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Glomerulonephritis

Glomerulonephritis is a collection of disorders with different pathogeneses and histomorphologies whose common denominator is inflammation of the glomeruli, the fitration barrier of the kidney. Often glomerulonephrites are caused by immunological disturbances and therefore increasingly genetic disorders are found to be essential for its development.

Diagnosis

Basic symptoms are proteinuria and glomerular hematuria. Theres two main symptoms underly the clinical classifiaction into nephrotic and nephritic kidney disease.

Histomorphology allows a more accurate diagnosis and classification into subtypes that differ by histomorphology. Histomorphological studies also allow immunehistochenistry which comes close to a pathogenetic classification.

Most recent genetic fidings allow an even deeper pathognetic classification.

Systematic

Hereditary glomerular disease
Fibronectin glomerulopathy
Glomerulonephritis
C3 glomerulopathy
C3 glomerulonephritis
ADAMTS13
C1QA
C1QB
C1QC
C3
CD46
CFB
CFD
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
CLU
DGKE
PIGA
THBD
Dense deposit disease
ADAMTS13
C1QA
C1QB
C1QC
C3
CD46
CFB
CFD
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
CLU
DGKE
PIGA
THBD
CFHR5 Nephropathy
CFHR5
Goodpasture syndrome
COL4A3
COL4A3BP
COL4A5
Lupus erythematosus nephritis
C1QA
C1QB
C1QC
CFHR1
CFHR3
Membranoproliferative glomerulonephritis (MPGN)
ADAMTS13
C1QA
C1QB
C1QC
C3
CD46
CFB
CFD
CFH
CFHR1
CFHR2
CFHR3
CFHR4
CFHR5
CFI
CLU
CR1 deficiency
CR1
Complement component C1q deficiency
C1QA
C1QB
C1QC
Complement component C1s deficiency
C1S
DGKE
PIGA
THBD
Membranous nephropathy
HLA-DQA1
PLA2R1
Mesangioproliferative glomerulonephritis
CXCR1
Complement component C1q deficiency
C1QA
C1QB
C1QC
IgA nephropathy
CFHR1
CFHR3
CFHR5
IgA nephropathy type 1
IgA nephropathy type 2
IgA nephropathy type 3
SPRY2
Schimke Immunoosseous dysplasia
SMARCAL1
Wiskott–Aldrich syndrome
WAS
Glomerulosclerosis
Lipoprotein glomerulopathy
Myoclonus-nephropathy syndrome
Nephritic syndrome
Nephrotic syndrome

References:

1.

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2.

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Gharavi AG et al. (2000) IgA nephropathy, the most common cause of glomerulonephritis, is linked to 6q22-23.

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Hiki Y et al. (2001) Mass spectrometry proves under-O-glycosylation of glomerular IgA1 in IgA nephropathy.

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Levy M et al. (1986) H deficiency in two brothers with atypical dense intramembranous deposit disease.

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45.

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46.

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47.

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48.

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49.

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51.

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52.

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53.

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54.

Stutchfield PR et al. (1986) X-linked mesangiocapillary glomerulonephritis.

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55.

Strife CF et al. (1977) Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane.

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56.

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57.

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58.

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59.

Wyatt RJ et al. (1982) Partial H (beta 1H) deficiency and glomerulonephritis in two families.

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60.

McRae JL et al. (2001) Human factor H-related protein 5 (FHR-5). A new complement-associated protein.

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61.

Rougier N et al. (1998) Human complement factor H deficiency associated with hemolytic uremic syndrome.

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62.

OMIM.ORG article

Omim 137940 external link
63.

Orphanet article

Orphanet ID 280569 external link
64.

Wikipedia article

Wikipedia EN (Glomerulonephritis) external link
Update: Aug. 14, 2020
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