Peripheral bile duct paucity and cholestasis in the liver of a patient with Alagille syndrome: further evidence supporting a lack of postnatal bile duct branching and …
L Libbrecht, NB Spinner, EC Moore… - The American journal …, 2005 - journals.lww.com
Alagille syndrome (AGS) is a developmental, multiorgan disease caused by mutations of the
Jagged1 gene. The liver is one of the major organs affected in AGS, and the hallmark of liver
pathology in AGS is an age-related increase in the proportion of portal tracts that have no
bile duct, but without evidence of prominent bile duct damage. The pathogenesis of this bile
duct paucity is currently not well understood.(Immuno) histochemical and molecular
analyses were performed on several liver biopsies that were taken during macroscopic …
Jagged1 gene. The liver is one of the major organs affected in AGS, and the hallmark of liver
pathology in AGS is an age-related increase in the proportion of portal tracts that have no
bile duct, but without evidence of prominent bile duct damage. The pathogenesis of this bile
duct paucity is currently not well understood.(Immuno) histochemical and molecular
analyses were performed on several liver biopsies that were taken during macroscopic …