Clinicopathological features and prognosis of minimal change nephropathy with IgA deposition in children
10.3760/cma.j.issn.2095-428X.2018.05.008
- VernacularTitle:伴IgA沉积的儿童微小病变肾病的临床病理特征及预后
- Author:
Sanlong ZHAO
1
;
Fei ZHAO
;
Chunhua ZHU
;
Guixia DING
;
Songming HUANG
Author Information
1. 210008,南京医科大学附属儿童医院肾内科
- Keywords:
Minimal change nephropathy with IgA deposition;
Minimal change disease;
IgA nephropathy;
Child;
Pathology
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(5):353-357
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinicopathological features and prognosis of minimal change nephropa-thy with IgA deposition(MCD-IgA)in children.Methods The clinical and pathological data of 10 cases in Chil-dren's Hospital of Nanjing Medical University from January 2010 to December 2015 with MCD-IgA were retrospective-ly 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 manifesta-tions: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 C3deposi-tion.In NS-IgAN group,IgA deposition intensity was stronger(more than + + +),and most of the cases were accom-panied with C3and 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 ster-oid-dependent or frequently relapsed,1 case showed steroid-resistance,6 patients required additional agents.Except 1 case lost,with an average of(61.5 ± 28.8)months were followed up,8 patients achieved complete remission;In MCD-NS group,20 cases were steroid-dependent or frequently relapsed,4 cases were steroid-resistant,23 cases re-quired 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.
