Renal amyloidosis pathophysiology
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Renal amyloidosis Microchapters |
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Treatment |
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Template:Renal amyloidosis On the Web |
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Risk calculators and risk factors for Renal amyloidosis pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
Overview
The kidney is the most involved organ in systemic amyloiosis. Suggested mechanisms of renal involvement include abnormal protein production or hereditary mutation.
Pathophysiology
Pathogenesis
- In systemic amyloidosis (AL/AH/AHL is much more common than AA) kidney is the most frequently involved organ.[1]
- In renal amyloidosis, the mechanisms of amyloidogenesis may include:[2]
- Abnormal protein production
- Overproduction wild-type proteins
- Decreased excretion of wild-type proteins
- Hereditary mutation
- In multiple myeloma, the cast nephropathy in distal tubules of nephrons results in renal impairment and deposition of AL amyloid protein in glomeruli can cause massive fibrillar involvement.[3]
Genetics
Hereditary amyloidosis due to amyloidogenic mutations:[4]
- Transthyretin (TTR) (most common inherited mutation)
- Fibrinogen
- Apolipoprotein A1
- Apolipoprotein A2
- Lysozyme
- Gelsolin genes
Associated Conditions
Gross Pathology
On gross pathology, amyloid looks "waxy" or "lardaceous" [7]
Microscopic Pathology
Microscopic Pathology of all types of amyloid after Congo red dye staining include: [2]
- Orange-red appearance by normal light microscopy
- Apple-green birefringence upon polarized light
For more general information about amyloidosis, click here.
References
- ↑ Said SM, Sethi S, Valeri AM, Leung N, Cornell LD, Fidler ME, Herrera Hernandez L, Vrana JA, Theis JD, Quint PS, Dogan A, Nasr SH (September 2013). "Renal amyloidosis: origin and clinicopathologic correlations of 474 recent cases". Clin J Am Soc Nephrol. 8 (9): 1515–23. doi:10.2215/CJN.10491012. PMC 3805078. PMID 23704299.
- ↑ 2.0 2.1 Khalighi MA, Dean Wallace W, Palma-Diaz MF (April 2014). "Amyloid nephropathy". Clin Kidney J. 7 (2): 97–106. doi:10.1093/ckj/sfu021. PMC 4377792. PMID 25852856.
- ↑ Hajra A, Bandyopadhyay D (2016). "An interesting case of renal amyloidosis". Indian J Nephrol. 26 (6): 467–469. doi:10.4103/0971-4065.177143. PMC 5131391. PMID 27942184.
- ↑ Mahmood S, Palladini G, Sanchorawala V, Wechalekar A (February 2014). "Update on treatment of light chain amyloidosis". Haematologica. 99 (2): 209–21. doi:10.3324/haematol.2013.087619. PMC 3912950. PMID 24497558.
- ↑ Ghiso J, Frangione B (December 2002). "Amyloidosis and Alzheimer's disease". Adv. Drug Deliv. Rev. 54 (12): 1539–51. PMID 12453671.
- ↑ Head E, Lott IT (April 2004). "Down syndrome and beta-amyloid deposition". Curr. Opin. Neurol. 17 (2): 95–100. PMID 15021233.
- ↑ Kyle RA (September 2001). "Amyloidosis: a convoluted story". Br. J. Haematol. 114 (3): 529–38. PMID 11552976.