The Clinical Analysis Of Fifteen Cases With Wilson's Disease

objective To study the clinical presentation ofWilson' s disease, increase the understanding of diagnosis andtreatment.Methods Fifteen patients with Wilson' s disease were diagnosedbetween 1998 and 2004 at our hospital. Their clinical featuresat presentation and laboratory findings were retrospectivelycollected.Results:(1)Two patients initially presented with hepaticsymptoms;two patients initially presented with renalsymptoms;eleven patients presented with neurological symptomsincluding tremor dysarthria . ataxia and psychiatricdiseases. (2) a pair of siblings had a family history of WD.three patients had parental consanguinity. (3) Ceruloplasmindecreased in 14 patients and Kayer-Fleischer ring occurred inall patients. (4) Penicillamine Zinc and low copper food weregenerally effective.Conclusions: Wilson' s disease is an autosomal recessive inherited disorder of copper metabolism, The clinical presentation of Wilson' s disease is very variable. The diagnosis of Wilson disease requires a combination of a variety of clinical symptoms, biochemical tests and family histories, and the pharmacological treatment of WD is commonly effective.