摘要目的 探讨伴C1q沉积的IgA肾病患儿的临床、病理改变特征及其预后分析.方法 回顾性分析2000年1月至2017年12月于东部战区总医院肾活检确诊为原发性IgA肾病患儿的临床病理资料,根据肾小球免疫荧光检查是否有C1q沉积分为C1q沉积组和C1q阴性组.随访终点事件包括血肌酐翻倍、或估算肾小球滤过率(eGFR)下降超过50％、或进入终末期肾脏病期、或接受肾脏替代治疗、或死亡.采用Kaplan-Meier曲线比较两组患儿肾脏生存率的差异;单因素及多因素Cox回归模型法分析C1q沉积对IgA肾病患儿预后的影响.结果120例IgA肾病患儿入选本研究,其中C1q沉积组60例,C1q阴性组60例.C1q沉积组基线eGFR、血浆白蛋白水平低于C1q阴性组,血肌酐、血总胆固醇、24 h尿蛋白量水平高于C1q阴性组(均P<0.05).C1q沉积组系膜细胞增殖(M)、肾小管萎缩/间质纤维化(T)、细胞/纤维细胞性新月体(C)评分高于C1q阴性组(均P<0.05).中位随访时间78.9(66.3,184.1)个月,Kaplan-Meier生存曲线分析结果显示,两组患儿肾脏累积生存率的差异有统计学意义(Log-rank检验 χ2=6.801,P<0.01).Cox回归分析结果显示,C1q沉积组患儿出现肾脏终点事件的风险较C1q阴性组患儿增加了5.772倍(HR=5.772,95％CI:1.353～24.6211,P=0.018).结论伴C1q沉积的IgA肾病患儿临床、肾脏病理改变程度较C1q阴性患儿严重,肾脏预后较差.C1q沉积是儿童IgA肾病肾脏预后不良的独立危险因素.

abstractsObjective To investigate the clinical and pathological features and prognosis of children with IgA nephropathy with C1q deposition. Methods The children with IgA nephropathy diagnosed by renal biopsy from January 1, 2000 to December 30, 2017 were retrospectively analyzed and divided into C1q deposit group and C1q negative group according to glomerular immunofluorescence examination. Follow-up until the patient's serum creatinine doubled, glomerular filtration rate decreased by more than 50％, entering end-stage kidney disease, renal replacement therapy or death. Kaplan-Meier survival analysis was used to evaluate the renal survival rate in two groups. Univariate and multivariate Cox proportional hazard regression models were used to analyze the effect of C1q deposition on the prognosis of patients with IgA nephropathy. Results There were 60 cases in C1q deposition group and 60 cases in C1q negative group. (1) the initial eGFR and plasma albumin in C1q deposition group were lower than those in C1q negative group, while the levels of serum creatinine, serum cholesterol and 24 hour urinary protein in C1q group were higher than those in C1q negative group (all P<0.05). (2) pathological indexes:Mesangial cell proliferation, tubular atrophy/interstitial fibrosis, and cell/fibrocytic crescein score in C1q negative group were significantly higher than those in C1q negative group (all P<0.0.5). (3) Kaplan-Meier analysis showed that there was significant difference in renal cumulative survival rate between the two groups (Log-rank test:χ2=6.801, P=0.009). Cox proportional hazard regression model showed that the risk of renal end-point events in IgAN children with C1q deposition group was 5.772 times higher than that in C1q negative group (HR=5.772, 95％CI: 1.353-24.6211, P=0.018). Conclusion C1q deposition is an independent risk factor for the progress of renal function in IgA nephropathy children.