<prompt> False statement about alagille syndromeComplete absence of bile ductsNormal liverMutations in Jagged 1 geneNo Risk of hepatocellular carcinoma  <response>: Ans. d No Risk of hepatocellular carcinomaRef: Robbins 9thpg 853; 8thpg 854Alagille Syndrome Syndromic Paucity of Bile Ducts; Arteriohepatic DysplasiaAn autosomal dominant disorder characterized by absence of bile ducts in portal tracts.Caused by mutations or deletion of gene encoding Jagged 1, on chromosome 20p.Patients can survive into adulthood but are at risk for hepatic failure hepatocellular carcinomaMajor clinical features:Chronic cholestasisPeripheral pulmonary artery stenosisButterfly-like vertebral arch defectsAn eye defect known as posterior embryotoxon,A peculiar hypertelic, triangular facies. 