The prevalence and clinical features of Wilson's disease in Scotland were investigated.
Thirty three cases were identified but adequate information was available on only 28.
In 1989, the prevalence rate was 4 per million.
Ten patients with a mean (SEM) age of 18 (1.9) years presented with neurological symptoms, 12 patients aged 14 (1.7) years presented with hepatic symptoms, and six patients aged 12 (0.9) years were asymptomatic siblings of patients with Wilson's disease.
Nine (56%) of the 16 patients who underwent liver biopsy on presentation were found to have cirrhosis.
Mots-clés Pascal : Wilson maladie, Prévalence, Symptomatologie, Pronostic, Epidémiologie, Métabolisme pathologie, Maladie héréditaire, Système nerveux pathologie, Appareil digestif pathologie, Elément minéral, Cuivre, Ecosse, Grande Bretagne, Royaume Uni, Europe
Mots-clés Pascal anglais : Wilson disease, Prevalence, Symptomatology, Prognosis, Epidemiology, Metabolic diseases, Genetic disease, Nervous system diseases, Digestive diseases, Inorganic element, Copper, Scotland, Great britain, United Kingdom, Europe
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 92-0320790
Code Inist : 002B22E03. Création : 199406.