U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Nephrotic Syndrome with Lesion of Segmental Hyalinosis

MedGen UID:
209026
•Concept ID:
C0866110
•
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 an inflammatory reaction in the filtering capillaries and the deposition of plasma components between the endothelium and glomerular basement membrane. 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 Segmental Hyalinosis

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
Treatment of the idiopathic nephrotic syndrome: regimens and outcomes in children and adults.
Tune BM, Mendoza SA
J Am Soc Nephrol 1997 May;8(5):824-32. doi: 10.1681/ASN.V85824. PMID: 9176855

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
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
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

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

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
C1q Nephropathy: The Unique Underrecognized Pathological Entity.
Devasahayam J, Erode-Singaravelu G, Bhat Z, Oliver T, Chandran A, Zeng X, Dakshinesh P, Pillai U
Anal Cell Pathol (Amst) 2015;2015:490413. Epub 2015 Nov 10 doi: 10.1155/2015/490413. PMID: 26640759Free 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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...