P0448CHARACTERISTICS OF PRIMARY GLOMERULAR DISEASE PATIENTS WITH HEMATURIA IN TURKEY: THE DATA FROM TSN-GOLD WORKING GROUP

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Abdullah Sumnu ◽  
Kultigin Turkmen ◽  
Egemen Cebeci ◽  
Aydin Turkmen ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Interstitial Fibrosis ◽  
Basal Membrane ◽  
National Database ◽  
Tubular Atrophy ◽  
Laboratory Findings ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
Serum Blood Urea Nitrogen ◽  
Primary Glomerular Diseases ◽  
Primary Glomerular Disease

Abstract Background and Aims Hematuria is one of the most common laboratory findings in nephrology practice. In different regions of the world, the etiologic causes differ. To date, there is no enough data regarding the clinical and histopathologic characteristics of primary glomerular diseases (PGD) patients with hematuria in our country. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) database between May 2009 and June 2019. The data of all PGD patients over the age of 16 years who were diagnosed with renal biopsy and had hematuria data were included in the study. The biopsy samples were processed using a light microscopy and immunofluorescence examination. Demographic characteristics such as age, sex, indications for biopsy, primary glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were also recorded. Hematuria was defined as the presence of at least 5 red blood cells/hpf. Results Data of 3394 patients were included to the study after the exclusion of patients with secondary glomerulonephritis and patients with missing biopsy findings. While 1699 (50.1%) patients had hematuria, 1695 (49.9%) patients did not have hematuria. Demographic, laboratory, and histopathological characteristics of patients with and without hematuria are given in Table. Patients with hematuria had statistically higher systolic blood pressure (SBP), serum blood urea nitrogen, creatinine, albumin, levels and urine pyuria, however, these patients had statistically lower age, body mass index, presence of hypertension and diabetes, eGFR, 24-hour proteinuria, serum total, HDL and LDL-cholesterol and C3 levels when compared with patients without hematuria. Figure depicted the etiologic causes of patients with and without hematuria. According to histopathological findings, number of global sclerotic glomeruli, cellular and fibrocellular crescents, the levels of mesangial proliferation, endocapillary proliferation, exudative changes in glomeruli, severe tubular atrophy, interstitial inflammation, subendothelial deposition, moderate and severe IgA and C3 deposition were found to be significantly higher and the levels of basal membrane thickening, interstitial fibrosis, subepithelial deposition, severe IgG staining were found to be significantly lower in patients with hematuria. Conclusion This is the first multicenter national report regarding the demographic and histopathologic data of PGD patients with or without hematuria. Hematuria, a feature of nephritic syndrome, was found at a higher than expected in the PGDs presenting with nephrotic syndrome in our national database.

scholarly journals P0470IS GLYCOSURIA A MARKER OF TUBULO-INTERSTITITAL FIBROSIS AND PROGNOSIS IN PRIMARY GLOMERULOPATHIES?

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Carolina Ormonde ◽  
Ivo Laranjinha ◽  
Augusta Gaspar ◽  
Margarida Gonçalves ◽  
Célia Gil ◽  
...  
Keyword(s):  
Interstitial Fibrosis ◽  
Glomerular Sclerosis ◽  
Similar Proportion ◽  
Tubular Damage ◽  
Tubular Dysfunction ◽  
Ckd Progression ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Glomerular Lesion ◽  
Ckd Stage

Abstract Background and Aims Multiple studies have shown that tubular damage is common in glomerular diseases and that it correlates better with chronic kidney disease (CKD) progression than glomerular lesion itself. The link between glomerular and tubular damage is not entirely established. Glycosuria can be found in (proximal) tubular dysfunction and may be used as a marker of tubular lesion and CKD progression. The aim of this study was to evaluate the association between glycosuria (at the diagnosis) and known histological prognostic markers (glomerular sclerosis (%GS) and interstitial fibrosis/tubular atrophy (IFTA)) and CKD progression, in patients with primary glomerulopathies (GP). Method We conducted a 36-month retrospective cohort study with 110 patients with primary GP confirmed by renal biopsy in the last 10 years in our centre – 39 (35.5%) IgA Nephropathy, 27 (24.5%) Membranous Nephropathy, 26 (23.6%) Focal Segmental Glomerulosclerosis and 18 (16.4%) Minimal Change Disease. Patients were divided in two groups according to their glycosuric status at the time of the diagnosis. Data was collected from patients’ charts. Exclusion criteria: patients with diabetes or glucose intolerance, use of SGLT2 inhibitors, secondary GP and transplant kidney patients. Results The global prevalence of glycosuria was 9.1% (n=10). Glycosuric patients had, at baseline, higher serum creatinine (3.9±5.1 vs 1.7±1.3mg/dL, p=0.001), higher baseline albuminuria (7.1±6.3 vs 3.2±3.4 g/g, p=0.002) and lower serum albumin (2.3±0.7 vs 3.2±1.1 g/dL, p=0.022). Both groups had similar proportion of patients that underwent immunosuppressive therapy. At the end of the follow-up, in glycosuric patients, only albuminuria was higher (3.3±0.6 vs 0.7±0.8 g/g, p<0.0001); the eGFR decline rate (ml/min/year), 3-year eGFR and 3-year CKD stage 5D incidence were not statistically different. Glomerular sclerosis (%GS) and interstitial fibrosis and tubular atrophy (IFTA) were not different between groups. These results were confirmed by multivariate analysis. Conclusion Patients with primary GP with glycosuria at diagnosis had higher baseline creatinine and albuminuria. Even though a worse clinical presentation, glycosuria was not associated with well-known prognostic factors (%GS and IFTA) or CKD progression. We can hypothesize that patients with primary GP with glycosuria have severe diseases at diagnosis, but the lesions may have greater reversibility. Prospective and longer studies are needed to confirm these results.

P0501THE EPIDEMIOLOGICAL FEATURES OF PIRMARY GLOMERULAR DISEASES IN TURKEY: THE MULTICENTER STUDY OF TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES (TSN-GOLD) WORKING GROUP

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Aydin Turkmen ◽  
Abdullah Sumnu ◽  
Egemen Cebeci ◽  
Halil Yazici ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Membranous Nephropathy ◽  
Working Group ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
The Mean ◽  
Primary Glomerular Diseases ◽  
Turkish Society

Abstract Background and Aims The largest data on the epidemiology of primary glomerular diseases (PGD) are obtained from the databases of countries or centers. Here, we presented the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD)Working Group. Method The data of patients who underwent renal biopsy and diagnosed as primary glomerular disease were recorded in the database prepared for the study. Between May 2009 and May 2019, a total of 4399 patients from 47 centers were evaluated. Basal data of 3875 patients were analyzed after exclusion of those lacking light microscopy and immunofluorescence findings. Results The mean age was 41.5 ± 14.9 years. Of the patients, 1690 were female (43.6%) and 2180 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGH was IgA nephropathy (25.7%), followed by membranous nephropathy (25.6%) and FSGS (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. Mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. Median proteinuria was 3300 (IQR: 1467-6307) mg / day, mean serum creatinine, estimated GFR and albumin values were 1.4 ± 1.5 mg / dl, 80.7 ± 39.1 ml / min and 3.2 ± 0.9 g / dl, respectively. Conclusion In Turkey, the incidence of IgA nephropathy patients have become more common than membranous nephropathy among PGD patients diagnosed with renal biopsy.

scholarly journals Epidemiological features of primary glomerular disease in Turkey: a multicenter study by the Turkish Society of Nephrology Glomerular Diseases Working Group

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Aydin Turkmen ◽  
Abdullah Sumnu ◽  
Egemen Cebeci ◽  
Halil Yazici ◽  
Necmi Eren ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Membranous Nephropathy ◽  
Working Group ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
The Mean ◽  
Primary Glomerular Diseases ◽  
Turkish Society

Abstract Background The largest data on the epidemiology of primary glomerular diseases (PGDs) are obtained from the databases of countries or centers. Here, we present the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. Methods Data of patients who underwent renal biopsy and received the diagnosis of PGD were recorded in the database prepared for the study. A total of 4399 patients from 47 centers were evaluated between May 2009 and May 2019. The data obtained at the time of kidney biopsy were analyzed. After the exclusion of patients without light microscopy and immunofluorescence microscopy findings, a total of 3875 patients were included in the study. Results The mean age was 41.5 ± 14.9 years. 1690 patients were female (43.6%) and 2185 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGD was IgA nephropathy (25.7%) followed by membranous nephropathy (25.6%) and focal segmental glomerulosclerosis (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. The mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. The median proteinuria, serum creatinine, estimated GFR, and mean albumin values were 3300 (IQR: 1467–6307) mg/day, 1.0 (IQR: 0.7–1.6) mg/dL, 82.9 (IQR: 47.0–113.0) mL/min and 3.2 ± 0.9 g/dL, respectively. Conclusions The distribution of PGDs in Turkey has become similar to that in other European countries. IgA nephropathy diagnosed via renal biopsy has become more prevalent compared to membranous nephropathy.

scholarly journals Gene Expression as a Guide to the Development of Novel Therapies in Primary Glomerular Diseases

2021 ◽  
Vol 10 (11) ◽  
pp. 2262
Author(s):  
Panagiotis Garantziotis ◽  
Stavros A. P. Doumas ◽  
Ioannis Boletis ◽  
Eleni Frangou
Keyword(s):  
Immunoglobulin A ◽  
Enrichment Analysis ◽  
Novel Therapies ◽  
Computational Systems Biology ◽  
Glomerular Diseases ◽  
Thin Basement Membrane Nephropathy ◽  
Novel Drugs ◽  
Primary Glomerular Diseases ◽  
Primary Glomerular Disease ◽  
Computational Systems

Despite improvements in understanding the pathogenic mechanisms of primary glomerular diseases, therapy still remains nonspecific. We sought to identify novel therapies targeting kidney-intrinsic injury of distinct primary glomerulonephritides through computational systems biology approaches. We defined the unique transcriptional landscape within kidneys from patients with focal segmental glomerulosclerosis (FSGS), minimal change disease (MCD), immunoglobulin A nephropathy (IgAN), membranous nephropathy (MN) and thin basement membrane nephropathy (TBMN). Differentially expressed genes were functionally annotated with enrichment analysis, and distinct biological processes and pathways implicated in each primary glomerular disease were uncovered. Finally, we identified novel drugs and small-molecule compounds that may reverse each glomerulonephritis phenotype, suggesting they should be further tested as precise therapy in primary glomerular diseases.

scholarly journals Glycosuria in primary glomerulopathies: prevalence and prognostic significance

2021 ◽  
Author(s):  
Carolina Ormonde ◽  
Ivo Laranjinha ◽  
Célia Gil ◽  
Margarida Gonçalves ◽  
August a Gaspar
Keyword(s):  
Interstitial Fibrosis ◽  
Prognostic Significance ◽  
Renal Outcome ◽  
Diabetic Patients ◽  
Tubular Damage ◽  
Tubular Dysfunction ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Transplant Patients

Abstract Introduction: Tubular damage is common in glomerular diseases (GD). Glycosuria is a marker of tubular dysfunction and may be used to detect tubular lesion and CKD progression. The aim of this study was to evaluate the prevalence and prognostic value of glycosuria at the time of diagnosis in primary glomerulopathies (PG). Methods: We conducted a 24-month retrospective study in patients diagnosed with PG in our center between 2009 and 2020. We excluded diabetic patients, use of SGLT2 inhibitors, transplant patients, and secondary GD. Patients were divided in two groups according to their glycosuria status at diagnosis. Results: We studied 115 patients. Global prevalence of glycosuria was 10% (n=11) and membranous nephropathy (MN) had the highest prevalence (n=5, 17.9%). We found that patients with glycosuria had higher serum creatinine (2.4 vs. 1.2 mg/dL, p=0.030), higher albuminuria (4.8 vs. 1.9 g/g, p=0.004), and lower serum albumin (2.3 vs. 3.2 g/dL, p=0.021). We did not find association with histological prognostic factors. At the end of follow-up, patients with glycosuria had higher prevalence of the composite outcome of stage 5D CKD or 50% increase in basal SCr (45.5% vs. 17.3%, p=0.037). In patients with MN, results were similar but we were able to find an association of glycosuria with more severe interstitial fibrosis and tubular atrophy (25.0 vs. 0.0 %, p=0.032). Conclusion: Ten percent of our patients with PG have glycosuria. Glycosuria at the time of diagnosis was associated with more severe clinical presentation and worst renal outcome. The association with higher albuminuria suggests that tubular function has an impact on the severity and outcomes of PG.

P0395DEMOGRAPHIC AND CLINICAL CHARACTERISTICS OF RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS IN TURKEY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Zeki Aydin ◽  
Kultigin Turkmen ◽  
Fatih Dede ◽  
Emre Yasar ◽  
Savas Ozturk ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Interstitial Fibrosis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Demographic Characteristics ◽  
Glomerular Diseases ◽  
Nephritic Syndrome ◽  
Number Of Patients ◽  
Type 3

Abstract Background and Aims Rapidly progressive glomerulonephritis (RPGN) is a clinical condition that develops due to different etiologic causes, characterized by a rapid and progressive decrease in renal function and progresses to end-stage renal failure in weeks to months if not treated. In our study, diagnostic and demographic characteristics of patients diagnosed with RPGN by biopsy, clinical and laboratory findings in our country were investigated. Method Data were obtained from national multicenter (47 centers) data entered into the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group database between May 2009 and June 2019. Demographic characteristics such as age, sex, indications for biopsy, diagnosis of the glomerular diseases, comorbidities, laboratory and biopsy findings of all patients were recorded. The data presented is cross-sectional and includes application data for the biopsy period. According to their types, RPGN patients were classified as type 1 (anti-GBM related), type 2 (immunocomplex related) and type 3 (immune-negative; “pauci-immune”). Results After exclusion of 46 patients with missing data, 200 patients (mean age 47.9 ± 16.7 years, 44% female) were included in the study which constitutes 5.2% of the total glomerulonephritis database (total number of patients: 3875). Hypertension was present in 62 patients (31.0%) and diabetes was present in 18 patients (9.0%). Renal biopsy was performed in 147 (73.5%) patients due to nephritic syndrome (RPGN included). 80.2% of the patients' biopsies were performed in nephrology clinics and 19.8% of them were performed in radiology clinics. ANCA positivity was found in 121 (60.5%) patients (proteinase 3-ANCA was positive in 55 and myeloperoxidase-ANCA positive in 66 patients). Type 1 RPGN was detected in 11 (5.5%), type 2 RPGN in 42 (21%) and type 3 RPGN in 147 (73.5%) patients. In 21 patients (10.5%), biopsy revealed RPGN with advanced chronic changes (fibrous global sclerotic glomeruli, advanced interstitial fibrosis and tubular atrophy). Mean serum creatinine was 4.2 ± 3.4 mg/dl, median glomerular filtration rate was 18 (10-37) ml/min and proteinuria 2100 (1229-3526) mg/day according to CKD-EPI formula. The mean number of glomeruli in the biopsies was 18.8 ± 10.6 and the number of crescentic glomeruli was 9.9 ± 7.7 (ratio: 52.7%) (Figure). The patients were divided into 3 groups according to their crescentic glomeruli ratios. The proportion of crescentic glomeruli is 10-50% in group 1, 50-80% in group 2, and >80% in group 3. The demographic, laboratory and histopathological characteristics of the groups are given in Figure. It was observed that urea and creatinine increased and calcium and hemoglobin decreased with increasing crescentic glomerular ratio. Conclusion Our study provides valuable demographic, clinical, laboratory and histopathological data about RPGN in our country. Our data are generally compatible with the literature. In our study, advanced chronic histopathological findings were prominent in the biopsy of 21 patients. Early biopsy should be performed to confirm the diagnosis of RPGN and to avoid unnecessary intensive immunosuppressive therapy. In addition to the treatments applied, detailed data, including patient and renal survival are needed.

P0440EPIDEMIOLOGICAL AND CLINICAL CHARACTERSTICS OF IGA NEPHROPATHY PATIENTS IN TURKEY: TSN-GOLD WORKING GROUP

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Necmi Eren ◽  
Meltem Gursu ◽  
Egemen Cebeci ◽  
Aydin Turkmen ◽  
Hasan Haci Yeter ◽  
...  
Keyword(s):  
Iga Nephropathy ◽  
Clinical Data ◽  
Working Group ◽  
Interstitial Fibrosis ◽  
Renal Outcome ◽  
Pathological Features ◽  
Oxford Classification ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Lower Egfr

Abstract Background and Aims According to the data of the Turkish Society of Nephrology-Glomerular Diseases Working Group (TSN-GOLD Working Group), IgA nephropathy is the most common primary glomerular disease in Turkey. The purpose of this study was to investigate the epidemiological and clinical data of IgA nephropathy patients in Turkey. Method 4399 patients with primary glomerular diseases from 47 centers who were followed up between May 2009 and May 2019 were included in the study conducted by TSN-GOLD Working Group. 524 patients were excluded due to lack of pathological data. Among the remaining patients, demographic, clinical and laboratory data of 994 patients with IgA nephropathy were analyzed. Results The median age of the patients was 37 (28-47) years, and 37.3% of them were female. The laboratory and clinical data at the time of diagnosis is presented in Figure-1, and biopsy indications are described in Figure-2. The median number of glomeruli was 16 (IQR: 3.5-4.3), sclerotic glomeruli was 2 (IQR: 1-5), and segmental sclerotic glomeruli was 1 (IQR: 1-2). Exudative changes, subendothelial and subepithelial deposition were present in 566 patients (56.9%), 46 patients (4.6%) and in 38 patients (3.8%), respectively. 662 (66.1%) and 611 of the patients (61.4%) had tubular atrophy and interstitial fibrosis in varying degrees, respectively. 672 (%67.6) and 416 patients (%41.9) had interstitial inflammation and vascular changes, respectively. In immunofluorescence staining, 18%, 30.1%, 4.4%, 68% of the patients had IgG, IgM, C1q and C3 positivity, respectively. Crescentic glomeruli were detected in 227 patients (3.3 ± 3.1 glomeruli). Patients with crescentic glomeruli had significantly higher proteinuria and lower eGFR than the patients without [2203 mg/day (15-26078) vs 1807 mg/day (15-29112); p=0.001; 55.3 ml/min/1.73 m2 (3.72-141.9) vs 72 ml/min/1.73 m2 (3.84-150.81); p<0.001, respectively]. Oxford classification was applied to 544 patients. Endocapillary hypercellularity (E1), mesengial hypercellularity (M1), tubular atrophy and interstitial fibrosis (T1 and T2), segmental sclerosis (S1) were present in 126 (13%), 425 (42.8%), 306 (30.8%) and 325 patients (%32.7), respectively. Proteinuria levels were higher in patients with endocapillary hypertrophy, mesengial hypercellularity, tubular atrophy-interstitial fibrosis and segmental sclerosis. eGFR levels were lower in patients with endocapillary hypertrophy, tubular atrophy-interstitial fibrosis and segmental sclerosis (Figure-3). Conclusion In this study we found that, the most common presentation of IgA nephropathy patients in our country was asymptomatic urinary abnormalities followed by nephritic and nephrotic syndrome. Higher proteinuria and lower eGFR values in patients with crescentic glomeruli, support the adoption of crescentic lesions in the new Oxford classification (MEST-C) to predict more precise outcome of IgA nephropathy patients. The high number of patients to whom the Oxford classification was applied provided us with the opportunity to examine the clinical reflections of pathological features. Evaluation of the follow-up data of the patients will give us the possibility to reveal the effect of initial clinical and pathological features on clinical findings and renal outcome.

scholarly journals RETROSPECTIVE STUDY OF HISTOLOGICAL ANALYSIS AND ITS CORRELATION WITH CLINICAL PRESENTATION AND OUTCOME OF IGA NEPHROPATHY FROM A TERTIARY CENTRE OF GUWAHATI ASSAM.

2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Manjuri Sharma ◽  
Manzoor Ahmad Parry ◽  
Hamad Jeelani ◽  
Pranab Jyoti Mahanta
Keyword(s):  
Retrospective Study ◽  
Iga Nephropathy ◽  
Clinical Presentation ◽  
Interstitial Fibrosis ◽  
Clinical Manifestations ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Mesangial Hypercellularity ◽  
Endocapillary Hypercellularity

Background: IgA nephropathy (IgAN) is one of the most common glomerular diseases with varied presentations. We aimed to study clinical presentation and outcome of IgAN and correlate with histopathology at the time of presentation. Methods: This is a retrospective study in which we analyzed kidney biopsy data, clinical manifestations and outcome of 137 patients with a diagnosis of primary IgAN from 2012 to 2016. Kidney biopsies were reviewed as per Oxford classification assessing mesangial hypercellularity, endocapillary hypercellularity, segmental sclerosis/adhesion, tubular atrophy/interstitial fibrosis. Correlation analysis was done for biopsy findings and clinical presentation/outcome. P score less than 0.05 was taken as significant. Results: Mean age for presentation was 27.35 years with 83 males and 54 females. Asymptomatic urinary abnormality was the most common clinical presentation (28.5%). Mean serum creatinine was 2.23 ± 2.06mg/dl with mean proteinuria of 1.49 ± 1.43g/day. Mesangial hypercellularity (M) and Endocapillary hypercellularity (E) lesions were significantly associated with proteinuria at the time of biopsy (p=0.02& 0.04 respectively). Segmental glomerulosclerosis (S) and tubular atrophy (T) were significantly associated with eGFR and mean arterial pressure at the time of biopsy. Mean time of follow up was 1.6 years. M1, E0, S1, T0 were the most common lesions. M, S and T lesions in biopsy were significantly associated with decrease in GFR at the end of follow up. Conclusion: In our study, most common presentation of IgAN was AUA with rarity of macroscopic hematuria. M, S and T lesions were associated with decreased GFR on follow up. Key words: IgA Nephropathy, Nephrotic Syndrome, Oxford MEST classification

scholarly journals Morphologic lesions of membranous nephropathy in association with various demographic and laboratory parameters of patients; a single center study

2019 ◽  
Vol 9 (1) ◽  
pp. e09-e09
Author(s):  
Sara Zamani ◽  
Hamid Nasri
Keyword(s):  
Nephrotic Syndrome ◽  
Serum Creatinine ◽  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Interstitial Fibrosis ◽  
Tubular Atrophy ◽  
Glomerular Diseases ◽  
Laboratory Parameters ◽  
Positive Correlation ◽  
End Stage

Introduction: Nephrotic syndrome is an important clinical presentation of glomerular diseases that is classified into several types based on the findings of renal biopsy. Membranous neuropathy is the most common cause of nephrotic syndrome, especially in adults over 40 years of age, which may lead to end-stage renal failure. Objectives: The present study aimed to assess the association of morphologic lesions of membranous nephropathy (MN) on renal biopsy with various demographic and laboratory parameters of the patients. Patients and Methods: This study was performed on renal biopsies, which were referred to the laboratory with the diagnosis of MN. To reach a definite diagnosis of MN, an immunofluorescence study (IgG, IgA, IgM, C1q and C3 antibody deposits) was conducted for all patients. Light microscopy was conducted to categorize the morphologic lesions of the glomeruli and interstitial area. The percentage of interstitial fibrosis/tubular atrophy was assessed too. Additionally, age, gender, and 24- hour urinary protein and serum creatinine were recorded. Results: Among 175 idiopathic MN patients, 98 were male (56%). The patients’ age was between 14 and 84 years (mean; 42±15 years). The mean of serum creatinine and 24-hour urine protein were 1.05 ± 0.31 mg/dL and 2779.56± 1495.80 mg/d, respectively. We found a significant correlation between gender and serum creatinine level, which was higher in men (P<0.001). Moreover, there was a significant, positive correlation between serum creatinine and age of patients (P<0.001, r=0.25). Additionally, there was a significant correlation between serum creatinine and interstitial fibrosis (P=0.001). We found a significant correlation between serum creatinine and the pathologic stage of glomeruli (P=0.003). The stages of glomeruli were also associated with the proportion of interstitial fibrosis (P=0.001) and C3 deposition rate (P=0.002). IgG deposition score was also significantly different in age ranges over and under 40 years of age (P=0.001). The 24-hours proteinuria had no correlation with other laboratory parameters and microscopic findings. Conclusion: In accordance with other studies, we found that MN is more common among male patients. The positive correlation between serum creatinine and proportion of interstitial fibrosis is in concordance with previous studies. We found a positive correlation between serum creatinine and glomerular morphologic stages. It may show the importance of glomerular damage intensity in prognosis and survival of patients.

scholarly journals Enyhe szövettani eltérések ellenére gyors progressziójú proliferativ glomerulonephritis monoklonális immunglobulin-G-depozitumokkal

2018 ◽  
Vol 159 (38) ◽  
pp. 1567-1572
Author(s):  
Dóra Bajcsi ◽  
Kypros Constantinou ◽  
László Krenács ◽  
Zsolt Barabás ◽  
Szabolcs Molnár ◽  
...  
Keyword(s):  
Kidney Failure ◽  
Interstitial Fibrosis ◽  
Rapidly Progressive Glomerulonephritis ◽  
Proliferative Glomerulonephritis ◽  
Caucasian Woman ◽  
Tubular Atrophy ◽  
Nephritic Syndrome ◽  
Dense Deposits ◽  
Tubulointerstitial Lesions ◽  
First Time

Abstract: Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits is characterized by granular deposits of monoclonal IgG; histologically it has typically a membranoproliferative or endocapillary pattern, and seen electronmicroscopically there are dense deposits without substructure. Here, we present the case of a 62-year-old Caucasian woman who was admitted with rapidly progressive kidney failure. The patient’s status, the laboratory and imaging examinations did not support prerenal, postrenal and – among the intrinsic causes – vascular and tubulointerstitial origin. The proteinuria and dysmorphic microhematuria suggested rapidly progressive glomerulonephritis. Tests for anti-neutrophil cytoplasmic antibodies, anti-glomerular basement membrane, antinuclear antibodies and cryoglobulins were negative, the C3 and C4 levels were normal. The biopsy evaluation diagnosed proliferative glomerulonephritis with monoclonal IgG deposits because of mesangial granular deposits of IgG3-kappa, C3, and C1q, and ultrastructurally electron-dense deposits (incidence in our adult native kidney biopsy series: 0.18%). 31 glomeruli were assessed histologically. 29 glomeruli displayed mild mesangial hypercellularity, 2 glomeruli were globally sclerotic. Crescents were not observed. Mild arteriolar hyalinosis, interstitial fibrosis and tubular atrophy accompanied the glomerular alterations. In the postbiopsy evaluation, paraprotein or multiple myeloma was not detected. Despite the mild histological findings, the kidney failure progressed, and hemodialysis had to be started two weeks after the biopsy. Steroids, cyclophosphamide and rituximab did not affect her kidney function, and she remained on hemodialysis during the follow-up of 39 months. This report presents for the first time proliferative glomerulonephritis with monoclonal IgG deposits as the possible cause of rapidly progressive nephritic syndrome in the absence of pronounced glomerular proliferative, sclerotic or tubulointerstitial lesions. Orv Hetil. 2018; 159(38): 1567–1572.

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