临床儿科杂志 ›› 2016, Vol. 34 ›› Issue (2): 115-.doi: 10.3969 j.issn.1000-3606.2016.02.008

• 综合报道 • 上一篇    下一篇

儿童IgM 肾病与IgA 肾病临床和病理的对比研究

陆春久1,李艳红1,李晓忠1,王兴东2   

  1. 苏州大学附属儿童医院1. 肾脏科,2. 病理科( 江苏苏州 215003)
  • 收稿日期:2016-02-15 出版日期:2016-02-15 发布日期:2016-02-15
  • 通讯作者: 王兴东 E-mail:etyyli2014@163.com

Clinicopathological comparative study of IgM nephropathy and IgA nephropathy in children 

 LU Cunjiu1, LI Yanhong1, LI Xiaozhong1, WANG Xingdong2   

  1. 1.Department of Nephrology, 2.Department of Pathology, Children's Hospital of Soochow University, Suzhou 215003, Jiangsu, China
  • Received:2016-02-15 Online:2016-02-15 Published:2016-02-15

摘要: 目的 比较IgM 肾病(IgMN) 与IgA 肾病(IgAN) 患儿在临床及病理方面的异同。方法 对经肾活检确诊的38 例IgMN 及40 例IgAN 患儿的临床表现、实验室检查及肾脏病理进行对比分析。结果 IgMN 患儿的平均发病年龄小于IgAN 患儿,平均肾活检前病程长于IgAN 患儿,肉眼血尿发生率、尿IgG 及尿白蛋白水平均低于IgAN 患儿,同时严重肾小球损伤发生率也低于IgAN 患儿,差异均有统计学意义(P<0.05)。IgMN 患儿中,有严重肾小球损伤患儿的血清白蛋白水平更低而尿白蛋白水平更高,与无严重肾小球损伤的同组患儿比较,差异有统计学意义(P<0.05);有严重肾小管损伤患儿以男性多见,肉眼血尿发生率、尿白蛋白和N- 乙酰-β-D 氨基葡萄苷酶(NAG)水平以及出现基底膜厚薄异常的比例高于无严重肾小管损伤的患儿,差异均有统计学意义(P<0.05),但发生严重肾小球损伤的差异无统计学意义(P>0.05)。在IgAN 患儿中,有严重肾小球损伤患儿的蛋白尿、肾小管见RBC 管型、C3 及Fibrinogen 显著沉积和足突融合的发生率均高于无严重肾小球损伤的同组患儿,差异有统计学意义(P<0.05);有严重肾小管损伤的患儿的肾功能受损程度、出现重度系膜细胞增生及肾小球纤维硬化情况比无严重肾小管损伤的同组患儿更严重,差异均有统计学意义(P<0.05)。结论 儿童IgMN 与IgAN 在临床和病理方面存在差异,IgMN 肾脏损伤程度较IgAN 轻。与IgAN 不同,IgMN 患儿的肾小管损伤与肾小球损伤无平行关系。

Abstract:  Objective To compare the clinical and pathological differences between IgM nephropathy (IgMN) and IgA nephropathy (IgAN) in children. Methods Clinical manifestations, laboratory examination results, and renal pathological data from 38 children with IgMN and 40 children with IgAN were compared. Results The mean age of onset in IgMN group was younger than that in IgAN group (P<0.05), the mean course before renal biopsy of IgMN group was longer than that of IgAN group (P<0.05), but the incidence of gross hematuria, the level of urinary IgG and albumin, and the incidence of severe glomerular injury were all higher in IgAN group than those in IgMN group (P<0.05). In IgMN group, the level of serum albumin was lower and the urine albumin was higher in the cases with severe glomerular injury than those in the cases without severe glomerular injury (P<0.05); there were more males in those cases with sever glomerular injuries; the incidences of gross hematuria, the level of urine albumin and NAG, and the abnormal basement membrane thickness was higher in the cases with severe tubular injury than those in the cases without severe tubular injury (P<0.05). However, the incidence of severe glomerular injury had no significant difference between the cases with severe and without severe renal tubular injury (P>0.05). In IgAN group, the incidence of proteinuria, RBC casts in tubular, C3 and fibrinogen deposition, and foot process effacement were higher in the cases with severe glomerular injury than those in the cases without severe glomerular injury (P<0.05); the degree of impairment of renal function, the incidence of severe mesangial cell proliferation, and glomerular sclerosis were more serious in the cases with severe tubular injury than those in the cases without severe tubular injury (P<0.05). Conclusions The clinical and pathological features are different between IgMN and IgAN in children. The renal damage is less in IgMN than that in IgAN children. Different from IgAN children, there is no parallel relationship between tubular and glomerular injury in IgMN children.