Serum and tissue tumor growth factor β 1 in children with biliary atresia
Abstract Background Biliary atresia (BA) is an infantile disorder characterized by the obstruction of a portion or the entirety of the extrahepatic bile ducts, leading to hepatic fibrosis and loss of liver function. The gold standard for diagnosing and grading fibrosis is liver biopsy, but there are...
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Published in: | Journal of pediatric surgery Vol. 45; no. 9; pp. 1784 - 1790 |
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Main Authors: | , , , , , , , |
Format: | Journal Article |
Language: | English |
Published: |
01-09-2010
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Subjects: | |
Online Access: | Get full text |
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Summary: | Abstract Background Biliary atresia (BA) is an infantile disorder characterized by the obstruction of a portion or the entirety of the extrahepatic bile ducts, leading to hepatic fibrosis and loss of liver function. The gold standard for diagnosing and grading fibrosis is liver biopsy, but there are many groups searching for noninvasive biomarkers that could replace and/or complement this procedure. Methods and Materials In this study, we evaluated serum and tissue transforming growth factor β 1 (TGF β 1) and aspartate aminotransferase [AST]-to-platelet ratio index (APRI) in patients with BA at the time of diagnosis and at liver transplantation and correlated these data with tissue collagen density, to verify if they could act as biomarkers for BA. Results At the time of diagnosis, TGF β 1 levels were highly variable in BA patients. However, serum values at transplantation were significantly decreased (13.75 ± 3.68 ng/mL) as compared to controls (34.36 ± 9.35 ng/mL) ( P = .01). No correlation was found between serum TGF1 β 1 and collagen density in both groups analyzed. Serum TGF β 1 showed no correlation with APRI at diagnosis. At the time of liver transplantation, all patients had low serum TGF β 1 and variable APRI, although all higher than 2.0. However, when platelet count was used, an inverse correlation with serum TGF β 1 was observed at the time of diagnostics ( r2 = 0.749; P = .03). Conclusions Our findings suggest that at the time of diagnosis the fibrogenic process is active, with higher levels of TGF β 1, whereas later on, there is scar tissue, with reduced TGF β 1 expression. Although our results should be confirmed in larger sets of patients with BA, the lack of TGF β 1 at the time of liver transplantation may have important consequences for the patient because it is a pleiotropic molecule, responsible for many functions in the body, mainly those related to immune response and cell growth. |
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ISSN: | 0022-3468 |
DOI: | 10.1016/j.jpedsurg.2010.04.007 |