Dang Xi-qiang, Cao Yan, Yi Zhu-wen, Xu Zi-chuan, He Xiao-jie, Huang Dan-lin
Department of Pediatrics, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2008 Mar;33(3):227-32.
To explore the relationship between pathological features and clinical manifestations in children with nephropathy under 6 years old.
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.
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.
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.
探讨6岁以下肾病患儿的病理特征与临床表现之间的关系。
对313例6岁以下临床诊断为14种不同类型肾脏疾病的患儿进行快速经皮肾穿刺活检。标本分为3份,分别用于光镜、电镜和免疫荧光检查,并进行HE、PAS、PASM和Masson染色。采用免疫荧光法检测肾组织中IgG、IgM、IgA、C3、C4、C1q和Fb的沉积。对部分血清HBs-Ag阳性病例,额外检测HBs-Ag、HBeAg和HBcAg的沉积情况。共290份标本(290/313,92.65%)进行了电镜检查。
所有肾穿刺活检操作均成功。临床表现包括持续性血尿(32.92%,103/313)、特发性肾病综合征(26.1%,82/313)、急性肾病综合征(20.14%,63/313)、过敏性紫癜性肾炎(8.32%,26/313)、乙肝相关性肾炎(4.79%,15/313)和孤立性蛋白尿(2.56%,8/313)。肾小球疾病的主要病理类型为系膜增生(51.75%,162/313)、IgM肾病(8.31%,26/313)、轻微病变和微小病变(7.99%,25/313)、IgA肾病(7.35%,23/313)、毛细血管内增生性肾小球肾炎(5.11%,16/313)、局灶节段性肾小球硬化(4.47%,14/313)、薄基底膜肾病(4.47%,14/313)和膜性肾病(4.47%,14/313)。Alport综合征、先天性肾病综合征和薄基底膜肾病可通过电镜诊断,IgA肾病、IgM肾病和C1q肾病可通过免疫病理学诊断。
相似的临床表现其病理可能不同,而同一病理诊断的临床特征也可能差异很大。肾活检对6岁以下肾病患儿的诊断、治疗及预后评估有很大帮助。电镜在肾病诊断中也发挥着重要作用。