作者: DJ Dolin , JR Asplin , L Flagel , M Grasso , DS Goldfarb
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摘要: Purpose: To determine the effect of cystine-binding thiol drugs (CBTD) on urinary cystine capacity in patients with cystinuria. Patients and Methods: Seven cystinuric patients performed two sets of urine collections while on and off CBTE while controlling for all other variables: diet and fluid and alkali intake. They monitored and recorded their diet for 3 days and performed urine collections on days 2 and 3. They then stopped the CBTD for 7 days. On days 8, 9, and 10, they replicated their diets of days I through 3 and performed two more urine collections on days 9 and 10. Two patients took D-penicillamine, four took tiopronin, and one took tiopronin and captopril. The cystine capacity was determined, and the values obtained when the patient was on and off the CBTE) were compared to determine whether CBTDs affect urinary cystine capacity. To measure the cystine capacity, we used a solid-phase assay in which …