Prognosis of Wilson's disease in childhood.

Wilson's disease in childhood has several characters distinct from those in adults. The progression of the disease tends to be rapid, hepatic manifestations are common, cerebral symptoms related to dystonia are predominant, and tremor is rare. Forty-nine children with Wilson's disease under the age of 15 were treated with D-penicillamine for 2 to 15 years. None of the presymptomatic patients subsequently developed any symptoms of the disease. The results of treatment in patients who had exhibited only hepatic symptoms were also excellent. However, neurological manifestations associated with a history of jaundice or ascites responded less well to chelation. These observations clearly indicate that early diagnosis and treatment are extremely important to ensure normal lives for children with Wilson's disease.