Book of Medical Disorders in Pregnancy - Tintash

10 mg per dL (17.1 to 171.0 µmol per
L).Some children of mothers with acute
fatty liver of pregnancy have been noted
to express homozygous deficiency of
long chain 3-hydroxyacyl-CoA
dehydrogenase, resulting in severe
metabolic and neurologic consequences
to the infants. 32,33 Their mothers were
found to exhibit a heterozygous
deficiency of long chain 3-hyd-roxyacyl-
CoA dehydrogenase, contributing to
acute fatty liver of pregnancy. Such
defects in fatty acid oxidation are
initially suggested by elevations in
urinary organic acid levels and in plasma
carnitine and acyl carnitine levels, detected
after an overnight fast. 32 Recurrent
acute fatty liver of pregnancy has
been reported in mothers expressing
heterozygous long-chain 3-hydroxyacyl-
CoA dehydrogenase deficiency. Ursodeoxycholic
acid, at dosages of 15 mg per
kg per day, has been the most successful
therapy for cholestasis of pregnancy.
The treatment of acute fatty liver of
pregnancy is expeditious delivery and
intensive care. Patients usually improves
promptly following delivery and, in the
absence of long chain 3hydroxyacylCoA
dehydrogenase deficiency,
the prognosis in pregnancies
following acute fatty liver of pregnancy
is good.
Hepatic rupture and infarction:
Hepatic rupture and infarction, extremely
rare complications of Pre
eclamptic liver disease, usually occur in
the third trimester. The incidence of
hepatic rupture varies from one in
40,000 to one in 250,000 pregnancies 35 ;
hepatic in-farction is even rarer. Older
multi-gravida mothers with preeclampsia
(75 to 85 percent) are at higher risk. Less
commonly, hepatic rupture complicates
growth of hepatic adenomata or other
120
masses during pregnancy. 3 Hepatic
rupture most commonly involves the
right lobe. It is believed to be a continuum
of preeclampsia, in which areas
of coalescing hemorrhage result in
thinning of the capsule and intraperitoneal
hemorrhage. Case reports
have docu-mented numerous pseudo a
eurysm in the area of hemorrhage,
raising the possibility of a vasculopathy
contributing to this rare disorder. Patients
with hepatic rupture typically present in
shock, with preceding right upper
quadrant pain, hypertension, elevated
transaminase levels (greater than 1,000
IU per L) and coagulopathy. 4 Therapy
for hepatic rupture has included
transfusion of blood products and intravenous
fluids, surgical evacuation and
arterial embolization. 4 These therapies
have met with only moderate success; a
59 to 70 percent maternal mortality rate
and a 75 percent perinatal mortality rate
have been noted in hepatic rupture. 4 Late
complications arising after treatment of
hepatic rupture include hepatic abscess
formation and pleural effusions. Hepatic
infarction is best detected by using
computed tomographic scans or magnetic
resonance imaging. Patients typically
present with fever and marked
elevations in transaminase levels. In
surviving patients, liver function and
histopathology are normal within six
months of delivery. Intrahepatic hemorrhage
has been reported to recur in a
minority of subsequent pregnancies.
Fig8.6: Shows cirrhosis of liver in the
specimen.