Clinicopathological Characteristic And Steroid Therapeutic Responses Of IgA Nephropathy With Nephrotic Syndrome In Children

Objective: Immunglobulin A nephropathy(IgAN)is defined as dominant or codominant staining with IgA in glomeruli by immunohistology.The distribution of IgA staining includes presence in the mesangium,with or without capillary loop staining.IgAN is one of the most common primary glomerulonephritis in the world,mainly in children and abolescent.IgAN episodes of gross hematuria in the most common,followed by nephrotic syndrome(NS).The common pathological abnormality of IgAN presented with NS is mesangial proliferative glomerulonephritis,and glomerular sclerosis,crescent formation,interstitial fibrosis and tubular atrophy and other pathological changes can occur simultaneously.Steroid combined immunosuppressive therapy is effective on the reduction of proteinuria of IgAN presented with NS.The aim of this study was to analysis clinical,pathological features and response to steroid treatment of IgAN with NS in children.Methods: Nineteen children diagnosed with IgAN were recruited into our study at Department of Pediatrics,Fuzhou General Hospital of Nanjing Command from January 1992 to May 2013.They were analyzed retrospectively.Clinical manifestations,kidney pathology and urine protein negative rate of steroid therapy for4 weeks,8 weeks in the 19 patients were investigated.Results: 1.According to clinical characteristic,4 cases presented with simple nephropathy,11 cases with nephritic nephropathy.2.According to Lee pathohistological classification,the pathological changes of 4 patients with simple nephropathy were gradeⅠ(1 case)and gradeⅡ(3 cases),respectively,and those of 15 patients with nephritic nephropathy were gradeⅠ(2 cases),grade Ⅲ(11 cases)and grade Ⅳ(2 cases),respectively.The Lee pathohistological classification grade of the IgAN patients presented with nephritic nephropathy was higher than that of the IgAN patients presented with simple nephropathy(P=0.02).3.For 4 weeks of steroid treatment,urine protein of 7 cases(36.84%)was negative,and that of 12 cases(63.16%)was positive.Urine protein negative rate of steroid therapy for the patients with IgAN with NS was lower than that for the children with primary NS in the study by ISKDC(P<0.005).For 8 weeks of steroid treatment,urine protein of 10 cases was negative,and that of 9 cases was positive.Urine protein negative rate of steroid therapy for the patients with IgAN with NS was also lower than that for the children with primary NS in the study by ISKDC(P<0.025).Conclusions: The Lee pathohistological grade of the IgAN children presented with nephritic nephropathy was higher than that of the IgAN cases presented with simple nephropathy,and Lee pathohistological Grade Ⅲ was frequently found in the IgAN children with NS.2.The percentage of IgAN children presented with NS with steroid-resistance accounts for about 50 percent,and the efficacy of steroid treatment for the IgAN children presented with NS is poorer than that for children with primary NS.