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Heart involvement in hepatolenticular degeneration
Author(s) -
Azevedo E. M.,
Scaff M.,
Barbosa E. R.,
Neto A. E. Gouveia,
Canelas H. M.
Publication year - 1978
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1978.tb02890.x
Subject(s) - degeneration (medical) , pathological , pathology , biopsy , medicine , h&e stain , staining
The case of a 10‐year‐old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin‐eosine and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and the presence of copper deposition in the tissue. Copper concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. Comments are made on the relationship of the clinical, laboratory and pathological findings with the degree of copper storage on the myocardium.