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C4 nephritic factor positivity

MedGen UID:
1814398
•Concept ID:
C5676834
•
Finding
HPO: HP:0034257

Definition

The presence of autoantibodies (immunoglobulins) in the blood circulation that recognize C4b2a, the C3 convertase of the classical pathway of complement. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVC4 nephritic factor positivity

Recent clinical studies

Etiology

C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.
Garam N, Prohászka Z, Szilágyi Á, Aigner C, Schmidt A, Gaggl M, Sunder-Plassmann G, Bajcsi D, Brunner J, Dumfarth A, Cejka D, Flaschberger S, Flögelova H, Haris Á, Hartmann Á, Heilos A, Mueller T, Rusai K, Arbeiter K, Hofer J, Jakab D, Sinkó M, Szigeti E, Bereczki C, Janko V, Kelen K, Reusz GS, Szabó AJ, Klenk N, Kóbor K, Kojc N, Knechtelsdorfer M, Laganovic M, Lungu AC, Meglic A, Rus R, Kersnik-Levart T, Macioniene E, Miglinas M, Pawłowska A, Stompór T, Podracka L, Rudnicki M, Mayer G, Romana Rysava, Reiterova J, Saraga M, Tomáš Seeman, Zieg J, Sládková E, Szabó T, Capitanescu A, Stancu S, Tisljar M, Galesic K, Tislér A, Vainumäe I, Windpessl M, Zaoral T, Zlatanova G, Csuka D
Orphanet J Rare Dis 2019 Nov 8;14(1):247. doi: 10.1186/s13023-019-1237-8. PMID: 31703608Free PMC Article
C4 Nephritic Factors in C3 Glomerulopathy: A Case Series.
Zhang Y, Meyer NC, Fervenza FC, Lau W, Keenan A, Cara-Fuentes G, Shao D, Akber A, Fremeaux-Bacchi V, Sethi S, Nester CM, Smith RJH
Am J Kidney Dis 2017 Dec;70(6):834-843. Epub 2017 Aug 24 doi: 10.1053/j.ajkd.2017.07.004. PMID: 28838767Free PMC Article

Diagnosis

C4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.
Garam N, Prohászka Z, Szilágyi Á, Aigner C, Schmidt A, Gaggl M, Sunder-Plassmann G, Bajcsi D, Brunner J, Dumfarth A, Cejka D, Flaschberger S, Flögelova H, Haris Á, Hartmann Á, Heilos A, Mueller T, Rusai K, Arbeiter K, Hofer J, Jakab D, Sinkó M, Szigeti E, Bereczki C, Janko V, Kelen K, Reusz GS, Szabó AJ, Klenk N, Kóbor K, Kojc N, Knechtelsdorfer M, Laganovic M, Lungu AC, Meglic A, Rus R, Kersnik-Levart T, Macioniene E, Miglinas M, Pawłowska A, Stompór T, Podracka L, Rudnicki M, Mayer G, Romana Rysava, Reiterova J, Saraga M, Tomáš Seeman, Zieg J, Sládková E, Szabó T, Capitanescu A, Stancu S, Tisljar M, Galesic K, Tislér A, Vainumäe I, Windpessl M, Zaoral T, Zlatanova G, Csuka D
Orphanet J Rare Dis 2019 Nov 8;14(1):247. doi: 10.1186/s13023-019-1237-8. PMID: 31703608Free PMC Article
C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic.
Ravindran A, Fervenza FC, Smith RJH, De Vriese AS, Sethi S
Mayo Clin Proc 2018 Aug;93(8):991-1008. doi: 10.1016/j.mayocp.2018.05.019. PMID: 30077216Free PMC Article
Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN).
Ohi H, Yasugi T
Clin Exp Immunol 1994 Feb;95(2):316-21. doi: 10.1111/j.1365-2249.1994.tb06530.x. PMID: 8306508Free PMC Article

Therapy

C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic.
Ravindran A, Fervenza FC, Smith RJH, De Vriese AS, Sethi S
Mayo Clin Proc 2018 Aug;93(8):991-1008. doi: 10.1016/j.mayocp.2018.05.019. PMID: 30077216Free PMC Article

Prognosis

Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN).
Ohi H, Yasugi T
Clin Exp Immunol 1994 Feb;95(2):316-21. doi: 10.1111/j.1365-2249.1994.tb06530.x. PMID: 8306508Free PMC Article

Clinical prediction guides

Occurrence of C3 nephritic factor and C4 nephritic factor in membranoproliferative glomerulonephritis (MPGN).
Ohi H, Yasugi T
Clin Exp Immunol 1994 Feb;95(2):316-21. doi: 10.1111/j.1365-2249.1994.tb06530.x. PMID: 8306508Free PMC Article

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