Focal Segmental Glomerulosclerosis: Genetics, Mechanism, and Therapies

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Focal Segmental Glomerulosclerosis: Genetics, Mechanism, and Therapies

1Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria 2Pediatric Nephrology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany 3Department of Microbiology and Immunology, KU Leuven and Department of Nephrology, UZ Leuven, 3000 Leuven, Belgium 4Department of Pediatric Nephrology, Severance Childr...

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Focal segmental glomerulosclerosis: molecular genetics and targeted therapies

Recent advances show that human focal segmental glomerulosclerosis (FSGS) is a primary podocytopathy caused by podocyte-specific gene mutations including NPHS1, NPHS2, WT-1, LAMB2, CD2AP, TRPC6, ACTN4 and INF2. This review focuses on genes discovered in the investigation of complex FSGS pathomechanisms that may have implications for the current FSGS classification scheme. It also recounts recen...

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Focal Segmental Glomerulosclerosis.

Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease worldwide. The presumed etiology of primary FSGS is a plasma factor with responsiveness to immunosuppressive therapy and a risk of recurrence after kidney transplant-important disease characteristics. In contrast, adaptive FSGS is associated with excessive nephron workload due to increased body size, reduced nephron ...

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Idiopathic focal segmental glomerulosclerosis.

Idiopathic focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that essentially represents a form of chronic, progressive renal fibrosis for which there is no discernible cause. Often presenting with or eventually manifesting the nephrotic syndrome, this disease is increasing in incidence in both children and adults. Therapy continues to be a challenge, although some patie...

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Collapsing Focal Segmental Glomerulosclerosis

Background and objectives: Treatment with IFN is rarely associated with nephrotic syndrome and renal biopsy findings of minimal-change disease or FSGS. Design, setting, participants, & measurements: We report 11 cases of collapsing FSGS that developed during treatment with IFN and improved after discontinuation of therapy. Results: The cohort consists of seven women and four men with a mean age...

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ژورنال

عنوان ژورنال: BioMed Research International

سال: 2016

ISSN: 2314-6133,2314-6141

DOI: 10.1155/2016/9643785