A study of cystinuria in two sisters

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Abstract
In 2 cases of eystinuria, neither increase in the cystine content of the diet nor the addition to an ordinary diet of pure cystine (prepared from animal hair) increased the cystine output. Increase of animal protein in the diet increased the elimination of cystine in almost direct ratio, probably owing to the stimulation of tissue metabolism with the liberation of more endogenous cystine. The urine was abnormally alkaline; the solubility of cystine in alkaline solns. and the fact that, by choice, the patients never had a high animal protein diet, may explain the absence of calculi. In both cases albuminuria was present, varying with the amts. of cystme excreted. The excretion of greater amts. of cystine may be associated with deposition of cystine crystals in the renal tubules, damaging them and causing a leak of protein into them.

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