Alagilleシンドロームのレバー病理学
Alagille syndrome (ALGS) is a genetic disorder that affects primarily the liver and the heart. Problems associated with the disorder generally become evident in infancy or early childhood. The disorder is inherited in an autosomal dominant pattern, and the estimated prevalence of Alagille syndrome is 1 in every 30,000 to 1 in every 40,000 live
Alagille syndrome (ALGS) is a rare multisystem genetic disorder caused by defects in the Notch signaling pathway and characterized by the buildup of bile in the liver and hepatic damage because of a paucity of intrahepatic bile ducts. 1 Although it mainly affects the liver, Alagille syndrome can also affect the heart, spine, eyes, face, kidneys, and vasculature.
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