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Jornal de Pediatria

Print version ISSN 0021-7557On-line version ISSN 1678-4782


SANTOS, Jorge Luiz dos et al. Factors related to the post-portoenterostomy prognosis of biliary atresia. J. Pediatr. (Rio J.) [online]. 2002, vol.78, n.4, pp.341-346. ISSN 0021-7557.

Objective: this study considered the presence of congenital anomalies, ductal plate malformation, area of fibrosis and, mainly, the patient's age in cases of biliary atresia submitted to surgery. The present study verified the influence of these factors on the follow-up of a biliary atresia sample. Methods: a sample of 47 patients with biliary atresia was evaluated in a cross-sectional study. Their histologic specimens were stained for antibody anticytokeratin 19 and CAM 5.2 through immunohistochemistry in order to study biliary structures, and for picrosirius red to evaluate the area of fibrosis. The study of biliary structures was performed by two pathologists and the first author of the study. They were "blind" with regard to the clinical follow-up. The area of fibrosis was quantitatively evaluated. Data on the patients with regard to age, death and occurrence of liver transplantation were searched on the patients records. Results: age at portoenterostomy varied between 24 and 251 days of life (90.4 ±44.8 days) and follow-up was available in 32 cases (72%). The nine cases (19%) with extrahepatic congenital anomalies associated to biliary atresia did not present different prognosis from the remaining patients. Age at portoenterostomy influenced the prognosis (p=0.016). The area of fibrosis was different on patients aged less than 60 days and those aged more than 90 days at portoenterostomy (p=0.023), but did not influence the prognosis. The presence of ductal plate malformation, as well, did not influence the follow-up. Conclusions: age at portoenterostomy was the only factor that influenced prognosis on this sample of biliary atresia. It is necessary to increase the biliary atresia sample to check the influence of congenital extrahepatic anomalies on the follow-up post-portoenterostomy.

Keywords : biliary atresia; post-portoenterostomy prognosis; fibrosis; ductal plate malformation.

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