The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis ☆ Jorge L. Santos a, ⁎ , Carlos O. Kieling a , Luise Meurer b , Sandra Vieira a , Cristina T. Ferreira a , Andrea Lorentz b , Themis R. Silveira b a Hospital de Clínicas de Porto Alegre, CEP-90035-903 Brazil b Universidade Federal do Rio Grande do Sul, CEP-90035-903 Brazil Received 4 May 2008; revised 12 September 2008; accepted 12 September 2008 Key words: Biliary atresia; Prognosis; Histopathology; CK7; Biliary proliferation; Age; Portoenterostomy; Liver fibrosis Abstract Background/Purpose: In biliary atresia (BA), a derangement in the biliary system remains, despite portoenterostomy performance. Many factors can influence the disease progression rate. This study aimed to analyze the association between biliary proliferation extent in biopsies from BA patients and postoperative prognosis. Methods: Biliary proliferation was evaluated by a morphometric analysis of the cytokeratin 7 positivity percentage (PCK7) in wedge liver biopsies from 47 BA patients. The extent of fibrosis was evaluated by a fibrosis score (FS). The outcome 1-year native liver survival was correlated, using a multivariable regression analysis, with PCK7, FS, and age at portoenterostomy. Results: The PCK7 ranged between 0.80% and 14.79% (M ± SD = 7.36% ± 4.15%). Patients who died or underwent transplantation had higher PCK7 than survivors with their native livers (P b .001). The area under the receiver operating characteristic curve for PCK7 in relation to the outcome was 0.845 (P b .001). The cutoff point of PCK7 for the maximal effect on postoperative prognosis was 10.18% (sensitivity = 0.71, specificity = 0.88). The PCK7 was the only studied variable associated with 1-year native liver survival, independently of age and FS (P = .002). Conclusion: The extent of biliary proliferation at portoenterostomy, evaluated by PCK7, was associated with 1-year native liver survival of BA patients. © 2009 Elsevier Inc. All rights reserved. Biliary atresia (BA) is an infantile disorder character- ized by the complete obstruction of a portion or the entirety of the extrahepatic biliary ducts. Since 1959, after the description of portoenterostomy by Dr Morio Kasai, the removal of the impediment to biliary flow has become feasible in all types of the disease, including the previously “uncorrectable type” [1]. However, regardless of timely performance of a portoenterostomy, all (or almost all) patients with BA experience progressive derangement of the intrahepatic biliary system, leading to increasing fibrogenesis and eventually to cirrhosis [2]. ☆ Sponsor: Fundo de Incentivo à Pesquisa e Eventos, Hospital de Clínicas de Porto Alegre. ⁎ Corresponding author. Tel.: +55 51 3312 2090, +55 51 2101 8847. E-mail address: kapars@terra.com.br (J.L. Santos). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.09.013 Journal of Pediatric Surgery (2009) 44, 695–701