Barbara A Haber1, Jessi Erlichman, Kathleen M Loomes. 1. Associate Professor of Pediatrics The Childrens Hospital of Philadelphia, Division of GI, Hepatology & Nutrition, Philadelphia, PA 19104, USA. haber@E-mail.chop.edu
Abstract
BACKGROUND: Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction before 2 months of age. Untreated BA is a uniformly fatal disease and even with our current therapies only 50% of children with BA will be transplant-free by 2 years of age. Despite descriptions of this disorder dating back to the 1800s our current therapies are palliative. They focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. OBJECTIVE: To present the literature supporting current treatment strategies and potential future therapies. METHOD: Each of the aspects of care is described and the literature about nuances of care is provided. CONCLUSION: Therapies will not improve outcomes until novel treatments are introduced, such as those suggested, which may intervene in the inflammatory or fibrotic steps of the disease process.
BACKGROUND:Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction before 2 months of age. Untreated BA is a uniformly fatal disease and even with our current therapies only 50% of children with BA will be transplant-free by 2 years of age. Despite descriptions of this disorder dating back to the 1800s our current therapies are palliative. They focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. OBJECTIVE: To present the literature supporting current treatment strategies and potential future therapies. METHOD: Each of the aspects of care is described and the literature about nuances of care is provided. CONCLUSION: Therapies will not improve outcomes until novel treatments are introduced, such as those suggested, which may intervene in the inflammatory or fibrotic steps of the disease process.