Open AccessALAGILLE'S SYNDROME
Author(s) -
Nishikawa Akiyoshi,
Mori Hideki,
Takahashi Masayoshi,
Ojima Akitsugu,
Shimokawa Kuniyasu,
Fueuta Tomohiko
Publication year - 1987
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1987.tb00464.x
Subject(s) - pathology , autopsy , medicine , nodule (geology) , alagille syndrome , cirrhosis , nodular regenerative hyperplasia , jaundice , intrahepatic bile ducts , etiology , lesion , portal hypertension , cholestasis , gastroenterology , biology , bile duct , paleontology
A male case of Alagille's syndrome associated with a hamartomatous nodule of the liver is described. The patient developed jaundice soon after birth, and was diagnosed as the syndrome with signs such as paucity of the intrahepatic bile ducts, pulmonary stenosis and embryotoxon in the cornea at 15 years of age. The liver was examined in recurrent biopsies and other tests. However, no evidence of liver cirrhosis was confirmed until his 15th year. The patient died of hepatic dysfunction when he was 17 years old. At autopsy, a large hamartomatous nodule was found in the liver showing biliary cirrhosis. Morphology of the nodule resembled that of focal nodular hyperplasia. Abnormalities of the large vessels were noted around the liver. Vascular abnormalities were also seen in the mass. The relation of these vascular abnormalities to etiological background of the syndrome and occurrence of the nodular lesion is discussed.