Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England

Backgruond/Purpose: Portoenterostomy is an accepted method of achieving bil e drainage in biliary atresia, but there is a paucity of data, including fo rmal quality-of-life (QoL) studies, on long-term survivors. This report inc ludes survival analysis and QoL studies from the world's largest series of cases treated in Japan (1951 to 1998). The Japanese QoL results are compare d with a matched group of UK patients from King's College Hospital, London. Methods: One hundred fifteen Japanese surviving portoenterostomy patients w ere studied and comparison of trends in survival calculated from 8-year per iod cohorts. Liver function and hematologic status in a group of 30 long-te rm survivors (14 to 24 years) were compared with 25 patients from England, (14 to 23 years). Twenty-five Japanese and 21 UK patients (SF-36) completed a QoL questionnaire. Results: Median survival times in Japanese patients before 1975 were less t han 1 year but increased to 18 years after 1975. Hematologic and liver func tion test results did not show any significant differences between the Japa nese and UK patients. QoL studies in the UK patients showed no significant difference from normative, general population data. Japanese patients under performed in general health (P=.01), role emotional (P=.05) and role physic al (P=.07) but, overall, there was no significant difference between the Ja panese and UK patients except for marginal differences in indices of genera l health and vitality (P =.06 and .04, respectively). Conclusions: Long-term survival rate in the Japanese patients increased dra matically from 1 year to 17 years after 1975. The QoL of survivors was comp arable in Japan and England. The satisfactory comparison with normative pop ulation data suggests that we should continue to use portoenterostomy as th e primary treatment for biliary atresia. J Pediatr Surg 36:892-897. Copyrig ht (C) 2001 by W.B. Saunders Company.