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Article

A Drug Regimen for Progressive Familial Cholestasis Type 2.

Pediatrics - 1 Jan 2018

Malatack J Jeffrey, Doyle Daniel

Abstract excerpt

Progressive familial cholestasis type 2 is caused by a genetically determined absence or reduction in the activity of the bile salt export pump (BSEP). Reduction or absence of BSEP activity causes a failure of bile salt excretion, leading to accumulation of bile salts in hepatocytes and subsequent hepatic damage. Clinically, patients are jaundiced, suffer from severe intractable pruritus, and evidence progressive...

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Share to ATP Binding Cassette Transporter, Subfamily B, Member 11
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 29284646
DOI: 10.1542/peds.2016-3877

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