Kidney Outcome in Primary Focal Segmental Glomerulosclerosis (FSGS) by Using a Predictive Model

2019 ◽  
Author(s):  
Shahrzad Ossareh ◽  
Mansoureh Yahyaei ◽  
Mojgan Asgari ◽  
Hanri Afghahi
Keyword(s):  
Kidney Disease ◽  
Predictive Model ◽  
Focal Segmental Glomerulosclerosis ◽  
Interstitial Fibrosis ◽  
Cox Model ◽  
End Stage Kidney Disease ◽  
Model Interaction ◽  
Segmental Glomerulosclerosis ◽  
End Stage ◽  
Adjusted Model

Abstract Background: Focal segmental glomerulosclerosis (FSGS) is one of the important causes of end stage kidney disease (ESKD). We evaluated the risk factors of progression of primary FSGS to chronic kidney disease (CKD) or ESKD with a predictive model including clinical and histological predictors. Methods: 201 patients with primary FSGS (59% male, mean age: 38±15 years), were studied. Time-dependent Cox model and C statistics were used for the predictive model. Interaction and correlation between independent variables were estimated. Results: During 55±27 months of follow-up, 82 patients (41%) developed CKD (46) or ESKD (36) patients. In adjusted model, 1 unit of higher serum creatinine (SCr) at baseline (HR:1.39, 95%CI: 1.15-1.70) and 1% increase in glomeruli with segmental glomerulosclerosis (SGS) (HR: 1.03, 95% CI: 1.02-1.04) or interstitial fibrosis/tubular atrophy (IF/TA) (HR: 1.03, 95% CI: 1.01-1.05) increased the risk of CKD/ESKD. In adjusted model, higher baseline proteinuria and collapsing variant were not associated with risk of CKD/ESKD. By adding SGS and IF/TA scores to baseline SCr in the model, discrimination by C statistics was 0.83 (95%CI: 0.77-0.90). Median renal survival was 3.1 years (95% CI: 2.2-4.1 years) in patients with highest risks score (baseline eGFR<25 ml/min/1.73 m2+ IF/TA/SGS> 50%), and 8.1 years (95% CI: 7.7-8.6 years).in those with lowest score (baseline eGFR>75 ml/min/1.73 m2+ IF/TA/SGS <5%). Conclusion: In primary FSGS, higher baseline SCr, increased SGS and IF/TA, but not baseline proteinuria and collapsing pathology, were the predictors for CKD/ESKD. These findings indicated the importance of timely detection and referral in prognosis of primary FSGS. Keywords: Focal segmental glomerulosclerosis, End stage kidney disease, Pathology

Kidney Outcome in Primary Focal Segmental Glomerulosclerosis (FSGS) by Using a Predictive Model

2020 ◽  
Author(s):  
Shahrzad Ossareh ◽  
Mansoureh Yahyaei ◽  
Mojgan Asgari ◽  
Hanri Afghahi
Keyword(s):  
Kidney Disease ◽  
Predictive Model ◽  
Focal Segmental Glomerulosclerosis ◽  
Interstitial Fibrosis ◽  
Cox Model ◽  
Tubular Atrophy ◽  
Model Interaction ◽  
Segmental Glomerulosclerosis ◽  
End Stage ◽  
Adjusted Model

Abstract Background: Focal segmental glomerulosclerosis (FSGS) is one of the important causes of end stage kidney disease (ESKD). We evaluated the risk factors of progression of primary FSGS to chronic kidney disease (CKD) or ESKD with a predictive model including clinical and histological predictors.Methods: 201 patients with primary FSGS (59% male, mean age: 38±15 years), were studied. Time-dependent Cox model and C statistics were used for the predictive model. Interaction and correlation between independent variables were estimated.Results: During 55±27 months of follow-up, 82 patients (41%) developed CKD (46) or ESKD (36) patients. In adjusted model, 1 unit of higher serum creatinine (SCr) at baseline (HR:1.39, 95%CI: 1.15-1.70) and 1% increase in glomeruli with segmental glomerulosclerosis (SGS) (HR: 1.03, 95% CI: 1.02-1.04) or interstitial fibrosis/tubular atrophy (IF/TA) (HR: 1.03, 95% CI: 1.01-1.05) increased the risk of CKD/ESKD. In adjusted model, higher baseline proteinuria and collapsing variant were not associated with risk of CKD/ESKD. By adding SGS and IF/TA scores to baseline SCr in the model, discrimination by C statistics was 0.83 (95%CI: 0.77-0.90). Median renal survival was 3.1 years (95% CI: 2.2-4.1 years) in patients with highest risks score (baseline eGFR<25 ml/min/1.73 m2+ IF/TA/SGS> 50%), and 8.1 years (95% CI: 7.7-8.6 years).in those with lowest score (baseline eGFR>75 ml/min/1.73 m2+ IF/TA/SGS <5%).Conclusion: In primary FSGS, higher baseline SCr, increased SGS and IF/TA, but not baseline proteinuria and collapsing pathology, were the predictors for CKD/ESKD. These findings indicated the importance of timely detection and referral in prognosis of primary FSGS.

scholarly journals Development of a clinical prediction model of chronic kidney disease in primary Focal Segmental Glomerulosclerosis

2020 ◽  
Author(s):  
Shahrzad Ossareh ◽  
Mansoureh Yahyaei ◽  
Mojgan Asgari ◽  
Hanri Afghahi
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Predictive Model ◽  
Focal Segmental Glomerulosclerosis ◽  
Interstitial Fibrosis ◽  
Cox Model ◽  
Tubular Atrophy ◽  
Model Interaction ◽  
Segmental Glomerulosclerosis ◽  
Adjusted Model

Abstract Background: Focal segmental glomerulosclerosis (FSGS) is one of the important causes of end stage kidney disease (ESKD). We evaluated the risk factors of progression of primary FSGS to chronic kidney disease (CKD) or ESKD with a predictive model including clinical and histological predictors. Methods: 201 patients with primary FSGS (59% male, mean age: 38±15 years), were studied. Time-dependent Cox model and C statistics were used for the predictive model. Interaction and correlation between independent variables were estimated. Results: During 55±27 months of follow-up, 82 patients (41%) developed CKD (46) or ESKD (36) patients. In adjusted model, 1 unit of higher serum creatinine (SCr) at baseline (HR:1.39, 95%CI: 1.15-1.70) and 1% increase in glomeruli with segmental glomerulosclerosis (SGS) (HR: 1.03, 95% CI: 1.02-1.04) or interstitial fibrosis/tubular atrophy (IF/TA) (HR: 1.03, 95% CI: 1.01-1.05) increased the risk of CKD/ESKD. In adjusted model, higher baseline proteinuria and collapsing variant were not associated with risk of CKD/ESKD. By adding SGS and IF/TA scores to baseline SCr in the model, discrimination by C statistics was 0.83 (95%CI: 0.77-0.90) for prediction of CKD/ESKD . Median renal survival was 3.1 years (95% CI: 2.2-4.1 years) in patients with highest risks score (baseline eGFR<25 ml/min/1.73 m 2 + IF/TA/SGS> 50%), and 8.1 years (95% CI: 7.7-8.6 years).in those with lowest score (baseline eGFR>75 ml/min/1.73 m 2 + IF/TA/SGS <5%). Conclusion: In primary FSGS, higher baseline SCr, increased SGS and IF/TA were the predictors for CKD/ESKD. Baseline proteinuria did not predict the risk of CKD/ESKD. Collapsing variant did not increase the risk of CKD/ESKD after adjustment for IF/TA score. These findings indicated the importance of baseline GFR and the degree of chronicity at biopsy as predictors of kidney outcome .

scholarly journals Focal Segmental Glomerulosclerosis, Risk Factors for End Stage Kidney Disease, and Response to Immunosuppression

Kidney360 ◽  
2020 ◽  
pp. 10.34067/KID.0006172020
Author(s):  
Benjamin M. Forster ◽  
Robert Nee ◽  
Dustin J. Little ◽  
Peter J. Greasley ◽  
James B. Hughes ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kidney Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
Renal Survival ◽  
End Stage Kidney Disease ◽  
Interstitial Inflammation ◽  
Segmental Glomerulosclerosis ◽  
End Stage

Background: Focal segmental glomerulosclerosis (FSGS) is a heterogeneic glomerular disease. Risk factors for end- stage kidney disease (ESKD) and impact of immunosuppression treatment (IST) has varied in previously published cohorts. These cohorts were limited by relatively small case numbers, short follow up, lack of racial/ethnic diversity, a mix of adult and pediatric patients, lack of RAAS inhibition, or lack of subgroup analysis of IST. Methods: We compared demographics, clinical characteristics, histopathology and IST to long term renal survival in a large, ethnically diverse, adult cohort of 338 biopsy-proven FSGS cases with long term follow up in the era of RAAS inhibition using data from the United States Department of Defense health care network. Results: Multivariate analysis showed that nephrotic range proteinuria (NRP), estimated glomerular filtration rate <60 ml/min/1.73m2, hypoalbuminemia, interstitial fibrosis and tubular atrophy, and interstitial inflammation at diagnosis as well as the absence of remission were all associated with worse long term renal survival. IgM, C3, and a combination of IgM/C3 immunofluorescence staining were not associated with reduced renal survival. IST was not associated with improved renal survival in the whole cohort, or in a subgroup with NRP. However, IST was associated with better renal survival in a subgroup of FSGS cases with both NRP and hypoalbuminemia and hypoalbuminemia alone. Conclusion: Our study suggests that IST should be reserved for FSGS patients with nephrotic syndrome. It also introduces interstitial inflammation as a potential risk factor for ESKD and does not support the proposed pathogenicity of IgM and complement activation.

scholarly journals Disease causing mutations in inverted formin 2 regulate its binding to G-actin, F-actin capping protein (CapZ α-1) and profilin 2

2016 ◽  
Vol 36 (1) ◽  
Author(s):  
Ruth Rollason ◽  
Matthew Wherlock ◽  
Jenny A. Heath ◽  
Kate J. Heesom ◽  
Moin A. Saleem ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Kidney Disease ◽  
Capping Protein ◽  
Segmental Glomerulosclerosis ◽  
Actin Capping Protein ◽  
End Stage ◽  
Actin Capping

Mutations in inverted formin 2 (INF2) cause focal segmental glomerulosclerosis (FSGS) a major cause of end-stage kidney disease. In the present study, we show that disease associated mutations reduce INF2 auto-inhibition and cause increased binding to monomeric G-actin, profilin 2 and the F-actin capping protein, CapZ α-1.

scholarly journals Clinical and laboratory features that differentiate familial from sporadic focal segmental glomerulosclerosis in adolescents and adults

2021 ◽  
Vol 6 (1) ◽  
pp. 1-5
Author(s):  
Maria Goretti Polito ◽  
Michelle Tiveron Passos ◽  
Danilo Euclides Fernandes ◽  
Gianna Mastroianni-Kirsztajn
Keyword(s):  
Serum Albumin ◽  
Focal Segmental Glomerulosclerosis ◽  
Steroid Resistance ◽  
End Stage Kidney Disease ◽  
Steroid Resistant ◽  
Best Management ◽  
Segmental Glomerulosclerosis ◽  
Laboratory Features ◽  
Adolescents And Adults ◽  
End Stage

Background: Focal segmental glomerulosclerosis (FSGS) is an important cause of end-stage kidney disease in children and adults. Although most cases are sporadic (s), familial (f) presentation is also described. The purpose of the present study was to establish clinical and laboratory profiles of fFSGS vs. primary sFSGS, contributing to the distinguishing diagnosis in clinical practice and best management, in particular when mutation analysis is not available. Methods: Demographic, clinical and laboratorial parameters were studied in 124 patients 12 years and older with FSGS, subdivided in sFSGS (n=89) and fFSGS (n=35). Results: General and clinical features were similar, as well as serum creatinine at disease presentation. Proteinuria levels were more frequently ≥ 3g/day in sFSGS (63.8%) than in fFSGS (44%, p=0.080), and serum albumin levels were < 3.0 g/dL in 45.8% and 20%, respectively (p=0.046). The groups were statistically different regarding steroid resistance, corresponding to 60% in sFSGS and 100% in fFSGS (p=0.001). Conclusions: The studied groups were clinically similar, except that proteinuria tended to be higher (nephrotic range) and serum albumin was lower in sFSGS vs. fFSGS. In addition, all treated fFSGS patients were steroid resistant. At presentation it is important to characterize if the patient has fFSGS, that will contribute to further disease management, and disease history will be the first clue for such differential diagnosis.

scholarly journals Chronic kidney disease: a review of proteomic and metabolomic approaches to membranous glomerulonephritis, focal segmental glomerulosclerosis, and IgA nephropathy biomarkers

2019 ◽  
Vol 17 (1) ◽  
Author(s):  
Amir Taherkhani ◽  
Reyhaneh Farrokhi Yekta ◽  
Maede Mohseni ◽  
Massoud Saidijam ◽  
Afsaneh Arefi Oskouie
Keyword(s):  
Chronic Kidney Disease ◽  
Kidney Disease ◽  
Iga Nephropathy ◽  
Focal Segmental Glomerulosclerosis ◽  
Membranous Glomerulonephritis ◽  
Renal Diseases ◽  
Segmental Glomerulosclerosis ◽  
Therapeutic Procedures ◽  
Definition Of ◽  
End Stage

AbstractChronic Kidney Disease (CKD) is a global health problem annually affecting millions of people around the world. It is a comprehensive syndrome, and various factors may contribute to its occurrence. In this study, it was attempted to provide an accurate definition of chronic kidney disease; followed by focusing and discussing on molecular pathogenesis, novel diagnosis approaches based on biomarkers, recent effective antigens and new therapeutic procedures related to high-risk chronic kidney disease such as membranous glomerulonephritis, focal segmental glomerulosclerosis, and IgA nephropathy, which may lead to end-stage renal diseases. Additionally, a considerable number of metabolites and proteins that have previously been discovered and recommended as potential biomarkers of various CKDs using ‘-omics-’ technologies, proteomics, and metabolomics were reviewed.

scholarly journals Sex and kidney ACE2 expression in primary focal segmental glomerulosclerosis: A NEPTUNE study

PLoS ONE ◽  
2021 ◽  
Vol 16 (6) ◽  
pp. e0252758
Author(s):  
Nicholas A. Maksimowski ◽  
James W. Scholey ◽  
Vanessa R. Williams ◽  
Keyword(s):  
Kidney Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
Current Knowledge ◽  
Interstitial Fibrosis ◽  
Linear Regression Analysis ◽  
Renin Angiotensin System ◽  
Multiple Linear Regression Analysis ◽  
Tubular Atrophy ◽  
Kidney Fibrosis ◽  
Segmental Glomerulosclerosis

Background Angiotensin-converting enzyme 2 (ACE2) has been implicated in the pathogenesis of experimental kidney disease. ACE2 is on the X chromosome, and in mice, deletion of ACE2 leads to the development of focal segmental glomerulosclerosis (FSGS). The relationship between sex and renal ACE2 expression in humans with kidney disease is a gap in current knowledge. Methods We studied renal tubulointerstitial microarray data and clinical variables from subjects with FSGS enrolled in the Nephrotic Syndrome Study Network (NEPTUNE) study. We compared relationships between ACE2 expression and age, estimated glomerular filtration rate (eGFR), urinary albumin to creatinine ratio (UACR), interstitial fibrosis, tubular atrophy, and genes implicated in inflammation and fibrosis in male and female subjects. Results ACE2 mRNA expression was lower in the tubulointerstitium of males compared to females (P = 0.0026). Multiple linear regression analysis showed that ACE2 expression was related to sex and eGFR but not to age or treatment with renin angiotensin system blockade. ACE2 expression is also related to interstitial fibrosis, and tubular atrophy, in males but not in females. Genes involved in inflammation (CCL2 and TNF) correlated with ACE2 expression in males (TNF: r = -0.65, P < 0.0001; CCL2: r = -0.60, P < 0.0001) but not in females. TGFB1, a gene implicated in fibrosis correlated with ACE2 in both sexes. Conclusions Sex is an important determinant of ACE2 expression in the tubulointerstitium of the kidney in FSGS. Sex also influences the relationships between ACE2, kidney fibrosis, and expression of genes involved in kidney inflammation.

Resveratrol Attenuates Adriamycin-Induced Focal Segmental Glomerulosclerosis through C3aR/C5aR- Sphingosine Kinase 1 Pathway

Pharmacology ◽  
2017 ◽  
Vol 100 (5-6) ◽  
pp. 253-260 ◽  
Author(s):  
Guoyong Liu ◽  
Qiang Wang ◽  
Yan Shi ◽  
Xiaofei Peng ◽  
Hong Liu ◽  
...  
Keyword(s):  
Body Weight ◽  
Focal Segmental Glomerulosclerosis ◽  
Interstitial Fibrosis ◽  
Sphingosine Kinase ◽  
Sphingosine Kinase 1 ◽  
Segmental Glomerulosclerosis ◽  
Adriamycin Nephropathy ◽  
Type Plasminogen Activator ◽  
Stage Renal Disease ◽  
End Stage

Background/Aim: Focal segmental glomerulosclerosis (FSGS) typically presents with nephrotic range proteinuria, which could eventually develop into end-stage renal disease. Resveratrol (RSV) is a natural polyphenol compound, which has been reported to suppress inflammatory response and renal interstitial fibrosis. This study is aimed at evaluating the renoprotective effect of RSV treatment on adriamycin-induced FSGS. Methods: In Balb/c mice, adriamycin nephropathy was induced by adriamycin (10 mg/kg body weight, diluted in normal saline) via a tail vein on day 0. Then the mice were treated with RSV (40 mg/kg body weight) once daily by oral gavage, again starting on the day of adriamycin injection and continued for 6 weeks. At 6 weeks, the mice were sacrificed; kidneys and blood samples were collected for further analysis. Results: When treated with adriamycin, the expressions of C3aR, C5aR, sphingosine kinase 1 (Sphk1), and soluble urokinase-type plasminogen activator receptor (suPAR) were upregulated, while RSV treatment could inhibit the expressions of C3aR, C5aR, Sphk1, and suPAR, eventually leading to anti-inflammatory and anti-fibrosis conditions. Conclusion: RSV attenuates adriamycin-induced FSGS through C3aR/C5aR-Sphk1 pathway.

scholarly journals Prognostic Factors of Renal Outcomes after Heart Transplantation: A Nationwide Retrospective Study

2021 ◽  
Vol 10 (21) ◽  
pp. 5110
Author(s):  
Junseok Jeon ◽  
Hyejeong Park ◽  
Youngha Kim ◽  
Danbee Kang ◽  
Jung Eun Lee ◽  
...  
Keyword(s):  
Cohort Study ◽  
Kidney Disease ◽  
Heart Transplantation ◽  
Retrospective Cohort ◽  
Perioperative Period ◽  
Poor Survival ◽  
End Stage Kidney Disease ◽  
Renal Outcomes ◽  
End Stage ◽  
Adjusted Model

Renal dysfunction after heart transplantation (HT) is associated with poor survival. We investigated the predictive factors of renal outcomes after HT using nationwide cohort data. In this retrospective cohort study using the Health Insurance Review and Assessment database of Korea, 654 patients who received HT between 2008 and 2016 and survived until discharge after HT were analyzed. The median (interquartile range) age was 52 (40–60) years, and 68.1% were male. Perioperative renal replacement therapy (RRT) was performed in 27.8% of patients. During 2.8 years of median followup, end-stage kidney disease (ESKD) developed in 12 patients (1.8%). In a fully adjusted model, RRT > 3 weeks, the use of inotropes/vasopressors and non-use of ACEi/ARB were associated with ESKD. Preexisting renal disease tended to be associated with ESKD. Among the 561 patients without preexisting CKD, 104 (18.5%) developed chronic kidney disease (CKD). Age, extracorporeal membrane oxygenation, and RRT were associated with the development of CKD after HT. Our nationwide cohort study demonstrated that perioperative RRT was a predictor of poor renal outcomes after HT. These results suggest that an active renoprotective strategy is required during the perioperative period.

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