Category Ranking
98%
Total Visits
921
Avg Visit Duration
2 minutes
Citations
20
Article Abstract
Focal segmental glomerulosclerosis (FSGS) is a histological pattern of injury that derives from various pathological processes that affect podocytes, resulting in loss of selectivity of the glomerular filtration membrane, proteinuria and the development of renal failure that progresses to end-stage kidney disease in a significant number of patients. The classification proposed by the 2021 KDIGO guidelines divides FSGS into four categories: primary, secondary, genetic, and FSGS of undetermined cause, thus facilitating its diagnosis and management. Genetic causes of FSGS present significant clinical variability, complicating their identification. Genetic testing is crucial to identify FSGS of genetic cause. The prevalence of genetic FSGS is significant in children and considerable in adults, highlighting the importance of early diagnosis to avoid unnecessary treatments and facilitate genetic counselling. Massive sequencing techniques have revolutionized genetic diagnosis, allowing the identification of more than 60 genes responsible for podocyte damage. This document proposes clinical recommendations for carrying out genetic studies in adults with FSGS, highlighting the need for a correct classification for adequate therapeutic planning and improvement of results in clinical trials.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.nefroe.2025.02.001 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Antagonistic interaction between Notch3 signaling and CREB/KLF15 pathway in regulating the phenotypic alterations of glomerular parietal epithelial cells in adriamycin-induced nephropathy.
Exp Cell Res
August 2025
Department of Pathology, School of Basic Medical Sciences, Fudan University, 131 Dongan Road, 200032, Shanghai, China. Electronic address:
Parietal epithelial cells (PECs) that line the Bowman's capsule are considered progenitor cells for podocytes, which exhibit limited regeneration capacity following injury. Notch3 receptor has been found to be co-expressed in both podocytes and PECs in focal segmental glomerulosclerosis (FSGS), suggesting a distinct regulatory role in this context. Our previous research indicated that Notch3 signaling is significantly negatively correlated with the cAMP-PKA-CREB-KLF15 pathway in adriamycin (ADR)-injured podocytes.
View Article and Find Full Text PDFUncommon Allies: Van der Knaap Syndrome and Focal Segmental Glomerulosclerosis.
J Assoc Physicians India
July 2025
Professor, Department of Medicine, Jawaharlal Institute of Postgraduate Medical Education & Research, Puducherry, India.
Background: Van der Knaap disease, or megalencephalic leukoencephalopathy with subcortical cysts (MLC), is a rare autosomal recessive leukodystrophy caused by mutations in the or genes. It is characterized by macrocephaly, developmental delays, ataxia, spasticity, seizures, progressive neurodegeneration, and subcortical cysts, particularly in individuals from consanguineous populations.
Objective: To report a unique case of a 26-year-old male with MLC who developed steroid-resistant focal segmental glomerulosclerosis (FSGS), an association not previously described.
Co-localization of IgG with nephrin in immune-mediated idiopathic nephrotic syndrome.
Clin Exp Nephrol
August 2025
Department of Pediatrics, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo, Kobe, Hyogo, 6500017, Japan.
Background: Increased serum anti-nephrin antibody titers and co-localization of nephrin and IgG in kidney tissues have been reported in minimal change disease (MCD) and post-transplant recurrent focal segmental glomerulosclerosis (FSGS). These results indicate an association of anti-nephrin antibodies with nephrotic syndrome (NS); however, the exact relationship remains unclear. Herein, we evaluated nephrin/IgG co-localization in the glomeruli of patients with various kidney diseases, including monogenic NS, to clarify the association between idiopathic nephrotic syndrome (INS) and anti-nephrin antibodies.
View Article and Find Full Text PDFControlling proteinuria in secondary focal segmental glomerulosclerosis with cyclosporine: A case report.
SAGE Open Med Case Rep
July 2025
Division of Nephrology, Hypertension, and Kidney Transplantation, Department of Medicine, University of California, Irvine, CA, USA.
Focal segmental glomerulosclerosis is a histopathological condition characterized by podocyte injury, which manifests as persistent proteinuria and progressive decline in renal function. It is classified into primary and secondary forms, with secondary focal segmental glomerulosclerosis often resulting from factors such as obesity, hypertension, or genetic mutations. The management of secondary focal segmental glomerulosclerosis remains challenging due to the lack of standardized therapeutic guidelines.
View Article and Find Full Text PDFDefining subgroups of pediatric nephrotic patients with urine proteomics.
Sci Rep
July 2025
Department of Medicine, Kidney Disease Program, University of Louisville Health Sciences Center, Room 102S Donald Baxter Research Building, Louisville, KY, 40202, USA.
The molecular pathophysiology of nephrotic syndrome remains largely elusive in pediatric patients. While most children with minimal change disease (MCD) show favorable responses to immunosuppressive therapy, those with focal segmental glomerulosclerosis (FSGS) often exhibit poorer treatment responses, with many experiencing either partial remission or no remission of proteinuria. The need for reliable glomerular disease biomarkers to predict treatment response and understand molecular pathways governing responsiveness and resistance is a critical unmet need in pediatric nephrology.
View Article and Find Full Text PDF