Advances in biliary atresia: from patient care to research
Braz. j. med. biol. res
; 43(6): 522-527, June 2010. tab
Article
in English
| LILACS
| ID: lil-548269
Responsible library:
BR1.1
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.
Full text:
Available
Collection:
International databases
Database:
LILACS
Main subject:
Biliary Atresia
Type of study:
Diagnostic study
/
Etiology study
/
Practice guideline
/
Prognostic study
/
Screening study
Limits:
Animals
/
Humans
Language:
English
Journal:
Braz. j. med. biol. res
Journal subject:
Biology
/
Medicine
Year:
2010
Document type:
Article
Affiliation country:
Brazil
/
United States
Institution/Affiliation country:
Cincinnati Children's Hospital Medical Center/US
/
Hospital de Base do Distrito Federal/BR
/
Universidade Federal of Rio Grande do Sul/BR