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Tip variant segmental glomerulosclerosis

MedGen UID:
1779765
•Concept ID:
C5539645
•
Disease or Syndrome
Synonym: Segmental glomerulosclerosis, tip variant
 
HPO: HP:0033497

Definition

Segmental solidification of the glomerular tuft characterized by an adhesion at the tip of the glomerular tuft abutting the proximal tubular taek-off can be accompanied by increased extracellular matrix, causing glomerular capillary obliteration at the glomerular tip. Features including hyalinosis, foam cells, hypertrophy of overlying glomerular epithelial cells can also be observed. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTip variant segmental glomerulosclerosis

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
Treatment with Glucocorticoids or Calcineurin Inhibitors in Primary FSGS.
Laurin LP, Gasim AM, Poulton CJ, Hogan SL, Jennette JC, Falk RJ, Foster BJ, Nachman PH
Clin J Am Soc Nephrol 2016 Mar 7;11(3):386-94. Epub 2016 Feb 16 doi: 10.2215/CJN.07110615. PMID: 26912551Free PMC Article
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
Renal comorbidities in collapsing variant focal segmental glomerulosclerosis: more than a coincidence?
Gougeon F, Singh HK, Nickeleit V
Nephrol Dial Transplant 2022 Jan 25;37(2):311-317. doi: 10.1093/ndt/gfaa327. PMID: 33370435
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

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
Non-glomerular Tip Lesion Focal Segmental Glomerulosclerosis as a Negative Predictor in Idiopathic Membranous Nephropathy.
Wang H, Wan C, Jiang M, Zhang C
Curr Med Sci 2022 Oct;42(5):1007-1014. Epub 2022 Sep 30 doi: 10.1007/s11596-022-2628-y. PMID: 36178577
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
Renal comorbidities in collapsing variant focal segmental glomerulosclerosis: more than a coincidence?
Gougeon F, Singh HK, Nickeleit V
Nephrol Dial Transplant 2022 Jan 25;37(2):311-317. doi: 10.1093/ndt/gfaa327. PMID: 33370435
Problems with 'focal segmental glomerulosclerosis'.
Howie AJ
Pediatr Nephrol 2011 Aug;26(8):1197-205. Epub 2010 Dec 2 doi: 10.1007/s00467-010-1701-0. PMID: 21125407

Therapy

New Onset Biopsy-Proven Nephropathies after COVID Vaccination.
Fenoglio R, Lalloni S, Marchisio M, Oddone V, De Simone E, Del Vecchio G, Sciascia S, Roccatello D
Am J Nephrol 2022;53(4):325-330. Epub 2022 Mar 30 doi: 10.1159/000523962. PMID: 35354140Free 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
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

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
Non-glomerular Tip Lesion Focal Segmental Glomerulosclerosis as a Negative Predictor in Idiopathic Membranous Nephropathy.
Wang H, Wan C, Jiang M, Zhang C
Curr Med Sci 2022 Oct;42(5):1007-1014. Epub 2022 Sep 30 doi: 10.1007/s11596-022-2628-y. PMID: 36178577
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
Renal comorbidities in collapsing variant focal segmental glomerulosclerosis: more than a coincidence?
Gougeon F, Singh HK, Nickeleit V
Nephrol Dial Transplant 2022 Jan 25;37(2):311-317. doi: 10.1093/ndt/gfaa327. PMID: 33370435
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
Non-glomerular Tip Lesion Focal Segmental Glomerulosclerosis as a Negative Predictor in Idiopathic Membranous Nephropathy.
Wang H, Wan C, Jiang M, Zhang C
Curr Med Sci 2022 Oct;42(5):1007-1014. Epub 2022 Sep 30 doi: 10.1007/s11596-022-2628-y. PMID: 36178577
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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