[Hepatic duct hypoplasia or Alagille-Odièvre disease].
Five children from one to seven years of age with intrahepatic ductular hypoplasia are presented. Clinical and biochemical characteristics of the disease are specifically considered as well as differential diagnosis. In three patients a severe hemolytic anemia, only partially corrected with vitamine E, was present. All subjects received continuous cholestyramine therapy. Variable follow up from nine months to seven years demonstrated persistent intrahepatic cholestasis with hypercholesterolemia in two cases, clinical and biochemical improvement in one case. One child died two years and six months after diagnosis was made an one patient was lost to follow up. The Authors confirm the literature clinic, diagnostic and therapeutic data concerning ductular hypoplasia, while, on the basis of their experience, the prognosis seems poor also in some cases of syndromic ductular hypoplasia.
D000743:Anemia, Hemolytic / Q000150:complications; D001649:Bile Duct Diseases / Q000150:complications*; D001653:Bile Ducts, Intrahepatic; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000209:etiology*; D006330:Heart Defects, Congenital / Q000150:complications; D006500:Hepatic Duct, Common; D006801:Humans; D008297:Male
La ipoplasia duttale epatica o malattia di Alagille-Odièvre.