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 pathophysiologic mechanism of glomerular hematuria might be further classified into 6 subtype depending 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
Microscopic pathology might differ depending on the underlying etiology of hematuria.
- On microscopic histopathological analysis,
- Diffuse thinning of the glomerular basement membrane (GBM) is characteristic finding of thin basal membrane disease[2]
- Lobular appearance with massive fibronectin deposits and the absence of immunoglobulin/complement deposition are characteristic findings of fibronectin nephropathy[3]
- Randomly aligned fibrillar deposits that measure 12-24 nm is characteristic finding of fibrillary nephropathy[4][5]
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.
- ↑ Foster K, Markowitz GS, D'Agati VD (May 2005). "Pathology of thin basement membrane nephropathy". Semin Nephrol. 25 (3): 149–58. doi:10.1016/j.semnephrol.2005.01.006. PMID 15880325.
- ↑ Lusco MA, Chen YP, Cheng H, Dong HR, Najafian B, Alpers CE, Fogo AB (November 2017). "AJKD Atlas of Renal Pathology: Fibronectin Glomerulopathy". Am J Kidney Dis. 70 (5): e21–e22. doi:10.1053/j.ajkd.2017.09.001. PMID 29055354.
- ↑ Klomjit, Nattawat; Alexander, Mariam Priya; Zand, Ladan (2020). "Fibrillary Glomerulonephritis and DnaJ Homolog Subfamily B Member 9 (DNAJB9)". Kidney360. 1 (9): 1002–1013. doi:10.34067/KID.0002532020. ISSN 2641-7650.
- ↑ Rosenstock JL, Markowitz GS (July 2019). "Fibrillary Glomerulonephritis: An Update". Kidney Int Rep. 4 (7): 917–922. doi:10.1016/j.ekir.2019.04.013. PMC 6611949 Check
|pmc=value (help). PMID 31317113.