The analysis of clinical features and outcomes of children with late steroid resistant primary nephrotic syndrome
YANG Qing, CHEN Min-guang ,YE Xiao-hua, LIN Hong-zhou
YANG Qing, CHEN Min-guang ,YE Xiao-hua, LIN Hong-zhou(Department of Nephrology, Yuying Children's Hospital of Wenzhou Medical University,Wenzhou 325027,China)
Abstract: Objective To analyze the clinical features and outcomes of children with late steroidresistant nephrotic syndrome. Method Retrospective 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)d vs (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 first relapse to first remission was more shorter in late steroid-resistant group(34.95 ± 39.44d vs 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 first steroid therapy, the interval from first relapse to first 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.
杨 青 陈敏广 叶晓华 林洪洲. 儿童迟发激素耐药型肾病综合征的临床和转归分析[J]. 发育医学电子杂志, 2015, 3(3): 160-163.
YANG Qing, CHEN Min-guang , YE Xiao-hua, LIN Hong-zhou. The analysis of clinical features and outcomes of children with late steroid resistant primary nephrotic syndrome. Journal of Developmental Medicine(Electronic Version), 2015, 3(3): 160-163.
2015, Vol. 3 
