儿童肾脏疾病临床与病理753例分析

Clinical and pathological analysis of 753 children with renal diseases

目的分析和总结儿童肾脏疾病的临床资料和病理类型,探讨肾穿刺活检术在儿童肾脏疾病中的重要性。方法回顾性分析1995—2015年在山东大学附属省立医院小儿肾脏风湿免疫科接受经皮肾穿刺活检的753例肾脏疾病患儿临床资料和病理资料。结果 753例经皮肾穿刺活检患儿中,原发性肾小球疾病428例(56.84%)、继发性肾小球疾病306例(40.64%)、先天性遗传代谢性肾脏疾病17例(2.26%)、肾小管间质性疾病2例(0.27%)。临床诊断最常见的为原发性肾病综合征。原发性肾小球疾病中最常见的临床表现和病理类型分别为原发性肾病综合征和IgA肾病,继发性肾小球疾病中常见的临床表现和病理类型分别为紫癜性肾炎和系膜增生性肾小球肾炎。先天性遗传代谢性肾脏疾病中最常见的为薄基底膜肾病和Alport综合征。8例重复肾活检患儿的病理类型及临床表现均出现改变。结论儿童肾脏疾病以原发性肾小球疾病为主,最常见的临床诊断和病理类型分别为原发性肾病综合征和系膜增生性肾小球肾炎。重复肾穿刺活检有助于及时掌握患儿病理类型转化,调整治疗方案。

Objective To explore and conclude the pathological categories and clinical data of childhood renal diseases and understand the importance of renal biopsy in childhood renal diseases. Methods Totally 753 patients who underwent percutaneous renal biopsy from 1995 to 2015 were selected as study subjects, and their clinical and pathological information was analyzed retrospectively. Results Among 753 patients who underwent percutaneous renal biopsy, 428 cases （56.84%） had primary glomerular disease, 306 cases （40.64%） had secondary glomerular disease, 17 cases （2.26%） had heritage glomerular disease, and 2 cases（0.27% ） had renal tubular interstitial disease. The most common clinical diagnosis were primary nephritic syndrome. The most common clinical diagnosis and pathological category in primary glomerulary disease were primary nephritic syndrome and IgA nephropathy respectively, and in secondary glomerulary disease they were purpura nephritis and mesangial proliferative glomerulonephritis respectively. Thin basement membrane disease and Alport＇ s syndrome are the most common pathological category of the heritage glomerular disease. The 8 repeated renal biopsies showed pathological and clinical progression. Conclusion The primary glomerulary dis- ease is the main type of childhood glomerulary diseases. The most common clinical diagnosis and pathological category are primary nephrotic syndrome and mesangial proliferative glomerulonephritis respectively. The repeated renal biopsy is beneficial to control the transformation of pathological types and adjust new treatments timely.