CAJ | 학술논문

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。
목적분석인동지발격소내약형신병종합정적림상특점화전귀。방법회고성분석본원2008년1월지2013년12월수치적지발격소내약형신병종합정환인적림상병리특점급치료전귀。결과동기수치적격소초치민감형신병종합정환인513례，기중지발격소내약형신병종합정21례，점4.1%。21례중남11례,녀10례，단순형신병16례,신염형신병5례。재출현지발성격소내약전，14례표현위빈복발혹격소의뢰，7례위비빈복발혹무복발，종격소민감지발생격소지발내약적시간위（206.10±212.64）천。여격소민감조비교，초차격소치료뇨단백음전시간경장,위（14.05±7.71）천비（10.26±5.66）천（P =0.003），녀성경다견（11/10비380/112，P =0.018），여신병복발조비교，수차복발거신병초차완해시간경단,위（34.95±39.44）천비（122.10±168.07）천（P=0.019）。12례접수신활검，병리현시미소병변（MCD）7례，국조절단성신소구경화(FSGS)4례，C1q신병1례。85.7%（18/21례）응용배포소화배린선알체도완전완해，1례FSGS격소연합마체맥고분지치료체부분완해，부1례C1q신병다약치료무효，3년후인종말기신장병(ESRD)사망。결론본조인동지발내약성신병종합정병비한견，초차격소치료뇨단백음전시간、수차복발거신병초차완해시간、성별혹가예측지발내약적발생，병리개변이MCD화FSGS위주，대격소연합면역억제제치료대다수반응량호，예후량호，단수주의소부분병례지속내약，예후불량。
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 mainly renal pathological changes are MCD and FSGS, majority cases are good response to combined steroid and immunosuppressive therapy, the prognosis is good, but also need pay attention to a small portion of the cases continued resistance and show poor prognosis.