• chronic kidney disease
• glomerulonephritis
• endodontic inflammation
• endodontics
• blood pressure
• wistar rats
• animal models

• Animals
• Rats, Wistar
• Male
• Rats
• Kidney/pathology
• Periapical Periodontitis/complications
• Periapical Periodontitis/pathology

• Adults
• End-stage kidney disease
• Immunoglobulin-associated mesangiocapillary glomerulonephritis
• Kidney replacement therapy
• Outcomes

• Chronic kidney disease (CKD)
• Complement-mediated MPGN (C-MPGN)
• Immune complex-mediated MPGN (IC-MPGN)
• Kidney Replacement therapy (KRT)
• Membranoproliferative Glomerulonephritis (MPGN)

• Humans
• Glomerulonephritis, Membranoproliferative/pathology
• Glomerulonephritis, Membranoproliferative/immunology
• Glomerulonephritis, Membranoproliferative/classification
• Male
• Female
• Retrospective Studies
• Adult
• Fluorescent Antibody Technique
• Middle Aged
• Immunosuppressive Agents/therapeutic use
• Biopsy
• Antigen-Antibody Complex
• Young Adult
• Complement System Proteins/analysis
• Kidney/pathology
• Kidney/immunology
• Treatment Outcome

• Biomarker
• Glomerular disease
• Liquid biopsy
• Proteinuria
• Protocol

• Humans
• Biomarkers/blood
• Biomarkers/urine
• Nephrotic Syndrome/urine
• Nephrotic Syndrome/blood
• Nephrotic Syndrome/diagnosis
• Prospective Studies
• Japan
• Glomerulosclerosis, Focal Segmental/urine
• Glomerulosclerosis, Focal Segmental/blood
• Glomerulosclerosis, Focal Segmental/diagnosis
• Receptors, Urokinase Plasminogen Activator/blood
• Glomerulonephritis, Membranous/urine
• Glomerulonephritis, Membranous/blood
• Glomerulonephritis, Membranous/diagnosis
• Adult
• Nephrosis, Lipoid/urine
• Nephrosis, Lipoid/blood
• Nephrosis, Lipoid/diagnosis
• Research Design
• Receptors, Phospholipase A2/immunology
• Thrombospondins/blood
• Glomerulonephritis, Membranoproliferative/blood
• Glomerulonephritis, Membranoproliferative/urine
• Glomerulonephritis, Membranoproliferative/diagnosis
• Male
• Female
• Lupus Nephritis/blood
• Lupus Nephritis/urine
• Lupus Nephritis/diagnosis
• East Asian People
• B7-1 Antigen

• 18ek0109354h0003 Japan Agency for Medical Research and Development
• 21ek0109544h0001 Japan Agency for Medical Research and Development

• Clinical characteristics
• Membranoproliferative glomerulonephritis
• Nephrotic syndrome
• Registry

• Adolescent
• Young Adult
• Humans
• Aged
• Adult
• Glomerulonephritis, Membranoproliferative/drug therapy
• Glomerulonephritis, Membranoproliferative/epidemiology
• Nephrotic Syndrome/drug therapy
• Nephrotic Syndrome/epidemiology
• Japan/epidemiology
• Prednisolone/therapeutic use
• Registries
• Demography

• FC001045 Arthritis Research UK
• FC001048 Arthritis Research UK
• Ministry of Health, Labour and Welfare

• focal segmental glomerulosclerosis
• membranous nephropathy
• minimal change disease

• Humans
• Nephrotic Syndrome/drug therapy
• Nephrotic Syndrome/epidemiology
• Japan/epidemiology
• Glomerulosclerosis, Focal Segmental/drug therapy
• Glomerulosclerosis, Focal Segmental/epidemiology
• Prednisolone
• Glomerulonephritis, Membranous/drug therapy
• Glomerulonephritis, Membranous/epidemiology
• Nephrosis, Lipoid
• Registries
• Demography

• FC001045 Arthritis Research UK
• FC001048 Arthritis Research UK
• Ministry of Health, Labour and Welfare,Japan
• Ministry of Health, Labour and Welfare

• C3 glomerulopathy
• Membranoproliferative glomerulonephritis
• children

• Glomerulonephritis
• immunofluorescence
• nephrotic

The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) based on immunofluorescence findings in kidney biopsies has provided insights into these two distinct diseases. C3G is further classified into dense deposit disease and C3 glomerulonephritis (C3GN) based on electron micrographic findings. Although these diseases have poor outcomes, limited Japanese literature confined to small, single-center cohorts exist on these diseases. We retrospectively analyzed 81 patients with MPGN type I and III from 15 hospitals in the Japan Renal Biopsy Registry to compare demographic, clinical characteristics and treatment outcomes of patients with IC-MPGN to those with C3GN. Of the 81 patients reviewed by immunofluorescence findings in kidney biopsies, 67 patients had IC-MPGN and 14 patients had C3GN. Age at diagnosis and systolic and diastolic pressure were higher and proteinuria and impaired renal function were significantly more prevalent in patients with IC-MPGN than those with C3GN. About 80% of the patients in both groups were treated with immunosuppressive therapy. At last follow-up (median 4.8 years), complete remission rate of proteinuria was significantly higher in patients with C3GN (64.3%) than in those with IC-MPGN (29.9%; P = 0.015). The renal survival rate was lower in patients with IC-MPGN when compared to C3GN (73.1% vs. 100%; log-rank, P = 0.031). Systolic blood pressure and renal function at baseline were independent predictors of progression to end-stage kidney disease. The overall prognosis of patients with C3GN is more favorable than for patients with IC-MPGN.

• Adolescent
• Adult
• Age Factors
• Aged
• Antigen-Antibody Complex
• Biopsy
• Blood Pressure
• Demography/methods
• Female
• Fluorescent Antibody Technique
• Follow-Up Studies
• Glomerulonephritis/diagnosis
• Glomerulonephritis/physiopathology
• Humans
• Japan
• Kidney
• Male
• Middle Aged
• Registries
• Remission Induction
• Retrospective Studies
• Treatment Outcome

• ministry of health, labour and welfare

To identify the clinical characteristics, histopathological features, and prognosis of kidney disease in a large cohort of elderly patients from Northeast China.

We retrospectively analyzed the renal disease spectrum in 7,122 patients who underwent renal biopsies at the Second Hospital of Jilin University from 2006 to 2020. Patients were grouped according to age: below 60 years (non-elderly group, n = 5923) and at least 60 years (elderly group, n = 1199). The clinical and pathological characteristics of renal biopsy patients in the groups were analyzed using the t-test and chi-square test.

Compared with the non-elderly group, the elderly group had significantly fewer patients with primary glomerulonephritis, but more patients with tubulointerstitial disorders (p < .05). The incidence of IgA nephropathy, mesangial proliferative glomerulonephritis, and lupus nephritis was significantly lower in elderly patients than in non-elderly patients. The incidence of membranous nephropathy, membranoproliferative glomerulonephritis, diabetic nephropathy, hypertensive nephropathy, systemic vasculitis-associated renal damage, and amyloid nephropathy was significantly higher in elderly patients than in non-elderly patients (p < .05). The incidence of perinephric hematoma (≥4 cm²) in elderly patients with renal biopsy was lower than that in non-elderly patients. We noted that 79.9% of primary glomerulonephritis patients who received immunosuppressive therapy showed a remission rate of 83.5%.

The spectrum of kidney disease in the elderly is different from that in the younger population.

• elderly
• pathology
• primary glomerulonephritis
• prognosis
• renal biopsy

Focal segmental glomerulosclerosis (FSGS) is a serious condition leading to kidney failure. We aimed to investigate the clinical characteristics of FSGS and its differences compared with minimal change disease (MCD) using cross-sectional data from the Japan Renal Biopsy Registry. In Analysis 1, primary FSGS (n = 996) were stratified by age into three groups: pediatric (< 18 years), adult (18–64 years), and elderly (≥ 65 years), and clinical characteristics were compared. Clinical diagnosis of nephrotic syndrome (NS) was given to 73.5% (97/132) of the pediatric, 41.2% (256/622) of the adult, and 65.7% (159/242) of the elderly group. In Analysis 2, primary FSGS (n = 306) and MCD (n = 1303) whose clinical diagnosis was nephrotic syndrome (NS) and laboratory data were consistent with NS, were enrolled. Logistic regression analysis was conducted to elucidate the variables which can distinguish FSGS from MCD. On multivariable analysis, higher systolic blood pressure, higher serum albumin, lower eGFR, and presence of hematuria associated with FSGS. In Japanese nationwide registry, primary FSGS patients aged 18–64 years showed lower rate of NS than those in other ages. Among primary nephrotic cases, FSGS showed distinct clinical features from MCD.

The reclassification of membranoproliferative glomerulonephritis (MPGN) into immune-complex MPGN (IC-MPGN) and C3 glomerulopathy (C3G) has provided insights into 2 distinct diseases. Although outcomes in adults are poor in both diseases, the pediatric literature is scarce and limited to small, single-center cohorts.

We conducted a retrospective analysis of 165 pediatric patients across 17 hospitals to compare outcomes between children with IC-MPGN and C3G.

Forty-two percent of patients initially diagnosed with MPGN were reclassified as C3G after a review of renal biopsy reports. There was a trend toward higher serum creatinine levels in patients with C3G compared with IC-MPGN both at diagnosis (mean 168.9 [range 45.4–292.4] vs. 93.7 [range 70.7–116.6] μmol/l, P = 0.25) and after a mean follow-up time of 4 years (mean 145.0 (range −8.1 to 298.1) vs 99.1 (range 46.3–151.9) μmol/l, P = 0.47), although the estimated glomerular filtration rate (eGFR) was not significantly different. Steroid treatment was associated with a significant improvement in eGFR versus no steroids in C3G (mean +43.0 (range 12.9–73.0) vs. −3.0 (range −23.1 to 17.2) ml/min per 1.73 m², P = 0.02) but not in IC-MPGN. Overall kidney function was preserved in both groups although hypertension remained prevalent in 42.5% of the cohort at the last follow-up, and the urine protein/creatinine ratio remained elevated (mean 253.8 [range 91.9–415.7] mg/mmol).

This large pediatric IC-MPGN/C3G cohort revealed nearly half of the patients were misclassified, and there may be a trend toward worse renal prognosis in C3G although they may have greater steroid responsiveness. The overall prognosis appears to be more favorable than in adults; however, persistent hypertension and proteinuria suggest suboptimal disease control.

• C3 glomerulonephritis
• C3 glomerulopathy
• dense deposit disease
• hypertension
• immune-complex membranoproliferative glomerulonephritis
• proteinuria

The Japan Renal Biopsy Registry (J-RBR), the first nation-wide registry of renal biopsies in Japan, was established in 2007, and expanded to include non-biopsy cases as the Japan Kidney Disease Registry (J-KDR) in 2009. The J-RBR/J-KDR is one of the biggest registries for kidney diseases. It has revealed the prevalence and distribution of kidney diseases in Japan. This registry system was meant to be revised after 10 years.

In 2017, the Committees of the Japanese Society of Nephrology started a project for the revision of the J-RBR/J-KDR. The revised system was designed in such a way that the diagnoses of the patients could be selected from the Diagnosis Panel, a list covering almost all known kidney diseases, and focusing on their pathogenesis rather than morphological classification. The Diagnosis Panel consists of 22 categories (18 glomerular, 1 tubulointerstitial, 1 congenital/genetical, 1 transplant related, and 1 other) and includes 123 diagnostic names. The items for clinical diagnosis and laboratory data were also renewed, with the addition of the information on immunosuppressive treatment.

The revised version of J-RBR/J-KDR came into use in January 2018. The number of cases registered under the revised system was 2748 in the first year. The total number of cases has reached to 43,813 since 2007.

The revised version 2018 J-RBR/J-KDR system attempts to cover all kidney diseases by focusing on their pathogenesis. It will be a new platform for the standardized registration of kidney biopsy cases that provides more systemized data of higher quality.

The online version of this article (10.1007/s10157-020-01932-6) contains supplementary material, which is available to authorized users.

• Pathology
• Registry
• Renal biopsy

Expression levels and changes of serum cystatin C (C's C) and soluble vascular endothelial growth factor receptor 1 (sVEGFR1) in treatment of patients with glomerulus nephritis (GN) were investigated. The medical records of 88 patients with GN who were diagnosed in Weifang People's Hospital from March 2014 to June 2017 were collected, and their medical records were considered as a study group. The study group was divided into secondary glomerulonephritis (SGN) group (52 cases) and primary glomerulonephritis (PGN) group (36 cases). Physical examination data of 50 healthy volunteers who were examined in the same hospital during the same period were considered as a control group. The correlation between expression of serum C's C and expression of sVEGFR1 of patients with GN was compared. Expression levels of serum C's C and sVEGFR1 of patients before treatment in the study group were higher than those in the control group (P<0.05). With the extension of the treatment cycle, C's C and sVEGFR1 expression levels in PGN and SGN groups reduced gradually (P<0.05). With the extension of the treatment cycle, the renal function indexes of the study group patients showed a downward trend (P<0.05). Expression of C's C was positively correlated with urea nitrogen and creatinine (P<0.05). In conclusion, C's C and sVEGFR1 are highly expressed in the serum of patients with GN. Expression of C's C and sVEGFR1 decrease as patients are treated. C's C and sVEGFR1 can be used as indicators for monitoring the condition of patients with GN. It is worthwhile to promote C's C and sVEGFR1 in clinical practice.

• cystatin C
• disease monitoring
• glomerulus nephritis
• soluble vascular endothelial growth factor receptor 1

Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of end stage kidney disease (ESKD) and the clinical outcomes of patients with MPGN who commence kidney replacement therapy have not been comprehensively studied.

All adult patients with ESKD due to glomerulonephritis commencing kidney replacement therapy in Australia and New Zealand from January 1, 1996 to December 31, 2016 were reviewed. Patients with ESKD due to MPGN were compared to patients with other forms of glomerulonephritis. Patient survival on dialysis and following kidney transplantation, kidney recovery on dialysis, time to transplantation, allograft survival, death-censored allograft survival and disease recurrence post-transplant were compared between the two groups using Kaplan Meier survival curves and Cox proportional hazards regression.

Of 56,481 patients included, 456 (0.8%) had MPGN and 12,660 (22.4%) had another form of glomerulonephritis. Five-year patient survival on dialysis and following kidney transplantation were similar between patients with ESKD from MPGN and other forms of glomerulonephritis (Dialysis: 59% vs. 62% p = 0.61; Transplant: 93% vs. 93%, p = 0.49). Compared to patients with other forms of glomerulonephritis, patients with MPGN had significantly poorer 5-year allograft survival (70% vs. 81% respectively, p = 0.02) and death censored allograft survival (74% vs. 87%, respectively; p < 0.01). The risk of disease recurrence was significantly higher in patients with MPGN compared to patients with other glomerulonephritidites (18% vs. 5%; p < 0.01). In patients with MPGN who had allograft loss, patients with MPGN recurrence had a significantly shorter time to allograft loss compared to patients with MPGN who had allograft loss due to any other cause (median time to allograft loss 3.2 years vs. 4.4 years, p < 0.01).

Compared with other forms of glomerulonephritis, patients with MPGN experienced comparable rates of survival on dialysis and following kidney transplantation, but significantly higher rates of allograft loss due to disease recurrence.

• Dialysis
• End-stage kidney disease
• Kidney transplantation
• Membranoproliferative glomerulonephritis
• Mesangiocapillary glomerulonephritis

• Cryofiltration
• Cryoglobulin
• Membranoproliferative glomerulonephritis
• Mizoribine

• Complement alternative pathway
• Immune complex
• Membranoproliferative glomerulonephritis
• New classification