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Nephrotic Syndrome with Lesion of Focal Glomerulosclerosis

MedGen UID:
167848
•Concept ID:
C0866108
•
Disease or Syndrome

Definition

A renal functional disorder characterized by proteinuria, edema, hyperlipidemia and hypoalbuminemia. It results from damage to the renal vascular filtration apparatus. It is further characterized by scarring of some of the glomerular capillaries. Sequelae may include hypertension, atherosclerosis, infection, hypercoagulablity and renal failure. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNephrotic Syndrome with Lesion of Focal Glomerulosclerosis

Professional guidelines

PubMed

Treatment with Glucocorticoids or Calcineurin Inhibitors in Primary FSGS.
Laurin LP, Gasim AM, Poulton CJ, Hogan SL, Jennette JC, Falk RJ, Foster BJ, Nachman PH
Clin J Am Soc Nephrol 2016 Mar 7;11(3):386-94. Epub 2016 Feb 16 doi: 10.2215/CJN.07110615. PMID: 26912551Free PMC Article
Treatment of focal segmental glomerulosclerosis.
Meyrier A
Expert Opin Pharmacother 2005 Aug;6(9):1539-49. doi: 10.1517/14656566.6.9.1539. PMID: 16086641
Primary focal segmental glomerulosclerosis: clinical course, predictors of renal outcome and treatment.
Shiiki H, Dohi K
Intern Med 2000 Aug;39(8):606-11. doi: 10.2169/internalmedicine.39.606. PMID: 10939532

Recent clinical studies

Etiology

Genetic Causes of Nephrotic Syndrome and Focal and Segmental Glomerulosclerosis.
Caparali EB, De Gregorio V, Barua M
Adv Kidney Dis Health 2024 Jul;31(4):309-316. doi: 10.1053/j.akdh.2024.04.001. PMID: 39084756
Impact of focal segmental glomerulosclerosis over the past decade.
Woo KT, Chan CM, Foo M, Lim C, Choo J, Chin YM, Teng EWL, Mok I, Kwek JL, Tan HZ, Loh AHL, Wong J, Kee T, Choong HL, Tan HK, Wong KS, Tan PH, Tan CS
Clin Nephrol 2023 Mar;99(3):128-140. doi: 10.5414/CN111022. PMID: 36633377
Focal segmental glomerulosclerosis: Risk for recurrence and interventions to optimize outcomes following recurrence.
Harshman LA, Bartosh S, Engen RM
Pediatr Transplant 2022 Sep;26(6):e14307. Epub 2022 May 19 doi: 10.1111/petr.14307. PMID: 35587003
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Collapsing glomerulopathy: update.
Morales E, Alonso M, Gutiérrez E
Med Clin (Barc) 2019 May 3;152(9):361-367. Epub 2018 Dec 13 doi: 10.1016/j.medcli.2018.10.021. PMID: 30554809

Diagnosis

State of the art in childhood nephrotic syndrome: concrete discoveries and unmet needs.
Vincenti F, Angeletti A, Ghiggeri GM
Front Immunol 2023;14:1167741. Epub 2023 Jul 12 doi: 10.3389/fimmu.2023.1167741. PMID: 37503337Free PMC Article
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Glomerulonephritis Histopathological Pattern Change.
AlYousef A, AlSahow A, AlHelal B, Alqallaf A, Abdallah E, Abdellatif M, Nawar H, Elmahalawy R
BMC Nephrol 2020 May 18;21(1):186. doi: 10.1186/s12882-020-01836-3. PMID: 32423387Free 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
Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach.
De Vriese AS, Sethi S, Nath KA, Glassock RJ, Fervenza FC
J Am Soc Nephrol 2018 Mar;29(3):759-774. Epub 2018 Jan 10 doi: 10.1681/ASN.2017090958. PMID: 29321142Free PMC Article

Therapy

State of the art in childhood nephrotic syndrome: concrete discoveries and unmet needs.
Vincenti F, Angeletti A, Ghiggeri GM
Front Immunol 2023;14:1167741. Epub 2023 Jul 12 doi: 10.3389/fimmu.2023.1167741. PMID: 37503337Free PMC Article
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Difficult-to-treat idiopathic nephrotic syndrome: established drugs, open questions and future options.
Kemper MJ, Valentin L, van Husen M
Pediatr Nephrol 2018 Oct;33(10):1641-1649. Epub 2017 Sep 6 doi: 10.1007/s00467-017-3780-7. PMID: 28879428
What are we missing in the clinical trials of focal segmental glomerulosclerosis?
Zand L, Glassock RJ, De Vriese AS, Sethi S, Fervenza FC
Nephrol Dial Transplant 2017 Jan 1;32(suppl_1):i14-i21. doi: 10.1093/ndt/gfw324. PMID: 28391338
Clinical trials in minimal change disease.
Ravani P, Bertelli E, Gill S, Ghiggeri GM
Nephrol Dial Transplant 2017 Jan 1;32(suppl_1):i7-i13. doi: 10.1093/ndt/gfw235. PMID: 28391333

Prognosis

Podocytopathies.
Kopp JB, Anders HJ, Susztak K, Podestà MA, Remuzzi G, Hildebrandt F, Romagnani P
Nat Rev Dis Primers 2020 Aug 13;6(1):68. doi: 10.1038/s41572-020-0196-7. PMID: 32792490Free PMC Article
Focal Segmental Glomerulosclerosis: State-of-the-Art and Clinical Perspective.
Shabaka A, Tato Ribera A, Fernández-Juárez G
Nephron 2020;144(9):413-427. Epub 2020 Jul 28 doi: 10.1159/000508099. PMID: 32721952
Glomerulonephritis Histopathological Pattern Change.
AlYousef A, AlSahow A, AlHelal B, Alqallaf A, Abdallah E, Abdellatif M, Nawar H, Elmahalawy R
BMC Nephrol 2020 May 18;21(1):186. doi: 10.1186/s12882-020-01836-3. PMID: 32423387Free 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
Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach.
De Vriese AS, Sethi S, Nath KA, Glassock RJ, Fervenza FC
J Am Soc Nephrol 2018 Mar;29(3):759-774. Epub 2018 Jan 10 doi: 10.1681/ASN.2017090958. PMID: 29321142Free PMC Article

Clinical prediction guides

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
Clinical and Pathological Features of Renal Presentations in Polycythemia Vera.
Yang J, Yu X, Hu N, Su T
Am J Med Sci 2022 Jan;363(1):33-41. Epub 2021 Oct 21 doi: 10.1016/j.amjms.2021.10.009. PMID: 34687616
Clinical and morphologic spectrum of renal involvement in idiopathic hypereosinophilic syndrome.
Dong JH, Xu ST, Xu F, Zhou YC, Li Z, Li SJ
Clin Exp Nephrol 2021 Mar;25(3):270-278. Epub 2021 Jan 4 doi: 10.1007/s10157-020-02012-5. PMID: 33398597
Glomerulonephritis Histopathological Pattern Change.
AlYousef A, AlSahow A, AlHelal B, Alqallaf A, Abdallah E, Abdellatif M, Nawar H, Elmahalawy R
BMC Nephrol 2020 May 18;21(1):186. doi: 10.1186/s12882-020-01836-3. PMID: 32423387Free PMC Article
Defining nephrotic syndrome from an integrative genomics perspective.
Sampson MG, Hodgin JB, Kretzler M
Pediatr Nephrol 2015 Jan;30(1):51-63; quiz 59. Epub 2014 Jun 3 doi: 10.1007/s00467-014-2857-9. PMID: 24890338Free PMC Article

Recent systematic reviews

Mercury-associated nephrotic syndrome: a case report and systematic review of the literature.
Miller S, Pallan S, Gangji AS, Lukic D, Clase CM
Am J Kidney Dis 2013 Jul;62(1):135-8. Epub 2013 Apr 18 doi: 10.1053/j.ajkd.2013.02.372. PMID: 23602193

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