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Titolo:
Expression of collagens type I and IV osteonectin and transforming growth factor beta-1 (TGF beta 1) in biliary atresia and paucity of intrahepatic bile ducts during infancy
Autore:
Lamireau, T; Le Bail, B; Boussarie, L; Fabre, M; Vergnes, P; Bernard, O; Gautier, F; Bioulac-Sage, P; Rosenbaum, J;
Indirizzi:
Univ Bordeaux 2, Grp Rech Etud Foie, F-33076 Bordeaux, France Univ Bordeaux 2 Bordeaux France F-33076 d Foie, F-33076 Bordeaux, France Hop Pellegrin, Unite Gastroenterol Pediat, F-33076 Bordeaux, France Hop Pellegrin Bordeaux France F-33076 l Pediat, F-33076 Bordeaux, France Hop Pellegrin, Anat Pathol Lab, F-33076 Bordeaux, France Hop Pellegrin Bordeaux France F-33076 thol Lab, F-33076 Bordeaux, France Hop Pellegrin, Serv Chirurg Pediat, F-33076 Bordeaux, France Hop Pellegrin Bordeaux France F-33076 g Pediat, F-33076 Bordeaux, France Hop Bicetre, Anat Pathol Lab, Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France l Lab, Le Kremlin Bicetre, France Hop Bicetre, Serv Hepatol Pediat, Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France ediat, Le Kremlin Bicetre, France Hop Bicetre, Serv Chirurg Pediat, Le Kremlin Bicetre, France Hop Bicetre Le Kremlin Bicetre France ediat, Le Kremlin Bicetre, France
Titolo Testata:
JOURNAL OF HEPATOLOGY
fascicolo: 2, volume: 31, anno: 1999,
pagine: 248 - 255
SICI:
0168-8278(199908)31:2<248:EOCTIA>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
HUMAN LIVER MYOFIBROBLASTS; EXTRACELLULAR-MATRIX; HEPATIC-FIBROSIS; ITO CELLS; PROTEIN; SPARC; GROWTH-FACTOR-BETA-1; PROCOLLAGEN; PROLIFERATION; INHIBITOR;
Keywords:
biliary atresia; cholestasis; fibrogenesis; infants;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
30
Recensione:
Indirizzi per estratti:
Indirizzo: Lamireau, T Univ Bordeaux 2, Grp Rech Etud Foie, 146 Rue Leo Saignat, F-33076 Bordeaux, France Univ Bordeaux 2 146 Rue Leo Saignat Bordeaux France F-33076 e
Citazione:
T. Lamireau et al., "Expression of collagens type I and IV osteonectin and transforming growth factor beta-1 (TGF beta 1) in biliary atresia and paucity of intrahepatic bile ducts during infancy", J HEPATOL, 31(2), 1999, pp. 248-255

Abstract

Background/Aims: Biliary atresia and paucity of intrahepatic bile ducts are the main causes of neonatal cholestasis leading to hepatic fibrosis. Fibrotic evolution is slow in paucity of bile ducts as compared to the rapid progression to biliary cirrhosis in biliary atresia when cholestasis persistsdespite hepatoportoenterostomy, Our aim was to compare the expression of collagens type I and IV, alpha-smooth muscle actin, osteonectin and transforming growth factor beta 1 in biliary atresia and paucity of bile ducts. Methods: Liver biopsies were obtained in 12 children with biliary atresia and in five with paucity of bile ducts. Collagens type I and IV alpha-smooth muscle actin were detected with immunostaining. Collagens type I and IV, osteonectin and transforming growth factor beta 1 mRNAs were detected by insitu hybridization. Results: Expression of mRNA and proteins was roughly parallel. In ductularproliferation areas of biliary atresia: (1) the expression of collagens type I and IV and osteonectin was increased, and was localized to periductular myofibroblasts; (2) transforming growth factor beta 1 was expressed around biliary ductules, probably in inflammatory cells, land also in biliary cells. Osteonectin expression was also increased in the lobules, In paucity of bile ducts, there was no overexpression of collagens type I and IV and transforming growth factor beta 1, except in the only child with marked fibrosis, However, osteonectin expression was enhanced at the periphery of the lobules, even when fibrosis was mild or absent. Conclusions: These findings suggest that in biliary atresia ductular proliferation areas are the site of a marked production of extracellular matrix proteins in periductular myofibroblasts, probably secondary to transforminggrowth factor beta 1 production by inflammatory cells and by biliary cells. The weak expression of transforming growth factor beta 1 could explain the slow progression of fibrosis in paucity of bile ducts.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 11/07/20 alle ore 16:01:22