Acute fatty liver of pregnancy (AFLP) is characterized histologically by microvesicular fatty cytoplasmic infiltration of hepatocytes with minimal hepatocellular necrosis.

• Acute fatty metamorphosis
• Acute yellow atrophy
• Acute fatty liver of pregnancy (AFLP)

• Initial manifestations:
  1. Nausea and vomiting (50% to 82%)
  2. Abdominal pain (32% to 70%)
  3. Malaise (40% to 78%)
  4. Jaundice (60% to 100%)
• Hypoglycemia
• Late manifestations:
  1. Fulminant hepatic failure
  2. Hepatic encephalopathy
  3. Acute kidney injury
  4. Pancreatitis
  5. Gastrointestinal and uterine bleeding
  6. Pulmonary edema
  7. Disseminated intravascular coagulation
  8. Seizures
  9. Coma
• Liver:
  1. Usually small
  2. Normal or enlarged in preeclampsia, eclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), and acute hepatitis
  3. Coexistent preeclampsia in up to 46% of patients
  4. Ascites can be present

• Fetal LCHAD deficiency results in transplacental passage of excess fetal fatty acids and subsequent accumulation in the maternal liver.
• Postulated that inhibition of mitochondrial oxidation of fatty acids may lead to microvesicular fatty infiltration of liver.
• Fatty metamorphosis of preeclamptic liver disease believed to be of different etiology.

• Acute gastroenteritis
• Preeclampsia or eclampsia with liver involvement
• HELLP syndrome
• Acute viral hepatitis
• Fulminant hepatitis
• Acetaminophen toxicity
• Drug-induced hepatitis caused by halothane, phenytoin, methyldopa, isoniazid, hydrochlorothiazide, or tetracycline
• Intrahepatic cholestasis of pregnancy
• Gallbladder disease
• Reye syndrome
• Hemolytic-uremic syndrome
• Budd-Chiari syndrome
• Systemic lupus erythematosus

• A clinical diagnosis is based predominantly on physical and laboratory findings (Swansea criteria).
• Most definitive diagnosis is through liver biopsy with oil red O staining and electron microscopy.
• Liver biopsy is reserved for atypical cases only and only after any existing coagulopathy is corrected with fresh frozen plasma because of concerns for excessive bleeding.

Tests to determine the following:

• Hypoglycemia (often profound <60 mg/dl)
• Hyperammonemia
• Elevated aminotransferases (usually <500 U/ml)
• Thrombocytopenia
• Leukocytosis (white blood cell count >15,000)
• Hyperbilirubinemia (usually <10 mg/dl)
• Hypoalbuminemia
• Hypofibrinogenemia (<300 mg/dl)
• Disseminated intravascular coagulation (DIC) (in 75%)

• Ultrasound: Best used to rule out other diseases in the differential diagnosis such as gallbladder disease or thrombosis
• Computed tomography scan: Plays minimal role because of a high false-negative rate

• Patient is admitted to intensive care unit for stabilization.
• Fetus is delivered; spontaneous resolution usually follows delivery.
• Mode of delivery is based on obstetric indications and clinical assessment of disease severity.

• Decrease in endogenous ammonia through dietary protein restriction; neomycin 6 to 12 g/day PO to decrease presence of ammonia-producing bacteria; magnesium citrate 30 to 50 ml PO or enema to evacuate nitrogenous wastes from colon.
• Administration of intravenous fluids with glucose to keep glucose levels >60 mg/dl.
• Coagulopathy corrected with fresh frozen plasma.
• Avoidance of drugs metabolized by liver.
• Aggressive avoidance and treatment for nosocomial infections; consideration of prophylactic antibiotics.
• Monitor closely for development of complications such as hepatic encephalopathy, pulmonary edema, DIC, and respiratory arrest.
• Plasma exchange after delivery may result in a lower maternal mortality.

Orthotopic liver transplantation is the only treatment for irreversible liver failure.

• Before 1980, both maternal and fetal mortality rates were approximately 85%
• Maternal mortality rates have improved to <2% to 15% and perinatal mortality rates have improved to 5% to 20%
• Usually, rapid return of liver function to normal after delivery
• Minimal risk of recurrence with future pregnancies

• To tertiary health care facility as soon as diagnosis is suspected.
• 20% of infants born to mothers with AFLP have LCHAD; after birth, all infants should be evaluated for the deficiency.

• Eclampsia (Related Key Topic)
• Preeclampsia (Related Key Topic)
• HELLP Syndrome (Related Key Topic)