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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:
1) occurs during the 3rd trimester
2) nausea
3) abdominal pain
4) jaundice
5) coagulopathy
6) encephalopathy
7) features of pre-eclampsia, eclampsia
8) hypoglycemia
Laboratory:
1) serum AST 100-1000 U/L
2) serum ALT 100-1000 U/L
3) serum bilirubin: normal,elevated if severe [1]
4) complete blood count
a) evaluate bleeding
b) leukocytosis
c) thrombocytopenia
d) hemolysis [1]
4) serum glucose may be low
5) serum uric acid may be high
6) prothrombin time, INR & aPTT may be increased
7) plasma D-dimer for disseminated intravascular coagulation (DIC)
8) HADHA gene mutation
Radiology:
- ultrasound: increased hepatic echogenicity
Complications:
1) disseminated intravascular coagulation (DIC)
2) acute onset of jaundice & hepatic failure
3) hepatic encephalopathy [1], coma
4) excessive bleeding
5) liver failure & excessive bleeding may occur for several days postpartum
6) often fatal
7) fetal loss
Differential diagnosis:
- intrahepatic cholestasis of pregnancy
- HELLP syndrome (microangiopathic hemolytic anemia)
- ultrasound: increased hepatic echogenicity distinguishes
Management: (of complications)
1) delivery of fetus for complication of DIC
2) supportive therapy
a) transfusions as needed
b) control of hypoglycemia
3) generally resolves with delivery of infant
4) recurrence with subsequent pregnancies is rare
General
liver disease during pregnancy
steatosis; fatty liver; nonalcoholic fatty liver disease (NAFLD); metabolic dysfunction-associated steatotic liver disease (MASLD)
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15,
16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006,
2009, 2012, 2015, 2018, 2022.
- Medical Knowledge Self Assessment Program (MKSAP) 19
Board Basics. An Enhancement to MKSAP19.
American College of Physicians, Philadelphia 2022
- Bacak SJ, Thornburg LL.
Liver Failure in Pregnancy.
Crit Care Clin. 2016 Jan;32(1):61-72. Review.
PMID: 26600444