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September 14, 1964


JAMA. 1964;189(11):851. doi:10.1001/jama.1964.03070110053016

Descriptions of specific characteristics of diseases are usually first achieved by identification of histological changes and later by recognition of functional and biochemical abnormalities. This procedure appears to have been reversed in understanding the structural changes in the kidney in hepatolenticular degeneration (Wilson's disease). There have been many reports of abnormalities in renal function in this disease but histological or histochemical changes in the kidneys have not been previously described. Wolff1 recently investigated the histological changes in postmortem specimens of five patients with the disease. All specimens showed renal tubular epithelial cellular damage and degeneration; the glomeruli, however, were normal. Rubeanic-acid staining for copper demonstrated copper granules in the tubular epithelial cells. The deposition was not uniform throughout the kidney, but whenever copper was present it was intracytoplasmic in localization.

The fact that there was often heavy deposition in the same areas in which the maximum histological alterations were noted suggests a causal relationship. This supposition is in accord with the experimental finding that copper toxicity produces renal tubular changes in rats. Wilson's original hypothesis of a possible toxic cause for alterations of the liver and basal ganglia may also offer an explanation for

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