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Non-immunoglobulin-mediated membranoproliferative glomerulonephritis

MedGen UID:
1672497
Concept ID:
C4087273
Disease or Syndrome
Synonyms: C3 glomerulopathy; non-Ig-mediated membranoproliferative glomerulonephritis; Non-Ig-mediated membranoproliferative glomerulonephritis; Non-Ig-mediated MPGN; non-Ig-mediated MPGN; non-immunoglobulin-mediated membranoproliferative glomerulonephritis; Non-immunoglobulin-mediated MPGN; non-immunoglobulin-mediated MPGN
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
 
Monarch Initiative: MONDO:0018013
Orphanet: ORPHA329918

Definition

A form of primary membranoproliferative glomerulonephritis characterized by the presence in renal biopsy samples of a glomerulonephritis with sole (or at least dominant) glomerular immunofluorescence staining for C3. Non-specific alterations or proliferative patterns with C3-dominant glomerular staining are also possible. Based upon electron microscopic findings, C3 glomerulopathy (C3G) may be further classified as C3 glomerulonephritis (C3GN) or Dense deposit disease (DDD). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Non-immunoglobulin-mediated membranoproliferative glomerulonephritis

Recent clinical studies

Etiology

Michels MAHM, van de Kar NCAJ, van Kraaij SAW, Sarlea SA, Gracchi V, Engels FAPT, Dorresteijn EM, van der Deure J, Duineveld C, Wetzels JFM, van den Heuvel LPWJ, Volokhina EB
Front Immunol 2021;12:715704. Epub 2021 Aug 11 doi: 10.3389/fimmu.2021.715704. PMID: 34456924Free PMC Article

Diagnosis

Michels MAHM, van de Kar NCAJ, van Kraaij SAW, Sarlea SA, Gracchi V, Engels FAPT, Dorresteijn EM, van der Deure J, Duineveld C, Wetzels JFM, van den Heuvel LPWJ, Volokhina EB
Front Immunol 2021;12:715704. Epub 2021 Aug 11 doi: 10.3389/fimmu.2021.715704. PMID: 34456924Free PMC Article
Lionaki S, Gakiopoulou H, Boletis JN
APMIS 2016 Sep;124(9):725-35. Epub 2016 Jun 30 doi: 10.1111/apm.12566. PMID: 27356907
Bomback AS, Appel GB
Nat Rev Nephrol 2012 Nov;8(11):634-42. Epub 2012 Oct 2 doi: 10.1038/nrneph.2012.213. PMID: 23026947

Therapy

Lionaki S, Gakiopoulou H, Boletis JN
APMIS 2016 Sep;124(9):725-35. Epub 2016 Jun 30 doi: 10.1111/apm.12566. PMID: 27356907

Prognosis

Michels MAHM, van de Kar NCAJ, van Kraaij SAW, Sarlea SA, Gracchi V, Engels FAPT, Dorresteijn EM, van der Deure J, Duineveld C, Wetzels JFM, van den Heuvel LPWJ, Volokhina EB
Front Immunol 2021;12:715704. Epub 2021 Aug 11 doi: 10.3389/fimmu.2021.715704. PMID: 34456924Free PMC Article
Bomback AS, Appel GB
Nat Rev Nephrol 2012 Nov;8(11):634-42. Epub 2012 Oct 2 doi: 10.1038/nrneph.2012.213. PMID: 23026947

Clinical prediction guides

Michels MAHM, van de Kar NCAJ, van Kraaij SAW, Sarlea SA, Gracchi V, Engels FAPT, Dorresteijn EM, van der Deure J, Duineveld C, Wetzels JFM, van den Heuvel LPWJ, Volokhina EB
Front Immunol 2021;12:715704. Epub 2021 Aug 11 doi: 10.3389/fimmu.2021.715704. PMID: 34456924Free PMC Article

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