Advances in biliary atresia: from patient care to research
Braz. j. med. biol. res
;
43(6): 522-527, June 2010. tab
Artigo
em Inglês
| LILACS
| ID: lil-548269
ABSTRACT
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.
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Atresia Biliar
Tipo de estudo:
Estudo diagnóstico
/
Estudo de etiologia
/
Guia de Prática Clínica
/
Estudo prognóstico
/
Estudo de rastreamento
Limite:
Animais
/
Humanos
Idioma:
Inglês
Revista:
Braz. j. med. biol. res
Assunto da revista:
Biologia
/
Medicina
Ano de publicação:
2010
Tipo de documento:
Artigo
País de afiliação:
Brasil
/
Estados Unidos
Instituição/País de afiliação:
Cincinnati Children's Hospital Medical Center/US
/
Hospital de Base do Distrito Federal/BR
/
Universidade Federal of Rio Grande do Sul/BR
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