Alagille症候群における肝内胆管低形成 : 乳児閉塞性黄疸における鑑別診断を中心に

概要

論文の詳細を見る
Five cases of Alagille's syndrome were experienced at Nagoya City University Hospital during from 1972 to 1979. Four cases were male and one female infant. Hypoplasia of the intrahepatic bile duct in Alagille's syndrome is usually recognized by pathological examination of the biopsied specimen, However, the size of the sample is not always large enough to detect changes of the bile ducts in the portal area. In our experience, we found the importance of cholangiographic studies for the diagnosis of various forms of obstructive jaundice. A new clasification of obstructive jaundice acording to cholangiographic findings in infants is proposed. 1) Biliary Alresia: Anywhere of the biliary system from the intrahepatic to common duct, one or more obstructions are found. 2) Bile duct Hypoplasia: There is no obstructive lesion in the total bile duct, but the diameter of the bile duct is very narrow or unclear on cholangiogram. 3) Infantile Hepatitis: There is no obstructive lesion in the total bile duct, and the cholangiogram can clearly demonstrates the second branch of intrahepatic bile duct. It is postulated that a successful out come of surgical treatment of biliary atresia will be expected if the diagnosis of intrahepatic bile duct hypoplasia is made in the early post natal period.
日本小児外科学会の論文
1980-10-20