Benign recurrent intrahepatic cholestasis.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis is a rare autosomal recessive disorder characterized by repeated episodes of intense pruritus and jaundice . Each attack lasts from several weeks to months before resolving spontaneously . Patients are completely asymptomatic for months to years between symptomatic periods . The disorder does not lead to progressive liver disease . Although attacks seem to be associated with a viral prodrome , an inciting viral agent or toxin has not been defined . Genetic studies have mapped the defect of this disorder to the long arm of chromosome 18 and a gene that codes for a P-type ATPase , which appears to be involved in aminophospholipid transport . Therapy during symptomatic periods is supportive and aimed at relief of severe pruritus until the episode resolves spontaneously .

Diseases
Validation

Diseases presenting "progressive liver disease" symptom

benign recurrent intrahepatic cholestasis

megacystis-microcolon-intestinal hypoperistalsis syndrome

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