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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

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
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
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

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
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
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
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
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

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
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
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
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
Han MH, Kim YJ
Biomed Res Int 2016;2016:9375753. Epub 2016 May 9 doi: 10.1155/2016/9375753. PMID: 27247945Free PMC Article

Therapy

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
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
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
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
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

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
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
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
Dettmar AK, Oh J
Biomed Res Int 2016;2016:7351964. Epub 2016 May 17 doi: 10.1155/2016/7351964. PMID: 27294131Free PMC Article
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

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
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
Krendel M, Leh S, Garone ME, Edwards-Richards A, Lin JJ, Brackman D, Knappskog P, Mikhailov A
Pediatr Nephrol 2023 Feb;38(2):439-449. Epub 2022 Jun 20 doi: 10.1007/s00467-022-05634-x. PMID: 35723736Free PMC Article
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
Pollak MR, Genovese G, Friedman DJ
Curr Opin Nephrol Hypertens 2012 Mar;21(2):179-82. doi: 10.1097/MNH.0b013e32835012ab. PMID: 22257798

Supplemental Content

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