Fatty Liver of Pregnancy, Acute

Acute liver failure in the third trimester of pregnancy

Mortality of 7–23% with early delivery

Likely cause is the result of poor placental mitochondrial function

Many cases may be due to fetal long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency

Fatty engorgement of hepatocytes seen on CT of liver and on biopsy

Incidence of 1:14,000 deliveries

Gradual onset of flu-like symptoms

Progression to jaundice, encephalopathy, disseminated intravascular coagulation, and death

Signs of liver failure are present on examination

All etiologies of acute liver failure should be considered, but transaminase levels are lower (500–1000 units/mL) in acute fatty liver of pregnancy

Marked elevation of alkaline phosphatase

Only moderate alanine aminotransferase and aspartate aminotransferase elevations

Hypocholesterolemia and hypofibrinogenemia are typical

Coagulopathy is also frequently seen with depressed procoagulant protein production

Kidney function should be assessed for hepatorenal syndrome

White blood cell count is elevated; platelet count is depressed

Hypoglycemia may be extreme

Intensive supportive care with ICU-level observation is essential and typically includes administration of blood products and glucose as well as correction of acidemia

Diagnosis mandates immediate delivery, preferably vaginally

Rare cases of liver transplantation have been reported

Resolution of encephalopathy occurs over days and laboratory derangements occurs over days with supportive care

Recurrence rates are unclear