1316例小儿肾脏病临床与病理分布特点

Clinicopathologic characteristics of 1316 children with renal disease

目的了解小儿肾小球疾病肾脏病理改变的特点及其与临床表现的关系。方法对近20年间该院的1316例患儿的肾活检资料,进行了回顾性分析。结果1316例患儿中临床主要表现为肾病综合征(383例,占29.09%)、急性肾炎综合征(291例,占22.00%)、孤立性血尿(224例,占17.21%)、紫癜性肾炎(209例,占15.87%)、乙型肝炎病毒相关肾炎(96例,占7.30%)等。病理改变主要为系膜增生(756例,占57.45%)、IgA肾病(113例,占8.59%)、毛细血管内增生(112例,占8.51%)、膜性肾病(66例,占5.02%)、微小和轻微病变(59例,占4.48%)等。通过超微结构检查,使Alport综合征、薄基底膜病、先天性肾病、纤维样肾小球病、Fabry病等,得以明确诊断。通过肾活检组织免疫病理学检查,使IgA肾病、IgM肾病及C1q肾病得以确诊。肾活检患儿中原发性肾小球疾病最为常见(915例,占69.53%)。原发性肾小球疾病病因以原发性肾病综合征为最常见(375/915例,占41.0%),而继发性肾小球疾病病因以紫癜性肾炎为最常见(209/344例,占60.8%)。结论在该次调查的肾活检资料中,原发性肾小球疾病最常见,肾病综合征是最常见的临床诊断,病理改变则以系膜增生最为多见。

Objective To investigate the clinicopathologic characteristics of childhood renal diseases. Methods A retrospective analysis of 1 316 renal biopsies performed over the past 20 years was performed. Results Of the 1 316 patients, 383 （ 29.09% ） were diagnosed as nephrotic syndrome, 291 （ 22.00% ） as acute nephritis syndrome, 224 （ 17.21% ） as isolated hematuria, 209 （ 15.87% ） as purpura nephritis, and 96 （7.30%） as hepatitis B virus-associated nephritis. Mesangial proliferation was the most common pathological change （756 cases; 57.45% ）, followed by IgA nephropathy （ 113 cases ; 8.59% ） , endothelial capillary proliferation （ 112 cases ; 8.51% ）, membranous nephropathy （ 66 cases; 5.02% ） , and various minor and minimal changes （ 59 cases ; 4.48% ）. Alport syndrome, congenital nephrotic syndrome, thin basement membrane nephropathy, fibrillary glomerulopathy disease, and Fabry disease were confirmed by eleetrenic microscopy. IgA, IgM and Clq nephropathy were definitely diagnosed using immune histochemistry or immunofluorescent. A diagnosis of primary glomerular disease was made in 69.53% of the cases （915 cases） ; secondary glomerular disease was noted in 26.14% （344 cases）. Of the 915 cases of primary glomerular disease, 375（41.0% ） had nephrotic syndrome. Secondary glomerular disease due to purura nephritis was common （209/344 ; 60.8% ）. Conclusions Primiary glomerular disease predominates in children. Nephrotic syndrome is the most common clinical diagnosis. Mesangial proliferation is the most common pathological patterns in children with renal disease.