胆道閉鎖症における葛西氏手術の予後および肝移植の問題について

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Development of surgical technique and postoperative management provides substantial improvement for at least five years in one third of the patients with biliary atresia, although progressive cholestasis, hepatocellular decompression or severe portal hypertension occur with increasing age in some of them. One hundred and three patients have undergone hepatoportoenterostomy for biliary atresia during the period from January 1970 to December 1986 in our institute. Eighty-six patients (83%) had a bile dranage and two thirds of them showed jaundice free. There are thirty-six patients living without jaundice and twenty-eight of them living for more than five years. The survival rate is 36% at five years. Twnty-five patients have been expected curable. In this paper we did retrospectively examine in seventy-one patients of all with biliary atresia whether success of this procedure could be predicted for the individual patients by means of the data of operative age, serum bilirubin decreasing rate, clinical signs and various hepatic functional tests. We concluded as follows : (1) We suppose that serum bilirubin decreasing rate could be an important index for prediction of the success of hepatoportoenterostomy in biliary atresia, but the operative age should not be. (2) Transplantation of the liver should be early considered for biliary atresia patients with rapid increase of serum bilirubin or progressive hyperbilirubinemia exceeding 5 mg% at 3 months after hepatoportoenterostomy, who gradually decompensate hepatic functional reserve after this procedure. (3) In the postoperative patients with biliary atresia in hyperbilirubinemia exceeding or below 5 mg% of serum total bilirubin, who do not deteriorate hepatic functional reserve, liver transplantation should be delayed as long as possible to permit the children to achieve maxim growth. But, it should be performed for these patients within keeping in normal level of tha hepatic functional reserve.
日本小児外科学会の論文
1989-12-20

胆道閉鎖症における葛西氏手術の予後および肝移植の問題について

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