For this study, researchers looked at the prognosis of biliary atresia (BA) in France after 1986, when both the Kasai procedure (KOp) and liver transplantation (LT) were more commonly available. All patients diagnosed with BA who were born between 1986 and 2015 and lived in France had their files examined. A total of 1,428 individuals were involved in the study, with 1,340 (94%) undergoing KOp. In 516 individuals, complete clearance of jaundice (total bilirubin 20 mol/L) was reported (39%). The median age at KOp (59 days, range 6–199) remained consistent across time. Survival with native liver following KOp was 41%, 35%, 26%, and 22% after 5, 10, 20, and 30 years, with the four groups remaining stable. In patients operated on in the first, second, or third month of life or later, 25-year survival with native liver was 38%, 27%, 22%, and 19%, respectively (P=0.0001). Only in the 1986–1996 cohort did center caseloads have a substantial influence on outcomes. In the four groups, 16%, 7%, 7%, and 8% of patients died without LT (P=0.0001). LT was performed on 753 individuals (55%). At 28 years, patient survival following LT was 79%. In cohorts 1 to 4, patient survival following LT was 76%, 91%, 88%, and 92%, respectively (P<0.0001). The actual BA patient survival rate (from diagnosis) was 81%. Five-year BA patient survival rates were 72%, 88%, 87%, and 87%, respectively, in cohorts 1986 to 1996, 1997 to 2002, 2003 to 2009, and 2010 to 2015 (P<0.0001).
In France, 87% of BA patients survive nowadays, and 22% live to the age of 30 without a transplant. The improved BA prognosis is mostly attributable to lower mortality before LT and better results after LT.
Reference:journals.lww.com/jpgn/Fulltext/2019/10000/Management_of_Biliary_Atresia_in_France_1986_to.6.aspx
