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Focal Segmental Glomerulosclerosis Perihilar Variant

MedGen UID:
889682
•Concept ID:
C4054944
•
Disease or Syndrome

Definition

A variant of FSGS characterized by scarring of the glomerulus where at least 50% of the scars are adjacent to the hilum and must have hyalinosis; this excludes cellular, 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 Perihilar Variant

Professional guidelines

PubMed

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
A spectrum of morphologic lesions of focal segmental glomerulosclerosis by Columbia criteria in human immunodeficiency virus infection.
Meehan SM, Kim L, Chang A
Virchows Arch 2012 Apr;460(4):429-35. Epub 2012 Mar 3 doi: 10.1007/s00428-012-1213-3. PMID: 22388441

Recent clinical studies

Etiology

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
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
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
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

Diagnosis

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 lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases.
Mungan S, Turkmen E, Aydin MC, Saglam AE, Baydar DE
Ren Fail 2015 Jun;37(5):858-65. Epub 2015 Apr 10 doi: 10.3109/0886022X.2015.1033635. PMID: 25857429
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

Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases.
Mungan S, Turkmen E, Aydin MC, Saglam AE, Baydar DE
Ren Fail 2015 Jun;37(5):858-65. Epub 2015 Apr 10 doi: 10.3109/0886022X.2015.1033635. PMID: 25857429
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
Clinical features and outcomes of focal segmental glomerulosclerosis pathologic variants in Korean adult patients.
Kwon YE, Han SH, Kie JH, An SY, Kim YL, Park KS, Nam KH, Leem AY, Oh HJ, Park JT, Chang TI, Kang EW, Kang SW, Choi KH, Lim BJ, Jeong HJ, Yoo TH
BMC Nephrol 2014 Mar 25;15:52. doi: 10.1186/1471-2369-15-52. PMID: 24666814Free PMC Article
Histological heterogeneity of glomerular segmental lesions in focal segmental glomerulosclerosis.
Taneda S, Honda K, Uchida K, Nitta K, Yumura W, Oda H, Nagata M
Int Urol Nephrol 2012 Feb;44(1):183-96. Epub 2011 Mar 19 doi: 10.1007/s11255-011-9932-y. PMID: 21424374Free PMC Article
Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience.
El-Refaey AM, Bakr A, Hammad A, Elmougy A, El-Houseeny F, Abdelrahman A, Sarhan A
Pediatr Nephrol 2010 Jul;25(7):1369-73. Epub 2010 Mar 2 doi: 10.1007/s00467-010-1448-7. PMID: 20195645

Prognosis

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
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
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
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

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 of cases with asymptomatic proteinuria not manifesting nephrotic syndrome at onset: a single-center retrospective study.
Watanabe Y, Fujinaga S, Sakuraya K, Ikeda H, Nozu K
Clin Exp Nephrol 2022 May;26(5):453-459. Epub 2022 Feb 3 doi: 10.1007/s10157-021-02174-w. PMID: 35113255
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
Tip lesion variant of primary focal and segmental glomerulosclerosis: clinicopathological analysis of 20 cases.
Mungan S, Turkmen E, Aydin MC, Saglam AE, Baydar DE
Ren Fail 2015 Jun;37(5):858-65. Epub 2015 Apr 10 doi: 10.3109/0886022X.2015.1033635. PMID: 25857429
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

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