Acta Pediátrica Española

ISSN 2014-2986

Información adicional

  • Num_publicacion 67(7)
  • Resumen_ingles

    Because of improved life expectancy of patients with cystic fibrosis (CF), the attention has increased towards other signs of the disease such as the hepatic affectation. The cystic fibrosis related liver disease (CFRLD) has become the second cause of mortality in these patients. Clinical diagnosis of liver disease in cystic fibrosis is difficult because the fibrotic liver lesion usually develops in asymptomatic individuals. In recent years, advances in the understanding of the pathogenesis of disease and of the mechanisms of liver fibrogenesis are leading to novel therapeutic approaches, as well as to identify patients at risk for the development of liver disease. However, at present ursodeoxicholic acid (UDCA) is the only available treatment for these patients. The aim of this work is to update the knowledge about the pathogenesis and diagnosis of CFRLD and to review the mechanisms of action of UDCA and its benefits when used in these patients.

  • Palabras_clave_ingles Cystic fibrosis liver disease ursodeoxycholic acid
  • Todos_autores M. García Peris, C. Ribes Koninckx
  • autores listados M. García Peris, C. Ribes Koninckx
  • Correspondecia
    M. García Peris. Pl. de Nou Moles 6, 24. 46018 Valencia.
    Correo electrónico: monika_gp@hotmail.com
  • Titulo_ingles Ursodeoxycholic acid and its application in liver disease associated with cystic fibrosis
  • Centros_trabajo Sección de Gastroenterología Pediátrica. Hospital Infantil «La Fe». Valencia
  • Publicado en Acta Pediatr Esp. 2009; 67(7): 315-318
  • copyright ©2009 Ediciones Mayo, S.A.
  • Fecha recepcion 28/01/08
  • Fecha aceptacion 08/02/08
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