摘要
目的分析伴IgA沉积的儿童微小病变肾病(MCD-IgA)的临床病理特征及预后。方法回顾性分析2010年1月至2015年12月在南京医科大学附属儿童医院收治的10例MCD-IgA患儿的临床及病理资料,并选取24例微小病变肾病综合征(MCD-NS)及21例临床表现为肾病综合征的IgA肾病(NS-IgAN)作为对照,分析3组患儿临床表现、病理特征及预后差异。结果1.临床表现:MCD-IgA组在起病年龄、性别、血尿发生率、24 h尿蛋白水平、血清清蛋白及胆固醇水平、血清IgA升高比例方面与MCD-NS组比较差异均无统计学意义。但NS-IgAN组起病年龄较MCD-IgA组及MCD-NS组大[(8.6±2.1)岁比(4.8±2.4)岁、(4.0±1.6)岁],血清清蛋白水平较MCD-IgA组及MCD-NS组高[(22.8±4.3) g/L比(19.0±1.9) g/L、(16.8±3.0) g/L],血清总胆固醇水平低于MCD-IgA组及MCD-NS组[(7.9±1.9) mmol/L比(9.9±2.7) mmol/L、(9.8±2.1) mmol/L],以上差异均有统计学意义(均P〈0.05),且均合并肉眼血尿。2.病理表现:MCD-IgA组及MCD-NS组光镜下病变均较轻微,但NS-IgAN组光镜病变较重(系膜增生基础上常合并内皮增生、节段或球性硬化、新月体、襻坏死及肾小管间质慢性病变);免疫荧光MCD-NS组基本阴性,MCD-IgA组IgA沉积强度较弱(≤++),仅3例(30.0%)合并C3沉积,而NS-IgAN组IgA沉积强度较强(≥+++),且多数合并C3及其他免疫球蛋白沉积;电镜下MCD-IgA组及MCD-NS组主要表现为足突广泛融合,MCD-IgA 9例系膜区见少量电子致密物沉积。而NS-IgAN组主要为系膜区大量电子致密物沉积,仅8例(38.0%)足突融合〉50%。3.预后:MCD-IgA组9例激素依赖或频复发,1例激素耐药,6例加用其他免疫抑制剂,除1例失访外,平均随访(61.5±28.8)个月,8例获得完全缓解;MCD-NS组20例激素依赖或频复发,4例激素耐药,23例加用其他免疫抑制剂,平均随访(36.4±12.5)个月,22例(91.7%)获得完全缓解;NS-IgAN组均表现为激素耐药,均加用环磷酰胺冲击治疗,平均随访(38.6±15.2)个月,19例(90.5%)完全缓解。结论MCD-IgA患儿临床表现及预后与MCD-NS相似,但其临床及病理表现与NS-IgAN存在明显不同。推测MCD-IgA本质还是属于微小病变,IgA沉积可能系非特异性。
Objective To analyze the clinicopathological features and prognosis of minimal change nephropathy with IgA deposition (MCD -IgA) in children. Methods The clinical and pathological data of 10 cases in Children's Hospital of Nanjing Medical University from January 2010 to December 2015 with MCD - IgA were retrospectively analyzed, and 24 cases of minimal change nephrotic syndrome (MCD -NS) and 21 cases of IgA nephropathy clini- cally manifested with nephrotic syndrome ( NS - IgAN) were selected as controls. Results ( 1 ) Clinical manifestations : there were no significant differences in age, gender, incidence of hematuria, level of 24 hours urine protein, serum albumin and cholesterol levels and elevated serum IgA ratio in MCD - IgA compared with MCD - NS group. Compared with MCD - IgA and MCD - NS, NS - IgAN group showed older age of onset [ ( 8.6 ± 2.1 ) years vs. (4.8 ± 2.4 ) years, (4.0 ± 1.6) years ], higher level of serum albumin [ (22.8 ± 4.3 ) g/L vs. ( 19.0 ± 1.9 ) g/L, ( 16.8 ± 3.0 ) g/L ], and lower level of serum total cholesterol [ (7.9 ± 1.9 ) mmol/L vs. (9.9 ± 2.7 ) mmol/L, (9.8 ± 2.1 ) mmol/L ], and all the differences were significant( all P 〈 0.05 ). NS- IgAN group was all associated with gross hematuria. (2) Pathology : the light microscope lesions in MCD - IgA and MCD - NS group were mild, but it was usually associated with severe histologic lesions in NS - IgAN, such as endocapillary proliferation, segmental sclerosis, crescent formation, tuft necrosis and chronic tubulointerstitial lesions ; in MCD - NS group, immunofluorescence was negative. In MCD - IgA group, IgA deposition intensity was weak ( less than ++ ), and 3 cases ( 30.0% ) were accompanied with C3 deposition. In NS - IgAN group, IgA deposition intensity was stronger ( more than +++ ) ,and most of the eases were accompartied with C3 and other immunoglobulins deposition. Under electron microscope, both MCD - IgA and MCD - NS showed wide foot process effacement,and a small amount of mesangial electron dense deposit was detected in 9 cases of MCD- IgA. In NS - IgAN group, large amount of electron dense deposit was found in the mesangial region, and only 8 cases ( 38.0% ) showed more than 50% of foot process effacement. ( 3 ) Prognosis: in MCD - IgA group, 9 patients were steroid -dependent or frequently relapsed, 1 case showed steroid -resistance,6 patients required additional agents. Except 1 case lost ,with an average of (61.5 ±28. g ) months were followed up, g patients achieved complete remission; In MCD- NS group,20 cases were steroid- dependent or frequently relapsed,4 cases were steroid -resistant,23 cases required additional immunosuppressive agents. Followed up for an average of (36.4 ± 12.5 ) months, 22 cases (91.7 % ) achieved complete remission ;In NS- IgAN group, all cases were steroid -resistant and combined with cyclophospha- mide treatment;followed up for an average of (38.6 ±15.2) months, 19 cases (90.5%) achieved complete remission. Conclusions The clinical manifestations and prognosis of MCD - IgA were similar to MCD - NS, but the clinical and pathological findings of MCD - IgA were different from those of NS - IgAN. It is deduced that the nature of MCD - IgA is still a MCD, and that the IgA deposition may be nonspecific.
作者
赵三龙
赵非
朱春华
丁桂霞
黄松明
Zhao Sanlong, Zhao Fei, Zhu Chunhua, Ding Guixia, Huang Songming(Department of Nephrology , Children's Hospital of Nanjing Medical University ,Nanjing 210008, Chin)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2018年第5期353-357,共5页
Chinese Journal of Applied Clinical Pediatrics
关键词
伴IgA沉积的微小病变肾病
微小病变
IGA肾病
儿童
病理
Minimal change nephropathy with IgA deposition
Minimal change disease
IgA nephropathy
Child
Pathology