Article
Bile Ducts in Regenerative Liver Nodules of Alagille Patients Are Not the Result of Genetic Mosaicism.
Journal of pediatric gastroenterology and nutrition - 1 Jul 2015
Rougemont Anne-Laure, Alvarez Fernando, McLin Valérie A, Guiochon-Mantel Anne, Bouligand Jérome, Clément Sophie, Tonson La Tour Aude, Wildhaber Barbara E, Rubbia-Brandt Laura, Sartelet Hervé
Abstract excerpt
Alagille syndrome (ALGS) is a complex, multisystem disease associated with mutations in the JAG1 gene. In the liver, ALGS is characterized by paucity of intrahepatic bile ducts. Gene dosage analysis performed on a large, central regenerative nodule with preserved interlobular bile ducts of 2 unrelated ALGS patients, and on surrounding cirrhotic and ductopenic liver parenchyma, showed in both cases complete JAG1...
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