BACKGROUND/PURPOSE: Portoenterostomy is an accepted method of achieving bile drainage in @entity2876 , but there is a paucity of data, including formal quality-of-life (QoL) studies, on long-term survivors. This report includes survival analysis and QoL studies from the world's largest series of cases treated in Japan (1951 to 1998). The Japanese QoL results are compared with a matched group of UK @entity1 from King's College Hospital, London. METHODS: One hundred fifteen Japanese surviving portoenterostomy @entity1 were studied and comparison of trends in survival calculated from 6-year period cohorts. Liver function and hematologic status in a group of 30 long-term survivors (14 to 24 years) were compared with 25 @entity1 from England, (14 to 23 years). Twenty-five Japanese and 21 UK @entity1 (SF-36) completed a QoL questionnaire. RESULTS: Median survival times in Japanese @entity1 before 1975 were less than 1 year but increased to 18 years after 1975. Hematologic and liver function test results did not show any significant differences between the Japanese and UK @entity1 . QoL studies in the UK @entity1 showed no significant difference from normative, general population data. Japanese @entity1 underperformed in general health (P = .01), role emotional (P = .05) and role physical (P = .07) but, overall, there was no significant difference between the Japanese and UK @entity1 except for marginal differences in indices of general health and vitality (P = .06 and .04, respectively). CONCLUSIONS: Long-term survival rate in the Japanese @entity1 increased dramatically from 1 year to 17 years after 1975. The QoL of survivors was comparable in Japan and England. The satisfactory comparison with normative population data suggests that we should continue to use portoenterostomy as the primary treatment for @entity2876 . J Pediatr Surg 36:892-897.

Survival patterns in XXXX and comparison of quality of life of long-term survivors in Japan and England.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity2876 :: ('MESH:D001656', 'Disease') :: ['biliary atresia']
@entity2876 :: (MESH:D001656,Disease) :: ['biliary atresia']
