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Primary focal segmental glomerulosclerosis

MedGen UID:
313617
•Concept ID:
C1709661
•
Disease or Syndrome
Synonyms: Idiopathic Focal Segmental Glomerulosclerosis; Primary Focal Segmental Glomerulosclerosis; primary focal segmental glomerulosclerosis
 
Monarch Initiative: MONDO:0100324

Definition

Focal scarring of the glomerulus, which may result in isolated proteinuria or nephrotic syndrome, which affects only part of the glomerulus and only some of the glomeruli. The condition is considered primary in cases for which no underlying cause has been identified. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary focal segmental glomerulosclerosis

Professional guidelines

PubMed

Rituximab treatment of adults with primary focal segmental glomerulosclerosis.
Wang L, Yu L, Wang Y, Guo Y, Zhai Z, Tang L
Sci Rep 2023 Apr 25;13(1):6740. doi: 10.1038/s41598-023-33678-y. PMID: 37185370Free PMC Article
Immune-mediated entities of (primary) focal segmental glomerulosclerosis.
Braun F, Homeyer I, Alachkar N, Huber TB
Cell Tissue Res 2021 Aug;385(2):423-434. Epub 2021 Apr 27 doi: 10.1007/s00441-021-03454-3. PMID: 33907872Free PMC Article
Immunosuppressive treatment in primary focal segmental glomerulosclerosis.
Xu S, Xu G, Zeng L
J Nephrol 2012 Sep-Oct;25(5):626-35. doi: 10.5301/jn.5000092. PMID: 22344540

Recent clinical studies

Etiology

Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis.
Boussetta A, Ben Njima K, Jellouli M, Gargah T
Tunis Med 2023 Mar 5;101(3):373-378. PMID: 38263915Free PMC Article
Patients with primary focal segmental glomerulosclerosis with detectable urinary CD80 are more similar to patients with minimal change disease in clinicopathological features.
Gong X, Huang J, Zhang Y, Wang F, Wang X, Meng L, Cheng X, Liu G, Cui Z, Zhao M
Ren Fail 2023;45(2):2279642. Epub 2023 Nov 9 doi: 10.1080/0886022X.2023.2279642. PMID: 37942512Free PMC Article
Current understanding of the molecular mechanisms of circulating permeability factor in focal segmental glomerulosclerosis.
Salfi G, Casiraghi F, Remuzzi G
Front Immunol 2023;14:1247606. Epub 2023 Sep 19 doi: 10.3389/fimmu.2023.1247606. PMID: 37795085Free PMC Article
Rituximab treatment of adults with primary focal segmental glomerulosclerosis.
Wang L, Yu L, Wang Y, Guo Y, Zhai Z, Tang L
Sci Rep 2023 Apr 25;13(1):6740. doi: 10.1038/s41598-023-33678-y. PMID: 37185370Free PMC Article
Primary Focal Segmental Glomerulosclerosis Recurrence After Pediatric Renal Transplantation.
Baskın E, Avcı B, Gülleroğlu K, Akdur A, Moray G, Haberal M
Exp Clin Transplant 2022 Mar;20(Suppl 1):55-60. doi: 10.6002/ect.MESOT2021.O24. PMID: 35384808

Diagnosis

Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis.
Boussetta A, Ben Njima K, Jellouli M, Gargah T
Tunis Med 2023 Mar 5;101(3):373-378. PMID: 38263915Free PMC Article
Rituximab treatment of adults with primary focal segmental glomerulosclerosis.
Wang L, Yu L, Wang Y, Guo Y, Zhai Z, Tang L
Sci Rep 2023 Apr 25;13(1):6740. doi: 10.1038/s41598-023-33678-y. PMID: 37185370Free PMC Article
Primary Focal Segmental Glomerulosclerosis Recurrence After Pediatric Renal Transplantation.
Baskın E, Avcı B, Gülleroğlu K, Akdur A, Moray G, Haberal M
Exp Clin Transplant 2022 Mar;20(Suppl 1):55-60. doi: 10.6002/ect.MESOT2021.O24. PMID: 35384808
Lupus Podocytopathy: An Overview.
Oliva-Damaso N, Payan J, Oliva-Damaso E, Pereda T, Bomback AS
Adv Chronic Kidney Dis 2019 Sep;26(5):369-375. doi: 10.1053/j.ackd.2019.08.011. PMID: 31733721Free PMC Article
Primary focal segmental glomerulosclerosis.
Korbet SM
J Am Soc Nephrol 1998 Jul;9(7):1333-40. doi: 10.1681/ASN.V971333. PMID: 9644647

Therapy

Patients with primary focal segmental glomerulosclerosis with detectable urinary CD80 are more similar to patients with minimal change disease in clinicopathological features.
Gong X, Huang J, Zhang Y, Wang F, Wang X, Meng L, Cheng X, Liu G, Cui Z, Zhao M
Ren Fail 2023;45(2):2279642. Epub 2023 Nov 9 doi: 10.1080/0886022X.2023.2279642. PMID: 37942512Free PMC Article
Current understanding of the molecular mechanisms of circulating permeability factor in focal segmental glomerulosclerosis.
Salfi G, Casiraghi F, Remuzzi G
Front Immunol 2023;14:1247606. Epub 2023 Sep 19 doi: 10.3389/fimmu.2023.1247606. PMID: 37795085Free PMC Article
Rituximab treatment of adults with primary focal segmental glomerulosclerosis.
Wang L, Yu L, Wang Y, Guo Y, Zhai Z, Tang L
Sci Rep 2023 Apr 25;13(1):6740. doi: 10.1038/s41598-023-33678-y. PMID: 37185370Free PMC Article
Primary Focal Segmental Glomerulosclerosis Recurrence After Pediatric Renal Transplantation.
Baskın E, Avcı B, Gülleroğlu K, Akdur A, Moray G, Haberal M
Exp Clin Transplant 2022 Mar;20(Suppl 1):55-60. doi: 10.6002/ect.MESOT2021.O24. PMID: 35384808
Immunotherapy in renal diseases.
Kher A, Kher V
Med Clin North Am 2012 May;96(3):545-64, x. Epub 2012 May 22 doi: 10.1016/j.mcna.2012.04.007. PMID: 22703855

Prognosis

Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis.
Boussetta A, Ben Njima K, Jellouli M, Gargah T
Tunis Med 2023 Mar 5;101(3):373-378. PMID: 38263915Free PMC Article
Lupus Podocytopathy: An Overview.
Oliva-Damaso N, Payan J, Oliva-Damaso E, Pereda T, Bomback AS
Adv Chronic Kidney Dis 2019 Sep;26(5):369-375. doi: 10.1053/j.ackd.2019.08.011. PMID: 31733721Free PMC Article
Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children.
Han KH, Kim SH
Biomed Res Int 2016;2016:3053706. Epub 2016 Apr 18 doi: 10.1155/2016/3053706. PMID: 27195285Free PMC Article
Immunotherapy in renal diseases.
Kher A, Kher V
Med Clin North Am 2012 May;96(3):545-64, x. Epub 2012 May 22 doi: 10.1016/j.mcna.2012.04.007. PMID: 22703855
Primary focal segmental glomerulosclerosis.
Korbet SM
J Am Soc Nephrol 1998 Jul;9(7):1333-40. doi: 10.1681/ASN.V971333. PMID: 9644647

Clinical prediction guides

Anti-nephrin antibodies in recurrence of focal segmental glomerulosclerosis: closer to discovering the Holy Grail?
Vivarelli M, Colucci M
Kidney Int 2024 Mar;105(3):440-442. doi: 10.1016/j.kint.2023.12.015. PMID: 38388144
Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis.
Boussetta A, Ben Njima K, Jellouli M, Gargah T
Tunis Med 2023 Mar 5;101(3):373-378. PMID: 38263915Free PMC Article
Predictors of long-term outcomes in pediatric focal segmental glomerulosclerosis.
Peng Y, Gao C, Xu C, Wu H, Wang M, Wang R, Wei Y, Li X, Ju T, Xia Z, Zhang P
J Nephrol 2023 Jul;36(6):1581-1590. Epub 2023 Apr 7 doi: 10.1007/s40620-023-01631-x. PMID: 37027095
Identification of Novel Genetic Risk Factors for Focal Segmental Glomerulosclerosis in Children: Results From the Chronic Kidney Disease in Children (CKiD) Cohort.
Durand A, Winkler CA, Vince N, Douillard V, Geffard E, Binns-Roemer E, Ng DK, Gourraud PA, Reidy K, Warady B, Furth S, Kopp JB, Kaskel FJ, Limou S
Am J Kidney Dis 2023 Jun;81(6):635-646.e1. Epub 2023 Jan 6 doi: 10.1053/j.ajkd.2022.11.003. PMID: 36623684
Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children.
Han KH, Kim SH
Biomed Res Int 2016;2016:3053706. Epub 2016 Apr 18 doi: 10.1155/2016/3053706. PMID: 27195285Free PMC Article

Recent systematic reviews

Efficacy of extracorporeal plasma therapy for adult native kidney patients with Primary FSGS: a Systematic review.
Miao J, Krisanapan P, Tangpanithandee S, Thongprayoon C, Mao MA, Cheungpasitporn W
Ren Fail 2023 Dec;45(1):2176694. doi: 10.1080/0886022X.2023.2176694. PMID: 36762994Free PMC Article
Treatment Strategies of Adult Primary Focal Segmental Glomerulosclerosis: A Systematic Review Focusing on the Last Two Decades.
Beer A, Mayer G, Kronbichler A
Biomed Res Int 2016;2016:4192578. Epub 2016 Apr 7 doi: 10.1155/2016/4192578. PMID: 27144166Free PMC Article
Mycophenolate mofetil for primary focal segmental glomerulosclerosis: systematic review.
Lau EW, Ma PH, Wu X, Chung VC, Wong SY
Ren Fail 2013 Jul;35(6):914-29. Epub 2013 May 28 doi: 10.3109/0886022X.2013.794687. PMID: 23751146

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