부산대학교 도서관

Objective Biliary atresia (BA) is a progressive fibro-obliterative disease of the biliary tract, which results in end-stage liver disease. However, liver fibrosis progression may continue even after Kasai surgery. Recent evidence showed that collagen plays a pivotal role in the progression of liver fibrosis in BA. However, most studies were conducted in developed countries. We investigated the expressions of the collagen gene cluster (COL6A1, COL6A2, COL6A3, and COL1A1) in BA patients in Indonesia. Results There was a significant down-regulated expression of COL6A1 ([DELA]C.sub.T 9.06 [+ or -] 2.64 vs. 5.42 [+ or -] 2.41; p = 0.0009), COL6A2 ([DELA]C.sub.T 8.25 [+ or -] 2.07 vs. 5.77 [+ or -] 3.51; p = 0.02), COL6A3 ([DELA]C.sub.T 11.2 [+ or -] 6.08 vs. 6.78 [+ or -] 3.51; p = 0.024), and COL1A1 ([DELA]C.sub.T 3.26 [+ or -] 1.71 vs. 0.19 [+ or -] 2.76; p = 0.0015) in BA patients compared to controls. Interestingly, the collagen gene cluster expressions were significantly associated with the presence of cirrhosis (p = 0.0085, 0.04, and 0.0283 for COL6A1, COL6A2, and COL6A3, respectively). In conclusion, our study shows the changes in the collagen gene cluster, particularly collagen type I and VI, expressions in patients with BA in a particular developing country. Our findings suggest the role of these collagen gene clusters in the liver fibrogenesis of BA. Keywords: Biliary atresia, Collagen gene cluster expressions, Developing country, Kasai procedure, Liver fibrogenesis
Author(s): Dyah Ayu Puspitarani[sup.1], Khanza Adzkia Vujira[sup.1], Fadila Dyah Trie Utami[sup.1], Edita Mayda Devana[sup.1], Fiqih Vidiantoro Halim[sup.1], Kristy Iskandar[sup.2] and Akhmad Makhmudi[sup.1] Introduction Biliary atresia (BA) is the most common [...]