Arteriohepatic dysplasia (syndromatic ductular hypoplasia, Alagille syndrome) is a condition
of chronic cholestasis dating from infancy accompanied by characteristic facies, pulmonic
stenosis, and other somatic abnormalities. The pathologic hallmark of arteriohepatic
dysplasia is a paucity or absence of intrahepatic bile ducts, wildely regarded as a congenital
deficiency. We present a longitudinal study of six infants and children with arteriohepatic
dysplasia, stressing evolution of the characteristic pathology on liver biopsy. All patients …