儿童原发性IgA肾病131例临床病理特征及预后影响因素分析OA
Clinical and Pathological Characteristics and Analysis of Prognostic Factors in 131 Children with Primary IgA Ne-phropathy
目的 探讨儿童原发性IgA肾病(IgAN)临床病理特征及预后影响因素,分析尿液四蛋白(α1-微球蛋白、微量白蛋白、转铁蛋白、β2-微球蛋白)的动态变化.方法 回顾性纳入我院2017年至2024年住院肾活检确诊的儿童原发性IgAN患儿共131例,收集患儿基线期一般资料、实验室指标、病理结果及随访期间尿四蛋白结果.通过卡方检验比较不同病理分层间的一般资料和实验室指标的差异,采用Kaplan-Meier法进行单因素生存分析,组间比较采用Log-rank检验,将单因素分析中P<0.10的变量及临床重要变量纳入Cox比例风险回归模型,采用向前逐步筛选法确定独立危险因素.结果 卡方检验显示,活动性病理组与非活动性病理组,以及Lee氏分级低级别组与高级别组在性别、上呼吸道感染史和发病季节等临床特征上差异无统计学意义(P>0.05).活动性与非活动性病理组在白细胞计数(WBC)、补体C3、纤维蛋白原(FIB)、纤维蛋白降解产物(FDP)、24 h尿蛋白、尿微量白蛋白(mAlb)和转铁蛋白(TRF)等指标上差异有统计学意义(P<0.05).根据Lee氏分级,低级别与高级别组在WBC、血清白蛋白(sAlb)、免疫球蛋白G(IgG)等指标上的差异有统计学意义(P<0.05).尿四蛋白动态分析显示,α1-微球蛋白(α1-MG)、β2-微球蛋白(β2-MG)在随访12个月时达峰值,随后逐渐下降,而微量白蛋白(mAlb)、转铁蛋白(TRF)在整个随访期间保持稳定.多因素Cox回归最终模型显示,年龄(HR=1.312,95%CI 1.117~1.543,P=0.001)、补体 C3(HR=0.064,95%CI 0.009~0.450,P=0.006)及 MEST-C 中的T评分(HR=28.711,95%CI 2.858~288.392,P=0.004)是影响儿童原发性IgAN预后的独立危险因素.结论 儿童原发性IgAN临床表现多样,α1-MG、β2-MG的动态变化可以反映疾病缓解趋势,而稳定指标mAlb、TRF可用于长期疾病监测.年龄较大、补体C3水平降低及肾小管萎缩/间质纤维化(T评分)是患儿预后不良的独立危险因素.临床实践中应重视病理T评分的评估,并结合年龄和补体C3进行危险分层,以指导个体化治疗.
Objective This study examines the clinical and pathological characteristics of primary IgA nephropathy(IgAN)in children and identifies factors influencing prognosis,while analyzing the dynamic changes in urinary tetraproteins(α1-microglobulin,microalbumin,transferrin,and β2-microglobulin).Methods A retrospective study included 131 pediatric pa-tients with primary IgAN diagnosed by renal biopsy during their hospitalization at our hospital between 2017 and 2024.Baseline de-mographic data,laboratory parameters,pathological findings,and urine protein levels during follow-up were collected.Chi-square tests were used to compare differences in general characteristics and laboratory parameters across different pathological subgroups.Univariate survival analysis was performed using the Kaplan-Meier method,with intergroup comparisons assessed using the Log-rank test.Variables with P<0.10 in the univariate analysis and clinically significant variables were included in the Cox pro-portional hazards regression model,and independent risk factors were identified using forward stepwise selection.Results Chi-square tests revealed no statistically significant differences in clinical characteristics,including gender,history of upper respiratory tract infection,and season of onset,between the active and inactive pathology groups,or between the low-grade and high-grade Lee classification groups(P>0.05).There were statistically significant differences between the active and inactive pathology groups in terms of white blood cell count(WBC),complement C3,fibrinogen(FIB),fibrin degradation products(FDP),24-hour urine pro-tein,urinary microalbumin(mAlb),and transferrin(TRF)(P<0.05).According to the Lee classification,there were statistically significant differences between the low-grade and high-grade groups in indicators such as WBC,serum albumin(sAlb),and immu-noglobulin G(IgG)(P<0.05).Dynamic analysis of urinary tetraproteins showed that α1-microglobulin(α1-MG)and β2-micro-globulin(β2-MG)peaked at 12 months of follow-up and then gradually declined,while microalbumin(mAlb)and transferrin(TRF)remained stable throughout the follow-up period.The final multivariate Cox regression model showed that age(HR=1.312,95%CI 1.117~1.543,P=0.001),complement C3(HR=0.064,95%CI 0.009~0.450,P=0.006),and the T-score on the MEST-C(HR=28.711,95%CI 2.858~288.392,P=0.004)were independent risk factors influencing the prognosis of pri-mary IgAN in children.Conclusion The clinical manifestations of primary IgAN in children are diverse,changes in α1-MG andβ2-MG levels over time can reflect the trend toward disease remission,while stable markers such as mAlb and TRF can be used for long-term disease monitoring.Advanced age,reduced complement C3 levels,and renal tubular atrophy/interstitial fibrosis(T-score)are independent risk factors for poor prognosis in pediatric patients.In clinical practice,emphasis should be placed on the assessment of the pathological T-score,combined with age and complement C3 levels for risk stratification,to guide individualized treatment.
李金凤;雷双霖;冯仕品
电子科技大学医学院附属妇女儿童医院·成都市妇女儿童中心医院,四川成都 611731电子科技大学医学院附属妇女儿童医院·成都市妇女儿童中心医院,四川成都 611731电子科技大学医学院附属妇女儿童医院·成都市妇女儿童中心医院,四川成都 611731
医药卫生
儿童IgA肾病病理预后
childrenIgA nephropathypathologyprognosis
《四川医学》 2026 (7)
732-738,7
2024年成都市医学科研课题(编号:2024500)
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