Membranoproliferative Glomerulonephritis, X-Linked

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Retrieved

2019-09-22

Source

Omim

Trials

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Genes

CFH, TSLP, ALB, CFB, PON1, DNASE2, C1QA, FN1, EP300, PFN1, IL1B, TGFBR2, CCL2, F5, SPARC, EDN1, MYOC, CCN2, CCR6, CDKN1B, CDK2, PRF1, ANGPT2, CXCR1, LMNB2, SOX18, PGM3, IGAN1, IL6, C3

CFH, TSLP, ALB, CFB, PON1, DNASE2, C1QA, FN1, EP300, PFN1, IL1B, TGFBR2, CCL2, F5, SPARC, EDN1, MYOC, CCN2, CCR6, CDKN1B, CDK2, PRF1, ANGPT2, CXCR1, LMNB2, SOX18, PGM3, IGAN1, IL6, C3, VWF, CFHR3, ADAMTS13, VTN, VEGFA, THY1, TLR9, CFHR5, TAGLN, AFP, MPO, RIT2, PTEN, PIK3CG, PIK3CD, PIK3CB, PIK3CA, AKT1, MMP9, IGHG3, HRAS, HLA-DRB1, HLA-DQB1, CFHR1, CSF3, CGA, RBM45

Drugs

(2S,4R)-1-(2-(3-acetyl-5-(2-methylpyrimidine-5-yl)-1H-indazol-1-yl)acetyl)-N-(6-bromopyridine-2-yl)-4-fluoropyrrolidine-2-carboxamide, 4-{(2S,4S)-4-ethoxy-1-[(5-methoxy-7-methyl-1H-indol-4-yl)methyl]piperidin-2-yl}benzoic acid-hydrogen chloride(1/1), Avacopan, PEGylated peptide inhibitor of complement C3, Recombinant human minibody against complement component C5, S3,S13-cyclo(D-tyrolsyl-L-isoleucyl-L-cysteinyl-L-valyl-1-methyl-L-tryptophyl-L-glutaminyl-L-aspartyl-L-tryptophyl-N-methyl-L-glycyl-L-alanyl-L-histidyl-L-arginyl-L-cysteinyl-N-methyl-L-isoleucinamide)

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Description

Habib et al. (1973) recognized 2 morphologic classes for the glomerular changes seen in patients with mesangiocapillary (membranoproliferative) glomerulonephritis (MPGN). Type I is characterized by double contour appearance of the capillary walls due to mesangial cell interposition, with nonargyrophilic subendothelial deposits which are finely granular on electron microscopy. Type II is characterized by linear dense deposits within the basement membrane and only rare double contours. These 2 types appear to be distinct with no conversion of one type to another on serial biopsy. Strife et al. (1977) described a third variety in which there are not only subendothelial deposits but also numerous subepithelial and intramembranous deposits, associated with replication of the lamina densa and frequently disruption of the whole basement membrane.

Inheritance

Stutchfield et al. (1986) described a kindred in which 3 half brothers, each with a different father, and a nephew of theirs had a variant of type I MCGN, which the authors interpreted as evidence of X-linked inheritance. Previous evidence for genetic factors predisposing to MCGN had been limited to the rarity of the disease in blacks (Mandalenakis et al., 1971) and the association with deficiency of various complement components.

Berry et al. (1981) observed membranoproliferative glomerulonephritis in 2 sibships, in each of which 2 children were affected. In their family 1, a boy had type III and a girl type I; in their second family, 2 brothers had type I.