先天性胆道閉鎖症術後長期生存例 : 10歳以上に達した症例の検討

抄録

Many recent articles describe an increase of survivors in biliary atresia. Very little is known, however, about the studies of long term follow up. This paper reports the results of follow-up studies of long term survivors who are living more than 10 years after surgery. During the period from June 1967 to August 1974, a total of 61 patients had been treated by Kasai's hepatic portoenterostomy operation in originany reported fashion. Among these, 21 patients, that is 34.4% of total, are alive and so all of these survivors are more than 10 years old now. The oldest 2 patients have become 16 year old. None of these 21 patients was accompanied with ascending cholangitis early after surgery, but mild cholangitis developed in 12 patients later. The height in 2 patients and the body weight in 5 patients are considerably lower than the average. Hepatomegary exists in 12 and splenomegary exists in 9 patients including a case with hypersplenism. Liver function tests are thoroughly normal in 10 patients, but some of transaminase, alkaline phosphatase or the colloid reaction still maintain higher level than that of normal range in all of the remaining 11. Portal hypertension has been diagnosed in 9 patients by the endoscopic examination of esophageal varices. Mild varices in one patient disappeared spontaneously, but 6 of these 9 patients bled varices. An esophageal transection was indicated for 4 and an endoscopic sclerosing therapy was performed for the remaining 2 successfully. Out of 21 long term survivors, 10 are free from any clinical symptoms, abnormality of the liver function tests or the sign of portal hypertension. The remaining 11 patients, however, have some problems in such aspects and they should be closely followed up hereafter.