[Wilson's disease]. / Morbus Wilson.
Ther Umsch
; 64(1): 57-61, 2007 Jan.
Article
em De
| MEDLINE
| ID: mdl-17221826
Wilson's disease is a rare autosomal recessive disorder of hepatic copper transport leading to a biliary excretion inhibition of copper. Overload of the metal mainly in liver and basal ganglia leads to hepatic but also to extrapyramidal motor as well as psychiatric clinical symptoms. Diagnosis is based on clinical suspicion, parameters of copper metabolism, ophthalmic examination and a liver biopsy. A radiocopper test is able to identify patients even with inconsistent laboratory results. For initial assessment and follow-up neurophysiological investigation and MRI are recommended additionally. Genetic analysis can be helpful to detect asymptomatic relatives of the index patient. Dependent on the stage of the disease for therapy chelating drugs and zinc are possible but must be given lifelong without longer interruptions. With early diagnosis and consequent treatment the prognosis of Wilson's disease is excellent and usually the need for liver transplantation can be prevented.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Degeneração Hepatolenticular
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
/
Screening_studies
Aspecto:
Patient_preference
Limite:
Adolescent
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Adult
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Child
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Female
/
Humans
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Male
/
Pregnancy
Idioma:
De
Revista:
Ther Umsch
Ano de publicação:
2007
Tipo de documento:
Article