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Focal Segmental Glomerulosclerosis, Not Otherwise Specified

MedGen UID:
887152
•Concept ID:
C4049702
•
Disease or Syndrome

Definition

Segmental 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. Additionally, the Not Otherwise Specified classification excludes FSGS tip, perihilar, collapsing, and cellular variants. (D''Agati VD, et al. ""Pathologic Classification of Focal Segmental Glomerulosclerosis: A Working Proposal."" Am J Kidney Dis 43.2 (2004): 368-82.) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFocal Segmental Glomerulosclerosis, Not Otherwise Specified

Professional guidelines

PubMed

Clinical presentation, treatment and outcome of focal segmental glomerulosclerosis in Far North Queensland Australian adults.
Greenwood AM, Gunnarsson R, Neuen BL, Oliver K, Green SJ, Baer RA
Nephrology (Carlton) 2017 Jul;22(7):520-530. doi: 10.1111/nep.12816. PMID: 27170059

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: 38263915
Clinical, Pathological, and Genetic Characteristics in Patients with Focal Segmental Glomerulosclerosis.
Nagano C, Hara S, Yoshikawa N, Takeda A, Gotoh Y, Hamada R, Matsuoka K, Yamamoto M, Fujinaga S, Sakuraya K, Kamei K, Hamasaki Y, Oguchi H, Araki Y, Ogawa Y, Okamoto T, Ito S, Tanaka S, Kaito H, Aoto Y, Ishiko S, Rossanti R, Sakakibara N, Horinouchi T, Yamamura T, Nagase H, Iijima K, Nozu K
Kidney360 2022 Aug 25;3(8):1384-1393. Epub 2022 May 24 doi: 10.34067/KID.0000812022. PMID: 36176665Free PMC Article
Focal segmental glomerulosclerosis histologic variants and renal outcomes based on nephrotic syndrome, immunosuppression and proteinuria remission.
Kawaguchi T, Imasawa T, Kadomura M, Kitamura H, Maruyama S, Ozeki T, Katafuchi R, Oka K, Isaka Y, Yokoyama H, Sugiyama H, Sato H
Nephrol Dial Transplant 2022 Aug 22;37(9):1679-1690. doi: 10.1093/ndt/gfab267. PMID: 34499164
Focal segmental glomerulosclerosis in children.
Jellouli M, Abidi K, Askri M, Ferjani M, Naija O, Hammi Y, Goucha R, Gargah T
Tunis Med 2016 May;94(5):356-359. PMID: 27801486
Morphologic variants of focal segmental glomerulosclerosis and their significance.
Stokes MB, D'Agati VD
Adv Chronic Kidney Dis 2014 Sep;21(5):400-7. doi: 10.1053/j.ackd.2014.02.010. PMID: 25168828

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: 38263915
Clinical, Pathological, and Genetic Characteristics in Patients with Focal Segmental Glomerulosclerosis.
Nagano C, Hara S, Yoshikawa N, Takeda A, Gotoh Y, Hamada R, Matsuoka K, Yamamoto M, Fujinaga S, Sakuraya K, Kamei K, Hamasaki Y, Oguchi H, Araki Y, Ogawa Y, Okamoto T, Ito S, Tanaka S, Kaito H, Aoto Y, Ishiko S, Rossanti R, Sakakibara N, Horinouchi T, Yamamura T, Nagase H, Iijima K, Nozu K
Kidney360 2022 Aug 25;3(8):1384-1393. Epub 2022 May 24 doi: 10.34067/KID.0000812022. PMID: 36176665Free PMC Article
Practical Application of Columbia Classification for Focal Segmental Glomerulosclerosis.
Han MH, Kim YJ
Biomed Res Int 2016;2016:9375753. Epub 2016 May 9 doi: 10.1155/2016/9375753. PMID: 27247945Free PMC Article
Tip variant of focal segmental glomerulosclerosis: outcome and comparison to 'not otherwise specified' variant.
Arias LF, Franco-Alzate C, Rojas SL
Nephrol Dial Transplant 2011 Jul;26(7):2215-21. Epub 2010 Nov 10 doi: 10.1093/ndt/gfq668. PMID: 21068139
Pathologic classification of focal segmental glomerulosclerosis.
D'Agati V
Semin Nephrol 2003 Mar;23(2):117-34. doi: 10.1053/snep.2003.50012. PMID: 12704572

Therapy

Clinicopathological differences in focal segmental glomerulosclerosis depending on the accompanying pathophysiological conditions in renal allografts.
Taneda S, Honda K, Koike J, Ito N, Ishida H, Takagi T, Nagashima Y
Virchows Arch 2023 Dec;483(6):809-819. Epub 2023 Nov 18 doi: 10.1007/s00428-023-03703-6. PMID: 37980299Free PMC Article
Nephrotic syndrome with focal segmental glomerular lesions unclassified by Columbia classification; Pathology and clinical implication.
Ozeki T, Nagata M, Katsuno T, Inagaki K, Goto K, Kato S, Yasuda Y, Tsuboi N, Maruyama S
PLoS One 2021;16(1):e0244677. Epub 2021 Jan 5 doi: 10.1371/journal.pone.0244677. PMID: 33400710Free PMC Article
Utility of Columbia classification in focal segmental glomerulosclerosis: renal prognosis and treatment response among the pathological variants.
Tsuchimoto A, Matsukuma Y, Ueki K, Tanaka S, Masutani K, Nakagawa K, Mitsuiki K, Uesugi N, Katafuchi R, Tsuruya K, Nakano T, Kitazono T
Nephrol Dial Transplant 2020 Jul 1;35(7):1219-1227. doi: 10.1093/ndt/gfy374. PMID: 30649467
Focal segmental glomerulosclerosis in children.
Jellouli M, Abidi K, Askri M, Ferjani M, Naija O, Hammi Y, Goucha R, Gargah T
Tunis Med 2016 May;94(5):356-359. PMID: 27801486
Focal and segmental glomerulosclerosis: does prognosis vary with the variants?
Swarnalatha G, Ram R, Ismal KM, Vali S, Sahay M, Dakshinamurty KV
Saudi J Kidney Dis Transpl 2015 Jan;26(1):173-81. doi: 10.4103/1319-2442.148772. PMID: 25579744

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: 38263915
Focal segmental glomerulosclerosis histologic variants and renal outcomes based on nephrotic syndrome, immunosuppression and proteinuria remission.
Kawaguchi T, Imasawa T, Kadomura M, Kitamura H, Maruyama S, Ozeki T, Katafuchi R, Oka K, Isaka Y, Yokoyama H, Sugiyama H, Sato H
Nephrol Dial Transplant 2022 Aug 22;37(9):1679-1690. doi: 10.1093/ndt/gfab267. PMID: 34499164
Focal segmental glomerulosclerosis in children.
Jellouli M, Abidi K, Askri M, Ferjani M, Naija O, Hammi Y, Goucha R, Gargah T
Tunis Med 2016 May;94(5):356-359. PMID: 27801486
Practical Application of Columbia Classification for Focal Segmental Glomerulosclerosis.
Han MH, Kim YJ
Biomed Res Int 2016;2016:9375753. Epub 2016 May 9 doi: 10.1155/2016/9375753. PMID: 27247945Free PMC Article
Morphologic variants of focal segmental glomerulosclerosis and their significance.
Stokes MB, D'Agati VD
Adv Chronic Kidney Dis 2014 Sep;21(5):400-7. doi: 10.1053/j.ackd.2014.02.010. PMID: 25168828

Clinical prediction guides

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: 38263915
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
Focal segmental glomerulosclerosis histologic variants and renal outcomes based on nephrotic syndrome, immunosuppression and proteinuria remission.
Kawaguchi T, Imasawa T, Kadomura M, Kitamura H, Maruyama S, Ozeki T, Katafuchi R, Oka K, Isaka Y, Yokoyama H, Sugiyama H, Sato H
Nephrol Dial Transplant 2022 Aug 22;37(9):1679-1690. doi: 10.1093/ndt/gfab267. PMID: 34499164
Focal segmental glomerulosclerosis in children.
Jellouli M, Abidi K, Askri M, Ferjani M, Naija O, Hammi Y, Goucha R, Gargah T
Tunis Med 2016 May;94(5):356-359. PMID: 27801486
Podocyte and endothelial injury in focal segmental glomerulosclerosis: an ultrastructural analysis.
Taneda S, Honda K, Ohno M, Uchida K, Nitta K, Oda H
Virchows Arch 2015 Oct;467(4):449-58. Epub 2015 Aug 13 doi: 10.1007/s00428-015-1821-9. PMID: 26266776Free PMC Article

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