CLINICAL STUDY ON CYSTINURIA

Abstract

Clinical study was conducted on 26 cases of cystine stone formers who were found during past 22 years. Urinary amino acid excretion was compared between cystine stone formers and patients with chemical cystinuria. The results were as follows.
1) Cystine stone formers account for 0.62% of all stone patients.
2) Male cystine stone formers were 1.6cm shorter than that of controls, and female patients were 4.5cm shorter as well.
3) Urinary cystine and uric acid excretion were correlated with that in cystine stone formers.
4) Cystine, lysine, arginine, and ornithine excretion in cystine stone formers were all elevated, and these amounts were significantly higher than that of chemical cystinuria except lysine.
5) Kidney stone formation was observed in one of 6 homozygous cystinuria discovered by screening.
6) Stone dissolution was successful in 64.3% of the patients and the prevention of stone recurrence in 38.9%.
7) Alkakinization of urine could be achieved by sodium bicarbonate administration when the urine was acidic.
8) Dietary methionine restriction resulted in 28% and 25% reduction of urinary cystine excretion in 2 patients.
9) Side effect of alpha-mercaptopropionylglycine was observed in 31% of patients including one case of nephrose and 2 cases of liver disfuncion.