Book of Medical Disorders in Pregnancy - Tintash
Book of Medical Disorders in Pregnancy - Tintash
Book of Medical Disorders in Pregnancy - Tintash
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women (<strong>in</strong> the second and third<br />
trimesters) with choledocholithiasis<br />
us<strong>in</strong>g m<strong>in</strong>imal fluoroscopy and lead<br />
aprons to shield the abdomen. All <strong>of</strong> the<br />
women delivered healthy babies at term.<br />
<strong>Pregnancy</strong> specific liver disease:<br />
Intrahepatic cholestasis <strong>of</strong> pregnancy:<br />
Intrahepatic cholestasis <strong>of</strong> pregnancy<br />
occurs <strong>in</strong> 0.01 percent <strong>of</strong> pregnancies <strong>in</strong><br />
the United States. It typically arises <strong>in</strong><br />
the third trimester <strong>of</strong> pregnancy,<br />
although it has been reported as early as<br />
13 weeks' gestation. The<br />
pathophysiology <strong>of</strong> <strong>in</strong>trahepatic<br />
cholestasis <strong>of</strong> pregnancy rema<strong>in</strong>s poorly<br />
understood. 19 Pruritus alone occurs <strong>in</strong> 80<br />
percent <strong>of</strong> patients; pruritus and jaundice<br />
develop <strong>in</strong> 20 percent <strong>of</strong> patients.<br />
Laboratory abnormalities <strong>in</strong>clude a<br />
bilirub<strong>in</strong> level less than 5 mg per dL<br />
(85.5 µmol per L), m<strong>in</strong>imal or no<br />
elevation <strong>in</strong> transam<strong>in</strong>ase, cholesterol<br />
and triglyceride levels, and <strong>in</strong>frequent,<br />
mild to moderate steatorrhea. Liver<br />
histopathology reveals centrilobular bile<br />
stasis. 20 Intrahepatic cholestasis <strong>of</strong><br />
pregnancy is associated with a 12 to 44<br />
percent <strong>in</strong>cidence <strong>of</strong> prematurity, a 16 to<br />
25 percent <strong>in</strong>cidence <strong>of</strong> fetal distress and<br />
an <strong>in</strong>creased per<strong>in</strong>atal mortality rate (1.3<br />
to 3.5 percent). A clear racial and<br />
genetic predisposition for this disorder<br />
has been described. Intrahepatic<br />
cholestasis complicates 0.01 to 0.02<br />
percent <strong>of</strong> pregnancies <strong>in</strong> North<br />
America, 1 to 1.5 percent <strong>of</strong> pregnancies<br />
<strong>in</strong> Sweden and 5 to 21 percent <strong>of</strong> pregnancies<br />
<strong>in</strong> Chile. 20 The disease is rare <strong>in</strong><br />
black patients. 20 A strong family history<br />
<strong>of</strong> cholestasis <strong>of</strong> pregnancy is typically<br />
described by the patient. 20 K<strong>in</strong>dred<br />
studies reveal alterations <strong>in</strong><br />
bromosulfophthale<strong>in</strong> clearance follow<strong>in</strong>g<br />
estrogen treatment <strong>in</strong> both male and<br />
118<br />
female relatives <strong>of</strong> women affected by<br />
<strong>in</strong>trahepatic cholestasis <strong>of</strong> pregnancy. 19<br />
Multiple medications have been tried as<br />
treatments for cholestasis <strong>of</strong> pregnancy. 19<br />
parenteral vitam<strong>in</strong> K (phytonadione;<br />
Aqua MEPHYTON) supplementation is<br />
advocated <strong>in</strong> patients with prolonged<br />
cholestasis (secondary to malabsorption<br />
<strong>of</strong> this fat-soluble vitam<strong>in</strong>). Ursodeoxycholic<br />
acid (Actigall), given at dosages<br />
<strong>of</strong> 15 mg per kg per day, has been the<br />
most successful therapy for cholestasis<br />
<strong>of</strong> pregnancy, as it ameliorates both the<br />
pruritus and liver function abnormalities<br />
and is well-tolerated by both mother and<br />
fetus. 21 Ursodeoxycholic acid has been<br />
proved safe <strong>in</strong> trials <strong>of</strong> cholestatic liver<br />
disease <strong>in</strong> <strong>in</strong>fants, children and adults.<br />
Studies <strong>in</strong> rats, mice and rabbits have<br />
revealed no teratogenicity or other negative<br />
effects on the develop<strong>in</strong>g fetus. Studies<br />
<strong>in</strong> humans exam<strong>in</strong><strong>in</strong>g the use <strong>of</strong><br />
ursodeoxycholic acid <strong>in</strong> pregnancy have<br />
been uncontrolled and limited by small<br />
patient numbers. However, <strong>in</strong> pregnant<br />
patients with cholestatic liver disease,<br />
the pruritus can be severely disabl<strong>in</strong>g,<br />
and ursodeoxycholic acid therapy<br />
provides safe and effective relief.<br />
Cholestyram<strong>in</strong>e (Questran) b<strong>in</strong>ds bile<br />
acids and may improve pruritus; how<br />
ever, it may exacerbate steatorrhea and<br />
does not alter liver function or fetal<br />
prognosis. 19 Phenobarbital has not been<br />
shown to improve pruritus or alter liver<br />
tests and may cause neonatal respiratory<br />
depression. Patients exhibit<strong>in</strong>g cholestasis<br />
<strong>of</strong> pre-gnancy should receive close<br />
fetal surveillance at delivery. Symptoms<br />
<strong>of</strong> cholestasis usually resolve with<strong>in</strong> two<br />
days <strong>of</strong> delivery. Elevated serum<br />
bilirub<strong>in</strong> and alkal<strong>in</strong>e phosphatase levels<br />
re-turn to normal four to six weeks after<br />
delivery. 3 Cholestasis <strong>of</strong> pregnancy re-