Orphanet: Intrahepatic cholestasis of pregnancy

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Intrahepatic cholestasis of pregnancy

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.

ORPHA:69665

Classification level: Disorder

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: 147480 614972
UMLS: -
MeSH: C535932
GARD: 9804
MedDRA: -

Summary

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 - Last update: May 2007

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Intrahepatic cholestasis of pregnancy

ORPHA:69665

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