Internal Medicine

0521779407-01 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 20:45

22 Acute Fatty Liver of Pregnancy

Signs and Symptoms

■Jaundice, asterixis, may be preeclamptic

tests

Laboratory

■True hepatic failure with prolonged PT, low fibrinogen (usually high

in pregnancy)

■AST<ALT, usually <1,000

■Elevated bilirubin

■Hyperammonemia, hypoglycemia

■Normal platelet count unless severe and DIC

Liver Biopsy

■Central pallor with microvesicular fat on special stains (plan these

with Pathology before doing the biopsy), disarray of hepatocytes,

may mimic viral hepatitis; biopsy usually not needed as diagnosis

based on clinical grounds.

differential diagnosis

■HELLP syndrome (not true hepatic failure, low platelet count)

■Viral hepatitis (+serologies, risk factors on history; possible hepatitis

E if from endemic area, or herpes simplex hepatitis) or drug induced

fulminant hepatic failure (eg acetaminophen ingestion)

management

What to Do First

■Obstetric consultation, consider delivery

General Measures

■Maximal support, may require ICU, dialysis, etc

specific therapy

■Delivery/support

■Liver transplantation not indicated except very rarely

follow-up

■R/O associated defect in beta oxidation of fatty acids, DNA testing

available for the most common defect associated with acute fatty

liver of pregnancy, long chain 3-hydroxyl-acyl CoA dehydrogenase.

Test patient, her baby and baby’s father

■Expect full recovery. May recur in subsequent pregnancies