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Focal Segmental Glomerulosclerosis Cellular Variant

MedGen UID:
891201
•Concept ID:
C4054946
•
Disease or Syndrome

Definition

A variant of FSGS characterized by hypercellularity of the glomerulus; this excludes the tip and collapsing FSGS. (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 Cellular Variant

Professional guidelines

PubMed

Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free PMC Article
APOL1 genotype-associated morphologic changes among patients with focal segmental glomerulosclerosis.
Zee J, McNulty MT, Hodgin JB, Zhdanova O, Hingorani S, Jefferson JA, Gibson KL, Trachtman H, Fornoni A, Dell KM, Reich HN, Bagnasco S, Greenbaum LA, Lafayette RA, Gipson DS, Brown E, Kretzler M, Appel G, Sambandam KK, Tuttle KR, Chen D, Atkinson MA, Hogan MC, Kaskel FJ, Meyers KE, O'Toole J, Srivastava T, Sethna CB, Hladunewich MA, Lin JJ, Nast CC, Derebail VK, Patel J, Vento S, Holzman LB, Athavale AM, Adler SG, Lemley KV, Lieske JC, Hogan JJ, Gadegbeku CA, Fervenza FC, Wang CS, Matar RB, Singer P, Kopp JB, Barisoni L, Sampson MG
Pediatr Nephrol 2021 Sep;36(9):2747-2757. Epub 2021 Mar 1 doi: 10.1007/s00467-021-04990-4. PMID: 33646395Free PMC Article
Integration of Genetic Testing and Pathology for the Diagnosis of Adults with FSGS.
Yao T, Udwan K, John R, Rana A, Haghighi A, Xu L, Hack S, Reich HN, Hladunewich MA, Cattran DC, Paterson AD, Pei Y, Barua M
Clin J Am Soc Nephrol 2019 Feb 7;14(2):213-223. Epub 2019 Jan 15 doi: 10.2215/CJN.08750718. PMID: 30647093Free PMC Article

Recent clinical studies

Etiology

Inaxaplin for Proteinuric Kidney Disease in Persons with Two APOL1 Variants.
Egbuna O, Zimmerman B, Manos G, Fortier A, Chirieac MC, Dakin LA, Friedman DJ, Bramham K, Campbell K, Knebelmann B, Barisoni L, Falk RJ, Gipson DS, Lipkowitz MS, Ojo A, Bunnage ME, Pollak MR, Altshuler D, Chertow GM; VX19-147-101 Study Group
N Engl J Med 2023 Mar 16;388(11):969-979. doi: 10.1056/NEJMoa2202396. PMID: 36920755
Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free PMC Article
Collapsing Focal Segmental Glomerulosclerosis in Viral Infections.
Muehlig AK, Gies S, Huber TB, Braun F
Front Immunol 2021;12:800074. Epub 2022 Jan 13 doi: 10.3389/fimmu.2021.800074. PMID: 35095882Free PMC Article
APOL1 and kidney cell function.
Kumar V, Singhal PC
Am J Physiol Renal Physiol 2019 Aug 1;317(2):F463-F477. Epub 2019 Jun 26 doi: 10.1152/ajprenal.00233.2019. PMID: 31241995Free PMC Article
Obesity-related kidney disease.
Snyder S, Turner GA, Turner A
Prim Care 2014 Dec;41(4):875-93. Epub 2014 Oct 3 doi: 10.1016/j.pop.2014.08.008. PMID: 25439539

Diagnosis

Collapsing glomerulopathy: unraveling varied pathogeneses.
Smith KD, Akilesh S
Curr Opin Nephrol Hypertens 2023 May 1;32(3):213-222. Epub 2023 Feb 9 doi: 10.1097/MNH.0000000000000873. PMID: 36811644
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
Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free PMC Article
Variations of type IV collagen-encoding genes in patients with histological diagnosis of focal segmental glomerulosclerosis.
Demir E, Caliskan Y
Pediatr Nephrol 2020 Jun;35(6):927-936. Epub 2019 Jun 28 doi: 10.1007/s00467-019-04282-y. PMID: 31254113
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

Inaxaplin for Proteinuric Kidney Disease in Persons with Two APOL1 Variants.
Egbuna O, Zimmerman B, Manos G, Fortier A, Chirieac MC, Dakin LA, Friedman DJ, Bramham K, Campbell K, Knebelmann B, Barisoni L, Falk RJ, Gipson DS, Lipkowitz MS, Ojo A, Bunnage ME, Pollak MR, Altshuler D, Chertow GM; VX19-147-101 Study Group
N Engl J Med 2023 Mar 16;388(11):969-979. doi: 10.1056/NEJMoa2202396. PMID: 36920755
Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free PMC Article
Focal segmental glomerulosclerosis and concurrent glomerular microangiopathy after long-term imatinib administration.
Morita N, Ozaki T, Yokota S, Sasaki H, Watanabe M, Takahashi K, Himuro N, Ito K, Yasuno T, Miyake K, Nawata A, Nakayama T, Uesugi N, Masutani K
CEN Case Rep 2022 Feb;11(1):134-140. Epub 2021 Sep 9 doi: 10.1007/s13730-021-00622-w. PMID: 34505278Free 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 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

Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free PMC Article
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
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
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

Inaxaplin for Proteinuric Kidney Disease in Persons with Two APOL1 Variants.
Egbuna O, Zimmerman B, Manos G, Fortier A, Chirieac MC, Dakin LA, Friedman DJ, Bramham K, Campbell K, Knebelmann B, Barisoni L, Falk RJ, Gipson DS, Lipkowitz MS, Ojo A, Bunnage ME, Pollak MR, Altshuler D, Chertow GM; VX19-147-101 Study Group
N Engl J Med 2023 Mar 16;388(11):969-979. doi: 10.1056/NEJMoa2202396. PMID: 36920755
Monogenic focal segmental glomerulosclerosis: A conceptual framework for identification and management of a heterogeneous disease.
Sambharia M, Rastogi P, Thomas CP
Am J Med Genet C Semin Med Genet 2022 Sep;190(3):377-398. Epub 2022 Jul 27 doi: 10.1002/ajmg.c.31990. PMID: 35894442Free 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 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
APOL1 and kidney disease.
Pollak MR, Genovese G, Friedman DJ
Curr Opin Nephrol Hypertens 2012 Mar;21(2):179-82. doi: 10.1097/MNH.0b013e32835012ab. PMID: 22257798

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