Hematuria pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Adnan Ezici, M.D [2]

Overview

Pathophysiology

Pathogenesis

It is understood that glomerular hematuria is caused by either the dysfunction or damage of the glomerular filtration barrier(GFB).^[1]

Major components of GFB include:
- Endothelial surface layer (composed of glycosaminoglycans)
- Endothelial cells
- Glomerular basement membrane (GBM)
- Slit diaphragms
- Subpodocyte space

The pathophsiologic mechanism of glomerular hematuria might be further classified into 6 subtype depends on the primary and histopathologic localization.^[1]

Injuries of the glomerular endothelial cell and surface layer
Primary and secondary GBM disorders
Diseases that can cause mesangial deposition
Diseases that can cause subendothelial and subepithelial deposition
Podocyte-associated disorders
Miscellaneous

Genetics

Molecular defects involved in the pathogenesis of glomerular hematuria include:^[1]

Diseases with structural GBM damage

In the pathogenesis of alport syndrome:
- COL4A5 (X linked)
- COL4A3/COL4A4 (autosomal recessive)
In the pathogenesis of thin basement membrane disease:
- COL4A3/COL4A4
In the pathogenesis of hereditary angiopathy, nephropathy, aneurysms, and muscle cramps syndrome:
- COL4A1

Diseases with structural podocyte damage

In the pathogenesis of MYH9-related disorder:
- Non muscle myosin IIA heavy chain

Storage disorders

In the pathogenesis of fibronectin glomerulonephritis:
- Fibronectin
In the pathogenesis of fibrillary glomerulonephritis:
- 10-30 nm fibrils
In the pathogenesis of fabry’s disease:
- lysosomal storage
In the pathogenesis of immunotactoid glomerulonephritis:
- Fibrils that are > 30 nm

Autoimmune disorders

In the pathogenesis of ANCA (antineutrophil cytoplasmic antibodies):
- Antibodies against endothelium
In the pathogenesis of anti-GBM:
- Antibodies against COL4

Complement mediated disorders

In the pathogenesis of C3 glomerulopathy:
- Alternative pathway

Infectious (endocapillary) diseases

In the pathogenesis of IgA nephritis:
- Galactose-deficient IgA1

Gross Pathology

Microscopic Pathology

References

↑ ^1.0 ^1.1 ^1.2 Yuste C, Gutierrez E, Sevillano AM, Rubio-Navarro A, Amaro-Villalobos JM, Ortiz A, Egido J, Praga M, Moreno JA (May 2015). "Pathogenesis of glomerular haematuria". World J Nephrol. 4 (2): 185–95. doi:10.5527/wjn.v4.i2.185. PMC 4419128. PMID 25949932.

[pmid25949932-1] 1.0 ^1.1 ^1.2 Yuste C, Gutierrez E, Sevillano AM, Rubio-Navarro A, Amaro-Villalobos JM, Ortiz A, Egido J, Praga M, Moreno JA (May 2015). "Pathogenesis of glomerular haematuria". World J Nephrol. 4 (2): 185–95. doi:10.5527/wjn.v4.i2.185. PMC 4419128. PMID 25949932.

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