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Focal Segmental Glomerulosclerosis Tip Lesion Variant

MedGen UID:
885369
•Concept ID:
C4054943
•
Disease or Syndrome

Definition

A variant of FSGS characterized by scarring of the glomerulus adjacent to the origin of the proximal convoluted tubule; this occurs in the absence of collapsing and perihilar 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 Tip Lesion Variant

Professional guidelines

PubMed

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

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
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
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
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
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

Complete remission of tip lesion variant focal segmental glomerulosclerosis (FSGS) with the Janus Kinase (JAK) inhibitor tofacitinib.
Sedlacek M, Pettus JR
CEN Case Rep 2022 May;11(2):225-230. Epub 2021 Nov 5 doi: 10.1007/s13730-021-00658-y. PMID: 34741283Free 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
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
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
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
Cellular focal segmental glomerulosclerosis: Clinical and pathologic features.
Stokes MB, Valeri AM, Markowitz GS, D'Agati VD
Kidney Int 2006 Nov;70(10):1783-92. Epub 2006 Oct 4 doi: 10.1038/sj.ki.5001903. PMID: 17021605

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