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For further information, see CMDT Part 19-35: Acute Fatty Liver of Pregnancy

Key Features

  • 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

Clinical Findings

  • 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

Diagnosis

  • 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

Treatment

  • 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

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