Renal amyloidosis pathophysiology: Difference between revisions
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{{CMG}}; {{AE}} {{SHH}} | {{CMG}}; {{AE}} {{SHH}} | ||
==Overview== | ==Overview== | ||
The kidney is the most involved organ in systemic amyloiosis. Suggested mechanisms of renal involvement include abnormal protein production or hereditary mutation. | |||
==Pathophysiology== | ==Pathophysiology== | ||
===Pathogenesis=== | === Pathogenesis === | ||
*In systemic amyloidosis (AL/AH/AHL is much more common than AA) kidney is the most frequently involved organ.<ref name="pmid23704299">{{cite journal |vauthors=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 |title=Renal amyloidosis: origin and clinicopathologic correlations of 474 recent cases |journal=Clin J Am Soc Nephrol |volume=8 |issue=9 |pages=1515–23 |date=September 2013 |pmid=23704299 |pmc=3805078 |doi=10.2215/CJN.10491012 |url=}}</ref> | *In systemic amyloidosis (AL/AH/AHL is much more common than AA) kidney is the most frequently involved organ.<ref name="pmid23704299">{{cite journal |vauthors=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 |title=Renal amyloidosis: origin and clinicopathologic correlations of 474 recent cases |journal=Clin J Am Soc Nephrol |volume=8 |issue=9 |pages=1515–23 |date=September 2013 |pmid=23704299 |pmc=3805078 |doi=10.2215/CJN.10491012 |url=}}</ref> | ||
*In renal amyloidosis, the mechanisms of amyloidogenesis may include:<ref name="pmid25852856">{{cite journal |vauthors=Khalighi MA, Dean Wallace W, Palma-Diaz MF |title=Amyloid nephropathy |journal=Clin Kidney J |volume=7 |issue=2 |pages=97–106 |date=April 2014 |pmid=25852856 |pmc=4377792 |doi=10.1093/ckj/sfu021 |url=}}</ref> | *In renal amyloidosis, the mechanisms of amyloidogenesis may include:<ref name="pmid25852856">{{cite journal |vauthors=Khalighi MA, Dean Wallace W, Palma-Diaz MF |title=Amyloid nephropathy |journal=Clin Kidney J |volume=7 |issue=2 |pages=97–106 |date=April 2014 |pmid=25852856 |pmc=4377792 |doi=10.1093/ckj/sfu021 |url=}}</ref> | ||
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**Hereditary mutation | **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.<ref name="pmid27942184">{{cite journal |vauthors=Hajra A, Bandyopadhyay D |title=An interesting case of renal amyloidosis |journal=Indian J Nephrol |volume=26 |issue=6 |pages=467–469 |date=2016 |pmid=27942184 |pmc=5131391 |doi=10.4103/0971-4065.177143 |url=}}</ref> | *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.<ref name="pmid27942184">{{cite journal |vauthors=Hajra A, Bandyopadhyay D |title=An interesting case of renal amyloidosis |journal=Indian J Nephrol |volume=26 |issue=6 |pages=467–469 |date=2016 |pmid=27942184 |pmc=5131391 |doi=10.4103/0971-4065.177143 |url=}}</ref> | ||
== Genetics == | |||
Hereditary amyloidosis due to amyloidogenic [[Mutation|mutations]]:<ref name="pmid24497558">{{cite journal |vauthors=Mahmood S, Palladini G, Sanchorawala V, Wechalekar A |title=Update on treatment of light chain amyloidosis |journal=Haematologica |volume=99 |issue=2 |pages=209–21 |date=February 2014 |pmid=24497558 |pmc=3912950 |doi=10.3324/haematol.2013.087619 |url=}}</ref> | |||
* [[Transthyretin|Transthyretin (TTR)]] (most common [[inherited]] [[mutation]]) | |||
* [[Fibrinogen]] | |||
* [[Apolipoprotein A1]] | |||
* [[Apolipoprotein A2]] | |||
* [[Lysozyme]] | |||
* [[Gelsolin]] [[Gene|genes]] | |||
== Associated Conditions == | |||
* [[Alzheimer's disease]]<ref name="pmid12453671">{{cite journal |vauthors=Ghiso J, Frangione B |title=Amyloidosis and Alzheimer's disease |journal=Adv. Drug Deliv. Rev. |volume=54 |issue=12 |pages=1539–51 |date=December 2002 |pmid=12453671 |doi= |url=}}</ref> | |||
* [[Down syndrome]]<ref name="pmid15021233">{{cite journal |vauthors=Head E, Lott IT |title=Down syndrome and beta-amyloid deposition |journal=Curr. Opin. Neurol. |volume=17 |issue=2 |pages=95–100 |date=April 2004 |pmid=15021233 |doi= |url=}}</ref> | |||
== Gross Pathology == | |||
On gross pathology, amyloid looks "waxy" or "lardaceous" <ref name="pmid11552976">{{cite journal |vauthors=Kyle RA |title=Amyloidosis: a convoluted story |journal=Br. J. Haematol. |volume=114 |issue=3 |pages=529–38 |date=September 2001 |pmid=11552976 |doi= |url=}}</ref> | |||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
Latest revision as of 16:50, 27 July 2018
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Renal amyloidosis Microchapters |
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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.