儿童胱氨酸尿症

Cystinuria in children.

作者信息

Pavanello L, Rizzoni G, Dussini N, Zacchello G, Passerini G, Tasca A, Pagano F

出版信息

Eur Urol. 1981;7(3):139-43. doi: 10.1159/000473204.

PMID:7202450

Abstract

7 children, 20 months to 11 years old, with cystinuria and renal calculi were studied. Surgical treatment and alpha-mercaptopropionylglycine (MPG) gave satisfactory results in 5 children. The causes of the recurrences in the other 2 children are discussed. MPG therapy is effective but can cause a nephrotic syndrome at a dose of more than 50 mg/kg/day. A cystinuria of less than 200 mg/day cannot always be considered safe in children. The alkalinization and dilution of urine remain extremely important in the treatment of cystinuria.

摘要

对7名年龄在20个月至11岁之间、患有胱氨酸尿症和肾结石的儿童进行了研究。手术治疗和α-巯基丙酰甘氨酸（MPG）治疗使5名儿童取得了满意的效果。讨论了另外2名儿童复发的原因。MPG治疗有效，但剂量超过50mg/kg/天时可导致肾病综合征。儿童每天胱氨酸尿症少于200mg并不总是被认为是安全的。尿液碱化和稀释在胱氨酸尿症的治疗中仍然极其重要。