Clinicopathological comparative study of IgM nephropathy and IgA nephropathy in children / 临床儿科杂志

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 patholog-ical 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 (P0.05). In IgAN group, the incidence of proteinuria, RBC casts in tubular, C3 and ifbrinogen 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.

Urinary protein markers predict the severity of renal histological lesions in children with IgA nephropathy and IgM nephropathy / 中华实用儿科临床杂志

Objective To compare the clinical manifestations,renal histological lesions,and the levels of urinary protein markers between the children with IgA nephropathy (IgAN) and those with IgM nephropathy (IgMN), and to determine whether urinary protein markers could predict the severity of renal histological lesions in children with IgAN and IgMN.Methods Seventy-four children with renal biopsy-proven IgAN and IgMN from January 2002 to October 2014 were enrolled in the study.The levels of IgG, albumin (Alb), transferrin (TRF), α1-microglobulin (α1-MG) ,β2-microglobulin (β2-MG) and N-acetyl-β-glucosaminidase (NAG) in morning urine samples before biopsy were measured.The semi-quantitative scores of mesangial hypercellularity (MC), glomerulosclerosis (GS), and tubule-interstitial damage (TID) were used to assess renal histological lesions.Multivariate Logistic regression analysis was used to determine whether urinary protein levels were independently associated with renal histological lesions.The area under the receiver-operating-characteristic curve (AUC) was calculated to assess the predictive ability of urinary protein markers.Results Seventy-four children (44 cases with IgAN,30 cases with IgMN) were included.The urinary levels of α1-MG and Alb were significantly higher in children with IgAN as compared to those with IgMN.The differences, however, did not remain significant after adjustment for age.The urine protein, as an independent factor associated with severe MC(＞ 5 mesangial cells per mesangial area) was TRF(B =0.010), and severe GS (≥ 10％ glomeruli showing segmental adhesion or sclerosis) was significantly correlated with Alb(B =0.001) ,and severe TID (focal or diffuse tubular and interstitial lesions) was significantly correlated with NAG(B =0.038).Urinary β2-MG was not significantly associated with severe MC, GS and TID.Urinary TRF, Alb and NAG achieved the best AUC of 0.85 (P ＜ 0.001) ,0.78 (P =0.002), and 0.78 (P =0.003), respectively, for predicting severe MC, GS, and TID.Conclusions Urinary proteins are useful to predict the severity of renal histological lesions in children with IgAN and IgMN.Urinary TRF, Alb and NAG have better predictive value.

IgM nephropathy with nephrotic syndrome in children / 国际儿科学杂志

Objective To investigate the clinical charactors and treatment of IgM nephropathy with nephrotic syndrome(NS) in children.Methods Thirty-six IgM nephropathy patients hospitalized in Hunan Children's Hospital as research group(group A),from June 2005 to June 2012.One hundred and six patients with minimal change disease (MCD) as control group (group B),followed up for 1 ～ 8 years.Results (1) Hematuria at presentation of the two groups respectly 3.8％ vs 30.6％ (x2 =20.403,P ＜ 0.05).(2) Renal pathology revealed that there were 26 (72.2％)patients with minimal change disease,9 cases (72.2 ％)with moderate membranoproliferative glomerulonephritis,1 cases with focal segmental glomerulosclerosis.(3) According to renal pathology,group A patients were divided into two sub groups:mild group and moderate group.To compared with group B,the steroid-resistant incidence of the 3 group were respectly 12.3％,19.2％,77.8％ (x2 =24.369,P ＜ 0.05).There was no significant difference between control group and mild group.(4)The remission rate of proteinuria in steroid-resistant patients who combined to use mycophenolate mofetil (MMF) with the two groups were respectively 50％ and 85.7 ％ (x2 =3.60,P ＞ 0.05).Conclusion Incidence of hematuria is higher in IgM nephropathy patients and patients with renal pathology for moderate lesions have a high steroid-resistant,and need use immunosuppressive early.MMF may be a good immunosuppressive for theses patients.

Rituximab Followed by Mycophenolate Mofetil in Children With IgM Nephropathy.

IgM nephropathy presents with refractory nephrotic syndrome and its treatment is a significant challenge for pediatricians. We present two patients with IgM nephropathy and frequently relapsing nephrotic syndrome treated with rituximab and subsequently mycophenolate mofetil. Both showed complete remission, which 24 to 30 months later, was still maintained. The role of mycophenolate mofetil therapy in maintaining remission after successful treatment of rituximab in IgM nephropathy needs to be examined.

Immunoglobulin M nephropathy nephropathy in adults and adolescents in India: A single-center study of natural history.

Background: Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis (GN) usually presenting clinically as steroid resistant/dependent nephrotic syndrome (NS) with pathology of mesangial proliferative GN or focal and segmental glomerulosclerosis with diffuse predominant mesangial IgM deposits. Not much information is available about its natural history. This is the first Indian study to our knowledge on IgMN in adults and adolescents. Materials and Methods: We evaluated renal biopsies performed at our center between January,'04 to September,'09. Biopsies of all adolescents and adults were evaluated for IgMN and we studied their age, gender distribution, blood pressure (BP), disease duration, steroid/immunosuppressive management and serial serum creatinine (SCr), urinary proteins, and BP values. Patients with other systemic diseases/infections and children were excluded. Results: IgMN constituted 4.3% of 2702 adult renal biopsies. No significant gender predilection was noted. Males presented at average age of 23.1 years, females at 30 years. Steroid-dependent NS was the commonest presentation noted in 75% followed by steroid-resistant NS. Hypertension was noted in 10% patients. Mesangial proliferative GN (MePGN) was commonest histopathological finding noted in 74.4%, followed by focal segmental glomerulosclerosis (FSGS) in 16.2%, and minimal change disease (MCD) in 9.4% biopsies. Sole IgM deposits were noted in 88.5%. All MCD, 35.6% MePGN reached remission, FSGS progressed to renal failure by 1 year. Hypertension, proteinuria, interstitial fibrosis, and FSGS were bad prognosticators. Conclusions: This is the first Indian study of IgMN in adults and adolescents carried out over a period of 5.8 years, which has shown that hypertension, proteinuria, and interstitial fibrosis at presentation have bad prognosis.

Hereditary IgM nephropathy: report of three cases in a kindred and review of literatures / 中华肾脏病杂志

Objective To recognize hereditary IgM glomerulopathy. Methods Three patients with hereditary IgM glomerulopathy were report first time in China. Genealogic investigation and light, immunofluorescence and electron microscopy were performed. Literatures concerned were reviewed. Results Kidney biopsies from three patients all revealed diffuse mesangial proliferative glomerulonephritis with intense IgM and C3 deposits. These siblings might be suffering from a hitherto undescribed clinico-pathological entity. Genealogic investigation enabled us to discover 10 additional affected members with clinical glomerulonephritis in the kindred of the family. The clinical picture was that of hematuria and/or proteinuria, meanwhile one patient died of uremia. Conclusion Genetic factors may be involved in the mechanism of IgM nephropathy .