title: Measurement of urinary cystine and cysteinyl-penicillamine in patients with cystinuria.
abstract: A high-performance liquid chromatographic method with electrochemical detection (LC/EC) was developed to measure cystine and cysteinyl-penicillamine disulfide in the urine of patients screened or treated for cystinuria. Urine was acidified, centrifuged to remove urinary protein, diluted and injected. The disulfides were separated on a reversed-phase column, reduced at the upstream electrode of a dual electrochemical detector with gold-mercury amalgam (Au/Hg) electrodes and the resultant thiols measured at the downstream electrode. The sample preparation is simple, the analysis rapid, specimens can be easily batched and the specificity of the method is better than those of two other separative procedures with which it was compared. The coefficient of variation for cystine in cystinuric urine is 6.7%, 5.5% and 3.2% for levels of 0.09, 0.52 and 1.02 mmol/l respectively, and for cysteinyl-penicillamine disulfide 2.6% and 7.5% for levels of 0.45 and 0.98 mmol/l respectively. Urine for analysis of these disulfides should not be collected within 24 hours of administration of the radiopaque agent diatrizoate but no other interference to the assay has been noted. This method is suitable as a screen for cystinuria in patients with renal tract calculi, for ongoing monitoring of cystinuric patients and to check patient compliance with d-penicillamine therapy.
mesh_terms: D000596:Amino Acids / Q000032:analysis; D002849:Chromatography, Gas; D002851:Chromatography, High Pressure Liquid; D003545:Cysteine / Q000031:analogs & derivatives* / Q000652:urine; D003555:Cystinuria / Q000652:urine*; D004563:Electrochemistry; D006801:Humans; D010396:Penicillamine / Q000031:analogs & derivatives* / Q000652:urine; D013438:Sulfhydryl Compounds / Q000652:urine
chemical_list: D000596:Amino Acids; D013438:Sulfhydryl Compounds; C030163:penicillamine cysteine disulfide; D010396:Penicillamine; D003545:Cysteine