A case of Wilson's disease associated with cholelithiasis / 대한내과학회지
Korean Journal of Medicine
;
: 82-87, 2008.
Artigo
em Coreano
| WPRIM
| ID: wpr-164622
ABSTRACT
Wilson's disease is a rare autosomal recessive metabolic disease. The ATB7B gene mutation results in a defect of biliary copper excretion and subsequent accumulation of copper in the liver, brain, and sclera. The usual clinical signs of Wilson's disease include hepatitis, liver cirrhosis, movement disorder, or a Kayser-Fleisher ring in the sclera, but patients occasionally present with hepatic failure or hemolytic anemia. Under such metabolic conditions, free copper induce chronic hemolysis with oxidative damage via free radical production, and chronic hemolysis, in turn, can cause secondary pigment bililary stone formation. Herein we report a case of Wilson's disease associated with cholelithiasis in a young female.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Esclera
/
Encéfalo
/
Colelitíase
/
Falência Hepática
/
Cobre
/
Hemólise
/
Hepatite
/
Degeneração Hepatolenticular
/
Anemia Hemolítica
/
Fígado
Limite:
Humanos
Idioma:
Coreano
Revista:
Korean Journal of Medicine
Ano de publicação:
2008
Tipo de documento:
Artigo
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