初诊为原发性肾炎型肾病综合征患儿的病因构成分析——对国内儿童原发性肾病综合征临床分型的商榷

目的总结初诊为原发性肾炎型肾病综合征患儿的病因构成情况,探讨国内儿童原发性肾病综合征临床分型的价值。方法纳入2013年1月1日至2017年12月31日北京大学第一医院儿科收治的病初临床诊断为原发性肾炎型肾病综合征患儿,且经肾脏病理检查或基因突变分析明确最终诊断者,排除继发性、遗传性肾病综合征及伴有明显肉眼血尿者。原发性肾病综合征的临床分型参照中华医学会儿科学分会肾脏病学组标准。截取患儿入院时一般情况、入院时诊断和临床分型、肾脏穿刺活检病理检查结果、基因检测结果和最终诊断。结果 28例临床分型为原发性肾炎型肾病综合征的患儿进入本文分析,占同期收治的原发性肾病综合征的10.6%(28/265)。分型依据:25例(89.3%)为镜下血尿,2例为高血压(7.2%),1例为肾功能异常(3.6%)。28例均进行了肾穿刺活检,11例行基因检测,最终诊断:原发性肾炎型肾病综合征5例(17.9%),Ig A肾病10例(35.7%),Alport综合征8例(28.6%),遗传性肾病综合征3例(10.7%),急性感染后肾小球肾炎和纤维素性肾小球肾炎各1例(3.6%)。结论儿童原发性肾炎型肾病综合征临床分型诊断的主要依据为镜下血尿,通过肾脏病理检查和基因检测明确病因,以Ig A肾病、遗传性肾脏疾病等居多,因此,临床诊断儿童原发性肾炎型肾病综合征应该慎重。

Etiological analysis of the nephritic type of primary nephrotic syndrome in children

Objective:To analyze the etiology of primary nephrotic syndrome(nephritic type) in children and to explore the value of Chinese clinical classification method for primary nephrotic syndrome in children. Methods:The children with first onset nephrotic syndrome(nephritic type), hospitalized in Peking University First Hospital from Jan 1, 2013 to Dec 31, 2016 were studied retrospectively, to summarize their clinical classification and final diagnosis. All cases were diagnosed clearly by renal biopsy or gene mutation analysis. Secondary and hereditary nephrotic syndrome were excluded, and those who presented with gross hematuria were also excluded. The classification of primary nephrotic syndrome (nephritic type) was according to the criterion of Kidney Pathology Group of Chinese Medical Science Branch. The clinical data were included such as general information, admitting diagnosis, clinical classification, renal pathology, gene mutation analysis and final diagnosis. Results:There were 265 cases of first onset nephrotic syndrome, including 237 cases (89.4%) of simple type and 28 cases (10.6%) of nephritic type. The classification basis of nephritic type were 25 cases (89.3%) of microscopic hematuria, 2 cases (7.2%) of hypertension，1 case (3.6%) of abnormal renal function. The final diagnosis was 5 cases (7.9%) of nephrotic syndrome (nephritic type), 10 cases (35.7%) of IgA nephropathy, 8 cases (28.6%) of Alport syndrome, 3 cases (10.7%) of hereditary nephrotic syndrome, 1 case (3.6%) of acute post-infectious glomerulonephritis and 1 case of fibrillary glomerulonephritis. Conclusion:Microscopic hematuria is the main classification basis of the nephritic type of primary nephrotic syndrome in children. The main causes of primary nephrotic syndrome (nephritic type) are IgA nephropathy and genetic renal diseases. The diagnosis of primary nephrotic syndrome (nephritic type) should be made cautiously.