Mexican infants with extrahepatic biliary atresia display
Yves Fabre, Miriam Ruth Bueno‐Topete, Ana Rosa Rincón‐Sánchez, Julián Saldaña-Cortés, Raúl Vargas, Juan Armendáriz‐Borunda
Abstract
Yves Fabre, Miriam Ruth Bueno‐Topete, Ana Rosa Rincón‐Sánchez, Julián Saldaña-Cortés, Raúl Vargas, Juan Armendáriz‐Borunda
Abstract
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 IFN- with 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 IFN- were 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. 12 13 14 15 16 17 18 19 20 21 22
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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 IFN- with 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 IFN- were 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. 12 13 14 15 16 17 18 19 20 21 22
Key concepts: Biliary atresia, Gastroenterology, Medicine, Extrahepatic Biliary Atresia, Atresia, Internal medicine, Fibrosis, Hepatic fibrosis