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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
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
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
Focal segmental glomerulosclerosis after renal transplantation.
Shimizu A, Higo S, Fujita E, Mii A, Kaneko T
Clin Transplant 2011 Jul;25 Suppl 23:6-14. doi: 10.1111/j.1399-0012.2011.01452.x. PMID: 21623907

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
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
Focal segmental glomerulosclerosis after renal transplantation.
Shimizu A, Higo S, Fujita E, Mii A, Kaneko T
Clin Transplant 2011 Jul;25 Suppl 23:6-14. doi: 10.1111/j.1399-0012.2011.01452.x. PMID: 21623907

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

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
A Clinicopathologic Study of Children with Idiopathic Focal Segmental Glomerulosclerosis.
Nickavar A, Rahbar M
Fetal Pediatr Pathol 2016;35(3):159-66. Epub 2016 Apr 7 doi: 10.3109/15513815.2016.1153177. PMID: 27054662
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
Morphological features of primary focal and segmental glomerulosclerosis.
Noël LH
Nephrol Dial Transplant 1999;14 Suppl 3:53-7. doi: 10.1093/ndt/14.suppl_3.53. PMID: 10382983

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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