摘要
目的观察激素耐药的儿童肾病综合征（SRNS）应用环孢素A（CsA）长疗程治疗的疗效并分析影响疗效的相关因素。方法回顾性分析疗程2年的CsA治疗SRNS病例20例，男：女为3：1；平均年龄5．5岁，肾病病理类型微小病变（MCNS）15例，局灶节段肾小球硬化（FSGS）4例，系膜增生性肾炎（MsPGN）1例。结果（1）完全缓解13例（65％），部分缓解4例（20％），CsA无效3例（15％），总有效率85％。平均随访时间40．5个月，复发率45％。（2）CsA治疗微小病变肾病的有效率为93％，非微小病变为60％，但两者差异无统计学意义。（3）毒副作用：多毛、齿龈增生和高血压的发生率分别为75％、25％和10％，2例出现可逆性的肾损伤，4例出现尿NAG／Cr增高，神经系统症状2例。3例重复肾活检未发现CsA相关的肾小管间质纤维化病变。结论对儿童激素耐药型肾病综合征CsA长疗程治疗有良好的疗效。小剂量CsA长期治疗未发现药物相关的肾小管间质纤维化和肾功能损伤。CsA长期治疗的安全性和预后有待进一步研究。
Objective To observe the effects of long-term cyclosporine A （CsA） treatment in 20 children with steroid-resistant idiopathic nephrotic syndrome （SRNS） and analyse the relevant influencing factors of CsA therapy. Methods Twenty children with SRNS received CsA therapy for 2 years between February 2001 and October 2006 in the Department of Nephrology. The mean age of children at initiation of CsA therapy was 5.5 years （30 months to 12 years）. The initial renal histology showed minimal change （MCNS） in 15 patients, focal segmental glomerulosclerosis （FSGS） in 4 patients and mesangial proliferative glomerulonephritis （MsPGN） in one. The starting dose of CsA was 3-5 mg/（kg · d）, adjusted to maintain a trough level of 100-200μg/L during the first 6 months. After one year, a low dose of CsA 1-3 mg/（kg · d） with a trough level of 40-70 μg/L was applied to maintain remission for 1 year. Liver function, serum albumin, serum cholesterol, serum creatinine, urinary NAG/Cr, 24 h urinary protein excretion and CsA whole blood trough level of the patients were monitored every one or three months. Results （1） Complete remission （proteinuria ≤ 0. 1 g/d or negative by dipstick for 3 consecutive days ）, partial remission （proteinuria between 0. 1 g/d and 50 mg/（ kg · d）, serum albumin 〉t 30 g/L） and resistance to CsA （proteinuria 〉t50 mg/（kg · d） , or t〉 3 ＋ by dipstick, after 6 months of CsA treatment over a trough level of 100μg/L） were observed in 65% , 20% and 15% , respectively. Eleven patients who had complete remission discontinued CsA, in 5 （45%） patients the disease relapsed, and resumption of CsA therapy was followed by remission in three of them. （2） MCNS showed a 93% response to CsA therapy while non-MCNS showed a 60% response, but the difference was not significant （ P 〉 0. 05 ）. （ 3 ） Hypertrichosis, gingival hyperplasia and hypertension occurred in 75%, 25% and 10% of the patients, respectively. Two patients were found to have renal impairment （ 〉 30% rise of serum creatinine） and recovered in 2 weeks. Four patients showed a rise of urinary NAG/Cr. The central nervous system adverse event occurred in 2 cases. Post-therapy biopsies performed in 3 patients （2 with FSGS and one with MCNS） did not show any relevant tubulointerstitial fibrosis. Two patients with FSGS of the twenty cases progressed into end-stage renal failure. Conclusious CsA treatment was confirmed to be effective in children with SRNS. Renal fibrosis was rare in patients treated with a low dose of CsA for 2 years.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2008年第9期698-701,共4页
Chinese Journal of Pediatrics
关键词
肾病综合征
激素耐药
环孢菌素
长疗程
儿童
Nephrotic syndrome
Steroid resistancu
Cyelosporine
Long-term
Child
