1. Biliary atresia: how medical complications and therapies impact outcome
- Author
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Kimberly Hohlweg, Barbara Haber, and Jessi Erlichman
- Subjects
Cholagogues and Choleretics ,medicine.medical_specialty ,Time Factors ,Palliative care ,Adolescent ,medicine.medical_treatment ,Psychological intervention ,Disease ,Liver transplantation ,Esophageal and Gastric Varices ,Young Adult ,Cholestasis ,Biliary Atresia ,Biliary atresia ,Hypertension, Portal ,medicine ,Humans ,Young adult ,Child ,Intensive care medicine ,Hepatology ,Nutritional Support ,business.industry ,Palliative Care ,Gastroenterology ,Infant ,medicine.disease ,Hepatoportoenterostomy ,Liver Transplantation ,Surgery ,Treatment Outcome ,Child, Preschool ,Disease Progression ,Steroids ,Clinical Competence ,Gastrointestinal Hemorrhage ,business - Abstract
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.
- Published
- 2009
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