Serinet Marie-Odile, Broué Pierre, Jacquemin Emmanuel, Lachaux Alain, Sarles Jacques, Gottrand Frédéric, Gauthier Frédéric, Chardot Christophe
From the French Observatory of Biliary Atresia, University Hospital of Bicêtre, Le Kremlin, Bicêtre, France.
Hepatology. 2006 Jul;44(1):75-84. doi: 10.1002/hep.21219.
This study analyzed the results of the decentralized management of biliary atresia (BA) in France, where an improved collaboration between centers has been promoted since 1997. Results were compared to those obtained in England and Wales, where BA patients have been centralized in three designated centers since 1999. According to their birth dates, BA patients were divided into two cohorts: cohort A, with patients born between 1986 and 1996, had 472 patients; and cohort B, with patients born between 1997 and 2002, had 271 patients. Survival rates were calculated according to the Kaplan-Meier method and compared by using the log rank test and the Cox model. Four-year overall BA patient survival was 73.6% (95% CI 69.5%-77.7%) and 87.1% (CI 82.6%-91.6%) in cohorts A and B, respectively (P < .001). Median age at time of the Kasai operation was 61 and 57 days in cohorts A and B, respectively (NS). Four-year survival with native liver after the Kasai operation was 40.1% and 42.7% in cohorts A and B, respectively (NS): 33.9% (cohort A) and 33.4% (cohort B) in the centers with two or fewer caseloads a year, 30.9% (cohort A) and 44.5% (cohort B) in the centers with 3-5 cases/year, 47.8% (cohort A) and 47.7% (cohort B) in the center with more than 20 caseloads a year. In cohorts A and B, 74 (15.7%) and 19 (7%) patients, respectively, died without liver transplantation (LT). Four-year survival after LT was 75.1% and 88.8% in cohorts A and B, respectively (P = .006). In conclusion, BA patients currently have the same chance of survival in France as in England and Wales. The early success rate of the Kasai operation remains inferior in the centers with limited caseloads in France, leading to a greater need for LTs in infancy and early childhood.
本研究分析了法国胆道闭锁（BA）分散管理的结果，自1997年以来法国各中心之间的协作得到了加强。研究结果与英格兰和威尔士的情况进行了比较，自1999年以来，英格兰和威尔士的BA患者被集中在三个指定中心。根据出生日期，BA患者被分为两个队列：队列A为1986年至1996年出生的患者，有472例；队列B为1997年至2002年出生的患者，有271例。采用Kaplan-Meier方法计算生存率，并通过对数秩检验和Cox模型进行比较。队列A和队列B中BA患者四年总生存率分别为73.6%（95%CI 69.5%-77.7%）和87.1%（CI 82.6%-91.6%）（P <.001）。队列A和队列B中葛西手术时的中位年龄分别为61天和57天（无统计学差异）。队列A和队列B中葛西手术后自体肝四年生存率分别为40.1%和42.7%（无统计学差异）：每年病例数为两个或更少的中心中分别为33.9%（队列A）和33.4%（队列B），每年有3 - 5例病例的中心中分别为30.9%（队列A）和44.5%（队列B），每年病例数超过20例的中心中分别为47.8%（队列A）和47.7%（队列B）。在队列A和队列B中，分别有74例（15.7%）和19例（7%）患者未进行肝移植（LT）而死亡。队列A和队列B中LT后四年生存率分别为75.1%和88.8%（P = 0.006）。总之，目前法国BA患者的生存机会与英格兰和威尔士相同。在法国病例数有限的中心，葛西手术的早期成功率仍然较低，导致婴幼儿期对LT的需求更大。
