Sloand J A, Izzo J L
Arch Intern Med. 1987 Aug;147(8):1409-12.
Cystinuria is characterized by cystine stone formation and loss of renal function. Conservative therapy is generally ineffective and penicillamine therapy can be complicated by serious side effects. To our knowledge, we report the first clinical use of captopril in the treatment of homozygous cystinuria in two siblings. In the first patient, a 70% reduction in cystine excretion was observed after 26 weeks of therapy with 150 mg/d of captopril. Discontinuation of use of the drug resulted in a return to baseline cystine excretion, further loss of renal function, and nephrotic range proteinuria. Repeated treatment with captopril stabilized renal function, reduced proteinuria, and returned cystine excretion to near normal levels. In the second patient, cystine excretion was reduced by 93% after nine weeks of therapy with 75 mg/d of captopril. No adverse side effects were observed in either patient. Formation of the captopril-cysteine disulfide accounts for part of the reduction in cystine excretion but other mechanisms probably contribute. Because captopril-cysteine disulfide is 200 times more soluble than cystine, long-term captopril therapy may be useful in the treatment of cystinuria.
胱氨酸尿症的特征是胱氨酸结石形成和肾功能丧失。保守治疗通常无效，而青霉胺治疗可能会出现严重副作用。据我们所知，我们报告了卡托普利在两名同胞纯合子胱氨酸尿症治疗中的首次临床应用。在首例患者中，使用150mg/d卡托普利治疗26周后，胱氨酸排泄量减少了70%。停药后，胱氨酸排泄量恢复到基线水平，肾功能进一步丧失，出现肾病范围蛋白尿。再次使用卡托普利治疗可稳定肾功能、减少蛋白尿，并使胱氨酸排泄量恢复到接近正常水平。在第二例患者中，使用75mg/d卡托普利治疗9周后，胱氨酸排泄量减少了93%。两名患者均未观察到不良副作用。卡托普利 - 半胱氨酸二硫化物的形成是胱氨酸排泄量减少的部分原因，但可能还有其他机制起作用。由于卡托普利 - 半胱氨酸二硫化物的溶解度比胱氨酸高200倍，长期使用卡托普利治疗可能对胱氨酸尿症有效。
