Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996
Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996
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DOI：
10.1002/hep.510300330
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发表时间：
1999-09-01
期刊：
影响因子：
13.5
通讯作者：
Auvert, B
中科院分区：
文献类型：
--
作者：
Chardot, C;Carton, M;Auvert, B
自加塞手术联合或不联合肝移植的序贯治疗成为可能以来，胆道闭锁的总体预后仍不清楚。本研究探讨了诊断的预后因素。对1986年至1996年出生于法国的所有胆道闭锁患者进行了回顾。计算自体肝存活率、肝移植后存活率和总存活率的精算存活率。使用对数秩检验和考克斯模型分析潜在的预后因素。共识别出472例患者。10年总生存率为68%。总生存期的独立预后因素为（S= 10年利率）加塞作业的业绩（进行：S= 69%;未进行：S= 50%），加塞手术时的年龄（≤ 45天：S= 80%;> 45天：S= 66%），肝外胆管解剖型，多脾综合征，中心经验（≤ 2例新发胆道闭锁[BA]患者/年[24家中心]：S= 54%; 3 - 5例[2家中心]：S= 60%;≥ 20例[1家中心]：S= 78%）。自体肝的生存取决于相同的独立预后因素。结论：（1）加塞手术仍然是BA的一线治疗方法;（2）在有经验的中心早期进行加塞手术和治疗可减少婴儿和儿童期肝移植的需要，并为儿童提供最佳的生存机会。
Since the sequential treatment of Kasai operation with or without liver transplantation became available, the overall prognosis of biliary atresia remains unclear. This study examined the prognostic factors from diagnosis. All patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed. Actuarial survival rates were calculated for survival with native liver, survival after liver transplantation, and overall survival. Potential prognostic factors were analyzed using the logrank test and the Cox model. A total of 472 patients were identified. Ten-year overall survival was 68%. Independent prognostic factors for overall survival were (S= 10-year rates) performance of Kasai operation (performed: S= 69%; not performed: S= 50%), age at Kasai operation (≤ 45 days: S= 80%;> 45 days: S= 66%), anatomical pattern of extrahepatic bile ducts, polysplenia syndrome, experience of the center (≤ 2 new biliary atresia [BA] patients/year [24 centers]: S= 54%; 3 to 5 [2 centers]: S= 60%;≥ 20 [1 center]: S= 78%). Survival with native liver depended on the same independent prognostic factors. In conclusion (1) Kasai operation remains the first line treatment of BA, and (2) early performance of Kasai operation and treatment in an experienced center reduces the need for liver transplantation in infancy and childhood and provides children with the best chance of survival.