Mexican infants with extrahepatic biliary atresia display different fibrosis activity

Although biliary obstruction in extrahepatic biliary atresia (EHBA) patients can be surgically alleviated by hepatoportoenterostomy (Kasai surgery), most patients will eventually develop severe hepatic fibrosis with short life expectancy. Cellular and molecular mechanisms involved in this process are largely unknown. The aim of this study was to determine the potential correlation between fibrogenic (collagens I, III, IV, and TIMP-1) and antifibrogenic proteins like IFNwith disease evolution. Open liver biopsies were obtained from seven extrahepatic biliary atresia and two control infants to determine fibrosis index (baseline). Six months after Kasai surgery, extrahepatic biliary atresia patients were reclassified as: Group I (good progress; Child A–B) and Group II (poor progress; Child C or deceased). Liver fibrosis index was higher in Group II (n = 4) than Group I (n = 3) (36.67 ± 4.72% versus 22.9 ± 4.45%; P < 0.05). Highest bilirubins, ALT and AST levels, and higher number of positive cells for -SMA were found in Group II patients versus Group I (P < 0.05). Levels of mRNA for Collagens I, II, IV, TIMP-1, and IFNwere higher in both groups compared with control group measured by semi-quantitative RT-PCR (P < 0.05). These results suggest that in extrahepatic biliary atresia patients, low fibrosis index and number of activated hepatic stellate cells (HSC) could be useful factors to determine the early outcome in biliary atresia patients. © 2003 Elsevier B.V. All rights reserved.