The DOI matches its Crossref record; 67 of the 67 references it cites were found in a registry or an index.
Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes
Open accessAbstract
Biliary atresia is a severe cholangiopathy of early infancy that destroys extrahepatic bile ducts and disrupts bile flow. With a poorly defined disease pathogenesis, treatment consists of the surgical removal of duct remnants followed by hepatoportoenterostomy. Although this approach can improve the short-term outcome, the liver disease progresses to end-stage cirrhosis in most children. Further improvement in outcome will require a greater understanding of the mechanisms of biliary injury and fibrosis. Here, we review progress in the field, which has been fuelled by collaborative studies in larger patient cohorts and the development of cell culture and animal model systems to directly test hypotheses. Advances include the identification of phenotypic subgroups and stages of disease based on clinical, pathological and molecular features. Stronger evidence exists for viruses, toxins and gene sequence variations in the aetiology of biliary atresia, triggering a proinflammatory response that injures the duct epithelium and produces a rapidly progressive cholangiopathy. The immune response also activates the expression of type 2 cytokines that promote epithelial cell proliferation and extracellular matrix production by nonparenchymal cells. These advances provide insight into phenotype variability and might be relevant to the design of personalized trials to block progression of liver disease.
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Cites
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Liver fibrosis and repair: Immune regulation of wound healing in a solid organ
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Loss of IGF2 Imprinting: A Potential Marker of Colorectal Cancer Risk
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Congenital Diseases of Intrahepatic Bile Ducts: Variations on the Theme “Ductal Plate Malformation”
Desmet, V. J. (1992) · Hepatology · doi:10.1002/hep.1840160434 · Cited by 592
Interleukin-33-Dependent Innate Lymphoid Cells Mediate Hepatic Fibrosis
Mchedlidze, T., Waldner, M., Zopf, S., Walker, J., Rankin, A. L., Schuchmann, M., Voehringer, D., McKenzie, A. N. J., Neurath, M. F., Pflanz, S., et al. (2013) · Immunity · doi:10.1016/j.immuni.2013.07.018 · Cited by 502
Reversal of Left-Right Asymmetry: a Situs Inversus Mutation
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The frequency and outcome of biliary atresia in the UK and Ireland
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A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
Shneider, B. L., Brown, M. B., Haber, B., Whitington, P. F., Schwarz, K., Squires, R., Bezerra, J., Shepherd, R., Rosenthal, P., Hoofnagle, J. H., et al. (2006) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2005.12.054 · Cited by 403
Self-citation
Cited by
Biliary organoids uncover delayed epithelial development and barrier function in biliary atresia
Amarachintha, S. P., Mourya, R., Ayabe, H., Yang, L., Luo, Z., Li, X., Thanekar, U., Shivakumar, P., & Bezerra, J. A. (2022) · Hepatology · doi:10.1002/hep.32107 · Cited by 98
Self-citationGut microbial profile in biliary atresia: a case‐control study
Wang, J., Qian, T., Jiang, J., Yang, Y., Shen, Z., Huang, Y., Chen, G., Zheng, S., & Dong, R. (2020) · Journal of Gastroenterology and Hepatology · doi:10.1111/jgh.14777 · Cited by 51
Cytokine‐Producing B Cells Promote Immune‐Mediated Bile Duct Injury in Murine Biliary Atresia
Bednarek, J., Traxinger, B., Brigham, D., Roach, J., Orlicky, D., Wang, D., Pelanda, R., & Mack, C. L. (2018) · Hepatology · doi:10.1002/hep.30051 · Cited by 38
Metadata
- Authors
- Asai, A., Miethke, A., & Bezerra, J. A.
- Venue
- Nature Reviews Gastroenterology & Hepatology
- Published
- 2015-05-26
- Volume
- 12
- Issue
- 6
- Pages
- 342-352
- ISSN
- 1759-5045 (ISSN-L)
- 1759-5053
- OpenAlex
- W1945197404
- Cited by
- 279
- References
- 67
- FWCI
- 3.04
- Type
- journal article
- Language
- en
- Open access
- green
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