单中心儿童激素耐药型肾病综合征临床特征与病理分析

Clinical features and pathological analysis of steroid resistant nephrotic syndrome in children in a single center

目的分析西北地区儿童激素耐药型肾病综合征(steroid resistant nephrotic syndrome,SRNS)的临床特征、病理类型、治疗及预后情况,为SRNS的治疗提供参考。方法采用回顾性调查方法,分析2018年1月1日至2020年12月31日陕西省西安市儿童医院肾脏科住院诊断为SRNS的102例患儿的临床资料、肾脏病理结果、治疗方案及疗效等。根据年龄、临床分型、病理类型、治疗方案及治疗转归对所有患儿进行分组,探讨影响SRNS患儿预后的危险因素。符合正态分布的计量资料以xˉ±s表示,组间比较采用t检验;不符合正态分布的计量资料以M(Q_(1),Q_(3))表示,组间比较采用秩和检验。计数资料组间比较采用χ;检验。危险因素采用多因素Logistic回归分析。结果102例SRNS患儿的中位发病年龄3.0岁,局灶性节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)占36.3%(37/102),微小病变占33.3%(34/102)、系膜增生性肾小球肾炎占23.5%(24/102)。FSGS组患儿高血压[35.1%(13/37)]、24 h尿蛋白定量[130.5(91.5,159.6)mg/(kg·24 h)]、肾功能不全的患病率[21.6%(8/37)]均高于非FSGS组[13.8%(9/65)、65.8(51.2,85.5)mg/(kg·24 h)、4.6%(3/65)],两组比较差异均有统计学意义(统计量值分别为χ^(2)=6.32、Z=5.90、χ^(2)=7.09;P值分别为0.012、<0.001、0.008)。Logistic多因素回归分析发现高血压(OR=4.055,95%CI 1.178~3.962)和24 h尿蛋白定量升高(OR=1.036,95%CI1.020~1.053)与FSGS的风险增加相关(P值分别为0.026、<0.001)。ROC曲线分析显示24 h尿蛋白定量评估FSGS的最佳临界值为85.65 mg/(kg·24 h)。经治疗,102例SRNS患儿中完全缓解61.8%(63/102),部分缓解14.7%(15/102),未缓解23.5%(24/102)。截至随访结束,微小病变组中治疗有效率[94.1%(32/34)]高于FSGS组[51.3%(19/37)],两组比较差异有统计学意义(χ^(2)=16.02,P<0.001)。在初始的免疫抑制治疗中,激素联合钙调磷酸酶抑制剂组治疗完全缓解率[77.1%(37/48)]高于激素联合环磷酰胺组[11.1%(3/27)],两组比较差异有统计学意义(Z=32.28,P<0.001)。结论SRNS患儿中最常见的病理类型是FSGS,且发病年龄普遍偏小,病理类型为FSGS的患者预后最差,微小病变预后较好。高血压和24 h尿蛋白定量是FSGS的危险因素,儿童SRNS的二线免疫抑制剂首选钙调神经磷酸酶抑制剂类药物。

0bjective To analyze the clinical characteristics,pathological types,treatment and prognosis in children with steroid resistant nephrotic syndrome(SRNS)in Northwest China,in order to provide reference for the treatment of SRNS.Methods The clinical data,renal pathological results,treatment plan and efficacy of 102 children diagnosed with SRNS in the Department of Nephrology,Xi'an Children's Hospital of Shaanxi Province from January 1st,2018 to December thirty-first,2020 were analyzed retrospectively.All children were divided into groups according to age,clinical classification,pathological type,treatment scheme and treatment outcome,and the risk factors affecting the prognosis of children with SRNS were discussed.The measurement datas conforming to normal distribution were expressed as x¯±s,and t test was used for comparison between groups.Measurement datas that did not conform to normal distribution were represented by M(Q_(1),Q_(3)),and Kruskall-Wallis test was used for comparison between groups.Enumeration datas were compared byχ^(2) test.Risk factors were analyzed by multiple factor Logistic regression analysis.Results The median age of onset of 102 children with SRNS was 3.0 years.Focal segmental glomerulosclerosis(FSGS)accounted for 36.3%(37/102),minimal lesions accounted for 33.3%(34/102),and mesangial proliferative glomerulonephritis accounted for 23.5%(24/102).The prevalence rates of hypertension(35.1%(13/37)),24-h urine protein quantification(130.5(91.5,159.6)mg/(kg·24 h)and renal insufficiency(21.6%(8/37))in FSGS group were higher than those in non-FSGS group(13.8%(9/65),65.8(51.2,85.5)mg/(kg·24 h),4.6%(3/65)).The differences between the two groups were statistically significant(statistical values wereχ^(2)=6.32,Z=5.90,χ^(2)=7.09;P values were 0.012,<0.001,0.008).Logistic multivariate regression analysis showed that the hypertension(OR=4.055,95%CI 1.178-3.962)and 24 hour urinary protein(OR=1.036,95%CI 1.020-1.053)were associated with the increased risk of FSGS(P values were 0.026 and<0.001).ROC curve ananlysis showed that the optimal critical value of 24 hour urinary protein was 85.65 mg/(kg·24 h)in FSGS.After treatment,complete remission was 61.8%(63/102),partial remission was 14.7%(15/102),and no remission was 23.5%(24/102).By the end of follow-up the treatment effective rate in the small lesion group(94.1%(32/34))was higher than that in the FSGS Group(51.3%(19/37)),and the difference between the two groups was statistically significant(χ^(2)=16.02,P<0.001).In the initial immunosuppressive treatment,the complete remission rate of hormone combined with calcineurin inhibitor group(77.1%(37/48))was higher than that of hormone combined with cyclophosphamide Group(11.1%(3/27)).There was significant difference between the two groups(Z=32.28,P<0.001).Conclusion The most common pathological type in children with SRNS was FSGS,and the age of onset was generally small.The prognosis of patients with pathological type FSGS was the worst,and the prognosis of small lesions was better.Hypertension and 24-hour urinary protein quantification were the risk factors of FSGS.Calcineurin inhibitors were the first choice for the second-line immunosuppressants of SRNS in children.