Effect of cystine-binding thiol capacity in patients drugs on urinary cystine with cystinuria
Effect of cystine-binding thiol capacity in patients drugs on urinary cystine with cystinuria
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DOI：
10.1089/end.2005.19.429
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发表时间：
2005-04-01
影响因子：
2.7
通讯作者：
Goldfarb, DS
中科院分区：
文献类型：
--
作者：
Dolin, DJ;Asplin, JR;Goldfarb, DS
目的：为了确定胱氨酸结合巯基药物（CBTD）对尿胱氨酸容量的影响，在患者cystinuria.Patients和方法：七个胱氨酸尿患者进行了两组尿液收集，而对和关闭CBTE，同时控制所有其他变量：饮食和液体和碱的摄入量。他们监测并记录了3天的饮食，并在第2天和第3天进行尿液收集。然后他们停止了CBTD 7天。在第8、9和10天，他们重复了第1天至第3天的饮食，并在第9和10天再进行了两次尿液收集。2例患者服用D-青霉胺，4例服用硫普罗宁，1例服用硫普罗宁和卡托普利。测定胱氨酸容量，并比较当患者开启和关闭CBTE时获得的值，以确定CBD是否影响尿胱氨酸容量。为了测量胱氨酸容量，我们使用固相测定法，其中将胱氨酸晶体加入尿液中并孵育48小时。将晶体离心并在高pH缓冲液中重新溶解，并计算晶体中胱氨酸的量。固相将从过饱和的尿中摄取胱氨酸（负胱氨酸容量），并将胱氨酸释放到不饱和的尿中（正胱氨酸容量）。CBTD后平均囊蛋白含量为-130.6 ± 280.8，CBTD后为43.1 ± 131.2（P < 0.05）。在CBTD方面，两名患者仍然有负值，但都有重要的改善。CBTD治疗前后的平均尿量相似，表明液体摄入充足且相似。尿pH值和尿排泄的钠和尿素也是可比的，表明一致性的柠檬酸盐摄入量和diet.Conclusions：我们的研究结果表明，CBTDs降低尿中胱氨酸过饱和，如所示的一个较少的负或更多的积极胱氨酸的能力。胱氨酸容量可以直接测量，即使在存在CBD的情况下。这种测量的价值在于监测对药物的反应，规定最低有效剂量，并可能减少通常与CBD相关的不良反应。
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 cystine crystals are added to the urine and incubated for 48 hours. The crystals are spun down and resolubilized in high-pH buffer, and the amount of cystine in the crystals is calculated. The solid phase will take up cystine from urine (negative cystine capacity) that is supersaturated and give up cystine to an undersaturated urine (positive cystine capacity).Results: All seven patients had significant improvement in urinary cystine capacity on CBTDs. The mean cystin capacity off CBTD was -130.6 ± 280.8, while the value during CBTD use was 43.1 ± 131.2 (P < 0.05). On CBTDs, two patients still had negative values, but both had important improvements. The mean urinary volumes were similar on and off CBTD, indicating adequate and similar fluid intake. Urine pH values and urinary excretion of sodium and urea also were comparable, indicating consistency of citrate intake and diet.Conclusions: Our results demonstrate that CBTDs lower the urinary supersaturation of cystine, as shown by a less-negative or more-positive cystine capacity. Cystine capacity can be measured directly, even in the presence of CBTDs. The value of this measurement lies in the potential to monitor the response to the drug, prescribe the minimum effective dose, and potentially decrease the adverse effects often associated with CBTDs.