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May 1955

Contribution to Study of Wilson's Disease

Author Affiliations

Madrid, Spain

From the Department of Internal Medicine, University of Madrid (Professor, Dr. Gilsanz).

AMA Arch Intern Med. 1955;95(5):727-734. doi:10.1001/archinte.1955.00250110097012

We regard it of interest to report our study of three brothers affected with Wilson's disease, in two of whom the process appeared with its most characteristic clinical picture and in one of whom the only finding in relation to this disease was the presence of amino-aciduria.

The nature of the hepatolenticular degeneration that appears in Wilson's disease is yet unknown, but in the last few years, by evaluation of the disturbances of the metabolism of copper and the amino acids eliminated in the urine, new concepts have been brought forth which are worthy of further research.

Baker,1 in 1948, summarized the theories which up to that time had tried to explain the nature of Wilson's disease as follows: 1. It is a degenerative process affecting the liver and brain. 2. It is an acquired disease, and both organs are damaged simultaneously by an endogenous toxin. 3. The primary

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