Medical treatment of cystinuria: Results of contemporary clinical practice
Medical treatment of cystinuria: Results of contemporary clinical practice
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DOI：
10.1016/s0022-5347(01)65451-x
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发表时间：
1996-11-01
影响因子：
6.6
通讯作者：
Streem, SB
中科院分区：
文献类型：
--
作者：
Chow, GK;Streem, SB
目的：我们确定了一个当代的医疗方案的有效性，为膀胱癌材料和方法：共16例胱氨酸尿症患者进行了为期7至141个月（平均78.1）。标准治疗包括水化和碱化。添加D-青霉胺或α-巯基丙酰甘氨酸用于水合和碱化失败，以防止新结石或结石生长，或引起溶解。对于D-青霉胺或α-巯基丙酰甘氨酸治疗失败或不耐受，添加卡托普利。每6 ~ 12个月进行一次放射学检查，记录结石事件。结果：在水化和碱化过程中，9例患者中有8例发生46例结石事件（每患者年1.6例）。加入巯基衍生物后，9例患者中有7例发生了24起结石事件，所有6例接受水化、碱化和卡托普利治疗的患者均发生了10起事件，5例接受碱化、巯基和卡托普利治疗的患者中有4例发生了8起事件（分别为0.52、0.71和0.54起事件/患者-年）。在104.1患者年的总治疗时间88结石事件发生在14 16例（0.84事件每患者年）。结论：D-青霉胺和α-巯基丙酰甘氨酸是有效的，在水化和碱化失败的患者中，降低结石形成率。虽然卡托普利在这种情况下也可能是有益的，但它似乎不如D-青霉胺或α-巯基丙酰甘氨酸有效，并且它没有明显地增加这些硫醇的临床益处。我们的研究表明，胱氨酸尿症患者在接受任何现代医疗方案治疗时复发的风险都很高。在评价新的或替代的医学和泌尿外科治疗模式的长期疗效时，必须考虑这种自然史。
Purpose: We determined the efficacy of a contemporary medical regimen for treaMaterials and Methods: A total of 16 patients with cystinuria was followed for 7 to 141 months (mean 78.1). Standard therapy included hydration and alkalization. D-penicillamine or alpha-mercaptoproprionylglycine was added for failure of hydration and alkalization to prevent new stones or stone growth, or to cause dissolution. Captopril was added for failure of or intolerance to D-penicillamine or alpha-mercaptopropionylglycine. Radiography was performed every 6 to 12 months, at which time stone events were documented.Results: During hydration and alkalization 46 stone events occurred in 8 of 9 patients (1.6 events per patient-year). With addition of thiol derivatives 7 of 9 patients experienced 24 stone events, all 6 treated with hydration, alkalization and captopril experienced 10 events, and 4 of 5 treated with alkalization, thiols and captopril experienced 8 events (0.52, 0.71 and 0.54 events per patient-year, respectively). During a total treatment time of 104.1 patient-years 88 stone events occurred in 14 of 16 patients (0.84 events per patient-year).Conclusions: D-penicillamine and alpha-mercaptopropionylglycine are effective in decreasing the rate of stone formation in patients in whom hydration and alkalization failed. While captopril may also be beneficial in this setting, it does not appear to be as effective as D-penicillamine or alpha-mercaptopropionylglycine, and it does not clearly add clinical benefit to those thiols. Our study demonstrates that patients with cystinuria are at high risk for recurrence when treated with any contemporary medical program. This natural history must be considered when evaluating the long-term efficacy of newer or alternative modes of medical and urological treatment.