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Biliary Atresia, Extrahepatic
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Copyright 1986, 1990, 1994, 2003, 2007, 2009

NORD is very grateful to Jorge A. Bezerra, MD, Professor of Pediatrics, Cincinnati Children's Hospital Medical Center, Division of Gastroenterology, Hepatology and Nutrition, for assistance in the preparation of this report.

Synonyms of Biliary Atresia, Extrahepatic
  • EHBA

Disorder Subdivisions



General Discussion
Extrahepatic biliary atresia is a rare gastrointestinal disorder characterized by destruction or absence of all or a portion of the bile duct that lies outside the liver (extrahepatic bile duct). The bile duct is a tube that allows the passage of bile from the liver into the gall bladder and, eventually, the small intestine. Bile is a liquid secreted by the liver that plays an essential role in carrying waste products from the liver, breaking down fats in the small intestine, and promoting absorption of vitamins. In extrahepatic biliary atresia, absence or destruction of the bile ducts results in the abnormal accumulation of bile in the liver. Affected infants have yellowing of the skin and whites of the eyes (jaundice) and scarring of the liver (cirrhosis). In some cases, additional abnormalities may be present, including heart defects and kidney and spleen malformations. The exact cause of extrahepatic biliary atresia is unknown.
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Organizations related to Biliary Atresia, Extrahepatic
  • American Liver Foundation
    75 Maiden Lane
    Suite 603
    New York NY 10038
    Phone #: 212-668-1000
    800 #: 800-465-4837
    e-mail: info@liverfoundation.org
    Home page: http://www.liverfoundation.org
  • Canadian Liver Foundation
    2235 Sheppard Avenue
    Suite 1500
    Toronto, Ontario Intl M2J 5B5
    Phone #: (41-6) -491-3353
    800 #: (80-0) -563-5483
    e-mail: clf@liver.ca
    Home page: http://www.liver.ca
  • Children's Liver Association for Support Services (C.L.A.S.S.)
    25379 Wayne Mills Place, Suite 143
    Valencia CA 91355
    Phone #: 661-263-9099
    800 #: 877-679-8256
    e-mail: info@classkids.org
    Home page: http://www.classkids.org
  • Children's Liver Disease Foundation
    36 Great Charles Street
    Birmingham Intl B3 3JY
    Phone #: +44- (0-) 121 212 3839
    800 #: --
    e-mail: info@childliverdisease.org
    Home page: http://www.childliverdisease.org
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx
    Home page: http://rarediseases.info.nih.gov/GARD
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: getinfo@madisonsfoundation.org
    Home page: http://www.madisonsfoundation.org
  • March of Dimes Birth Defects Foundation
    1275 Mamaroneck Avenue
    White Plains NY 10605
    Phone #: 914-997-4488
    800 #: 888-663-4637
    e-mail: Askus@marchofdimes.com
    Home page: http://www.marchofdimes.com
  • NIH/National Institute of Diabetes, Digestive & Kidney Diseases
    Endocrine Diseases Metabolic Diseases Branch
    2 Information Way
    Bethesda MD 20892-3570
    Phone #: 301-654-3810
    800 #: --
    e-mail: NDDIC@info.niddk.nih.gov
    Home page: http://www.niddk.nih.gov

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Last modified Tuesday, September 07, 2010