Biliary Atresia Intrahepatic Syndromic Form


Type of disease: Rare conditions

Bile Atresia, Intrahepatic, Syndromic Form is a rare digestive disorder characterized by the incomplete development of the bile duct system as well as other related birth defects. The bile duct system is responsible for storing and transporting bile from the liver, where it is made, to the intestines, where it helps to digest food. Incomplete development of the ducts inhibits the flow of bile. Individuals with this defect have digestion problems as well as a yellow discoloration of the eye and skin (jaundice). Doctors diagnose this defect early in life, as the resulting symptoms are quickly apparent. Treatment is developed on a case-by-case basis and depends on the specific needs of the patient. Digestion specialists may suggest individuals eat a lower-fat diet, or undergo surgery to relieve the blockage or create a tube to allow bile to reach the digestive system.

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