Current and emerging adjuvant therapies in biliary atresia

Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure. Preventing liver transplantation, or even delaying eventual liver transplantation, is the key to improving long-term outcom...

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Published inFrontiers in pediatrics Vol. 10; p. 1007813
Main Authors Fligor, Scott C, Hirsch, Thomas I, Tsikis, Savas T, Adeola, Andrew, Puder, Mark
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 14.10.2022
Subjects
biliary atresia
cholangitis
cholestasis
glucocorticoids
liver transplant
Pediatrics
therapeutics
biliary atresia
therapeutics
children
cholestasis
survival
glucocorticoids
cholangitis
liver transplant
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Summary:Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure. Preventing liver transplantation, or even delaying eventual liver transplantation, is the key to improving long-term outcomes. This review first examines the commonly used adjuvant therapies in post-HPE biliary atresia and the strength of the evidence supporting these therapies. Next, it examines the evolving frontiers of management through a comprehensive evaluation of both recently completed and ongoing clinical trials in biliary atresia. Promising therapies used in other cholestatic liver diseases with potential benefit in biliary atresia are discussed. Improving post-HPE management is critical to prevent complications, delay liver transplantation, and ultimately improve the long-term survival of patients with biliary atresia.
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Specialty Section: This article was submitted to Pediatric Gastroenterology, Hepatology and Nutrition, a section of the journal Frontiers in Pediatrics
Edited by: Satoshi Ieiri, Kagoshima University, Japan
Reviewed by: Magd Kotb, Cairo University, Egypt Kenneth KY Wong, The University of Hong Kong, SAR China
ISSN:2296-2360
2296-2360
DOI:10.3389/fped.2022.1007813