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January 1967

Picture of the Month

Am J Dis Child. 1967;113(1):177-NP. doi:10.1001/archpedi.1967.02090160227040

Denouement and Discussion 

WILSON'S DISEASE  (Hepato-lenticular Degeneration)

MANIFESTATIONS  Symptoms may range from only hepatic or neurological manifestations to involvement of both systems. In childhood, Wilson's disease presents frequently as an acute hepatitis which becomes chronic or recurrent, or as cirrhosis. Subsequently neurological changes appear. However, the initial symptomatology may consist of neurologic or psychologic signs and symptoms, but this type of onset is more characteristic in adult life than in childhood. In those patients who present with a "liver picture," acute viral hepatitis is frequently diagnosed because of mild jaundice and liver enlargement. As the disease progresses with the development of a nodular cirrhosis, splenomegaly, gastrointestinal bleeding, esophageal varices, ascites, anorexia, and lassitude appear. The neurological signs may be insidious and overlooked because of the liver involvement. There is intellectual deterioration which may first be noted by the schoolteacher. Initially, dystonia or abnormal posturing may take place. There

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