145.

Bickel H, Neale FC, Hall G. Clinical and biochemical study of hepatolenticular degeneration (Wilson's disease). Quart. J Med. 1957; 26:527-528. (CA52:3994f). [Of 12 patients studied, all showed cupruria and reduced serum-Cu levels, but the same conditions were found in near relatives without clinical manifestations. In 3 cases Cu was high in various organs. Cu-balance expts. showed a diminished Cu output in the stool. A characteristic amino-aciduria was present in all but 3 cases. Normal levels of a-amino N and of individual amino acids in the blood suggest a renal mechanism, although occasional raised values, and certain changes in the amino-acid pattern of the urine indicate that an addnl. hepatic factor may develop. The similarity of the amino aciduria to that seen in Pb poisoning and it absence in 2 of the youngest patients, suggest that it is a secondary feature of the disease due to tubular damage by C11. Proteinuria and glucosuria may have a similar origin, although small amts. of other sugars in the urine may be due to liver insufficiency. There is no correlation between amino-aciduria and the degree of damage to liver or brain. In an extensive trial of Cu-removing agents, intravenous Versene (EDTA) was more successful than BAL or Mo. If given parenterally, EDTA did not seem to lose its Cu-removing powers, even after months of continuous administration, nor to produce toxic effects; but when given orally it led to an increased cupruria in 3 of 6 patients. There was no evidence of the production of amino-aciduria. However, there was no clinical improvement in spite of Cu removal, which may be due to failure to produce a neg. Cu balance for a sufficiently long time.]

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