Improving outcomes of biliary atresia: French national series 1986-2009

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• 作者：Christophe Chardot¹ ; ^{christophe.chardot@nck.aphp.fr} ; Chantal Buet¹ ; Marie-Odile Serinet¹ ; Jean-Louis Golmard² ; Alain Lachaux³ ; Bertrand Roquelaure⁴ ; Fré ; dé ; ric Gottrand⁵ ; Pierre Broué ; ⁶ ; Alain Dabadie⁷ ; Fré ; dé ; ric Gauthier⁸ ; Emmanuel Jacquemin⁸
• 关键词：BA ; biliary atresia ; LT ; liver transplantation ; CI ; confidence interval ; SE ; standard error ; SNL ; survival with native liver
• 刊名：Journal of Hepatology
• 出版年：2013
• 出版时间：June, 2013
• 年：2013
• 卷：58
• 期：6
• 页码：1209-1217
• 全文大小：1600 K

文摘

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Background & Aims

This study analyses the prognosis of biliary atresia (BA) in France since liver transplantation (LT) became widely available.

Methods

The charts of all BA patients living in France and born between 1986 and 2009 were reviewed. Patients were divided into 3 cohorts according to their years of birth: 1986-1996, 1997-2002, and 2003-2009.

Results

1107 BA children were identified, 990 born in metropolitan France (incidence 1/18,400 live births). Kasai operation was performed in 1044 (94 % ), leading to complete clearance of jaundice (total serum bilirubin ?20 ¦Ìmol/L) in 38 % of patients. Survival with native liver (SNL) after Kasai operation was 40 % , 36 % , and 30 % at 5, 10, and 20 years, stable in the 3 cohorts. Median age at Kasai operation was 59 days, unchanged over time. Twenty-year SNL was 39 % , 32 % , 28 % , and 19 % after Kasai operation performed in the first, second, third months of life or thereafter (p = 0.0002). 588 children underwent 692 LTs. Mortality without transplantation decreased over time: 16 % , 7 % , and 4 % in the 3 cohorts (p <0.0001). Survival after transplantation was 83 % , 82 % , and 77 % at 5, 10, and 20 years in the whole series. Five-year post-transplant survival was 75 % , 90 % , and 89 % in the 3 cohorts (p <0.0001). In the whole series, overall BA patient survival was 81 % , 80 % , and 77 % at 5, 10, and 20 years. Five-year BA patient overall survival increased over time: 72 % , 88 % , and 89 % in the 3 cohorts (p <0.0001).

Conclusions

BA patients currently have an 89 % live expectancy, and a 30 % chance to reach adulthood without transplantation. Early Kasai operation, without age threshold, reduces the need for liver transplantation until adulthood.