Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Renal amyloidosis pathophysiology
Introduction
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
- 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
Genetics
Hereditary amyloidosis due to amyloidogenic mutations:[4]
- Transthyretin (TTR) (most common inherited mutation)
- Fibrinogen
- Apolipoprotein A1
- Apolipoprotein A2
- Lysozyme
- Gelsolin genes
Microscopic Pathology
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
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.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]