影响cystine-binding硫醇药物对胱氨酸尿患者尿胱氨酸能力。

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格拉索道林DJ, Asplin JR,来自um L, M,戈德法布DS

影响cystine-binding硫醇药物对胱氨酸尿患者尿胱氨酸能力。

J Endourol。2005年4月,19 (3):429 - 32。

PubMed ID
15865542 (在PubMed
]
文摘

目的:确定的影响cystine-binding硫醇药物(CBTD)尿胱氨酸胱氨酸尿患者的能力。病人和方法:七cystinuric患者进行两组尿液收集同时开关CBTD并控制所有其他变量:饮食和流体和碱的摄入量。他们监控和记录他们的饮食上3天,进行尿液收集天2和3。然后他们停止CBTD 7天。8天8,9,10,他们复制天1到3的饮食和执行两个天9和10上尿液收集。两个病人D-penicillamine,四个tiopronin,和一个带tiopronin和卡托普利。胱氨酸能力决定,当病人获得的值在CBTD进行比较,以确定CBTD影响尿胱氨酸能力。测量胱氨酸能力,我们使用了一个固相测定胱氨酸晶体被添加到48小时尿液和孵化。晶体旋转下来resolubilized在减缓冲区,和胱氨酸晶体计算。固相将从尿胱氨酸(负胱氨酸能力)过饱和而放弃胱氨酸欠饱和尿液(积极胱氨酸能力)。 RESULTS: All seven patients had significant improvement in urinary cystine capacity on CBTDs. The mean cystine 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.

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药物