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intrahepatic cholestasis of pregnancy (obstetric cholestasis)
Epidemiology: 2nd most common cause of jaundice in pregnancy
Pathology:
- multifactorial liver disorder of pregnancy
- cholestasis results from abnormal biliary transport from the liver into the small intestine
Genetics:
- genetically determined
- associated with defects in ABCB4
Clinical manifestations:
1) generally presents in 2nd or 3rd trimester
2) pruritus, often intense with or without jaundice
3) jaundice may be profound
4) pruritus may become more severe with advancing gestation & cholestasis
5) fetal manifestations
a) fetal distress
b) spontaneous premature delivery
c) intrauterine death
6) patients have spontaneous & progressive disappearance of cholestasis after delivery
Laboratory:
- pattern of cholestasis
- elevated serum bile acids
- indication for early delivery [1]
- serum bilirubin elevated, < 6 mg/dL (case with serum bilirubin of 2.5 mg/dL)
- serum alkaline phosphatase markedly elevated
- serum AST < 200-500 U/L (case with serum ALT of 55 U/L)
- serum ALT < 200-500 U/L (case with serum AST of 30 U/L)
- complete blood count: no thrombocytopenia
- no evidence of hemolysis
- no evidence of disseminated intravascular coagulation
Differential diagnosis:
- fatty liver of pregnancy
- HELLP syndrome
- cholestatic features, pruritus & markedly increased serum alkaline phosphatase distinguishes
Complications:
- benign disorder for mother, but associated with increased fetal mortality
Management:
1) ursodeoxycholic acid
2) cholestyramine for pruritus
3) generally resolves promptly after delivery
4) recurrence is common is subsequent pregnancies or with the use of oral contraceptives
5) delivery at 1st sign of fetal distress [1]
General
liver disease during pregnancy
cholestasis
dermatoses of pregnancy
Database Correlations
OMIM 147480
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15,
16, 18. American College of Physicians, Philadelphia 1998, 2006,
2009, 2012, 2018
- OMIM :accession 147480
- Bacq Y, le Besco M, Lecuyer AI et al
Ursodeoxycholic acid therapy in intrahepatic cholestasis
of pregnancy: Results in real-world conditions and factors
predictive of response to treatment.
Dig Liver Dis. 2017 Jan;49(1):63-69..
PMID: 27825922