Pathology and treatment of idiopathic membranous nephropathy in children
10.3760/cma.j.issn.2095-428X.2015.05.009
- VernacularTitle:儿童特发性膜性肾病病理学特点分析与治疗
- Author:
Qian FU
;
Ying SHEN
;
Hui WANG
;
Libing FU
- Publication Type:Journal Article
- Keywords:
Idiopathic membranous nephropathy;
Atypical membranous nephropathy;
Pathology;
Treatment;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2015;30(5):346-350
- CountryChina
- Language:Chinese
-
Abstract:
Objective To study features of clinical manifestation,pathology and treatment effects of idiopathic membranous nephropathy(IMN).Methods A retrospective study was performed in patients with membranous nephropathy through renal biopsy diagnosis in Beijing Children's Hospital Affiliated to Capital Medical University from November 2009 to February 2013.Patients were diagnosed according to renal biopsy apart from hepatitis B virus-associated glomerulonephritis,Henoch-Schonlein purpura nephritis,lupus nephritis,tumor and drug associated nephritis.The study summed the clinical and pathological features of IMN and evaluated the effect of treatment according the follow up.Results (1) There were 13 patients,with the ratio of male and female being 1.0 ∶ 1.6,among them 1 patient was younger than 6 years old,3 cases were between 6 and 12 years old,and 9 cases were elder than 12 years old.Nine patients were nephrotic syndrome,and 3 patients had renal insufficiency.(2)Ten patients had infection,among them 5 cases were upper respiratory infection with agent unfound,4 patients were mcoplasma infection,1 patient was influenza B virus infection,1 patient had positive result of Hepatitis B surface antibody(HBsAb) and Hepatitis B core antibody (HBcAb),and 2 patients had positive result of purified protein derivative (PPD).(3)Pathological feature of IMN was atypical membranous nephropathy.Atypical membranous nephropathy was characterized by an irregular,thickened glomerular basement membrane(GBM),mesangial cells,and a mesangial matrix with mild to moderate hyperplasia.Immune complexes were stained in the subepithelial region,GBM,and mesangial region under light microscopy.Electron microscopy showed various sizes,density,and shapes of electron-dense deposits in multiple regions.The cases of immunofluorescence were 13 for IgG,10 for C3,9 for IgM,9 for C1q,5 for IgA,2 for C4,and 0 for fibrinogen.Seven patients were strongly positive(3 + and above) for IgG and one patient was strongly positive for C3.(4)Seven patients had complete remission,three patients had partial remission,and three patients had non-remission.Conclusions (1) In this study,children with IMN were mainly girls,and the age distribution was beyond 12 years.Nephrotic syndrome was the most common clinical manifestation.(2)Atypical membranous nephropathy was the most common pathological feature in children with IMN,which should be distinguished from hepatitis B virus-associated glomerulonephritis by testing HBV antigen in renal biopsy.(3) Prednisone was effective for IMN children,but it was suggested to give enough and long course of application.Effect of prednisone combined with cyclophosphamide was still undefined.
