Diagnosis and management of Alagille and progressive familial intrahepatic cholestasis

Author:

Cheng Katherine1,Rosenthal Philip12

Affiliation:

1. Department of Pediatrics Gastroenterology, Hepatology and Nutrition, University of California San Francisco, San Francisco, California, USA

2. Department of Surgery, University of California, San Francisco, San Francisco, California, USA

Abstract

Alagille syndrome and progressive familial intrahepatic cholestasis are conditions that can affect multiple organs. Advancements in molecular testing have aided in the diagnosis of both. The impairment of normal bile flow and secretion leads to the various hepatic manifestations of these diseases. Medical management of Alagille syndrome and progressive familial intrahepatic cholestasis remains mostly targeted on supportive care focusing on quality of life, cholestasis, and fat-soluble vitamin deficiency. The most difficult therapeutic issue is typically related to pruritus, which can be managed by various medications such as ursodeoxycholic acid, rifampin, cholestyramine, and antihistamines. Surgical operations were previously used to disrupt enterohepatic recirculation, but recent medical advancements in the use of ileal bile acid transport inhibitors have shown great efficacy for the treatment of pruritus in both Alagille syndrome and progressive familial intrahepatic cholestasis.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Hepatology

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