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Thin glomerular basement membrane

MedGen UID:
478451
Concept ID:
C3276821
Finding
Synonym: Thinning of the glomerular basement membrane
 
HPO: HP:0012577

Definition

Decreased thickness of the glomerular basement membrane (GBM), measured from endothelial to visceral epithelial plasma membrane and mainly attributable to a decrease in thickness of the lamina densa, generally to an overall thickness more than 2 standard deviations less than that of the normal mean GBM thickness for health age- and sex matched individuals. May be focal or diffuse, although the term thin GBMs generally implies thinning of over 50% of GBMs. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThin glomerular basement membrane

Conditions with this feature

Benign familial hematuria
MedGen UID:
66039
Concept ID:
C0241908
Disease or Syndrome
Benign familial hematuria (BFH) is an autosomal dominant condition manifest as nonprogressive isolated microscopic hematuria that does not result in renal failure. It is characterized pathologically by thinning of the glomerular basement membrane (GBM), and can be considered the mildest end of the spectrum of renal diseases due to type IV collagen defects of the basement membrane. The most severe end of the spectrum is represented by Alport syndrome (301050; 203780, 104200), which results in end-stage renal failure and may be associated with hearing loss and ocular anomalies (review by Lemmink et al. (1996)). Genetic Heterogeneity of Benign Familial Hematuria See also benign familial hematuria-2 (BFH2; 620320), caused by mutation in the COL4A3 gene (120070).
Nephrotic syndrome, type 8
MedGen UID:
815283
Concept ID:
C3808953
Disease or Syndrome
Any nephrotic syndrome in which the cause of the disease is a mutation in the ARHGDIA gene.
Alport syndrome 3b, autosomal recessive
MedGen UID:
1848447
Concept ID:
C5882699
Disease or Syndrome
Autosomal recessive Alport syndrome-3B (ATS3B) is a progressive hematuric glomerulonephritis characterized by glomerular basement membrane abnormalities. Sensorineural hearing loss and ocular manifestations may be present (summary by Boye et al., 1998). For a general phenotypic description of Alport syndrome, see the X-linked dominant form (ATS1; 301050).

Professional guidelines

PubMed

Kashtan CE
Am J Kidney Dis 2021 Feb;77(2):272-279. Epub 2020 Jul 22 doi: 10.1053/j.ajkd.2020.03.026. PMID: 32712016
Li Y, Wang Y, He Q, Dang X, Cao Y, Wu X, Mo S, He X, Yi Z
Mol Med Rep 2018 Jan;17(1):1513-1526. Epub 2017 Nov 10 doi: 10.3892/mmr.2017.8023. PMID: 29138824Free PMC Article

Recent clinical studies

Therapy

Yuen LK, Lai WM, Lau SC, Tong PC, Tse KC, Chiu MC
Hong Kong Med J 2008 Oct;14(5):348-55. PMID: 18840904
Haas M
Arch Pathol Lab Med 2006 May;130(5):699-706. doi: 10.5858/2006-130-699-TGBMNI. PMID: 16683888
Hebert LA, Betts JA, Sedmak DD, Cosio FG, Bay WH, Carlton S
Kidney Int 1996 Jan;49(1):168-73. doi: 10.1038/ki.1996.23. PMID: 8770964

Prognosis

Deltas C, Papagregoriou G, Louka SF, Malatras A, Flinter F, Gale DP, Gear S, Gross O, Hoefele J, Lennon R, Miner JH, Renieri A, Savige J, Turner AN
Genes (Basel) 2023 Aug 25;14(9) doi: 10.3390/genes14091686. PMID: 37761826Free PMC Article
Kfoury H, Arafah M
Ultrastruct Pathol 2017 Jan-Feb;41(1):51-54. Epub 2016 Dec 28 doi: 10.1080/01913123.2016.1258021. PMID: 28029267
Haas M
Arch Pathol Lab Med 2009 Feb;133(2):224-32. doi: 10.5858/133.2.224. PMID: 19195966
Gandhi S, Kalantar-Zadeh K, Don BR
South Med J 2002 Jul;95(7):768-71. PMID: 12144088
Kashtan CE
J Am Soc Nephrol 1998 Sep;9(9):1736-50. doi: 10.1681/ASN.V991736. PMID: 9727383

Clinical prediction guides

Hirabayashi Y, Katayama K, Mori M, Matsuo H, Fujimoto M, Joh K, Murata T, Ito M, Dohi K
Genes (Basel) 2022 Oct 2;13(10) doi: 10.3390/genes13101779. PMID: 36292665Free PMC Article
Kfoury H, Arafah M
Ultrastruct Pathol 2017 Jan-Feb;41(1):51-54. Epub 2016 Dec 28 doi: 10.1080/01913123.2016.1258021. PMID: 28029267
Zurawski J, Salwa-Zurawska W, Woźniak A, Bortkiewicz E, Maciejewski J, Idasiak-Piechocka I, Urbański B
Pol J Pathol 2009;60(1):35-42. PMID: 19670702
Frascà GM, Onetti-Muda A, Mari F, Longo I, Scala E, Pescucci C, Roccatello D, Alpa M, Coppo R, Li Volti G, Feriozzi S, Bergesio F, Schena FP, Renieri A; Italian Renal Immunopathology Group
Nephrol Dial Transplant 2005 Mar;20(3):545-51. Epub 2004 Dec 23 doi: 10.1093/ndt/gfh617. PMID: 15618242
Frascá GM, Onetti-Muda A, Renieri A
J Nephrol 2000 Jan-Feb;13(1):15-9. PMID: 10720210

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