Abstract
Summary
A brother and sister with Wilson's disease (hepatolenticular degeneration) were found to have low serum urate levels and were injected with isotopic uric acid for pool size and turnover rate determination. The pool size of both was well below normal and the turnover rate was approximately twice the normal value. Benemid accentuated these findings in the female but was not tried in the male because of its adverse clinical effect. The rapid rate of removal of uric acid is compared to the phenomenon of aminoaciduria that is common in this disease. When ribose nucleic acid was fed to the male subject there was a statistically significant increase in urinary uric acid and this “extra” uric acid was equivalent to about 55% of the calculated purine ingested. There was no significant rise in the excretion of nonuric acid purines after nucleic acid feeding and this suggests that exogenous purine is mainly converted to uric acid before excretion.
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