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Intrahepatic cholestasis of pregnancy

ORPHA:69665
Synonym(s) Gravidic intrahepatic cholestasis
Pregnancy-related cholestasis
Recurrent intrahepatic cholestasis of pregnancy
Prevalence Unknown
Inheritance Multigenic/multifactorial
or Not applicable
Age of onset Adult
ICD-10
  • O26.6
OMIM
UMLS -
MeSH
  • C535932
GARD
MedDRA -

Summary

Disease definition

Intrahepatic cholestasis of pregnancy (ICP) is a cholestatic disorder characterized by (i) pruritus with onset in the second or third trimester of pregnancy, (ii) elevated serum aminotransferases and bile acid levels, and (iii) spontaneous relief of signs and symptoms within two to three weeks after delivery.

Epidemiology

ICP is observed in 0.4-1% of pregnancies in most regions of Central and Western Europe and in North America. In Chile and Bolivia, as well as Scandinavia and the Baltic states, roughly 5-15% and 1-2%, respectively, of pregnancies are associated with ICP.

Clinical description

Intrahepatic cholestasis of pregnancy increases the risk of preterm delivery (19-60%), meconium staining of amniotic fluid (27%), fetal bradycardia (14%), fetal distress (22-41%), and fetal loss (0.4-4.1%), particularly when associated with fasting serum bile acid levels >40 micromol/L.

Etiology

Genetic and hormonal factors, as well as environmental effects, may contribute to the pathogenesis of ICP.

Management and treatment

The hydrophilic bile acid, ursodeoxycholic acid (10-20 mg/kg/d), is today regarded as the first line treatment for intrahepatic cholestasis of pregnancy. Induction of delivery is recommended in the 38th week when lung maturity has been established.

Expert reviewer(s)

  • Pr U.H.W. [Ulrich] BEUERS
  • Pr Thomas PUSL

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Detailed information

Review article
Clinical genetics review
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