fatty liver of pregnancy

Epidemiology

rare

Pathology

• cholestasis
• microvesicular fat accumulation in hepatocytes
• liver biopsy shows necrosis, fibrosis, steatosis & hemorrhage

Genetics

• associated with defects in HADHA gene

Clinical manifestations

• occurs during the 3rd trimester
• nausea
• abdominal pain
• jaundice
• coagulopathy
• encephalopathy
• features of pre-eclampsia, eclampsia
• hypoglycemia

Laboratory

• serum AST 100-1000 U/L
• serum ALT 100-1000 U/L
• serum bilirubin: normal,elevated if severe
• complete blood count
  • evaluate bleeding
  • leukocytosis
  • thrombocytopenia
  • hemolysis
• serum glucose may be low
• serum uric acid may be high
• prothrombin time, INR & aPTT may be increased
• plasma D-dimer for disseminated intravascular coagulation (DIC)
• HADHA gene mutation

Radiology

• ultrasound: increased hepatic echogenicity

Complications

• disseminated intravascular coagulation (DIC)
• acute onset of jaundice & hepatic failure
• hepatic encephalopathy, coma
• excessive bleeding
• liver failure & excessive bleeding may occur for several days postpartum
• often fatal
• fetal loss

Differential diagnosis

• intrahepatic cholestasis of pregnancy
• HELLP syndrome (microangiopathic hemolytic anemia)
• ultrasound: increased hepatic echogenicity distinguishes

Management

(of complications)

• delivery of fetus for complication of DIC
• supportive therapy
  • transfusions as needed
  • control of hypoglycemia
• generally resolves with delivery of infant
• recurrence with subsequent pregnancies is rare

More general terms

• liver disease during pregnancy
• steatosis