Urinary cystine excretion and capacity in patients with cystinuria

Kidney International
D GoldfarbJ Asplin

Abstract

The treatment of cystinuria is hampered by methods used to measure urinary lithogenicity. Most cystine assays cannot reliably distinguish cystine from soluble thiol drug-cysteine complexes. We used a solid-phase assay of urinary cystine capacity in a large sample of patients with cystinuria. A known amount of solid-phase cystine is added to urine. In supersaturated urine, cystine precipitates onto added crystals, so the solid phase recovered after incubation will be greater than that added. We studied the effect of cystine-binding thiol drugs (CBTD) to solubilize cystine and determined correlates of cystine capacity in patients who were and were not taking CBTD. Increasing concentrations of D-penicillamine, tiopronin and captopril dissolved cystine in urine with similar efficacy. A general linear model in which 24 h cystine excretion was the dependent variable showed that creatinine, urea nitrogen, and sodium excretions were associated with cystine excretion (P<0.02, all three). Urine volume, pH, and cystine excretion strongly correlated with cystine capacity (P<0.001). Tiopronin had no effect on supersaturation in a cross-sectional analysis. A subset of supersaturated samples, with negative cystine capacity, occurred mainly am...Continue Reading

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Citations

Jan 22, 2013·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Vidar O EdvardssonRunolfur Palsson
Aug 7, 2007·The Urologic Clinics of North America·Alexandra RogersDean G Assimos
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Apr 30, 2021·Néphrologie & thérapeutique·Caroline Prot-BertoyeMarie Courbebaisse

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