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Nephrotic Syndrome - CFH Associated

MedGen UID:
889445
•Concept ID:
C4054395
•
Disease or Syndrome

Definition

Nephrotic syndrome attributed to mutation(s) in the CFH gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNephrotic Syndrome - CFH Associated

Recent clinical studies

Etiology

Association between anti-complement factor H antibodies and renal outcome in primary membranous nephropathy.
Kagaya Y, Hayashi N, Fujimoto K, Adachi H, Furuichi K, Yokoyama H
Clin Nephrol 2021 Aug;96(2):82-89. doi: 10.5414/CN110479. PMID: 34032209
C3 nephritic factor associated with C3 glomerulopathy in children.
Nicolas C, Vuiblet V, Baudouin V, Macher MA, Vrillon I, Biebuyck-Gouge N, Dehennault M, Gié S, Morin D, Nivet H, Nobili F, Ulinski T, Ranchin B, Marinozzi MC, Ngo S, Frémeaux-Bacchi V, Pietrement C
Pediatr Nephrol 2014 Jan;29(1):85-94. Epub 2013 Sep 26 doi: 10.1007/s00467-013-2605-6. PMID: 24068526
Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature.
Manenti L, Gnappi E, Vaglio A, Allegri L, Noris M, Bresin E, Pilato FP, Valoti E, Pasquali S, Buzio C
Nephrol Dial Transplant 2013 Sep;28(9):2246-59. Epub 2013 Jun 19 doi: 10.1093/ndt/gft220. PMID: 23787552
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association?
Bridoux F, Desport E, Frémeaux-Bacchi V, Chong CF, Gombert JM, Lacombe C, Quellard N, Touchard G
Clin J Am Soc Nephrol 2011 Sep;6(9):2165-74. Epub 2011 Jul 22 doi: 10.2215/CJN.06180710. PMID: 21784830Free PMC Article

Diagnosis

Anti-complement factor I antibody associated atypical hemolytic uremic syndrome - A new insight for future perspective!
Govindarajan S, Rawat A, Ramachandran R, Hans R, Dawman L, Tiewsoh K
Immunobiology 2020 Sep;225(5):152000. Epub 2020 Aug 11 doi: 10.1016/j.imbio.2020.152000. PMID: 32962820
Monoclonal immunoglobulin mediates complement activation in monoclonal gammopathy associated-C3 glomerulonephritis.
Li LL, Li ZY, Wang SX, Yu XJ, Tan Y, Wang Y, Yu F, Zhao MH
BMC Nephrol 2019 Dec 10;20(1):459. doi: 10.1186/s12882-019-1640-3. PMID: 31823738Free PMC Article
Novel factor H mutation associated with familial membranoproliferative glomerulonephritis type I.
Alfandary H, Davidovits M
Pediatr Nephrol 2015 Dec;30(12):2129-34. Epub 2015 Aug 20 doi: 10.1007/s00467-015-3166-7. PMID: 26289290
A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl.
Besbas N, Gulhan B, Gucer S, Korkmaz E, Ozaltin F
J Nephrol 2014 Aug;27(4):457-60. Epub 2013 Dec 5 doi: 10.1007/s40620-013-0008-1. PMID: 24536001
Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature.
Manenti L, Gnappi E, Vaglio A, Allegri L, Noris M, Bresin E, Pilato FP, Valoti E, Pasquali S, Buzio C
Nephrol Dial Transplant 2013 Sep;28(9):2246-59. Epub 2013 Jun 19 doi: 10.1093/ndt/gft220. PMID: 23787552

Therapy

Novel factor H mutation associated with familial membranoproliferative glomerulonephritis type I.
Alfandary H, Davidovits M
Pediatr Nephrol 2015 Dec;30(12):2129-34. Epub 2015 Aug 20 doi: 10.1007/s00467-015-3166-7. PMID: 26289290
Atypical HUS due to factor H antibodies in an adult patient successfully treated with eculizumab.
Green H, Harari E, Davidovits M, Blickstein D, Grossman A, Gafter U, Gafter-Gvili A
Ren Fail 2014 Aug;36(7):1119-21. Epub 2014 May 15 doi: 10.3109/0886022X.2014.917574. PMID: 24828571
A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl.
Besbas N, Gulhan B, Gucer S, Korkmaz E, Ozaltin F
J Nephrol 2014 Aug;27(4):457-60. Epub 2013 Dec 5 doi: 10.1007/s40620-013-0008-1. PMID: 24536001
Pulse cyclophosphamide therapy and clinical remission in atypical hemolytic uremic syndrome with anti-complement factor H autoantibodies.
Boyer O, Balzamo E, Charbit M, Biebuyck-Gougé N, Salomon R, Dragon-Durey MA, Frémeaux-Bacchi V, Niaudet P
Am J Kidney Dis 2010 May;55(5):923-7. Epub 2010 Mar 3 doi: 10.1053/j.ajkd.2009.12.026. PMID: 20202729
Late-onset thrombocytic microangiopathy caused by cblC disease: association with a factor H mutation.
Guigonis V, Frémeaux-Bacchi V, Giraudier S, Favier R, Borderie D, Massy Z, Mougenot B, Rosenblatt DS, Deschênes G
Am J Kidney Dis 2005 Mar;45(3):588-95. doi: 10.1053/j.ajkd.2004.12.004. PMID: 15754282

Prognosis

Association between anti-complement factor H antibodies and renal outcome in primary membranous nephropathy.
Kagaya Y, Hayashi N, Fujimoto K, Adachi H, Furuichi K, Yokoyama H
Clin Nephrol 2021 Aug;96(2):82-89. doi: 10.5414/CN110479. PMID: 34032209
Atypical haemolytic uraemic syndrome with underlying glomerulopathies. A case series and a review of the literature.
Manenti L, Gnappi E, Vaglio A, Allegri L, Noris M, Bresin E, Pilato FP, Valoti E, Pasquali S, Buzio C
Nephrol Dial Transplant 2013 Sep;28(9):2246-59. Epub 2013 Jun 19 doi: 10.1093/ndt/gft220. PMID: 23787552
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association?
Bridoux F, Desport E, Frémeaux-Bacchi V, Chong CF, Gombert JM, Lacombe C, Quellard N, Touchard G
Clin J Am Soc Nephrol 2011 Sep;6(9):2165-74. Epub 2011 Jul 22 doi: 10.2215/CJN.06180710. PMID: 21784830Free PMC Article

Clinical prediction guides

Association between anti-complement factor H antibodies and renal outcome in primary membranous nephropathy.
Kagaya Y, Hayashi N, Fujimoto K, Adachi H, Furuichi K, Yokoyama H
Clin Nephrol 2021 Aug;96(2):82-89. doi: 10.5414/CN110479. PMID: 34032209
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association?
Bridoux F, Desport E, Frémeaux-Bacchi V, Chong CF, Gombert JM, Lacombe C, Quellard N, Touchard G
Clin J Am Soc Nephrol 2011 Sep;6(9):2165-74. Epub 2011 Jul 22 doi: 10.2215/CJN.06180710. PMID: 21784830Free PMC Article

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