Rizzoni G, Pavanello L, Dussini N, Chiandetti L, Zacchello G
J Urol. 1979 Sep;122(3):381-2. doi: 10.1016/s0022-5347(17)56420-4.
We report 2 cases of cystinuria in which a nephrotic syndrome developed during treatment with alpha-mercaptopropionylglycine. This syndrome resolved after withdrawal of the drug and it did not recur when the alpha-mercaptopropionylglycine was given again in lower doses. The hypothesis is made that the nephrotic syndrome was dose-related. The alpha-mercaptopropionylglycine must be used in doses less than 50 mg./kg per day with regular monitoring of 24-hour urinary protein in cystinuric children.
我们报告2例胱氨酸尿症患者,他们在使用α-巯基丙酰甘氨酸治疗期间出现了肾病综合征。停药后该综合征得以缓解,当再次给予较低剂量的α-巯基丙酰甘氨酸时未复发。据此提出肾病综合征与剂量相关的假说。对于胱氨酸尿症患儿,使用α-巯基丙酰甘氨酸时剂量必须小于每日50mg/kg,并定期监测24小时尿蛋白。