Focal segmental glomerulosclerosis: pathogenesis and treatment

Benchimol, Corinne

doi:10.1097/00008480-200304000-00006

Cited by 40 publications

(34 citation statements)

References 119 publications

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“…FSGS is characterized by proteinuria at the early stage and by glomerular sclerosis at the later stages. Haemodynamic problems and oxidative stress contribute to the proteinuria by enhancement of glomerular filtration rate and damage of podocytes respectively, while disturbed metabolism of ECM proteins and dysregulation of sclerotic factors lead to the glomerular sclerosis [1,2].…”

Section: Discussionmentioning

confidence: 99%

“…Since sclerosis of glomeruli is the key determinant of FSGS [1,2], the proteins that appeared in the urine before the onset of glomerular sclerosis (day 7) have higher diagnostic values as early biomarkers than those appearing after the onset (days 7-20). These potential early biomarkers are highlighted in Table 1.…”

Section: Proteins With Potential As Early Diagnostic Biomarkers Of Fsgsmentioning

confidence: 99%

“…It is caused by a wide range of factors, including environmental toxins, genetic factors, infectious agents, haemodynamic problems, or other types of nephritis [1]. Clinically, FSGS is classified as a type of nephrotic syndrome, which is characterized by massive proteinuria without substantial nephritic features.…”

Section: Introductionmentioning

confidence: 99%

“…Clinically, FSGS is classified as a type of nephrotic syndrome, which is characterized by massive proteinuria without substantial nephritic features. Microscopically, the first glomerular cell to be affected in FSGS is the podocyte (glomerular visceral epithelial cells) that could be damaged by the pathogenic factors, leading to breakdown of the renal filtration barrier, and thus leakage of blood and tissue proteins into the urine [1][2][3]. So far, diagnosis of FSGS still relies on biopsy.…”

Section: Introductionmentioning

confidence: 99%

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Urinary proteome and potential biomarkers associated with serial pathogenesis steps of focal segmental glomerulosclerosis

Shui

Huang

et al. 2007

Nephrology Dialysis Transplantation

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Background. Focal segmental glomerulosclerosis (FSGS) is a chronic nephropathy showing characteristic glomerular sclerosis. So far, the diagnosis and prognosis of FSGS rely mainly on the invasive biopsy. Searching for potential FSGS-associated urinary biomarkers representing pre-sclerotic and serial sclerotic stages of FSGS could be helpful to the non-invasive diagnosis and prognosis of FSGS. Methods. In the present study, we used a 2D gel-based proteomic approach to identify urinary proteins at pre-sclerotic and different sclerotic stages of an FSGS mouse model in order to find FSGS-related urinary proteins. The FSGS mouse model was established in Balb/c mice by a single injection of adriamycin, and disease severity was monitored by renal biological parameters and histopathological features. Urine was collected on days 0, 4, 7, 11, 15 and 20, and subjected to two-dimensional electrophoresis (2-DE) analysis. Proteins were identified by matrix-assisted laser desorption ionization/time of flight mass spectrometry (MALDI-TOF MS) and a protein database search. Some of the identified proteins were confirmed by western blot analysis. Results. We identified 37 urinary proteins showing characteristic patterns of dynamic changes along the disease course of FSGS. Early urinary proteins appearing before glomerular scleoris were noticed. Importantly, 11 urine proteins are novel to FSGS and have known functions highly associated with different pathogenetic steps of the disease, including haemodynamic disturbance, podocyte apoptosis, ECMprotein deposition and glomerular sclerosis. Conclusions. Some urinary proteins appearing earlier than glomerular sclerosis could serve as potential early diagnostic biomarkers. The proteins with the pathogenic roles could serve as potential non-invasive prognostic markers of FSGS, and give an insight into pathogenic mechanisms of this sclerosis disease.

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Section: Discussionmentioning

confidence: 99%

Section: Proteins With Potential As Early Diagnostic Biomarkers Of Fsgsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Urinary proteome and potential biomarkers associated with serial pathogenesis steps of focal segmental glomerulosclerosis

Shui

Huang

et al. 2007

Nephrology Dialysis Transplantation

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“…[1][2][3] It can occur as a primary disorder, consequent to genetic mutations in specific podocyte proteins, secondary to a wide range of medical problems, or as a consequence of adaptive changes to reduced kidney mass. 4,5 Intensive research for .50 years has focused mainly on genetic, immunologic, and systemic causes of FSGS but the cause of primary disease remains elusive.…”

mentioning

confidence: 99%

Circulating suPAR in Two Cohorts of Primary FSGS

Wei¹,

Trachtman

Li³

et al. 2012

Journal of the American Society of Nephrology

202

183

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Overexpression of soluble urokinase receptor (suPAR) causes pathology in animal models similar to primary FSGS, and one recent study demonstrated elevated levels of serum suPAR in patients with the disease. Here, we analyzed circulating suPAR levels in two cohorts of children and adults with biopsyproven primary FSGS: 70 patients from the North America-based FSGS clinical trial (CT) and 94 patients from PodoNet, the Europe-based consortium studying steroid-resistant nephrotic syndrome. Circulating suPAR levels were elevated in 84.3% and 55.3% of patients with FSGS patients in the CT and PodoNet cohorts, respectively, compared with 6% of controls (P,0.0001); inflammation did not account for this difference. Multiple regression analysis suggested that lower suPAR levels associated with higher estimated GFR, male sex, and treatment with mycophenolate mofetil. In the CT cohort, there was a positive association between the relative reduction of suPAR after 26 weeks of treatment and reduction of proteinuria, with higher odds for complete remission (P=0.04). In the PodoNet cohort, patients with an NPHS2 mutation had higher suPAR levels than those without a mutation. In conclusion, suPAR levels are elevated in geographically and ethnically diverse patients with FSGS and do not reflect a nonspecific proinflammatory milieu. The associations between a change in circulating suPAR with different therapeutic regimens and with remission support the role of suPAR in the pathogenesis of FSGS.

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Therapeutic Plasma Exchange

McLeod

2009

Rossi's Principles of Transfusion Medicine

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Focal segmental glomerulosclerosis: pathogenesis and treatment

Cited by 40 publications

References 119 publications

Urinary proteome and potential biomarkers associated with serial pathogenesis steps of focal segmental glomerulosclerosis

Urinary proteome and potential biomarkers associated with serial pathogenesis steps of focal segmental glomerulosclerosis

Circulating suPAR in Two Cohorts of Primary FSGS

Therapeutic Plasma Exchange

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