Genetic background and biliary atresia
Biliary atresia (BA) is a lethal hepatobiliary disorder in infants characterized by progressive destruction of intrahepatic and extrahepatic bile ducts and obstructive biliary fibrosis. Although hepatic portoenterostomy (Kasai procedure) can temporarily reconstruct bile drainage, persistent postoper...
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| Format: | Article |
| Language: | English |
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BMJ Publishing Group
2025-06-01
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| Series: | World Journal of Pediatric Surgery |
| Online Access: | https://wjps.bmj.com/content/8/3/e001023.full |
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| _version_ | 1850231660178571264 |
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| author | Jianghua Zhan Shaowen Liu Yu Meng Qianhui Yang Xingyuan Ke |
| author_facet | Jianghua Zhan Shaowen Liu Yu Meng Qianhui Yang Xingyuan Ke |
| author_sort | Jianghua Zhan |
| collection | DOAJ |
| description | Biliary atresia (BA) is a lethal hepatobiliary disorder in infants characterized by progressive destruction of intrahepatic and extrahepatic bile ducts and obstructive biliary fibrosis. Although hepatic portoenterostomy (Kasai procedure) can temporarily reconstruct bile drainage, persistent postoperative inflammation and hepatic fibrosis still lead to over half of the patients requiring liver transplantation for survival. Epidemiological studies reveal significant geographical and ethnic disparities in BA incidence, suggesting that genetic susceptibility plays an indispensable role in its pathogenesis. This article is based on the multidimensional interactive pathogenic hypothesis of BA of ‘embryonic developmental abnormalities, perinatal injury, and dysregulated immune microenvironment’ in addition to progressive hepatobiliary fibrosis. We review advances in the genetic and epigenetic regulatory networks of BA with the aim of providing ideas for future genetic research on this disease. |
| format | Article |
| id | doaj-art-711264e5e66a4d7e8dbea870bcb7315c |
| institution | OA Journals |
| issn | 2516-5410 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | World Journal of Pediatric Surgery |
| spelling | doaj-art-711264e5e66a4d7e8dbea870bcb7315c2025-08-20T02:03:27ZengBMJ Publishing GroupWorld Journal of Pediatric Surgery2516-54102025-06-018310.1136/wjps-2025-001023Genetic background and biliary atresiaJianghua Zhan0Shaowen Liu1Yu Meng2Qianhui Yang3Xingyuan Ke4Clinical School of Pediatrics, Tianjin Medical University, Tianjin, ChinaClinical School of Paediatrics, Tianjin Medical University, Tianjin, ChinaNational Engineering Research Center of Personalized Diagnostic and Therapeutic Technology, Changsha, Hunan Province, ChinaClinical School of Pediatrics, Tianjin Medical University, Tianjin, ChinaClinical School of Pediatrics, Tianjin Medical University, Tianjin, ChinaBiliary atresia (BA) is a lethal hepatobiliary disorder in infants characterized by progressive destruction of intrahepatic and extrahepatic bile ducts and obstructive biliary fibrosis. Although hepatic portoenterostomy (Kasai procedure) can temporarily reconstruct bile drainage, persistent postoperative inflammation and hepatic fibrosis still lead to over half of the patients requiring liver transplantation for survival. Epidemiological studies reveal significant geographical and ethnic disparities in BA incidence, suggesting that genetic susceptibility plays an indispensable role in its pathogenesis. This article is based on the multidimensional interactive pathogenic hypothesis of BA of ‘embryonic developmental abnormalities, perinatal injury, and dysregulated immune microenvironment’ in addition to progressive hepatobiliary fibrosis. We review advances in the genetic and epigenetic regulatory networks of BA with the aim of providing ideas for future genetic research on this disease.https://wjps.bmj.com/content/8/3/e001023.full |
| spellingShingle | Jianghua Zhan Shaowen Liu Yu Meng Qianhui Yang Xingyuan Ke Genetic background and biliary atresia World Journal of Pediatric Surgery |
| title | Genetic background and biliary atresia |
| title_full | Genetic background and biliary atresia |
| title_fullStr | Genetic background and biliary atresia |
| title_full_unstemmed | Genetic background and biliary atresia |
| title_short | Genetic background and biliary atresia |
| title_sort | genetic background and biliary atresia |
| url | https://wjps.bmj.com/content/8/3/e001023.full |
| work_keys_str_mv | AT jianghuazhan geneticbackgroundandbiliaryatresia AT shaowenliu geneticbackgroundandbiliaryatresia AT yumeng geneticbackgroundandbiliaryatresia AT qianhuiyang geneticbackgroundandbiliaryatresia AT xingyuanke geneticbackgroundandbiliaryatresia |