Knowra Biliary atresia Biliary atresia Biliary atresia is an infantile disease in which bile ducts are absent or become scarred and blocked. Bile then accumulates in the liver, causing progressive damage unless bile drainage is restored or the liver is transplanted.
Bile : A digestive fluid made by the liver that carries bile acids, bilirubin, and other substances into the intestine. Its inability to reach the intestine drives jaundice, pale stools, and liver injury.
Direct bilirubin : Bilirubin that has been processed by the liver and is water-soluble for excretion in bile. Elevated direct bilirubin helps distinguish obstructive cholestasis from common newborn jaundice.
Bile duct : A tube that carries bile from the liver or gallbladder toward the small intestine. These are the structures that become absent, narrowed, or obliterated in biliary atresia.
Alagille syndrome : A genetic disorder that can cause cholestasis, characteristic facial and skeletal features, and abnormalities in several organs. It can resemble biliary atresia in infancy but arises from a different developmental and genetic disorder.
Ciliary biology : The study of cellular cilia, their structure, movement, and roles in signaling and development. Research considers whether ciliary defects contribute to a subset of biliary atresia cases.
Cholestasis : Reduced or stopped bile flow that causes bile components to accumulate in the liver or blood. Biliary atresia causes cholestasis by obstructing the ducts that carry bile.
Abdominal ultrasonography : An imaging method that uses reflected high-frequency sound waves to show structures inside the abdomen. Ultrasound can assess liver and gallbladder features during evaluation for biliary atresia.
Extrahepatic bile ducts : Bile ducts located outside the liver, including the common hepatic and common bile ducts. Their obstruction or obliteration is central to the form of disease treated by the Kasai procedure.
Progressive familial intrahepatic cholestasis : A group of inherited disorders that impair bile formation or transport within liver cells. It causes infantile cholestasis without the characteristic obliteration of extrahepatic ducts.
Rotavirus : A group of viruses that can cause severe gastrointestinal infection, especially in young children. Animal models and human studies have examined viral triggers, but a single cause is not established.
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