Guez S, Giani M, Melzi M L, Antignac C, Assael B M
Department of Pediatrics, University of Milan, Italy.
Pediatr Nephrol. 1998 Feb;12(2):130-2. doi: 10.1007/s004670050420.
The combination of captopril and indomethacin has been shown to control nephrotic proteinuria in an infant with congenital nephrotic syndrome of the Finnish type. We report the satisfactory control of congenital nephrotic syndrome by enalapril, maintaining normal serum albumin levels without albumin infusions. The haplotype data of our patient were consistent with the diagnosis of a Finnish-type nephrotic syndrome. After 21 months, during which daily infusions of albumin allowed partial control of the symptoms, captopril treatment was started. No adverse effects were noted. Serum creatinine levels remained normal. Within 8 weeks, albumin infusions were completely stopped. After 1 month the treatment was changed to a single dose of enalapril (0.8 mg/kg per day). During the next 15 months, the serum protein concentration was maintained around 6.5-7 g/dl, although proteinuria persisted (0.3-0.5 g/day). Weight and length gain are now satisfactory. We conclude that enalapril may be safely used in infants with severe forms of congenital nephrotic syndrome and might allow the avoidance of aggressive treatments for prolonged periods.
卡托普利和吲哚美辛联合使用已被证明可控制一名患有芬兰型先天性肾病综合征婴儿的肾病性蛋白尿。我们报告了依那普利对先天性肾病综合征的良好控制效果，在不输注白蛋白的情况下维持了正常的血清白蛋白水平。我们患者的单倍型数据与芬兰型肾病综合征的诊断一致。在21个月期间，每日输注白蛋白可部分控制症状，之后开始卡托普利治疗。未观察到不良反应。血清肌酐水平保持正常。8周内，白蛋白输注完全停止。1个月后，治疗改为单剂量依那普利（每天0.8mg/kg）。在接下来的15个月里，血清蛋白浓度维持在6.5 - 7g/dl左右，尽管蛋白尿持续存在（每天0.3 - 0.5g）。体重和身高增长目前令人满意。我们得出结论，依那普利可安全用于患有严重先天性肾病综合征的婴儿，并可能避免长期进行积极治疗。
