儿童迟发激素耐药型肾病综合征的临床和转归分析  

The analysis of clinical features and outcomes of children with late steroid resistant primary nephrotic syndrome

作　　者：杨青[1] 陈敏广[1] 叶晓华[1] 林洪洲[1] 

机构地区：[1]温州医科大学附属育英儿童医院肾内科,温州325027

出　　处：《发育医学电子杂志》2015年第3期160-163,共4页Journal of Developmental Medicine (Electronic Version)

摘　　要：目的分析儿童迟发激素耐药型肾病综合征的临床特点和转归。方法回顾性分析本院2008年1月至2013年12月收治的迟发激素耐药型肾病综合征患儿的临床病理特点及治疗转归。结果同期收治的激素初治敏感型肾病综合征患儿513例,其中迟发激素耐药型肾病综合征21例,占4.1%。21例中男11例,女10例,单纯型肾病16例,肾炎型肾病5例。在出现迟发性激素耐药前,14例表现为频复发或激素依赖,7例为非频复发或无复发,从激素敏感至发生激素迟发耐药的时间为(206.10±212.64)天。与激素敏感组比较,初次激素治疗尿蛋白阴转时间更长,为(14.05±7.71)天比(10.26±5.66)天(P=0.003),女性更多见(11/10比380/112,P=0.018),与肾病复发组比较,首次复发距肾病初次缓解时间更短,为(34.95±39.44)天比(122.10±168.07)天(P=0.019)。12例接受肾活检,病理显示微小病变(MCD)7例,局灶节段性肾小球硬化(FSGS)4例,C1q肾病1例。85.7%(18/21例)应用环孢素和环磷酰胺达到完全缓解,1例FSGS激素联合吗替麦考酚酯治疗达部分缓解,仅1例C1q肾病多药治疗无效,3年后因终末期肾脏病(ESRD)死亡。结论本组儿童迟发耐药性肾病综合征并非罕见,初次激素治疗尿蛋白阴转时间、首次复发距肾病初次缓解时间、性别或可预测迟发耐药的发生,病理改变以MCD和FSGS为主,对激素联合免疫抑制剂治疗大多数反应良好,预后良好,但需注意少部分病例持续耐药,预后不良。ObjectiveTo analyze the clinical features and outcomes of children with late steroid-resistant nephrotic syndrome.MethodRetrospective analyze the clinical and pathological characteristics and treatment outcomes of children with late steroid-resistant nephrotic syndrome in our hospital from January 2008 to December 2013.Results There were 513 cases with steroid-sensitive nephrotic syndrome in initial treatment, 21 of them with late steroid-resistant nephrotic syndrome, accounting for 4.1%. In all of 21 patients, there were 11 males and 10 females, 16 cases were simple type while 5 cases with nephritis type.Before late steroid resistance,14 cases showed frequency relapse or steroid-dependent, seven cases were non-frequency relapse or relapse-free. The interval from steroid-sensitive to late steroid-resistant was (206.10± 212.64) d. Compared with steroid-sensitive group, the proteinuria negative time more longer in initial steroid treatment[(14.05± 7.71)dvs (10.26± 5.66)d,P=0.003], more common in women (11/10 vs 380/112,P=0.018). Compared with the relapse group, the interval from ifrst relapse to ifrst remission <br> was more shorter in late steroid-resistant group(34.95± 39.44dvs 122.10± 168.07d,P= 0.019). There were 12 cases received renal biopsy and histology showed minimal change disease(MCD) in 7 cases, focal segmental glomerulosclerosis(FSGS) in 4 cases, C1q nephropathy in one case. About 85.7% (18/21 patients) achieved complete remission after treated by cyclosporine and cyclophosphamide, 1 case (4.8%) with FSGS reached partial remission after combined steroid and mycophenolate mofetil therapy, only one case with C1q nephropathy still treatment futility after multidrug therapy and death due to ESRD after three years. Conclusions Children with late steroid -resistant nephrotic syndrome are not uncommon. It's probably useful to predict the occurrence of late steroid-resistant by the proteinuria negative time after ifrst steroid therapy, the interval from ifrst relapse to ifrst remission and gender. The mai

关 键 词：肾病综合征 迟发激素耐药 儿童 临床特征 转归 

分 类 号：R726.9[医药卫生—儿科]