Alagille 症候群と胆道閉鎖症の1合併例

概要

論文の詳細を見る
Paucity of intrahepatic bile duct with characteristic face, peripheral pulmonary stenosis, vertebral anomaly and physical and mental retardation has been named Alagille's syndrome. This disease is now thought to be a different from extrahepatic biliary atresia. Recently we experienced one cae o Alagille's syndrome with extrahepatic billiary atresia, so we reported here. A 44-day-old girl was admitted to our hospital because of heart murmur and jaundice. Her characteristic face, peripheral pulmonary stenosis and vertebral anomaly highly suspected Alagille's syndrome. But preoperative ERCP examination showed the abscence of in-trahepatic bile ducts and bilateral hepatic ducts, so ex-trahepatic biliary atresia could not be ruled out. In operation, bilateral hepatic ducts was replaced with granulation tissue and the finding of surgical cholangiography was same as that of ERCP. Hepatoenterostomy (SURUGA Ilnd procedure) was performed. Pathological investigation demonstrated the marked reduction of intralobular bile ducts in portal areas in biopsied liver and no ductular stuructures in granulation tissue. So the patient was diagnosed as Alagille's syndrome with ex-trahepatic biliary atresia. Her post-operative course had become worse and she died from massive intestinal bleeding with liver cirrhosis after 3 years.
特定非営利活動法人日本小児外科学会の論文
1989-02-20