313例6岁以下小儿肾脏疾病病理特点及其与临床表现的关系

目的:了解6岁以下小儿肾脏疾病病理特点及其与临床表现的关系.方法:对313例临床诊断为14种肾脏疾病的6岁以下小儿进行肾组织病理检查.采用快速经皮肾活检术,将穿刺取得的组织分成3部分,按常规方法所有病例分别进行光镜、电镜及免疫荧光检查,标本均作HE,PAS,PASM及Masson染色,均应用免疫荧光检测肾组织中IgG,IgM,IgA,C3,C4,C1q及Firibn,部分病例根据血化验乙肝抗原阳性者加做免疫荧光检测肾组织中的HBsAg,HBeAg和HBcAg.本组290例(92.65%)进行了电镜检查.结果:313例肾活检成功率为100%;临床主要表现为持续性血尿103例(32.92%),单纯性肾病82例(26.21%),急性肾炎综合征63例(20.14%),紫癜性肾炎26例(8.32%),乙肝相关性肾炎15例(4.79%),孤立性蛋白尿8例(2.56%)等;病理改变主要为系膜增生性肾A炎162例(51.75%),IgM肾病26例(8.31%),微小病变扣轻微病变25例(7.99%),IgA肾病23例(7.35%),毛细血管内增生性肾小球肾炎16(5.11%),局灶节段性肾小球硬化14例(4.47%),薄基底膜病14例(4.47%),膜性肾病14例(4.47%)等.通过电镜检查,使Alport综合征、先天性肾病、薄基底膜病得以明确诊断.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.结论:临床表现类似的疾病病理类型不同,同一病理类型的疾病,临床表现可以多样.肾活检病理诊断对6岁以下小儿肾脏疾病的诊断、治疗、估计预后均有重要价值.电镜在肾病理检查中起到不可忽视的作用.

Pathological features and clinical manifestations in 313 children with nephropathy under 6

OBJECTIVE: To explore the relationship between pathological features and clinical manifestations in children with nephropathy under 6 years old. METHODS: Renal biopsy by rapid percutaneous puncturation was performed on 313 children under 6 who were all diagnosed clinically as kidney diseases of 14 different kinds. The specimens were divided into 3 parts for microscope, electron microscope and immuno fluorescence examination respectively and processed by HE, PAS, PASM, and Masson staining. Immunofluorescence was used to detect the deposition of IgG, IgM, IgA, C3, C4, C1q, and Fb in the renal tissues. Additional examinations were done to detect HBs-Ag, HBeAg and HBcAg deposition in some cases with positive serum HBs-Ag. Altogether 290 of the specimens (290/313, 92.65%) were examined by electron microscope. RESULTS: All the renal biopsy performances were successful. The clinical manifestations comprised of persistent haematuria (32.92%, 103/313), idiopathic nephritic syndrome (26.1%, 82/313), acute nephritic syndrome (20.14%, 63/313), Henoch Schonlein purpura nephritis (8.32%, 26/313), HBV-nephritis (4.79%, 15/313), and isolated proteinuria (2.56%, 8/313). The main pathological patterns of glomerular disease were identified as mesangial proliferation (51.75%, 162/313), IgM nephropathy (8.31%,26/313), minor and minimal change (7.99%, 25/313), IgA nephropathy (7.35%, 23/313), endocapillary proliferative glomerulonephritis (5.11%, 16/313), focus segmental glomerulosclerosis (4.47%, 14/313), thin basement membrane nephropathy (4.47%, 14/313), and membrane nephropathy (4.47%, 14/313). Alport syndrome, congenital nephrotic syndrome, and thin basement membrane nephropathy can be diagnosed by electron microscope, white IgA nephropathy, IgM nephropathy and C1q nephropathy by immunopathology. CONCLUSION: Similar clinical manifestations may differ in the pathology and the clinical features of one pathological diagnosis may vary greatly. Renal biopsy is of great help to the diagnosis, treatment and the prognosis evaluation for children with nephropathy under 6. Electron microscopes also play an important role in the diagnosis of nephropathy.