Abstract

SILVA, Ermelinda Santos et al. Biliary atresia: retrospective clinical study. Nascer e Crescer [online]. 2011, vol.20, n.1, pp.9-15. ISSN 0872-0754.

Objective: To obtain further insight into the natural history of patients with biliary atresia and to determine survival and prognostic factors. Patients and Methods: we performed a retrospective cross-sectional, descriptive, case series study, that included all patients with biliary atresia attended in our hospital from January 1992 to December 2007. This period was divided in two parts: P1 from January 1992 to December 1999 and P2 from January 2000 to December 2007. The following variables were analysed: clinical biochemical, imagiological, histological and surgical data; age of Kasai proce­dure; need of liver transplant and outcome. Results: There were 15 patients attended (six in P1 and nine in P2). Median time between admission and Kasai procedure was 10 days (14 days in P1; 7days in P2). Median age at Kasai procedure was 58 days (89 days in P1; 45 days in P2). Ka­sai procedure was successful in 46,67% (33,33% in P1; 55.56% in P2). Overall ve years survival was 83.33% (66,67% in P1; 100% in P2). Five years survival with native liver was 41.67% (16,67% in P1; 66.67 % in P2). Conclusions: Despite the low rate of admissions (1,125 patients/year) the P2 results are similar to those of Reference European Centers. This improvement was achieved with an early diagnosis (early reference of patients and more rapid investigation, with consequent lowering of Kasai procedure`s median age); it was also important the increased experience of the team within the years.

Keywords : Biliary atresia; neonatal cholestasis; liver transplantation.

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