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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: 38263915Free PMC Article
FSGS and COVID-19 in Non-African American Patients.
Medina E, Rueda C, Batlle D
Kidney360 2023 May 1;4(5):687-699. doi: 10.34067/KID.0000000000000104. PMID: 37229730Free PMC Article
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
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
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

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
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
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

Therapy

Evidence from the large VALIGA cohort validates the subclassification of focal segmental glomerulosclerosis in IgA nephropathy.
Bellur SS, Troyanov S, Vorobyeva O, Coppo R, Roberts ISD; Validation in IgA Nephropathy study group
Kidney Int 2024 Jun;105(6):1279-1290. Epub 2024 Mar 28 doi: 10.1016/j.kint.2024.03.011. PMID: 38554992
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
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: 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
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
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

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: 38263915Free PMC Article
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
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

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