
cardiomyopathy is a challenging syndrome, since it mimicks typical obstructive ACS in
early phases. However, appropriate diagnosis is crucial, since it has different
therapeutic and prognostic implications.
Hematological, Neurological and Electrocardiographic Findings in Secondary
Hypothermia
The association between hypothermia and pancytopenia has been reported in the
English literature in eleven cases; in five cases pancytopenia occurred in association
with secondary hypothermia. Herein we describe a 6th such case in which
hematological changes were also accompanied by new neurological and previously
unreported electrocardiographic (ECG) findings. A 23 year old female presented with
chronic central hypothermia (range, 28.8°C-34.6°C) secondary to surgical
craniopharyingioma resection 16 years previously. Associated findings included
pancytopenia (Hemoglobin 8.1g/dl, Leukocytes 3500/mm3, Platelets 63000/mm3),
ataxia, upper motor neuron signs, decreased level of consciousness and new ECG
changes. An extensive evaluation failed to reveal any cause of pancytopenia other than
chronic hypothermia. The pancytopenia and neurological changes improved after active
re-warming to 35.6°C. Given the paucity of reported cases, pancytopenia occurring in
association with hypothermia is rare, and pancytopenia in association with secondary
hypothermia as was observed in our patient is even less common. The hematological
and neurological changes in published cases were similar to the findings in our patient,
but the ECG findings noted in our patient have not previously been reported. In most
cases, both the hematological and neurological disturbances resolved after the body
temperature was restored. The association of pancytopenia, neurolgical disturbance
and ECG changes with hypothermia is rare, but its recognition is essential in order to
initiate appropriate therapy (re-warming and supportive management). However, this
same syndrome may occur in conjunction with secondary hypothermia, a condition the
prevention of which is complex.
Deceived by Question of Gallstone Pancreatitis
Deceived by Question of Gallstone Pancreatitis *Nicole M. Gentile MD, **Amindra S.
Arora, M.B., B.Chir *Department of Medicine, **Division of Gastroenterology, Mayo
Clinic College of Medicine, Rochester MN A 51 year old male with history of
hyperlipidemia, type 2 diabetes, recurrent episodes of acute on chronic pancreatitis
since 2009, presents with left upper quadrant pain and a markedly elevated lipase. CT
imaging in 2009 revealed a dilated pancreatic duct. In 2011, he had a recurrent acute
attack. During this time, MRCP showed a diffusely dilated pancreatic duct and an
abdominal ultrasound demonstrated sludge in the gallbladder. Cholecystectomy was
performed secondary to suspected gallstone pancreatitis. In early 2012, he developed
another attack and endoscopic ultrasound was performed revealing a dilated pancreatic
duct up to the papilla with question of ampullary stenosis. At admission, he was
94

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Aditi Ringwala, MD
Melissa Wells, MD
Christopher M. Wittich,
MD
hemodynamically stable and rated his pain at a 7/10, consistent with his prior attacks.
Physical exam was notable for left upper quadrant abdominal tenderness with no
rebound or guarding. Laboratory evaluation was remarkable for a lipase of 20,525 U/L,
amylase 4,043 U/L, and a total bilirubin of 1.2 mg/dL. He was aggressively hydrated and
made nil per os. He denied any alcohol, tobacco, or illicit drug use. Review of systems
was positive for a 15 pound weight loss over the last year. Over the next few days he
clinically improved. Upon resolution of the acute attack, endoscopic retrograde
cholangiopancreatography was performed and revealed a periductal communicating
cyst at the genu, normal biliary tree, dilated pancreatic duct, and intraductal mucus
with question of intraductal papillary mucinous neoplasm. A pancreatic and biliary
sphincterotomy were performed and pancreatic duct stent was placed. Cytology was
nondiagnostic. Post ERCP he developed another attack of acute pancreatitis and was
hospitalized. Subsequent investigation included another endoscopic ultrasound,
revealing a cyst in the head of the pancreas posterior to the ampulla. Fine-needle
aspiration was done and negative for malignancy. CT Abdomen suggested a 1.6 cm
multiloculated cystic lesion in the head of the pancreas with a 1.2 cm periportal lymph
node. The ultimate plan was to proceed with surgical resection. A pylorus sparing
Whipple procedure was performed. Pathology revealed a signet ring cell carcinoma of
solid and mucoid components measuring 2.5 x 2.5 x 2 cm in the pancreatic head with a
positive uncinate margin, perineural invasion, and involved the distal common bile duct
with 9 of 35 regional lymph nodes positive. Ultimately, what was thought to be
gallstone pancreatitis proved to be stage III, T4, N1, M0 pancreatic carcinoma. While
gallstones and alcohol are common etiologies of acute pancreatitis, in individuals with
recurrent attacks of acute on chronic pancreatitis, a more aggressive investigation
should be pursued and differential broadened to include pancreatic cancer.
Boils of Blastomycosis
Introduction: Blastomyces dermatitidis is a thermally diphormic fungus that is endemic
to North America. While lung findings are common, the rarity of skin and bone
manifestation can make the diagnosis a challenge. Case description: A 23-year-old man
of Hmong heritage presented with “boils” and weight loss that began six weeks before
presenting. At the time, patient went to an ER where he was diagnosed and treated for
pneumonia. Patient’s pneumonia symptoms resolved but he continued to have painful
ulcerated plaques in the trunk and extremities. A biopsy was completed and he was
found to have lobular panniculitis. Tissue culture was negative for bacteria, fungi, and
atypical mycobacteria. A chest X-ray performed at this time found a left lung opacity
consistent with pneumonia. Patient was started on dapsone and ibuprofen.
Subsequently, patient’s urinary Histoplasma antigen and Blastomycosis serology
returned positive. A CT found lytic lesion in the left 10th rib, associated with a soft
tissue mass. This mass was biopsied and found to have an acute necrotic process with
evidence of granulomatous formation. Given radiologic, pathologic, and clinical
findings, patient was believed to have blastomycosis. Discussion: This case illustrates
the relevance of a thorough history in a patient with predominantly extrapulmonary
manifestation of blastomycosis. While the patient’s pulmonary symptoms resolved
weeks before being seen in clinic for his ulcerations, an extensive history helped make
the connection between pulmonary and dermatologic findings.
A Surprise Diagnosis in a Patient with Elevated Transaminases
Introduction: The diagnosis of inflammatory myopathies often begins in the primary
care physician’s office. Evaluation of symptoms may lead to discovery of other
abnormalities, as highlighted in this case. Case: A 77-year old woman with a history of
hyperlipidemia, hypothyroidism, and microscopic colitis, presented with two years of
progressive leg weakness. She described difficulty climbing stairs and rising from floor
when playing with her grandchildren. Outside evaluation found mildly elevated
transaminases (AST 55 and ALT 89, normal alkaline phosphatase). Her medications at
presentation included levothyroxine, aspirin, fish oil, calcium, and vitamin D. She was
not receiving statin therapy. On exam, she had decreased strength in the proximal
muscles of the upper and lower extremities. Reflexes were normal reflexes. She did not
have a rash. Her bilateral hip and knee exams revealed slight decrease in range of
motion consistent with degenerative joint disease, but no signs of inflammatory
arthritis. Initial evaluation revealed mildly elevated sedimentation rate of 36 mm/1h,
AST 74 U/L, ALT 103 U/L, creatine kinase 976 U/L, and normal TSH. Electromyography
95

(EMG) revealed widespread, axonal, chronic-active neurogenic process, consistent with
a motor-predominant polyradiculopathy or progressive motor neuron disease with
superimposed small motor unit potentials in selected muscles, indicating a
superimposed myopathic process. She then proceeded to neurological evaluation for
possible neuropathy. Evaluation including MRI of lumbar spine, CSF analysis, and
paraneoplastic evaluation was unrevealing for a cause of her neuropathic process.
Rheumatological evaluation revealed elevated rheumatoid factor of 30 IU/ml, but other
autoantibodies were negative. During evaluation, EKG was obtained and revealed T
wave abnormalities in inferior leads. Echocardiogram showed inferior wall hypokinesis.
Biopsy of the left quadriceps revealed inflammatory myopathy without signs of
inclusion body myositis. Prednisone 60 mg daily was initiated and her creatine kinase
was followed to normal levels. Discussion: The patient in this case had inflammatory
myopathy. This case highlights diagnostic abnormalities that are associated with
myopathies. Elevated transaminases have been found to be associated with disease
severity in polymyositis. Also, cardiac abnormalities may be more common than
previously anticipated. Cardiac findings have been associated with up to 20% of
morbidity and mortality in patients with inflammatory myopathies. Recognition of
disease severity and prompt treatment may have significant impact on patient
outcomes.
QI1-
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Daniel Chan, MD
Vitaly Herasevich, M.D.,
Ph.D., Ognjen Gajic,
M.D., Brian W.
Pickering, M.B.,
Daryn Collins, MD
Aaron Graumann, Katie
Lorentz, Adam Foss, Eric
Quality Improvement
The Error of Omission: A study of inpatient hospital team task accountability
Define: On hospital inpatient teams at Mayo Clinic, Minnesota, there is a daily
generation of team tasks, or items "to do" for patient care. Despite our best efforts and
the use of current electronic tools to facilitate task completion there continues to be
frequent errors of omission of intended team tasks. The purpose of our study was to
characterize inpatient team tasks and study the rates of omission in order to propose
and develop more effective tools for team accountability. Measure: Over the course of
two weeks, we studied four inpatient medicine teaching services at Saint Mary’s
Hospital. Each team was comprised of three junior and one senior internal medicine
resident and a staff physician who were responsible for up to 14 hospitalized patients.
Each junior resident of each team (n=12) was observed during morning rounds on a
non-admitting post-call service day during which the observed junior resident was
solely responsible for completing all team tasks by the end of the day. The study
observer engaged in no communication or interaction with the team during rounds, and
the junior resident was surveyed at the end of the work day with a customized and
comprehensive online form to account for each task that had been generated by team
members during rounds that day. Over the course of twelve consecutive hospital days
we had a 100% survey completion rate. The total number of managed patients was
129. There were 521 total team tasks generated averaging 4 tasks per patient and 43
tasks per resident on their clean-up day. The overall task completion rate was 79.7%
with 10.6% of tasks intentionally deferred or cancelled. The overall omission rate was
9.8% with 6.1% of tasks un-intentionally incomplete or delayed, and 3.7% not
successfully communicated between team members. Of the omitted tasks,
Consultations (21.9%), Laboratory (21.4%), and Medication (15.6%) were the most
frequently missed task categories. Analyze: Results shows that on our inpatient
teaching services, nearly one in ten tasks are missed on a daily basis, of which almost
half are due to lack of communication potentially leading to serious medical
consequences. Team task completion is not currently optimized in the inpatient setting
due to limitations in completing and communicating team tasks. There currently is not
a synchronized and centralized methodology of creating and accounting for team tasks
as current electronic tools are limited by fragmentation, lack of memory, and lack of
accountability. Improve: We seek to improve the electronic environment using the
existing electronic EMR platform (AWARE 360) to integrate an accessible, centralized,
and integrated inpatient team task list with accountability, persistence, and intelligence
to minimize errors of omission in the inpatient setting.
Venous thromboembolism prophylaxis for medical patients: Implications for a
standardized order set
Introduction: Each patient that is admitted to Regions Hospital in St. Paul, MN, is
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Bomberg, Paula Skarda
John Egan, MD
William Dickey, MD
evaluated for risk of venous thromboembolism (VTE). Based on the degree of risk, a
patient receives mechanical prophylaxis, chemical prophylaxis, both, or neither. The
current admission order set utilizes the Institute for Clinical Systems Improvement (ICSI)
guidelines to determine degree of risk. Using the newest guidelines, published in the
journal Chest in 2012, which make use of the Padua Prediction Score, we set out to
determine if Regions Hospital is over or under utilizing chemical prophylaxis for VTE.
Methods: A chart review was performed on patients admitted to a general medicine or
critical care service at Regions Hospital (St. Paul, MN) from February 1, 2012 to March
31, 2012. Risk factors for VTE were tabulated based on the Padua Prediction Score for
each patient. It was noted if the patient received VTE prophylaxis (mechanical,
chemical or both). The type of chemical prophylaxis was recorded (unfractionated
heparin, enoxaparin, dalteparin, fondaparinux or warfarin continued from the
outpatient setting). Data analysis was performed comparing those patients in high risk
vs. low risk groups, as defined by the Padua Prediction Score. Results: Four hundred and
eighty-six patients were admitted to a general medicine or critical care service at
Regions Hospital during the aforementioned time period. Patients were then excluded
from further analysis if they were admitted to the intensive care unit or if they were on
chronic anticoagulation. Final analysis included 322 patients. High risk for VTE was
defined as a score greater than or equal to four based on the Padua Prediction Score.
Of the 322 patients, 269 were considered low risk. One hundred forty of these patients
(52%) did not receive chemical prophylaxis, while 129 patients (48%) did receive
chemical prophylaxis. Fifty-three patients met high risk criteria. Eighteen of these
patients (34%) did not receive chemical prophylaxis, while 35 (66%) did. Conclusion: A
significant number of patients who were considered low risk for VTE, based on the
Padua Prediction Score, received chemical prophylaxis. This may have implications for
modification of the current hospital order set and for significant cost savings.
Acquired understanding: tracking improvement of intern discharge summaries
Discharge summaries are essential means of communicating information and
facilitating continuity of care. Few providers are aware that JCAHO requires discharge
summaries include certain minimum standards. More concrete information, such as
diagnoses, procedures, and treatment courses are mandated, though more subjective
and stylistic elements are also recommended, including legibility and concision. There
is considerable disagreement among inpatient providers regarding what constitutes an
optimal discharge summary, and many training programs lack formal education on
discharge summary composition. Residents are nevertheless expected to develop
proficiency, learning from attending staff as well as resident peers. The lack of
validated tools to evaluate discharge summaries are a further challenge. We surveyed
our residents’ knowledge and attitudes about discharge summary writing. We then
modified an existing discharge summary evaluation tool and tracked intern-level
discharge summaries. METHODS: A voluntary, electronic survey was sent to all
residents (n=37) at Abbott Northwestern Hospital’s Internal Medicine Residency
program. Multiple-choice questions concerned knowledge about discharge summary
JCAHO requirements, perceived preparedness for discharge summary composition, and
interest in formal training. A grading instrument was developed, based on the
Discharge Summary Evaluation Tool used by O’Leary et al. (2006) with a five-point Likert
scale from 1 (unacceptable) to 5 (exemplary). Ten discharge summaries were arbitrarily
selected for each intern (n=12), and all were reviewed and graded by one reader. Five
summaries were taken from period at the onset of discharge summary writing mid-year
and five from the period at the end of the intern year. Scores were averaged by time
period. RESULTS: Of survey respondents (n=24), 83% (n=20) had some level of
awareness of JCAHO requirements discharge summaries; 70% (n=17) felt neither well
nor poorlyprepared for discharge summary writing, with the remainder split among
feeling well (n=3) and poorly (n=4) prepared; and 92% (n=22) believed that interns
should receive formal training on discharge summary writing. No discharge summary
was graded at less than acceptable quality (less than 2 on 5-point Likert scale). All
contained required components which were prompted by electronic health record
template. Individual means ranged from 2.5 to 4.2 in the early period to 3.5 to 4.6 in
the late period. There was significant within-intern and among-intern variation in both
early and late period scores. Nearly all interns (92%, n=11) demonstrated an overall
increase in mean discharge summary scores over the study time. CONCLUSION: Interns
desire formal education in discharge summary composition in their training. While they
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James Emerson, MD
Ying Jin, MD
Megha Prasad, MD
Jody Holmen RN, Ryan
Lennon BS, John
Bresnahan MD, Peter
Brady MD, Henry Ting
MD,and Malcolm Bell
MD
largely demonstrate a trend of improvement in discharge summary skills with practice,
a formal education program could lead to improved quality of initial discharge
summaries. Further evaluation of senior residents and interns undergoing formal
education would be fruitful.
Do inpatient cardiology consults reduce 30 day readmissions for acute heart failure
exacerbations?
Background: Congestive Heart Failure (CHF) remains the most common reason for
admission in US hospitals. Admissions for CHF account for approximately 80 percent of
total cost for heart failure care, which is estimated at more than 37 billion dollars.1 The
Center for Medicare and Medicaid Services has established a hospital readmission
reduction program which will directly affect Medicare reimbursement to
underperforming hospitals. Here we assess the impact of cardiology consultation in
reduction of 30 day admission rates and analyze the possible mechanisms that lead to
this effect. Methods: Using retrospective chart review of patients admitted at a
university affiliated tertiary care hospital from August to October of 2010 we conducted
a case control study to determine the impact of cardiology consultation on CHF
readmissions. Patients with systolic/diastolic heart failure exacerbation coded as their
primary inpatient diagnosis were included. Readmissions were defined as inpatient
admission within 30 days of discharge. Results: Cardiology consultation was obtained
in 81 of 190 reviewed charts. Readmission rate for patients receiving cardiology
consultation was 14.8% (12/81) compared to 25.7% (28/109). The interventions
recommended by the cardiology team are included in figure and graph 1. There was a
trend toward shorter follow-up ( 5.8 days vs 6.5 days). Increased rates of cardiology
clinic follow-up (68% [55/81] vs 24% [26/109]). The readmission rate for those patients
with receiving cardiology consultation as inpatients and with cardiology clinic follow-up
was 9 % (5/55). Specific interventions were able to be identified in 74/81 consultations
Conclusions: Here we report retrospective data that suggests that cardiology
consultation reduces 30 day readmission rates for congestive heart failure
exacerbation. In our review, the primary interventions that lead to improved outcome
were increased diuretic dosing 50% and modification of medical management of CHF
(86%). In addition, patients tended to have shorter intervals of follow-up and
increased rates of follow-up in cardiology clinic. Patients receiving both inpatient
cardiology consultation and outpatient CHF clinic followup had the lowest readmission
rate (9%). Although no causation can be established by thit review, this data suggests
that more aggressive medical management by hospitalist and increased cardiology
consultation rates, when available, for more complex patients can reduce acute CHF
readmission rates.
Door in Door Out (DIDO) times in the Mayo Health System
Patients with ST-elevation myocardial infarction (STEMI) presenting to emergency
departments without on-site percutaneous coronary intervention (PCI) availability
should be transferred to a PCI center in a timely fashion. It is recommended that the
Door-In-Door-Out (DIDO) time, the time elapsed from arrival at the emergency
department to discharge to a STEMI referral hospital, be less than 30 minutes. A recent
study has shown that DIDO times less than 30 minutes are associated with prompter
reperfusion and lower in-hospital mortality . Methods DIDO times and ECG-toactivation
times were recorded from July 1, 2009 to June 30, 2012 at 28 regional
hospitals that refer patients requiring PCI to Mayo Clinic Rochester, St. Mary’s Hospital.
The DIDO time was defined as the time from arrival at the outside hospital to the time
the patient was discharged to the referral center. ECG-to-activation time was defined as
the time elapsed from ECG acquisition to activation of the STEMI protocol and transport
system. Results There was a total of 179 patients at 28 medical centers intended for
PCI who had measured DIDO times. The median DIDO time was 73 minutes (IQR: 36
min). The median ECG-to-activation time was 14 minutes (IQR: 21 minutes) Each center
was studied individually. None of the 28 centers studied had a DIDO time less than 30
minutes. Discussion This study served as a magnitude assessment, and identified
long DIDO times in the Mayo Health System. The DIDO time is an important
performance measure, and low DIDO times have been associated with improved
survival. Long DIDO times naturally translate to longer times to reperfusion. Based on
these findings, key stakeholders were identified, and various interventions with the goal
of reducing DIDO times have been explored. Chief among these is instant transmission
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Elizabeth Koffel, MD
Angelene Mellick, MD;
Richard Adair, MD;
Loren Bosmans, MD
Brenda Olson, MD
of ECG to a 24- hour ECG reading center at Mayo Clinic, in an effort to activate the
STEMI protocol in a more timely fashion. Further investigation is necessary to identify
additional interventions that may reduce DIDO times in the Mayo Health System.
References 1. Wang TY, Nallamothu BK, Krumholz HM, Li S, Roe MT, Jollis JG, Jacobs
AK, Holmes DR, Peterson ED, Ting HH, “Assocation of door-in to door-out time with
reperfusion delays and outcomes among patients transferred for primary percutaneous
coronary intervention,” JAMA 2011.
“How many emails do you delete without reading?”
Diabetes is a leading cause of mortality and morbidity in the United States, with 25
million people in the United States suffering from the disease. Cardiovascular disease,
nephropathy, neuropathy, and retinopathy are a few of the comorbidities which can
cause significant debility and even death in many patients. It is an important job of a
primary care physician to try to prevent morbidity by appropriate screening, and
implementing treatment when needed. Care guides have been implemented to help
diabetic patients meet the American Diabetes Association care goals, and they
communicate by meetings, letters, and phone calls with the patients. However, the
physician is another piece to the puzzle, and we wanted to examine the best way in
which to communicate with the physician when goals where not being met. Methods:
Patients from the ANGMA Resident Clinic with diabetes were chosen to participate with
Care Guides in an effort to better control their diabetes, specifically the ADA care goals
for A1c, LDL, SBP, DBP, microalbumin test, ACEi/ARB usage, and pneumonia vaccine.
Based on chart review, 266 patients with a diagnosis of diabetes who had been seen
within the past 3 years were included in the study. The real subjects of the study,
however, were the residents. Each patient chart was assigned to one of two methods
of communication between the care guide and the resident physician. First was an
inbox message which stated which goals for a particular patient were met, and which
were unmet. The second method was an addition to the EMR problem list, which
would state the same information. We hypothesized that having the information in the
patient’s chart, more accessible when the patient was seen in the clinic, would lead to a
higher percentage of improvement in care guide goals met. The goals were further
separated into those that depended on patient participation (A1c, SBP, DBP, LDL) and
those which required only the physician’s intervention (microalbumin test, prescription
of ACEi/ARB if presence of microalbuminuria, and pneumonia vaccine), in order to
further characterize the effect of the care guides’ interventions on the physicians.
Results: We did not find a significant difference between the groups, either in patient
controlled or in resident controlled interventions. Conclusions: Primary care
physicians hold an important responsibility in helping prevent significant comorbidity in
a common disease. With more time, we may show a difference in goals met. It is vital
that we find efficient methods of communicating with each other in order to better
care for our patients. With special thanks to Jane Skinner and Lil Krel.
Palliative Care Consults in End Stage COPD
Background: Chronic Obstructive Pulmonary Disease (COPD) is the third leading cause
of death in the United States. Prevalence in Minnesota is 7.8 percent in patients greater
than 65 leading to 84 hospitalizations per 10,000 in the state in 2010 and 1881 deaths
in 2009. Palliative care improves patient experience with end stage diseases. Medicare
covers hospice for patients who have FEV1

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Jordan Schaefer, MD
Rajiv K. Pruthi MD,
Pedro J. Caraballo MD
Bradley Ternus, MD
Dennis Manning, MD.
Clinical Impact of a Clinical Decision Support System (CDSS), Increasing Awareness of
Heparin Induced Thrombocytopenia
Immune mediated heparin induced thrombocytopenia (HIT) typically has an onset of
thrombocytopenia between day 5 to 14 of initiation of heparin, but is often overlooked
by clinicians, potentially resulting in significant morbidity and mortality.
Implementation of CDSS based on patient specific data may result in an early HIT
diagnosis. Methods: A CDSS, designed to identify patients at risk for HIT, was studied.
The CDSS was integrated into the electronic medical record (Centricity Enterprise, GE)
at Mayo Clinic Rochester. Inpatients, with heparin listed on their medication profile in
the preceding two weeks and a 50% drop in platelet count (PC), had an automatic
message generated alerting their care team of the possibility of HIT. A retrospective
chart review, to assess message effect, of consecutive patients identified by the CDSS
over an 18 day period was performed. Message effect was classified into three
categories: 1) Objective, 2) Probable, or 3) None. 1) Objective effect: a) an action
(discontinuing heparin, testing for HIT antibodies, subspecialty consult for HIT), b)
documentation (discussing the possibility of HIT), or c) both, after the message had
been received. 2) Probable effect: new documentation of an explanation for
thrombocytopenia without mention of HIT or documentation or action more than
seven days after the message. 3) None: no evidence of clear action or change in
documentation. Results: Over the study period, 2,785 patients had a total of 37,060 PC
(13.31 PC/patient) the CDSS triggered a total of 125 unique messages. A total of 117
charts were reviewed. Message effect consisted of 1) objective effect: 21% (16% overall
change in management and 5% documenting HIT was felt to be unlikely). The most
common actions included HIT antibody testing, withholding heparin, subspecialty
consultation or changing anticoagulants; 2) probable effect: 14%. HIT had been
considered before the message was sent in 11% of patients. 3) no effect: for the
remaining 54% of patients. 15% of patients experienced arterial or venous thrombosis
and 6% had suspected thrombosis or progression of a pre-existing thrombus on
heparin. HIT antibody testing was ordered for 26% of the patients, with 7% of the PF4
assays being positive (2% of patients). 7% of the assays were equivocal results and the
rest tested negative. Conclusion: In this pilot study, the CDSS resulted in no effect in
54% of patients. With this as a baseline, planned educational efforts may increase the
awareness and documentation of the possibility of HIT in patients on heparin and may
lower the morbidity and mortality associated with HIT.
Stratifying the burden of carrying the service pager: A Quality Improvement Project
Introduction Communication between physicians, nursing staff, and other hospital
services are vital to provide the best care to hospitalized patients. The traditional
method for contacting members of the patient’s care team has been to send that
person a page. Newer text paging systems allows the sender to deliver a short message
without necessitating a return call. Although the use of text pages has provided some
improvement in communication in the hospital there is still a lot of room for
improvement. Throughout the day the resident services are inundated with pages to
the point that patient care can be affected. At this point in time the status of this
project is to define the extent of the problems in the paging system. Methods In order
to define the extent of the problem, the pages that were sent to the 2 resident run
inpatient Cardiology service pagers were monitored. These pages were tracked over a 2
week period and then analyzed to determine whether there were certain time periods
with an increased page burden. Also the reason for the page was tracked to determine
what the most common types of pages were. The total number of pages received on
call days were also compared to the total number of pages received on non-call days. In
addition, a root cause analysis and a critical to quality tree were done to better
understand the procedures and people affected by the current paging system. Analysis
The data that has been collected showed that there is a spike in pages between 0600-
0700 as well as 1800-1900. Upon further review of these anomalies the increased
number of pages can be attributed to transferring of the service pager to the resident
that is on call. Other than these spikes the number of pages to the service pager seems
to be relatively constant between 0600 and 2000. The available page details showed
that the most common type of page received were related to new patient admissions.
The 2nd most common reason for the page was for questions regarding medications,
and the 3rd most common page were digital pages. On average there were 14 more
pages sent to the service pager on days that the team was on call compared to their
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post-call day. Discussion It is clear that the page burden is too high; however the true
cause of this problem is still not evident. Further work to define the extent of the
problem will need to be undertaken. The ultimate goal of this project is to reduce the
number of pages by 30% to improve the efficiency of the residents on the inpatient
services.
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William Allen, MD
Scott Davies, M.D., Tom
Arneson, M.D., Allyson
Kats, M.S., Laxmana
Godishala, M.D., M.
Kathryn McCulloch,
M.D.
Wobo Bekwelem, MD
Lindsay G. Smith, Niki C.
Oldenburg, Tamara J.
Winden, Hong H. Keo,
Alan T. Hirsch, Sue
Duval
Research
Interventions to Improve D5 Scores Provides Significant, Unmeasured Benefits for
Ongoing Smokers
“Interventions to Improve D5 Scores Provides Significant, Unmeasured Benefits for
Ongoing Smokers.” William Allen, MD; Scott Davies, MD; Tom Arneson, MD; Allyson
Kats, MS; Laxmana Godishala, MD; M. Kathryn McCulloch, MD. Hennepin County
Medical Center, Department of Internal Medicine In an effort to improve D5 scores,
Clinic A undertook a quality initiative to create a registry of all diabetic patients
including appropriate health measures. Patients who were not achieving optimal goal
in the 5 domains of D5 (smoking status, Hg A1C < 8, ASA use if appropriate, LDL < 100,
and BP < 140/90) were contacted and asked to schedule labs and appointments to
specifically address deficiencies. New clinic protocols were developed to streamline,
standardize, and improve care management. Staff time was assigned for these
activities. We compared the results in this clinic to another Clinic (B) within our health
system where no specific quality initiative was done. Over one year, we found a
modest improvement in D5 scores in the Clinic A group compared to no change in Clinic
B. (24% improvement versus 8% improvement). However, in Clinic A, among those who
continued to smoke (by definition never reaching a positive D5 status), there was
improvement in BP control and LDL control resulting in a marked improvement in
Diabetic D4 (meeting all targets except smoking cessation). There was a 53%
improvement in Clinic A versus 4% improvement in Clinic B. This work suggests that the
major benefit of a quality program to improve D5 scores may be unmeasured benefits
for ongoing smokers (likely to improve health outcomes because of the independent
and additive positive impact of improved BP and LDL). Incentive programs looking at
improved D5 as the only endpoint would ignore this accomplishment. Improved results
of non-smoking related variables should also be recognized.
An innovative method using claims based algorithms to identify patients with critical
limb ischemia
Background: The prevalence of peripheral artery disease (PAD) in the United States
among individuals aged &#8805;40 years is 4.3%, approximately 5 million people.
Among these, 3% are diagnosed with critical limb ischemia (CLI) and one year after
diagnosis, approximately 25% of patients will have died and a further 30% will have
required a major amputation. Despite these numbers, major knowledge gaps exist in
management of CLI and it continues to be difficult to identify CLI patients for clinical
and research purposes. No study has determined the validity of an algorithm based on
ICD-9 billing codes from a claims database to identify patients with CLI. Methods: CLI
cases (n = 126), diagnosed by a vascular specialist, were identified through a registry.
Controls, those without diagnosed CLI (based on chart review), were frequency
matched to cases on age and sex in a 2:1 ratio. Billing codes for all patients were
extracted. We formulated six (6) algorithms defined on the basis of ICD-9 diagnosis
codes for CLI, (440.22, 440.23, 440.24, 443.9), diabetes (250.xx) and procedure codes
for CLI (39.25, 39.39, 39.5, 38.18). The sensitivity, specificity, positive predictive value
and negative predictive value were calculated for each algorithm. Results: The
sensitivity of the algorithms ranged from 0.29 to 0.92; the specificity was at least 0.99
for each algorithm. Algorithm 5 had the best overall performance with a sensitivity of
0.92, specificity of 0.99, positive predictive value of 0.98 and negative predictive value
of 0.96. This algorithm has been successfully applied to multiple patient databases, is
currently being used to recruit patients for the REVIVE clinical trial and has shown
promise in improving access to these patients. Conclusion: Billing codes can be used
effectively and efficiently to identify CLI patients for population based surveillance,
epidemiological studies and recruitment for clinical trials. An algorithm based on any
diagnosis or procedure code for CLI (440.22, 440.23, 440.24, 443.9, 39.25, 39.39, 39.5
or 38.18), is the most accurate in identifying these patients.
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Malini DeSivla, MD
Ann Settgast, Jose
Debes, Christopher
Anderson, and William
Stauffer
Ben Zhang, MD
Christine Lohse, John
Cheville, M.D., R.
Houston Thompson,
M.D., Brian Costello,
M.D.
Elizabeth Jeffrey, MD
Misti L. Paudel, Brent C.
Taylor, Jane A. Cauley,
Steven R. Cummings,
Kristine E. Ensrud
Asymptomatic Transaminitis in Newly Arrived Refugees to Minnesota
The Minnesota Refugee Health Assessment is a clinical evaluation performed within 90
days of entry to the U.S. to identify health issues in refugees. More than 95% of newly
arriving refugees in Minnesota undergo this examination. HealthPartners Center for
International Health (CIH) in Saint Paul, Minnesota, provides care for many newly
arrived refugees. Since 2008 Burmese and Bhutanese Nepali refugees have been two of
the largest refugee groups arriving in Minnesota. There is limited published data
examining the health status of this group, however a recent study of Karen refuges in
Australia showed elevated ALT in 16.1% of patients using an upper limit of normal of
40U/L. Unpublished observations from health practitioners at CIH suggest there are a
significant number of new Bhutanese and Burmese refugees have asymptomatic, mild
elevations of ALT and AST discovered during new arrival screening. We performed a
retrospective cohort study of refugees presenting for new arrival screening between
5/1/09 and 9/30/11 at CIH to evaluate for any significant differences between refugee
groups during initial health assessment and if present to explore possible associations
related to asymptomatic transaminitis. This study was approved by the HealthPartners
Research Foundation with a waiver of informed consent due to the retrospective data
collection and large study size. Demographic and clinical data obtained during initial
new arrival screening were extracted and audited to ensure accuracy. Statistical
analysis was performed using SAS v9.2. There was a statistically significant difference
of ALT levels between groups with prevalence appearing to be much higher in the
Bhutanese Nepali and Karen/Karenni/Burmese groups than all other refugee groups.
Correlation analysis was suggestive of possible association between female gender and
decreased prevalence of transaminase elevation, other associations may be found
between hemoglobin level or BMI. We are currently performing a multivariate analysis
to evaluate for other significant associations. Hypothesized etiologies of transaminitis
include alcohol use in refugee camps and lack of access to alcohol when arriving in
Minnesota with slower degradation of ALT than AST, a possible viral hepatitis not
routinely screened for in new arrivals, the administration of Hepatitis B vaccination
given in refugee camps prior to departure, a possible nutritional deficiency, or
environmental/toxin exposures in refugee camps. Further analysis of collected data and
future prospective analysis will help further elucidate the etiology and clinical
implications of this asymptomatic transaminitis.
Survival and Treatment Outcomes in Advanced Sarcomatoid-variant Renal Cell
Carcinoma
Sarcomatoid-variant renal cell carcinoma is a rare, but aggressive phenotype of renal
cell carcinoma (RCC) associated with a poor prognosis. Recent scientific evidence has
established newer anti-angiogenic agents such as tyrosine kinase inhibitors and mTOR
inhibitors as first-line agents in treating patients with metastatic renal cell carcinoma.
However, there is a dearth of clinical data on the treatment outcomes of systemic
therapy for metastatic sarcomatoid-variant RCC. Our study identified 206 postnephrectomy
patients with sarcomatoid-variant RCC, and demonstrated that this study
population had significantly worse median cancer-specific survival in comparison to
their non-sarcomatoid counterparts. Similarly, the M0 patients in this group had much
lower estimated distant metastases-free survival rates compared to the M0 patients
from the non-sarcomatoid RCC group (0.6 vs. 1.3 years). Furthermore, we identified
173 patients with metastatic sarcomatoid-variant RCC from our database. Patients who
received targeted therapy for their first occurrence of distant metastases were less
likely to die from RCC compared with patients did not receive targeted therapy
(P=0.039), with median cancer-specific survival of 2.2 and 0.6 years, respectively. In
conclusion, treatment of sarcomatoid-variant renal cell carcinoma with anti-angiogenic
agents resulted in a moderate response. However, larger studies comparing the
effectiveness of specific agents as well as the development of novel treatment
strategies are warranted.
Biomarkers of Kidney Function and Risk of Falls in Older Men
Advancing age is associated with declining renal function and an increased risk of falls,
but the association between these conditions is uncertain. To determine the association
between kidney function and the subsequent risk of falls, we measured serum cystatin
C (cysC) (higher levels indicate poorer renal function) and creatinine (Cr) in a random
sample of 1,566 men aged &#8805;65 years (mean age 73.7 years) enrolled in the
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Tossapol
Kerdsirichairat, MD
Martin Freeman, M.D.,
FACG, FASGE
baseline exam of MrOS and followed them prospectively for falls. After the baseline
examination, men were contacted about falls every 4 months (>99% of these follow-up
contacts were completed). All falls reported in the one year after baseline examination
were included in this analysis. CysC, Cr, and estimated glomerular filtration rate
calculated using the creatinine-based CKD-EPI equation (eGFRCr) were expressed in
quartiles. Fall status was analyzed as an ordinal outcome of none, 1, and 2 or more
using a multinomial logistic regression model to simultaneously evaluate the odds of 1
fall vs. none and &#8805;2 (recurrent) falls vs. none. Analyses included a base model
adjusted for age and clinical site, and a multivariable (MV) adjusted model further
adjusted for fall history, use of psychotropic medications, functional impairments,
prostate symptoms and body mass index. At follow-up, 1,147 men (73.2%) were nonfallers,
219 (14.0%) fell once, and 200 (12.8%) were recurrent fallers. In unadjusted
models, men with higher cystatin C had an increased odds of recurrent falls (odds ratio
[OR] quartile 4 vs. quartile 1 [referent group] 2.13, 95% confidence interval [CI] 1.38-
3.27, p linear trend across cysC quartiles 0.002). This association was in part due to
older age among men with higher cysC. After adjustment for age and site, the
association was somewhat attenuated and no longer significant (OR quartile 4 vs.
quartile 1 [referent group] 1.52, 95% CI 0.96-2.41, p-trend 0.16). Further adjustment
for additional confounders did not substantially alter these results. On the other hand,
there was no evidence of an association between eGFRCr and risk of falls in unadjusted
models (p-trend >0.70). In fact, after adjustment for age and other confounders, there
was some evidence that men with lower eGFRCr were at lower risk of recurrent falls
(MV-adjusted OR quartile 1 vs. quartile 4 [referent group], 0.69, 95% CI 0.40-1.21, p-
trend 0.09). In conclusion, our findings suggest that men with higher cysC levels have
an increased risk of recurrent falls due in part to older age among men with higher cysC
concentrations. In contrast, there appeared to be some evidence that fall risk was lower
among men with lower eGFRCr (higher serum Cr); this relationship may be related to
the dependence of serum creatinine on muscle mass.
Gangliocytic paraganglioma of the ampulla of Vater: case series and a systematic
review
Introduction: Gangliocytic paraganglioma (GP) of the ampulla of Vater is an extremely
rare tumor originating from neuroectodermal remnants and is usually considered as
benign. We report case series diagnosed in our institution, a systematic review of the
disease, and the effectiveness of endoscopic approach. To our knowledge, this is the
first systematic review of the disease. Methods: Two reviewers independently
conducted a detailed literature search using MeSH terms: gangliocytic paraganglioma,
neuroendocrine tumor, periampullary and duodenal neoplasm on major electronic
databases including PubMed, Ovid Medline, Cochrane Library, and Embase. The
reference list of each article was then hand searched. Exclusion criteria are incomplete
clinical data and lack of histology. We also identified 2 cases from our institution.
Results: A total of 42 studies were scanned. We identified 28 studies (31 patients, 11
female, 20 male) with the mean age of 53 years (interquartile, 43-60), published
between 1980 and 2012 to characterize natural history and response to treatments. In
our series, a 54-year-old male and a 47-year-old male, presented with persistent
gastroesophageal reflux symptoms requiring upper endoscopy which revealed a 20-mm
and a 15-mm ampullary masses, respectively. Endoscopic ultrasonography (EUS),
computer tomography (CT), and magnetic resonance imaging (MRI) showed no invasion
or metastasis. Endoscopic ampullectomy was performed in both patients. In our
systematic review, presenting symptoms included abdominal pain in 33%, jaundice in
24%, gastrointestinal bleeding in 18%, weight loss in 3%, and pancreatitis in 3%.
Interestingly, neurofibromatosis was found in 4 patients. Treatments included
pancreaticoduodenectomy in 21 patients, transduodenal laparoscopic ampullectomy in
5 patients, and endoscopic ampullectomy via endoscopic retrograde
cholangiopancreaticography (ERCP) in 7 patients (including our series). All patients who
underwent endoscopic approach had tumor size of less than 20 mm and no evidence of
tumor invasion or metastasis on EUS, CT, and MRI. The mean of tumor size was 20 mm
(16-37). There were 4 patients with regional lymph node metastasis. The mean of
follow up was 13 months (12-24) revealing no recurrence. Conclusion The majority of
ampullary GP has benign course and excellent response to excision. Our series and
recent studies showed that endoscopic resection of the tumor is feasible in selected
patients with reassuring results on comprehensive evaluation including EUS with
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Niyada Naksuk, MD
Prangthip Charoenpong,
Monica Colvin-Adams,
Michael Petty, Marc
Priztker, Sophia Carolina
Masri
John Stendahl, MD
Brooke Heubner,
Elizabeth Amiot, Lauren
McPherson, Kate Miley,
Anne Murray
evidence of submucosal confinement, tumor size of less than 20 mm, and CT and MRI
without evidence of metastasis, to avoid unnecessary major operation such as
pancreaticoduodenectomy and its complications.
Predictors at Admission and Impact of Worsening Renal Function in Acute
Decompensate Heart Failure Patients who Underwent Ultrafiltration Therapy
Introduction: Worsening renal function (WRF) in patients with acute decompensate
heart failure (ADHF) is associated with an increase in mortality. Several studies have
demonstrated the clinical utility of ultrafiltration (UF) in patients with ADHF. However,
despite UF, WRF occurs in some patients. The aim of this study is to determine the
clinical predictors and hospital outcomes associated with WRF and UF. Methods: From
2006 to 2011, 87 consecutive ADHF patients who underwent UF were identified.
Patients with ventricular assist device and with heart transplantation were excluded.
Selection of UF as the treatment method was the choice of the attending cardiologist
and all the patients had a trial of loop diuretics. WRF was defined as creatinine rising
>0.3 mg/dl from baseline. Results: Patients were 67±18 years old. After UF therapy, 28
(32%) patients met the criteria for WRF; their creatinine worsened from 1.8+0.8 to
3.41+.7 (p= 45 mL/min) were
recruited from two urban hospital clinics in Minneapolis, MN. Cognitive performance in
the domains of memory, language, and executive function was assessed via the
following neuropsychological tests: Hopkins Verbal Learning Test (memory), Controlled
Oral Word Association Test (language), Color Trails 2 (executive function), Brief Visual-
Spatial Memory Test-Revised (memory). Cognitive function levels were classified as
either normal or mild/moderate/severe CI according to an algorithm based on DSMIV-R
criteria for dementia and Mayo criteria for mild CI. Logistic regression was used to test
for risk factors for moderate-severe CI. Results: Ninety-six patients were enrolled (CKD
N = 50, Control N = 46). The mean ages of CKD patients and controls were 66.2 +/- 11.0
yrs and 62.0 +/- 10.8 yrs, respectively (p = 0.06). Baseline eGFR among CKD subjects
was 26.2 +/- 10.0 mL/min (Stage 4 CKD). In comparison to controls, CKD patients
demonstrated a higher prevalence of hypertension (88.0% vs. 69.6%, p = 0.03), a lower
rate of employment (24.0% vs. 45.7%, p = 0.03), a higher prevalence of walking
difficulty (20.0% vs. 2.2%, p = 0.01), and used greater numbers of prescription
medications (12.3 +/- 7.1 vs. 6.8 +/- 5.6, p < 0.01). The baseline rates of both stroke
(12.0% in CKD patients vs. 10.9% in controls, p = 0.86) and diabetes mellitus (64% vs.
47.8%, p = 0.11) were similar. While there was a higher prevalence of moderate-severe
CI in CKD patients than in controls (48% vs. 39%, respectively), this difference was not
statistically significant. Overall, in a multivariate analysis, moderate-severe CI was
positively associated with non-white race (OR = 4.0, 1.2-13.3 95% CI) and negatively
associated with years of education (OR = 0.7, 0.6-0.9 95% CI). This finding is consistent
with those of other published non-CKD population studies. Conclusion: Moderatesevere
CI is strongly associated with non-white race and fewer years of education.
Other CI risk factors may be identified as the target sample size is achieved (CKD N =
350, Control N = 100).
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Ian Cheng-Yi Chang,
MD
Erin Austin, Balaji
Krishnan, Lieng H. Ling,
David G. Benditt, Chwee
Neo Quay, Lin Y. Chen
Brian Hilgeman, MD
Anna Cox M.D.
P-wave Indices and Lone Atrial Fibrillation
Background Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia in
the general population and is frequently encountered by general internists. P-wave
indices from surface electrocardiography are readily available and could potentially be a
non-invasive tool to evaluate the risk of AF. Increased P-wave dispersion (PWD) and
maximum P-wave duration (maxPWd) are associated with common acquired atrial
fibrillation (AF). The association of P-wave indices with lone paroxysmal AF is unclear.
We sought to determine whether alterations in PWD, maxPWd, and minimum P-wave
duration (minPWd) are also associated with lone AF. Methods Consecutive patients
referred to the arrhythmia clinic for lone AF (AF in patients

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David Stoy, MD
Marshall Hertz, MD
Prevalence of Sleep Disordered Breathing in Lung Transplant Recipients
Introduction: Lung transplant recipients are now living longer, and many develop
chronic medical conditions which may be related to weight gain due to
immunosuppressive drugs. Our clinical observations suggest the hypothesis that sleep
disordered breathing (SDB) is a common but under-recognized condition in lung
transplant recipients. METHODS: All patients visiting the University of Minnesota-
Fairview pulmonary transplant clinics for routine follow up assessments during a two
month period (September - October 2012) were asked to participate in a sleep disorder
screening questionnaire. We used the eight-point STOP-Bang questionnaire. Subjects
were excluded if they currently required high flow supplemental oxygen, were currently
using CPAP/APAP at night, demonstrated acute graft dysfunction, or had been
hospitalized in the previous four months. Patients who met criteria and agreed to
participate were interviewed, and their demographic data were obtained. RESULTS: 64
patients have been interviewed to date. Two subjects declined due to lack of interest.
We anticipate 120-130 patients to be interviewed by the end of next month. Thus far,
the prevalence of SDB based on a STOP-Bang score of 3 or greater is 72% (46 out of 64).
Recipients with positive screening questionnaire had a greater change in pre- vs posttransplant
BMI compared to negative questionnaire patients (5.2+/- 4.2 vs 1.8+/- 2.0
kg/m2, p=0.04). At this point, duration from transplant date as well as current
immunosuppression drugs and doses do not seem to correlate with a positive screen.
CONCLUSION: Positive screening for sleep disordered breathing is highly prevalent after
lung transplantation, particularly in patients who have gained weight. Weaknesses of
this study include incomplete data set to date (pending October 2012 interviews) and
lack of correlation to polysomnography results (which we hope to complete in screenpositive
patients).
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Christopher Bailey
Catherine Newman,
M.D.
Medical Students
A Tale of Two Tongues: Geographic Tongue Copresenting with Black Hairy Tongue
This report describes the unusual case of a patient presenting with benign migratory
glossitis (AKA geographic tongue) on the right hemitongue, and black hairy tongue (AKA
furred tongue) on the left. Both are benign disorders which are diagnosed clinically, and
frequently cause patients distress prior to diagnosis. This 76 year old Caucasian male
presented to our facility with a complaint of burning tongue and discoloration over the
course of 3-4 months. The burning and tenderness were isolated mainly to the right
dorsum and side of the tongue. The patient reported a tongue laceration on the left
side with possible nerve damage six years prior. The tongue did not suffer from any
deviation or impaired movement. Prior to his evaluation in the dermatology
department, he was prescribed a mouthwash containing nystatin, diphenhydramine,
tetracycline, and prednisone three times daily for 7-10 days without improvement.
Upon examination, the left dorsum of the patient’s tongue was found to have dark
elongated filiform papillae on the left and sharply demarcated gyrated patches on the
right side of the tongue. The patient denied starting any medications other than the
mouthwash. The patient used a standard, mild toothpaste (Crest), had dental fillings,
and did not have dentures. The patient denied use of alcohol and tobacco. The patient
did not have a history of psoriasis or atopy. The patient was counseled that both
geographic tongue and black hairy tongue are benign disorders, and was advised
regarding substances that might aggravate burning mouth symptoms. Tests were
ordered to rule out any underlying condition(s). The patient was found to be deficient
in vitamins B2 and B6. The patient was prescribed fluocinonide gel 0.05% (an antiinflammatory,
antipruritic vasoconstrictor corticosteroid) to be administered three
times daily to the right hemitongue for treatment of the benign migratory glossitis, as
well as a daily B-complex vitamin, and 2% lidocaine gel. He was advised to practice
proper oral hygiene, brush his tongue or use a commercially-available tongue scraper to
gently treat the black hairy tongue. At a follow-up appointment 4 weeks later, filliform
papillae were noticeable shorter and discoloration was reduced. The patient returned 5
weeks later with continued sensitivity on the right tongue. White plaques were noted
on the lateral aspect, suspicious for candidiasis due to the fluocinonide gel, and the
patient was prescribed nystatin 5 mg to be swished and swallowed four times a day for
a week and then twice a day for treatment of this possible candida infection. The
patient continued treatment with fluocinonide when symptomatic. This case illustrates
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Max Gordon
Juan Reyes Genere,
Ryan Kelly MD, Steven
Miles MD
Laura Gorsuch
Tufia Haddad, M.D.
the importance of addressing patient anxiety regarding visually striking, yet benign
disorders. It is also important to have a wide differential diagnosis when seeing patients
with unconventional presentations.
A Bumpy Hospital Course: When an Outpaitient Complaint Grows into an Inpatient
Problem
Our patient is a 25 year old female with a medical history of heavy alcohol use and
severe malnutrition, as well as end stage liver disease, on chronic prednisone secondary
to prior episodes of alcoholic hepatitis. She was admitted to the hospital with fevers,
hypotension, cough, leukocytosis, and infiltrates on CXR. She was initially diagnosed
with sepsis secondary to pneumonia. Initial workup included broad spectrum
antibiotics, blood cultures, CXR, Chest CT, and a diagnostic thoracentesis. Blood and
pleural cultures were negative, but she did have an induced sputum culture that grew
enterococcus species. She had a prolonged hospitalization while awaiting placement to
a behavioral health institution. On hospital day 14, she complained of left breast pain
(that she said had been there for some time), and was found to have a palpable,
fluctuant breast mass. An Ultrasound was performed, and an abscess was documented.
This was drained, with improvement in symptoms. Cultures grew enterococcus species,
which were pansensitive to antibiotics. On review of outside records, one day prior to
admission, she had complained of breast tenderness to an outside provider; an
outpatient ultrasound had been ordered at that time, but this had not carried over to
her inpatient admission. Although her admission chest CT 14 days prior had not
documented a breast abscess, on review, the abscess was visible on this CT scan. She
required a second ultrasound guided drainage when fluid re-accumulated, but since
then, several months later, the fluid had not re-accumulated. Our case illustrates how
“outpatient complaints” can be overlooked in the hospitalized patient, sometimes with
surprising results. We are aware of only two cases of nonpeurperal breast abscesses
(NPBA) in which enterococcus was isolated. In recent years, rates of lactational breast
abscesses have declined while nonpuerperal breast abscesses have increased. To
better treat NPBAs, a more complete understanding of the microbiology is needed.
Previously, Staphylococcus aureus was thought to be the predominant pathogen, but
multiple studies have shown a predominance of polymicrobial infections, with
anaerobic bacteria playing an important role in the pathogenesis of many NPBAs. Since
the realization of the importance of anaerobes, many facultative and aerobic pathogens
have been isolated as well. To conclude, this case demonstrates the importance of a
thorough approach to the hospitalized patient, and adds to our understanding of the
microbiology of nonpeurperal breast abscesses.
Resveratrol-Induced Hepatotoxicity
RESVERATROL-INDUCTED HEPATOTOXICITY Laura Gorsuch, B.A., University of
Minnesota Medical School, Minneapolis, MN, and Tufia Haddad, M.D., Mayo Clinic,
Department of Oncology, Rochester, MN Introduction: Cancer survivors are 36% more
likely to utilize complementary and alternative therapies than the general population.
Resveratrol is a supplement recognized for its antioxidant and potential cancer
preventative effects, yet very few human studies evaluating its safety and efficacy have
been published. Case Description: A 72 year old woman with a history of stage II
breast cancer presented for an annual examination. Routine labs were notable for an
asymptomatic elevation of her aspartate aminotransferase (AST) at 73 u/l and alanine
aminotransferase (ALT) at 65 u/l. Subsequent ultrasound identified multiple lesions in
the liver, and a biopsy diagnosis of metastatic breast adenocarcinoma was made.
Staging PET CT scan demonstrated her disease to be confined to the liver, and baseline
tumor marker (CA27.29) level was 3937 U/ml. She began palliative therapy with an
aromatase inhibitor, letrozole, and had no other prescription medications at the time.
Within 2 weeks of therapy her hepatic transaminases normalized. Repeat CT confirmed
a substantial response to treatment. Five months into therapy blood work
demonstrated recurrent hepatic transaminitis; however, her levels were now
profoundly elevated with AST 991 u/l and ALT 1174 u/l. Meanwhile the CA27.29
demonstrated ongoing decline to 233 U/ml. Chart review was notable for prior
negative viral hepatitis serologies. Further history elicited that since her cancer
recurrence, she started taking resveratrol 100mg by mouth daily. Due to the successful
anti-tumor response from the letrozole and lack of knowledge about the possible
toxicities of resveratrol, she was asked to first stop the resveratrol and continue
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Brandon McCafferty
Kevin Rank, PGY-2
Medicine
Pulin Shah
Paul Di Marco, Enzo
Fallone, Pulin Shah
letrozole. Follow-up two weeks later showed a stable ALT, but 50% reduction in AST.
Six weeks after stopping resveratrol, the levels were both within normal range. After an
additional 12 months, there have been no elevations in her transaminases and tumor
marker is 122 U/ml. Discussion: To our knowledge, resveratrol-induced hepatotoxicity
has not previously been reported. Limited human studies exist, but animal studies have
shown that resveratrol can cause liver toxicity by the formation of glutathione-trapped
reactive intermediates. Further, resveratrol can elevate ALT levels when administered
to rats for 28 days. Lack of strict manufacturing oversight and extensive variation in
preparations make it likely that some preparations have doses great enough to induce
hepatotoxicity via this mechanism. Additionally, the ability of resveratrol to interact
with CYP3A4, a common metabolic pathway for pharmaceuticals, poses a dangerous
situation for patients who self-prescribe the supplement as an adjunct for their cancer
treatment or for its potential cancer preventative properties. This case illustrates the
importance of asking about complementary therapies and considering resveratrol on
the differential for hepatotoxicity in appropriate patients.
Abdominal Pain and the Utility of Reassessment
A 27-year-old male with a past medical history of small bowel obstruction at age 4
presented to the ED with abdominal pain. This was his third visit to the ED for
abdominal pain since it began 3 months prior. At that time, he experienced an abrupt
onset of severe right lower quadrant pain while on vacation. The pain gradually abated
but never fully resolved and he presented for initial evaluation 8 weeks ago. Abdominal
X-ray revealed moderate colonic stool without evidence of obstruction and with normal
labs, and the patient was discharged home on a laxative regimen. He reported mild
intermittent pain over the following 2 months until his return to the ED for 8/10
abdominal pain 2 days prior to the latest visit. Due to the sudden intensifying of his
pain, he underwent abdominal CT, which revealed the presence of a RLQ cystic mass
suspect for a possible mucocele from a mucinous or adenomatous malignancy. As his
pain had stabilized and he was eating post-CT, he was sent home and instructed to
follow up with colorectal as an outpatient. However, sharp, severe post-prandial pain in
the upper abdomen began the next day and he returned to the ED. He reported no
vomiting, fever, or chills, and had a normal bowel movement the day before.
Abdominal exam was remarkable only for moderate suprapubic tenderness, and his
labs were again unremarkable aside from a 2-day drop in hemoglobin of 2gm. The
patient was admitted to medicine, and GI and surgery were consulted. Subsequent EGD
and RLQ ultrasound inconclusively indicated a cystic mass. Surgery assessed him not to
have a surgical abdomen and close monitoring for worsening pain or bleeding was
recommended. However, upon re-reviewing films with radiology, the idea of a
previously asymptomatic Meckel''s diverticulum now causing problems was discussed
within the GI team, and the need for a second look by surgery became more evident.
Additionally, over the next hour his pain increased and he was reassessed. Exploratory
laparoscopy was scheduled, and during surgery a strangulated Meckel''s diverticulum
was discovered and excised. The patient made a full recovery. "When you hear hoof
beats, look for horses, not zebras" is a common idiom in medicine. This case reminds us
how important it is not to ignore the zebras visible only in the periphery. It also
reiterates the oft-forgotten fact that many congenital abnormalities may remain
asymptomatic for years, only later causing quite emergent complications. Had this
patient''s abnormal "cyst" been treated as merely warranting monitoring with follow-up
biopsy, he may not have received necessary interventional care. Lastly, this case
illustrates the utility of personally re-reviewing films with radiology, if only to aid the
radiologists with the "flavor" of the patient''s situation.
Chronic Xanthogranulomatous Cystitis in a 63-year-old Caucasian American Male: A
Case Report
INTRODUCTION First described in 1932, chronic xanthogranulomatous cystitis is a rare
condition with only 29 cases reported in the literature. Presenting symptoms are not
specific to XC and include those of a cystitis pattern of dysuria, urgency and increased
urinary frequency. Urinalysis suggests an infectious cause despite and negative urine
culture. Presenting as an irregular and necrotic growth on the bladder wall, it may
mimic carcinoma, resulting in misdiagnosis. Fortunately, XC has unique
immunohistochemical features that allow for its accurate diagnosis. CASE
PRESENTATION Patient presented on multiple occasions with suprapubic and flank
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Gregory Thibodeau
Pulin Shah,Sanjay R.
Singh
Daniel Kebed
Jennifer Hsu AB, Mark
Wieland MD
tenderness, low grade fever and lower urinary tract symptoms. Urinanalysis
consistently suggested infection despite negative urine cultures. His symptoms had
been treated with multiple courses of antibiotics for presumed urinary tract infection
without improvement. A computed tomography scan of the abdomen and pelvis
without contrast demonstrated thickening of the anterolateral and bilateral obstruction
of the ureters with atrophy of the right kidney and left hydronephrosis. Cystoscopy
demonstrated an white necrotic extrinsic dome mass with the consistency of
cardboard. Biopsy was performed and histology demonstrated chronic inflammatory
cells, large lipid laden histiocytes staining positive for CD68, revealing
xanthogranulatomous cystitis. DISCUSSION Of the limited cases of
xanthogranulomatous cystitis reported, the major clinical symptoms were suprapubic
tenderness and symptoms of cystitis, such as dysuria, urgency and increased urinary
frequency. The development of XC is not well understood but is thought to be due to an
immunological defect in macrophages resulting in chronic inflammatory processes,
inducing local metaplasia of the urothelium. Although xanthogranulomatous cystis is
rare, physicians should be aware of this condition as it can be mistaken for malignancy.
Definitive diagnosis is by microscopic analysis and exlusion of similar process such as
malakoplakia. Currently, conservative therapy with broad spectrum antibiotics have
become mainstay, although the literature indicates no therapeutic benefit. Therefore,
treatment has been centered on complete surgical excision providing resolution of the
disease, with no recurrence reported.
Left atrial compression secondary to a thymoma mimicking a ventricular aneurysm: a
case report.
INTRODUCTION: Thymoma, a neoplasm of thymic epithelial cells, accounts for 50% of
mediastinal masses1. The presentation of thymomas can be variable depending on their
location and proximity to peripheral structures. Extrinsic left atrial compression by a
thymoma is an uncommon source of hemodynamic compromise rarely reported in the
literature3. We present a patient experiencing presyncope with an X-ray suggestive of a
left ventricular aneurysm. CASE PRESENTATION: A 59-year-old patient with a history of
vertigo presents with dizziness and vomiting. He denies chest pain, dyspnea, blurred
vision, or sensorimotor deficits. On examination, nystagmus and Dix-Hallpike
maneuvers were negative. X-ray suggests a large ventricular aneurysm (Figure 1).
Electrocardiogram, brain MRI and carotid duplex were negative. Chest CT reveals a wellcircumscribed,
extra-cardiac mass compressing the left atrium (Figure 2). Ejection
fraction was normal. Biopsy confirmed the mass to be a B2 Type thymoma. A diagnosis
of presyncope, secondary to vertigo was made, with an incidental finding of a
thymoma. The patient’s thymoma was resected resulting in complete resolution of
symptoms. DISCUSSION: Thymomas are commonly incidental findings and nearly onehalf
are asymptomatic1. Chest X-ray offers limited information. Accurate diagnosis
requires CT scans and echocardiography. Among those presenting with symptoms, the
primary determinant is location of the thymoma to peripheral structures. The
presyncopal symptoms, initially thought to be secondary to vertigo, and the CT
diagnosed thymoma were perceived to be two separate entities. However, the
following lines of thought would suggest otherwise. Firstly, the left atrial compression
by the thymoma would result in obstruction of left atrial inflow. Secondly, the left
ventricular function would not be impaired. In this case, the echocardiography revealed
a normal ejection fraction. With compromised inflow, the total ventricular preload
decreases and leads to functional tamponade2,3. With significant hemodynamic
compromise, cerebral blood flow is reduced precipitating a presyncopal episode.
Complete resolution of symptoms following thymus resection also supports this
conclusion. CONCLUSIONS: Although thymomas are primarily asymptomatic, cardiac
compression is a possible manifestation that cannot be ignored. Fortunately, accurate
diagnoses can be made with chest CT and echocardiography. Furthermore, surgical
resection can lead to complete resolution of symptoms.
Disseminated Nocardiosis
A 72 year old Caucasian woman was admitted to the hospital directly from an
outpatient bronchoscopy suite for acute onset of fever, cough and tachycardia
following transtracheal biopsies of a right upper lung mass. Prior to admission she
described six months of progressive fatigue, chills, functional decline, and 40 pounds of
unintentional weight loss. Review of systems was otherwise unremarkable. Past
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Kathleen Mahan
medical history was significant for polymyalgia rheumatica on chronic glucocorticoid
therapy, chronic kidney disease , hypertension, and type 2 diabetes.After fluid
resuscitation, she remained hemodynamically stable throughout the hospitalization
with initial intermittent low-grade fever. Physical examination was pertinent for a
tender 3x5 cm fluctuant mass associated with the right deltoid muscle, fluctuance and
tenderness of the right gastrocnemius muscle, and a 1x1 cm tender nodule associated
with the left parotid gland. The cardiopulmonary, abdominal, and lymphatic
examinations were unremarkable. Admission laboratory evaluation was remarkable
for a normocytic anemia, mild neutrophilic leukocytosis, elevated sedimentation rate
(82 mg/dL), elevated creatinine (2.4 mg/dL), and elevated hemoglobin A1C (8.3%).
Prior CT of the chest showed a large right upper lung mass with multiple bilateral
pulmonary nodules and mediastinal lymphadenopathy. Gram stains of fluid aspirated
from the left deltoid and left leg demonstrated branching gram positive bacilli. Cultures
of this fluid and of tissue from the lung and parotid gland grew nocardia farcinica. She
underwent surgical debridement of the deltoid abscess and needle drainage of the left
lower extremity and parotid abscesses. Antimicrobial therapy included trimethoprimsulfamethoxazole
(TMP-SMX), imipenem and moxifloxacin for disseminated
nocardiosis. The hospital course was complicated by TMP-SMX induced hyperkalemia
that responded to dose adjustment. She was discharged on a prolonged antimicrobial
course. At outpatient follow-up, her symptoms had largely resolved and there was no
evidence of new metastatic infection. Nocardiosis is a rare bacterial opportunistic
infection that predominantly afflicts patients with compromised immune systems.
Common sites of infection include the lungs, central nervous system, and soft tissue.
Our patient had disseminated nocardiosis, which typically carries a poor prognosis.
TMP-SMX is the backbone of antimicrobial therapy for nocardiosis; treatment duration
may be up to 12 months.
“Bac-pedaling” from the Diagnosis of Anoxic Brain Injury
Electrophysiological studies such as the EEG are invaluable tools for assessment of the
unresponsive patient. Even with recent studies suggesting reliability, questions remain
about the use of EEG in prognostication and as a tool for determining withdrawal of
care. A 58 year old woman with advanced multiple sclerosis (MS) with associated
quadriplegia and Type 2 Diabetes Mellitus presented to the Emergency Department
(ED) with respiratory distress secondary to aspiration pneumonia. She was intubated in
the ED and was treated for severe sepsis with fluids, antibiotics, and vasopressors. After
being weaned from all paralytic agents, vasopressors, and sedatives, she remained
apneic during pressure support trials (PST). The neurological exam revealed
unresponsiveness, absence of almost all brainstem reflexes, flaccid musculature, and
deep tendon areflexia. CT of the head without contrast showed no acute intracranial
pathology. An EEG was significant for a rare burst-suppression pattern known as
generalized periodic epileptiform discharges (GPED) which is associated with hypoxic
brain injury and most often a poor prognosis. After two weeks of mechanical
ventilation, the question of extubation vs. tracheostomy placement was discussed.
What was the significance of apnea on PST and why did the EEG show GPEDs?
Medication review revealed that the patient was receiving high doses of oral baclofen
as well as intrathecal (IT) administration for MS-associated spasticity. All oral baclofen
was discontinued with no improvement in mental status. Two days after significant
reduction of intrathecal dosing of baclofen the patient became responsive to voice.
Complete resolution of the GPED pattern was seen on repeat EEG three days after IT
baclofen was reduced by 57% and the patient had spontaneous respirations during PST.
This case illustrates the limitations of EEG as a sole tool for establishing prognosis in the
unresponsive patient. Although the coma and apnea seen in this patient are
uncommon, with 0.5 - 1.5% incidence(1), they are predictable side effects of intrathecal
baclofen. Recognizing that drug toxicity can sometimes mimic hypoxic brain injury is
critical when considering end-of-life decision making in an unresponsive patient.
References: (1). Product Information: LIORESAL(R) INTRATHECAL intrathecal injection,
baclofen intrathecal injection. Medtronic, Inc (per FDA), Minneapolis, MN, 2011.
MSV2-
26
Dhruti Mankodi
Andre Dias, MD, Spyros
Smith, MD, Eugene
Aorta to Right Atrial Fistula: A Rare Complication of Native Bicuspid Aortic Valve
Endocarditis
INTRODUCTION: Endocarditis usually involves an abnormal valve and its extension can
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Fernandes, MD, Cary
Passik, MD
Govind Pandompatam
Maxine Miller, Maulin
Patel, Kathir
Balakumaran, Jonathan
Bress, MD
cause complications such as heart block and rarely, fistulas between chambers. In
general, these complications cause heart failure and require surgical intervention. We
present a patient with native bicuspid aortic valve endocarditis that developed both
heart block and a sub-aortic fistula to the right atrium. This unusual extensive infection
was successfully treated with antibiotics and aggressive surgery. CASE PRESENTATION:
A 54-year-old male with a known bicuspid aortic valve presented with a ten day history
of fever, fatigue and exertional dyspnea. There were no recent dental procedures and
no history of IV drug abuse. He was treated for suspected Lyme disease as an
outpatient, but was referred for admission as blood cultures grew alpha streptococci.
At admission, he was afebrile with a WBC count of 9.5. A grade 3/6 systolic murmur
loudest at right second intercostal space was noted upon exam. Electrocardiogram
(EKG) revealed normal sinus rhythm (NSR) at 79bpm, a 0.36 PR interval and an
incomplete right bundle branch block. Transthoracic echocardiogram (TTE) showed
significant aortic valve stenosis, with new vegetations on tricuspid and aortic valves.
Transesophageal echocardiogram (TEE) confirmed an aortic annular abscess with aortoright
atrial shunt. The patient received twelve days of antimicrobial therapy prior to
surgery. At operation, a thrill was felt over the aorta. A bicuspid aortic valve with heavy
calcification and acute infectious debris, especially in the tissue under the non-coronary
and right-coronary cusps was noted. There was also an inflamed area found in the right
atrium which was debrided and it was at this site that a surgical clamp was passed into
the aorta. Infectious debris was gently removed from under the tricuspid valve but the
valve itself was intact and competent. The sub-aortic to right atrial fistula was repaired
with 2 bovine pericardial patches placed under the aortic valve and in the right atrium.
Finally, the aortic valve was replaced with a bioprosthesis. Intra-operative cultures were
negative for bacterial organisms. The patient completed a course of 2 grams of
Ceftriaxone daily for 42 days. He has done well postoperatively with imaging studies
confirming a functional aortic valve, no tricuspid regurgitation and no signs of fistula.
CONCLUSION: Aorta to right atrial fistula is a relatively rare entity in clinical practice
and is an unusual complication of native bicuspid aortic valve endocarditis. Its
presentation can be nonspecific but aggressive surgical repair with debridement of the
infection and antimicrobial therapy is curative. Our case report illustrates the
importance of early recognition of this complication of endocarditis and emphasizes the
fact that a low threshold of suspicion must be maintained in patients with fever and
abnormal cardiac valves to avoid potential deadly complications.
Acute Chest Syndrome Complicating a Preterm Pregnancy in the Setting of Hemoglobin
SC Disease: A Case Report
Sickle Cell Disease (SCD) can lead to serious complications in pregnancy. Sickle Cell
Anemia (HbSS) is the most severe of these disorders, however variants such as
Hemoglobin SC Disease (HbSC) can also manifest with complications. Though typically
milder, severe presentations do occur. Acute Chest Syndrome (ACS) is one of the most
common and severe of these complications. We report the clinical course and
management of a preterm pregnancy in a HbSC patient with ACS. A 31-year-old Haitian
female G2P1, at 29 weeks gestation, with a history of HbSC, presented with a dry cough
and severe bone pain. Physical exam revealed an ill appearing patient, tachycardic,
tachypneic, normotensive and afebrile with oxygen saturation of 89% on room air.
Lung exam revealed bilateral wheezing and crackles. Labs showed: Hb electrophoresis:
S46%, C54%, Hb 9.2 (11-15 g/dL), WBC 14 (4-10 x10^3uL), ALT 116 (9-52U/L), AST 404
(14-36U/L), total bilirubin 3.9 (0.2-1.3mg/dL), and LDH 2676 (313-618U/L). The patient
was transfused with packed red blood cells, given oxygen, analgesics, IV fluids and
antibiotics. Hours later she developed a fever, increasing fetal heart rate and severe
dyspnea prompting intubation. Tocolytics and steroids were administered and manual
exchange transfusion was initiated. Over the next two days, chest x-rays showed
development of interstitial markings and retro cardiac infiltrates. Labs revealed: Hb 10.4
and LDH 5324, prompting machine exchange transfusion. Post transfusion
electrophoresis showed: A65%, S14%, C21%. On hospital day four, FHR showed
persistent late decelerations despite attempts at intrauterine resuscitation; emergency
cesarean section was performed. The patient’s postoperative course was complicated
by ongoing respiratory failure. She was eventually discharged on hospital day 34 after
Hb stabilized and LDH trended down. The neonate weighed 1455g with Apgar scores of
1 and 3 and developed respiratory distress syndrome among other complications.
ACS is a severe pulmonary complication seen in 7-20% of SCD pregnancies. It typically
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Jason Prudom
Sameet Sangha
presents with new pulmonary infiltrates, chest pain, fever and cough, making it difficult
to distinguish from other conditions including pneumonia and pulmonary embolism.
Initial treatment of ACS includes IV antibiotics and fluids, oxygen therapy, pain-control
and blood transfusion with a goal of HbS

MSV3-
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MSV3-
56
Kathir Balakumara
Gregory Thibodeau,
Sanjay R. Singh, Pulin
Shah
Kirk Wyatt
John Bundrick, MD
presentation in the past month for this problem; he had previously been told that his
discomfort was due to constipation. On exam, he had mild tenderness to palpation
diffusely across the abdomen; his abdomen remained soft and non-distended with
normal bowel sounds and without organomegaly. A CT scan revealed a distended,
unopacified portal vein, with collateral vessel formation, consistent with chronic portal
vein thrombosis. A follow-up ultrasound showed no flow in the portal vein and no
evidence of cirrhosis. The patient had no history of blood clots and no family history of
coagulopathy. He denied any history of liver disease. He was started on anticoagulation
and referred to hematology. Exhaustive coagulability testing including Jak-
2 mutation and PNH screen were negative. Hemoglobin was 17.1. Previous
hemoglobin readings from several months earlier had been above normal. The patient
reported having taken multiple testosterone injections in the past year for
hypogonadism. Testosterone is known to induce erythrocytosis; the mechanism of this
is postulated to be through down-regulation of hepcidin, allowing for increased
mobilization of iron stores. Erythrocytosis can predispose to portal vein thrombosis.
The patient agreed to discontinue testosterone. His abdominal pain resolved several
weeks after starting anti-coagulation. This case emphasizes three important learning
points. First, a thorough diagnostic work-up is indicated in a patient presenting
multiple times for the same chief complaint. Second, testosterone administration can
produce erythrocytosis and create a hypercoaguable state. Finally, the decision to anticoagulate
a patient with portal vein thrombosis is difficult; there is little data on the
efficacy of anti-coagulation in patients with chronic occlusion who do not have cirrhosis.
Atypical presentation of acute thromboembolism complicating idiopathic arterial
pulmonary hypertension in an elderly bird fancier.
INTRODUCTION: Early identification of pulmonary arterial hypertension (PAH) is
paramount for effective treatment. Primary assessment includes exercising capacity,
right-sided heart function and pulmonary artery systolic pressure. We present an
elderly patient with severe PAH in the setting of latent thromboembolism causing
frequent episodes of syncope. CASE PRESENTATION: A 79 year-old gentleman
presents with syncope, dyspnea, history of pulmonary hypertension and farmer''s lung.
He is on home oxygen. His syncopal episodes exclude tongue biting, bowel or urinary
incontinence. He denies chest pain but admits having palpitations. He has a smoking
history, but denies use of alcohol and illicit drugs. He is an avid bird keeper.
Transthoracic echocardiogram reveals PAH of 120 mmHg, oxygen saturation is 80% and
EKG reveals right ventricular hypertrophy. Further work up shows left femoral DVT and
positive ANA. Physical examination shows clear lungs, mild JVD with lower limb pitting
edema. DISCUSSION: This is an unusual case of an elderly male with PAH, WHO Stage
III, complicated by many risk factors. In spite of these risk factors for secondary PAH, he
likely has Idiopathic Arterial Pulmonary Hypertension (IAPH) which is more common in
younger adults. This patient has a normal pulmonary function test, V/Q scan and CT
angiogram showing no segmental stenosis ruling out COPD and chronic
thromboembolism. Although positive for ANA, other tests were negative for systemic
autoimmune diseases. Elevated ANA is present in 40% of IAPH and 6% of secondary
PAH1. This patient has severe right ventricular and atrial hypertrophy from years of
compensation for his IAPH. His recent pulmonary embolism exacerbated the PAH to
120 mmHg causing severely reduced global systolic function of the right ventricle. If he
had chronic thromboembolism causing PAH, his right ventricle would compensate
without failure2. The decompensated right ventricle resulted in a decreased prediastolic
volume, diminished cardiac output and frequent syncopal episodes.
CONCLUSIONS: IAPH in an elderly male is rare. This patient has compensated IAPH.
However, his acute pulmonary embolism exacerbated his IAPH with right ventricular
systolic dysfunction, resulting in syncopal episodes, edema and dyspnea. There is
limited literature on the treatment of acute thromboembolism in IAPH, thus surgical
embolectomy is a possible consideration2. Although IAPH is rare in the elderly, it should
be considered when patients present with dyspnea and syncope without cardiac and
neurological problems.
Puritis as a manifestation of iron-deficiency anemia
The patient is a 79 year old woman who presented with a two months of diffuse
pruritus (without primary rash) and ice craving along with one week of progressive
exertional dyspnea. She had a prior history of large hiatal hernia and Cameron’s
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Michael Bradshaw
Elizabeth Lieser, Gary
Croghan, Francis
Nichols, John Mullon,
Aaron Mansfield,
Svetomir Markovic
Maros Cunderlik
erosions, and on the present admission her hemoglobin was 5.8 g/dl. She had been on
low dose aspirin for primary prevention of cardiovascular events, and that was stopped
at admission. On examination, she had pallor and an early-peaking aortic flow murmur.
There was no evidence of any rash, and the examination was otherwise unremarkable.
She was treated with omeprazole, blood transfusion, and 400 mg of intravenous iron
sucrose. On discharge, she was symptom-free, including resolution of her pruritus. She
was placed on long-term oral iron replacement. One month after dismissal, a follow-up
phone call revealed that she had generally done well in the interim, apart from recent
return of mild exertional dyspnea and pruritus, for which she was scheduled for repeat
testing of her hemoglobin (results pending at time of this abstract submission). As
evidenced in this case and in several other reports and series in the medical literature,
iron deficiency is a known cause of generalized pruritus and should be included in the
differential diagnosis of patients presenting with this symptom. The pruritus in these
cases generally responds promptly to iron replacement.
Angiogenesis in Malignant Pleural Effusions: Pathogenesis and Implications for Novel
Management Strategies
Background: Malignant pleural effusions are a significant source of cancer morbidity,
debilitating patients by impairing respiratory function and decreasing quality of life
dramatically in over 150,000 patients in the US yearly. Current management strategies
are of palliative value, but do not address the pathobiology of the effusion, nor do they
improve survival. Further elucidation of the pathogenic mechanisms, coupled with
novel treatments such as intrapleural chemotherapeutics targeting implicated signaling
pathways has the potential to improve the efficacy of our current management options.
The role of vascular endothelial growth factor in malignant pleural effusion formation is
currently under investigation, with current data strongly implicating vascular
endothelial growth factor-A (VEGF-A) as a critical cytokine in the formation of malignant
pleural effusions. Bevacizumab, a monoclonal antibody targeting VEGF-A, may have a
potential role in the management of malignant pleural effusions. Methods: Pleural
effusions samples from nine patients were collected and evaluated by ELISA for VEGF
(R&D, Minneapolis, MN). An angiogenesis protein array was completed in order to
perform a semi-quantitative comparison of peptide expression in malignant and nonmalignant
effusions (R&D, Minneapolis, MN). Cells were isolated from PE fluid and
phenotypes were evaluated using fluorescent cell sorting. Results: Malignant pleural
effusions were found to have significantly higher levels of VEGF-A as compared to
benign effusions. VEGF-A levels varied greatly between malignant pleural effusions.
Through semi-quantitative protein array, we found that malignant pleural effusions had
elevated levels of Leptin, CXCL4 and FGF-7; and decreased levels of MCP-1, IL-8, and IL-
1&#946; compared to benign pleural effusions. Malignant pleural effusions possessed
low levels of cytotoxic T cells and high levels of dendritic cells, monocytes and
mesothelial cells compared to benign effusions. Conclusions: These data reveal
increased levels of pro-angiogenic factors in malignant pleural effusions that may play a
role in the transmigration of malignant cells and formation of malignant pleural
effusions. These and other data, coupled with a few case reports, suggest the potential
benefit of intrapleural bevacizumab for the treatment of malignant pleural effusions.
Diabetes and Hypertension Screening in Local Immigrant Population
Diabetes and hypertension significantly contribute to the overall disease burden in the
United States and Minnesota (1, 2). The prevalence of diabetes in Hennepin County
stands at 6% (3, 4). While the health outcomes of recent immigrants are often same if
not better (so-called ''immigrant paradox''), the evidence suggests the risk of
developing these diseases in immigrant populations is positively correlated with the
length of residence in the United States (4, 5). As such, education about the risk factors
and longitudinal screening for the risk factors of these diseases amongst globally mobile
populations is of high importance to the overall healthcare quality and delivery. In an
attempt to contribute to addressing the stated needs in our local community, a
partnership between University of Minnesota Medical Residents and Common Bond
Communities in Skyline Tower – Skyline Health and Awareness Resident Project
(Doctors S.H.A.R.E) – was formed in 2010. Skyline Tower is a 24 floors housing complex
with over 500 units managed by Common Bond Communities with a high percentage of
residents of Somali and Oromo backgrounds. In cooperation with Common Bond and a
group of local elders, we identified an opportunity to improve housing residents
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David Shin
Emily H Shin, Gregory T
Christopherson, Wesley
M Jackson, Youngmi Ji,
Leon J Nesti
Alexa Weingarden
Matthew J. Hamilton,
Tatsuya Unno, Michael
J. Sadowsky, and
Alexander Khoruts
understanding of disease risk factors and an early identification of disease via onsite
screening. Consequently, UMN medical residents and students conducted monthly
education sessions and bi-weekly health screenings. Health screenings consisted of
measurements of blood pressure, and blood glucose as an indication of glycemic
control. Any abnormal readings were referred for follow-up. During the period from
February to May 2011, eight such screenings were performed. During this period, there
were a total of 191 encounters (note: encounters were counted, not unique patients)
where blood pressure or glucose, or both were checked. Out of 191 encounters, 142
had blood glucose checked and 45 had levels > 130 mg/dL. Skyline Tower residents with
blood glucose levels above 130 mg/dL were counseled to seek follow-up with their
primary care physician, if they had one; or to follow-up with one of the neighborhood
or free clinics. Based on the screening results, the insulin resistance appears to be of a
significant concern amongst the Skyline Tower population. To better understand its
scope and improve quality of care provided, future plans include use of HgA1c
measurement at screenings as a better marker of glycemic level and development of
pre and post educational intervention testing to measure the efficacy of educational
intervention sessions. References: (Omitted due to word count restriction, will provide
as a part of the poster)
An In Vitro Fibroproliferative Model for Studying Heterotopic Ossification
Introduction: Heterotopic ossification (HO) is characterized by ectopic bone formation
in soft tissue, as seen in a variety of conditions such as fibrodisplaysia ossificans
progressive, traumatic myositis ossificans, CNS injuries, and as a consequence
orthopaedic procedures. However, more serious forms of HO have been observed in
amputee patients suffering blast injuries, in which severe fibrosis has preceded clinical
presentation of HO within their affected extremities. These fibrotic regions appear to
create an osteoinductive environment that is responsible for the dysregulated wound
healing response, leading to inappropriate bone formation. A model of fibrosis has
previously been described showing TGF-ß1 dependent nodule formation in several
different cell lines. The purpose of this study was to confirm that our multipotent
progenitor cell line, isolated directly from traumatized muscle tissue, conformed to this
fibroproliferative model, in order to further study the cellular events that characterize
early-phase HO. Methods: Fibroblasts and mesenchymal progenitor cells (MPC)
isolated from traumatized muscle tissue were seeded on poly-L-lysine coated plates and
treated with TGF-ß1 with or without a small molecule inhibitor (SB431542) of TGFßR1.
Protein lysates were extracted at various time points following TGF-ß1 treatment.
Fibrotic nodules were assessed after 72h and 35d using light, fluorescence, and
scanning electron microscopy. Traumatized muscle tissue was visualized using the
same methodology. Results: Fibroblasts and MPCs were shown to form nodules in a
TGF-ß1 dose-dependent manner via the Smad2/3 pathway, which was actively inhibited
by the addition of SB431542. Microscopy revealed similar extracellular matrix
composition both histologically and morphologically between nodules and traumatized
muscle tissue. Conclusions: Previous studies have confirmed increased levels of native
TGF-ß1 gene expression within traumatized muscle tissue, and literature states TGF-ß1
driven nodule formation is indicative of fibrosis, a necessary precursor for HO. We have
created an in vitro model replicating the fibroproliferative lesions observed in early
stage HO, which will allow us to further investigate the underlying pathology of this
disease.
Changes in the fecal microbiome following fecal transplantation for severe Clostridium
difficile infection
Title: Changes in the fecal microbiome following fecal transplantation for severe
Clostridium difficile infection. Introduction: Clostridium difficile infection (CDI) is an
antibiotic-associated diarrheal disease which continues to increase in incidence and
severity worldwide. Clinically severe forms of CDI can lead to fulminant colitis, which is
associated with a high mortality rate (~50%) and necessitates surgical colectomy. Fecal
microbiota transplantation (FMT), which is generally recognized as a highly effective
treatment for recurrent CDI, may also present a non-surgical alternative for treatment
of severe disease. This study examines the changes in clinical parameters and fecal
microbiota following FMT in one such severe CDI case. Methods: The patient was
prepped for colonoscopy. Frozen fecal slurry from a standard donor, 50 g in 250 ml PBS,
was thawed and inserted via colonoscope into the cecum. C-reactive protein (CRP) and
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Elizabeth Gorecki
white blood cell (WBC) count were tracked daily during hospitalization. The patient was
treated with vancomycin for two weeks before receiving a second FMT as an
outpatient. Fecal samples were collected before FMT, for five days following the first
FMT, on the day of the second FMT, and for two days after the second FMT. DNA was
extracted from all samples, PCR amplified using primers specific to the V6 region of the
bacterial 16S rDNA gene, and sequenced on the Illumina platform. Sequences were
analyzed with mothur software, clustered into operational taxonomic units (OTUs), and
classified using Ribosomal Database Project 7. Mothur-derived FASTA, name, and group
files were converted into a UniFrac format using a custom Perl script. Results:
Colonoscopic images taken during the first FMT revealed pseudomembranous colitis in
the proximal colon, indicative of severe CDI, which had resolved by the second FMT.
CRP and WBC decreased following the first FMT. Principal coordinate analysis based on
UniFrac results revealed that bacterial communities on the first day after the first FMT
and after the second FMT were closer to the donor sample than the initial patient
sample. At the phylum level, OTUs in the donor sample, pre-FMT sample, and samples
following the second FMT were dominated by Bacteroidetes and Firmicutes, while
samples two days after the first FMT until the second FMT were dominated by
Proteobacteria. Conclusions: FMT is a potential alternative to colectomy for the
treatment of severe CDI which avoids the morbidity and mortality associated with
surgery. Recovery after the second FMT in this patient was concurrent with decreases
in Proteobacteria. Patients treated for severe CDI with FMT may require two
procedures: one for stabilization of the patient, and one to ensure long-lasting clinical
recovery. Two FMT procedures may also be necessary to ensure engraftment of donor
microbiota.
The physiology of the Creighton Model Fertility Care System: physiologic knowledge of
patients and their appreciation for their chosen fertility management or family planning
system
Background: Most sexually experienced American women have used some method of
contraception to manage their fertility. Oral contraceptives (OC) are the most popular
method but often produce side effects. The Creighton Model Fertility Care System
(CrMS) is an alternative with no side effects and is comparatively effective to OC. The
CrMS is a fertility awareness based method (FABM) that requires a woman to monitor
her cervical mucus as indicator of fertility. The CrMS has method and use effectiveness
rates comparable to those of OC, yet is has a lower discontinuation rate after one year
than that for OC. Purpose: This study analyzed the relationship between participants’
satisfaction in their current fertility management method (FMM) and their knowledge
of the physiology of reproduction and fertility by using a descriptive survey design.
Methods: Females of childbearing age who were using either the CrMS or OC were
recruited in primary care clinics. Participants were invited to log in to an online survey
and answer questions about what type of FMM they used, their reproductive
knowledge, and their satisfaction with their FMM using a visual analogue scale (VAS).
Participants also answered two open ended questions about why they chose either OC
or FABM and what it was they appreciated about their method of choice. Quantitative
data were analyzed with descriptive statistics and qualitative data were analyzed for
themes. Results: Forty-nine women completed the entire survey and an additional 9
women partially filled out the survey. Of the women who completed the survey, 41
used the CrMS, 7 used another type of FABM and 1 used OC. Participants scored and
average of 7.94 questions correctly out of a possible 10 questions. Participants
expressed high satisfaction with their method of choice (84 to 96 out of 100 on the
VAS). Several themes emerged from the qualitative data collected from participants
using the CrMS: importance of a method that fit with their religious view, importance of
a method that was natural and without side effects, increased diagnostic power for
women’s health and infertility issues, versatility of the method to both achieve and
avoid pregnancy, and the strengthening of communication and relationship with one’s
partner because of shared responsibility for FMM. Discussion: Our study provides
evidence that women using CrMS have high satisfaction in their FMM. Exploration of
the themes in this study many shed light on why CrMS users have a low discontinuation
rate. In this sample, more of the participants used CrMS than OC, and therefore, a
comparison of the two methods was not statistically possible. Given the high
satisfaction, evidence for efficacy, and the inexpensiveness of the CrMS method,
women with concerns about hormonal side effects may be able to effectively manage
fertility with CrMS.
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Mariel Lougee
Gina Piscitello
Duong CD, Henderson
KM, Clark CJ, Janssen I,
Matthews KA, Sutton-
Tyrrell K, Everson-Rose
SA
Healthcare Usages in the Chronically and Transiently Homeless: Barriers to Healthcare
Utilization
Introduction: Current data from the National Alliance to End Homelessness found that
636,017 Americans experience homelessness on any given night. Research has
previously found that persons who are chronically homeless and disabled are at greater
risk of negative health consequences due to increased prevalence of mental health
issues, substance abuse disorders, and a greater level of disconnection from
preventative medical care. Stemming from this previous work, the goals of this research
project were to probe the barriers to utilization of healthcare by the transiently and
chronically homeless in Los Angeles, CA. By examining individuals’ perspectives and
perceptions of the medical care that was available to them as well as their assumptions
of the provider system, we hoped to help uncover what challenges exist for
practitioners and what could be done to help such a vulnerable patient population.
Methods: Surveys were administered over an 8 week period at four local drop-in
centers. Respondents included 106 men and women who currently self-identified as
homeless in the LA area. Individuals were selected at random at each site and only
excluded for age (under 18), current intoxication, or if they were not orientated to
space and time through the use of a mini-mental status exam. The survey assessed past
medical history, health care usage, and attitudes towards the healthcare system.
Results: The first and most basic result showed that in almost every demographic
criterion this population is different from traditional epidemiological and demographic
data. From issues of insurance and employment, to conditions of mental illness and
hypertension, these individuals suffer from more medical conditions and less access to
care. Secondly, recognizing that these individuals represented a non-traditional patient
population, it became clear that they also had different and unique barriers to receiving
care. Their increased use of emergency rooms, diminished continuity of primary care,
and increased need for assistance with transportation and cost of appointments all lead
to generally inadequate and inappropriate utilization of care. Thirdly, a majority of
individuals perceived that clinic staff and physicians were judgmental about their
homeless status. This feeling of being defined by one’s homelessness seemed to be
seen in many facets of their medical care, all of which suggested different strategies
from the medical profession towards this population. Conclusions: These results
showed a need for a different approach for a very different patient population who
have very different needs compared to the traditional patient. An interdisciplinary
approach with a constant awareness of these individuals’ multi-factorial barriers will
allow physicians and clinic staff to begin to improve the physical and mental health of
the homeless as well as alter the perceptions about the way the label of “homeless”
interferes with their medical experiences.
Effect of Psychosocial Stressors on Aortic Pulse Wave Velocity Progression in Black and
non-Hispanic White Women
Introduction: Increased arterial stiffening, described by aortic pulse-wave velocity
(aPWV) progression, may be affected by psychosocial stressors in middle-aged women.
aPWV is associated with cardiovascular disease (CVD) and mortality, and is known to
differ by race. This study examined associations of five psychosocial stressors previously
linked to CVD risk in women with progression of aPWV in a sample of 280 Black and
non-Hispanic White women, and whether associations differed by race. Methods: Data
were from the Study of Women’s Health Across the Nation Heart Study, a longitudinal
study of subclinical CVD progression in middle-aged women. The five psychosocial
stressors assessed at baseline were anger, anxiety, depressive symptoms, and
perceived discrimination, and hostility. Linear regression models examined the
associations of these five stressors with progression of aPWV (values were logtransformed,
due to skewness) over an average of 2.3 years. Analyses included baseline
measurements of CVD risk factors and demographic information. Results: In the risk
factor-adjusted models, with covariates for age, race, and cardiovascular risk factors,
the associations of anger (estimate, 0.0008), anxiety (estimate, -0.0072), depressive
symptoms (estimate, -0.0002), perceived discrimination (estimate, -0.0255), and
hostility (estimate, -0.0042) with aPWV progression all were non-significant (all P>0.3),
and no difference was seen by race. Compared to White women, Black women had
significantly higher baseline hostility and perceived discrimination measurements with
anxiety trending toward significance. aPWV increased by 94.2 cm/s and 46.0 cm/s in
Black and White women, respectively, between baseline and follow up. Traditional CVD
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Juan Reyes Genere
Monica Maalouf,
Natalia Lipin, Brian Sick,
MD
Joshua Dorn, MD
Brian Sick, MD
risk factors, including waist circumference, age, and systolic blood pressure, were
predictors of aPWV progression (all P

least one group visit, those that had received a diabetic foot exam in the past year
increased from 68.8% to 83.3%. Outcomes of the individuals who attended 9 or more
group visits compare favorably to those who attended 1-8 group visits and those who
were not part of the group visit cohort. Of the patients who attended 9 or more group
visits 45.5% reached optimal care whereas only 16.7% of those attending less than 9
group visits and 21% of those not attending a group visit reached optimal care.
Conclusion: The results show that there is a positive correlation between attending
group visits and meeting goals of care. This is further true with the more group visits
one attends. Group visits provide increased time with the provider, an avenue to have
questions answered, and camaraderie with fellow patients who are facing similar
problems. They are also very feasible to organize and a low cost intervention that has
great benefit both subjectively, as noted by patients, and objectively in outcomes.
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