Biliary atresia: how medical complications and therapies impact outcome.
Biliary atresia (BA) is a progressive fibro-obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. Untreated, BA is a uniformly fatal disease and, yet, even with our existing therapies, at least 50% of children with BA will undergo liver transplantation by the age of 2 years. Current treatment strategies are, at best, palliative; they focus on prompt diagnosis, supportive nutritional care and interventions for sequelae. The purpose of this article is to discuss the current treatment paradigm for BA and to assess the impact these strategies have on outcomes. As more children with BA survive into adulthood with their native liver, it is important to understand which factors predict good and poor outcomes.
Erlichman
Jessi
J
Division of GI, Hepatology and Nutrition, The Childrens' Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104, USA.
Hohlweg
Kimberly
K
Haber
Barbara A
BA
D000293:Adolescent; D001656:Biliary Atresia / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D002983:Clinical Competence; D018450:Disease Progression; D004932:Esophageal and Gastric Varices / Q000209:etiology / Q000628:therapy; D006471:Gastrointestinal Hemorrhage / Q000209:etiology / Q000628:therapy; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000628:therapy; D007223:Infant; D016031:Liver Transplantation; D018529:Nutritional Support; D010166:Palliative Care; D013256:Steroids / Q000627:therapeutic use; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D002756:Cholagogues and Choleretics; D013256:Steroids