Liver transplantation in Japanese and Australian/New Zealand children with biliary atresia: a 10-year comparative study.

摘要

OBJECTIVE: To compare Japanese with Australian/New Zealand (ANZ) children with biliary atresia who were treated by liver transplantation, and evaluate the indications for and timing of transplantation. DESIGN: Retrospective study. SETTING: Queensland Liver Transplant Service (QLTS), Australia. SUBJECTS: 43 Japanese and 30 ANZ children with biliary atresia who required transplantation between 1985 and 1992. INTERVENTIONS: The 43 Japanese children had 52 transplants, and the 30 ANZ children had 33. MAIN OUTCOME MEASURES: Morbidity, mortality, and long term survival. RESULTS: The Japanese children had significantly lower serum albumin concentrations than the ANZ children preoperatively (mean (SD) 32 (7) g/L compared with 37 (5), p<0.05). The actuarial survival at 7 years of the ANZ children was significantly higher than that of the Japanese children (79% compared with 49%, p<0.05). There were 24 deaths (17 Japanese, 40%, and 7 ANZ, 23%); 2 of the ANZ and 7 of the Japanese children died more than a year after transplantation. All 26 children who were well-nourished at the time of transplantation defined as a Z-score (weight or height minus mean weight or height for age, sex, and race, divided by the SD) of -1 or more were alive at 1 month compared with 11 of the 47 poorly-nourished children (Z-score <-1). Survival among the Japanese declined after 1 year, and there was no association with Z-scores. Overall, Z-scores for weight improved significantly after transplantation, whereas those for height improved a little, but not significantly so. Japanese children were significantly shorter than ANZ children, and their Z-scores for height did not improve after transplantation. CONCLUSION: liver transplantation should be done as soon as possible for children with biliary atresia to maximise survival and growth.