Wen-Jun Shen1, Gong Chen1, Min Wang1, Shan Zheng2. 1. Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China. 2. Department of Pediatric Surgery, Children's Hospital of Fudan University, 399 Wanyuan Road, Minhang District, Shanghai, 201102, China. szheng@shmu.edu.cn.
Abstract
BACKGROUND: Biliary atresia (BA) is the most common cause of obstructive jaundice in infants. Although the Kasai procedure has greatly improved the prognosis, most patients still need liver transplantation (LT) for long-term survival. The pathogenesis of BA has not been fully clarified, and liver fibrosis in BA is far beyond biliary obstructive cirrhosis. DATA SOURCES: Literature reviews were underwent through PubMed. Persistent inflammation, immune response, biliary epithelial-mesenchymal transition, matrix deposition, decompensated angiogenesis, and unique biliary structure development all contribute to the fibrosis process. Observed evidences in such fields have been collected and form the backbone of this review. RESULTS: Interactions of the multiple pathways accelerate this process. CONCLUSIONS: Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.
BACKGROUND:Biliary atresia (BA) is the most common cause of obstructive jaundice in infants. Although the Kasai procedure has greatly improved the prognosis, most patients still need liver transplantation (LT) for long-term survival. The pathogenesis of BA has not been fully clarified, and liver fibrosis in BA is far beyond biliary obstructive cirrhosis. DATA SOURCES: Literature reviews were underwent through PubMed. Persistent inflammation, immune response, biliary epithelial-mesenchymal transition, matrix deposition, decompensated angiogenesis, and unique biliary structure development all contribute to the fibrosis process. Observed evidences in such fields have been collected and form the backbone of this review. RESULTS: Interactions of the multiple pathways accelerate this process. CONCLUSIONS: Understanding the mechanisms of the liver fibrosis in BA may pave the way to improved survival after the Kasai procedure.
Authors: Magd A Kotb; Ahmed El Henawy; Sahaar Talaat; Mona Aziz; Gamal H El Tagy; Mohamed M El Barbary; Wael Mostafa Journal: J Pediatr Surg Date: 2005-08 Impact factor: 2.545
Authors: Benjamin L Shneider; Morton B Brown; Barbara Haber; Peter F Whitington; Kathleen Schwarz; Robert Squires; Jorge Bezerra; Ross Shepherd; Philip Rosenthal; Jay H Hoofnagle; Ronald J Sokol Journal: J Pediatr Date: 2006-04 Impact factor: 4.406
Authors: Cara L Mack; Rebecca M Tucker; Brandy R Lu; Ronald J Sokol; Andrew P Fontenot; Yoshiyuki Ueno; Ronald G Gill Journal: Hepatology Date: 2006-11 Impact factor: 17.425
Authors: Lin He; Patrick Ho Yu Chung; Vincent Chi Hang Lui; Clara Sze Man Tang; Paul Kwong Hang Tam Journal: Int J Mol Sci Date: 2022-04-27 Impact factor: 6.208
Authors: Lin He; Dennis Kai Ming Ip; Greta Tam; Vincent Chi Hang Lui; Paul Kwong Hang Tam; Patrick Ho Yu Chung Journal: Sci Rep Date: 2021-06-03 Impact factor: 4.379