Advances in biliary atresia : from patient care to research
Autor(a) principal: | |
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Data de Publicação: | 2010 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFRGS |
Texto Completo: | http://hdl.handle.net/10183/28276 |
Resumo: | Biliary atresia, the most common cause of liver transplantation in children, remains a challenge for clinicians and investigators. The development of new therapeutic options, besides the typical hepatoportoenterostomy, depends on a greater understanding of its pathogenesis and how it relates to the clinical phenotypes at diagnosis and the rate of disease progression. In this review, we present a perspective of how recent research has advanced the understanding of the disease and has improved clinical care protocols. Molecular and morphological analyses at diagnosis point to the potential contributions of polymorphism in the CFC1 and VEGF genes, to the pathogenesis of the disease, and to an association between the degree of bile duct proliferation and long-term outcome. In experimental models, cholangiocytes do not appear to have antigen-presenting properties despite a substantial innate and adaptive immune response that targets the biliary epithelium and produces duct obstruction. Initial clinical trials assessing the efficacy of corticosteroids in decreasing the inflammation and improving outcome do not show a superior effect of corticosteroids as an adjuvant treatment following hepatoportoenterostomy. The best outcome still remains linked to an early diagnosis and surgical treatment. In this regard, the Yellow Alert campaign by the Sociedade Brasileira de Pediatria and the inclusion of the Stool Color Card in the health booklet given to every neonate in Brazil have the potential to decrease the age of diagnosis, shorten the time between diagnosis and surgical treatment, and improve the long-term outcome of children with this devastating disease. |
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Santos, J. L.Carvalho, E.Bezerra, J. A.2011-03-26T06:01:41Z20100100-879Xhttp://hdl.handle.net/10183/28276000758593Biliary atresia, the most common cause of liver transplantation in children, remains a challenge for clinicians and investigators. The development of new therapeutic options, besides the typical hepatoportoenterostomy, depends on a greater understanding of its pathogenesis and how it relates to the clinical phenotypes at diagnosis and the rate of disease progression. In this review, we present a perspective of how recent research has advanced the understanding of the disease and has improved clinical care protocols. Molecular and morphological analyses at diagnosis point to the potential contributions of polymorphism in the CFC1 and VEGF genes, to the pathogenesis of the disease, and to an association between the degree of bile duct proliferation and long-term outcome. In experimental models, cholangiocytes do not appear to have antigen-presenting properties despite a substantial innate and adaptive immune response that targets the biliary epithelium and produces duct obstruction. Initial clinical trials assessing the efficacy of corticosteroids in decreasing the inflammation and improving outcome do not show a superior effect of corticosteroids as an adjuvant treatment following hepatoportoenterostomy. The best outcome still remains linked to an early diagnosis and surgical treatment. In this regard, the Yellow Alert campaign by the Sociedade Brasileira de Pediatria and the inclusion of the Stool Color Card in the health booklet given to every neonate in Brazil have the potential to decrease the age of diagnosis, shorten the time between diagnosis and surgical treatment, and improve the long-term outcome of children with this devastating disease.application/pdfengBrazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas. Ribeirão Preto. Vol. 43, n. 6 (jun. 2010), p. 522-527Atresia biliarPrognósticoTerapêuticaBiliary atresiaEtiologyPrognosisTherapeuticsAdvances in biliary atresia : from patient care to researchinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/otherinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT000758593.pdf.txt000758593.pdf.txtExtracted Texttext/plain32220http://www.lume.ufrgs.br/bitstream/10183/28276/2/000758593.pdf.txt3a58663233136f6034c37f19d348df0bMD52ORIGINAL000758593.pdf000758593.pdfTexto completo (inglês)application/pdf373488http://www.lume.ufrgs.br/bitstream/10183/28276/1/000758593.pdfcda86d6095ab602852858e2e0456cc58MD51THUMBNAIL000758593.pdf.jpg000758593.pdf.jpgGenerated Thumbnailimage/jpeg1950http://www.lume.ufrgs.br/bitstream/10183/28276/3/000758593.pdf.jpg665317d5b1690badb9387bcf3af87c22MD5310183/282762023-10-28 03:34:30.626531oai:www.lume.ufrgs.br:10183/28276Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2023-10-28T06:34:30Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.pt_BR.fl_str_mv |
Advances in biliary atresia : from patient care to research |
title |
Advances in biliary atresia : from patient care to research |
spellingShingle |
Advances in biliary atresia : from patient care to research Santos, J. L. Atresia biliar Prognóstico Terapêutica Biliary atresia Etiology Prognosis Therapeutics |
title_short |
Advances in biliary atresia : from patient care to research |
title_full |
Advances in biliary atresia : from patient care to research |
title_fullStr |
Advances in biliary atresia : from patient care to research |
title_full_unstemmed |
Advances in biliary atresia : from patient care to research |
title_sort |
Advances in biliary atresia : from patient care to research |
author |
Santos, J. L. |
author_facet |
Santos, J. L. Carvalho, E. Bezerra, J. A. |
author_role |
author |
author2 |
Carvalho, E. Bezerra, J. A. |
author2_role |
author author |
dc.contributor.author.fl_str_mv |
Santos, J. L. Carvalho, E. Bezerra, J. A. |
dc.subject.por.fl_str_mv |
Atresia biliar Prognóstico Terapêutica |
topic |
Atresia biliar Prognóstico Terapêutica Biliary atresia Etiology Prognosis Therapeutics |
dc.subject.eng.fl_str_mv |
Biliary atresia Etiology Prognosis Therapeutics |
description |
Biliary atresia, the most common cause of liver transplantation in children, remains a challenge for clinicians and investigators. The development of new therapeutic options, besides the typical hepatoportoenterostomy, depends on a greater understanding of its pathogenesis and how it relates to the clinical phenotypes at diagnosis and the rate of disease progression. In this review, we present a perspective of how recent research has advanced the understanding of the disease and has improved clinical care protocols. Molecular and morphological analyses at diagnosis point to the potential contributions of polymorphism in the CFC1 and VEGF genes, to the pathogenesis of the disease, and to an association between the degree of bile duct proliferation and long-term outcome. In experimental models, cholangiocytes do not appear to have antigen-presenting properties despite a substantial innate and adaptive immune response that targets the biliary epithelium and produces duct obstruction. Initial clinical trials assessing the efficacy of corticosteroids in decreasing the inflammation and improving outcome do not show a superior effect of corticosteroids as an adjuvant treatment following hepatoportoenterostomy. The best outcome still remains linked to an early diagnosis and surgical treatment. In this regard, the Yellow Alert campaign by the Sociedade Brasileira de Pediatria and the inclusion of the Stool Color Card in the health booklet given to every neonate in Brazil have the potential to decrease the age of diagnosis, shorten the time between diagnosis and surgical treatment, and improve the long-term outcome of children with this devastating disease. |
publishDate |
2010 |
dc.date.issued.fl_str_mv |
2010 |
dc.date.accessioned.fl_str_mv |
2011-03-26T06:01:41Z |
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Brazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas. Ribeirão Preto. Vol. 43, n. 6 (jun. 2010), p. 522-527 |
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