Heart involvement in hepatolenticular degeneration

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Abstract
The case of a 10 yr old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. Microscopic examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin-eosin and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and Cu deposition in the tissue. Cu concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. The relationship of clinical, laboratory and pathological findings to the degree of Cu storage in the myocardium is discussed.