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Wilson disease as a cause of liver injury in cystic fibrosis.
Kotalová, Radana; Jirsa, Milan; Vávrová, Vera; Vrábelová-Pouchlá, Slávka; Macek, Milan.
Afiliación
  • Kotalová R; Department of Pediatrics, Charles University 2nd Medical School and Faculty Hospital Motol, V Uvalu 84, 150 06 Praha 5, Czech Republic. radana.kotalova@seznam.cz
J Cyst Fibros ; 8(1): 63-5, 2009 Jan.
Article en En | MEDLINE | ID: mdl-18948065
Cystic fibrosis-related liver disease affects approximately one third of all patients with cystic fibrosis. Initial signs of other liver diseases including the genetically determined disorders of the liver co-inherited with cystic fibrosis may be obscured by or ascribed to cystic fibrosis-related liver disease. We report a patient shown to suffer simultaneously from cystic fibrosis and hepatic Wilson disease. Our case documents that in patients with cystic fibrosis presenting with liver disease, when unusual clinical and/or laboratory abnormalities appear and fail to respond to standard therapy, a second disease, including rare inherited metabolic disorders such as the hepatic form of Wilson disease or alpha(1)-antitrypsin deficiency, should be suspected.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fibrosis Quística / Degeneración Hepatolenticular Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Child / Child, preschool / Humans / Male / Newborn Idioma: En Revista: J Cyst Fibros Año: 2009 Tipo del documento: Article País de afiliación: República Checa Pais de publicación: Países Bajos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fibrosis Quística / Degeneración Hepatolenticular Tipo de estudio: Diagnostic_studies / Prognostic_studies Límite: Child / Child, preschool / Humans / Male / Newborn Idioma: En Revista: J Cyst Fibros Año: 2009 Tipo del documento: Article País de afiliación: República Checa Pais de publicación: Países Bajos