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Extrahepatic Biliary Atresia

Abstract

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NORD is very grateful to Jorge A. Bezerra, MD, William and Rebecca Balistreri Chair of Pediatric Hepatology, Professor of Pediatrics; Director, Digestive Health Center, Cincinnati Children's Hospital Medical Center, Division of Gastroenterology, Hepatology and Nutrition, for assistance in the preparation of this report.

Synonyms of Extrahepatic Biliary Atresia

  • EHBA

Disorder Subdivisions

  • No subdivisions found.

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 and promoting absorption of fats and vitamins by the intestines. 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 (fibrosis). In some cases, additional abnormalities may be present, including heart defects and intestinal, spleen and kidney malformations. The exact cause of extrahepatic biliary atresia is unknown.

Extrahepatic Biliary Atresia Resources

Organizations:

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