Overview of systemic and localized amyloidosis

Reviews in Health Care

View Publication Info
 
 
Field Value
 
Title Overview of systemic and localized amyloidosis
 
Creator Girnius, Saulius
 
Subject Internal Medicine
Localized amyloidosis; Rare disease; Systemic amyloidosis
 
Description Amyloidosis is a family of protein misfolding disorders, in which insoluble fibrillar proteins deposit extracellularly and cause end organ damage. Depending on the precursor protein, clinical manifestations in amyloidosis vary significantly. In systemic amyloidosis, the heart, kidneys, and nerves are most commonly affected, resulting in congestive heart failure, arrhythmia, nephrotic syndrome, renal failure, and peripheral and autonomic neuropathies. In localized amyloidosis, amyloid deposits at the site of production, so only one organ is disrupted. Once amyloidosis is confirmed histologically, the precursor subtype must be identified using immunohistochemistry, immunofixation, electron microscopy, or laser microdissection and mass spectrometry. Treatment should not be initiated prior to the identification of the type of amyloidosis. Currently, treatment focuses on the suppression of the precursor protein: in AL amyloidosis, chemotherapy or autologous stem cell transplants suppress production of immunoglobulin light chains; in AA amyloidosis, anti-microbial and anti-inflammatory agents suppress amyloid A production; and in AF amyloidosis, a liver transplantation removes the source of mutant transthyretin protein production. Newer drugs are being developed to target amyloidosis at an epigenetic level or stabilize folding intermediates, but there are currently in development.
 
Publisher SEEd
 
Contributor
 
Date 2013-10-21
 
Type info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion

Narrative Reviews
 
Format text/html
application/pdf
 
Identifier https://journals.edizioniseed.it/index.php/rhc/article/view/662
10.7175/rhc.v4i4.662
 
Source Reviews in Health Care; Vol 4, No 4 (2013); 231-247
2038-6702
2038-6699
 
Language eng
 
Relation https://journals.edizioniseed.it/index.php/rhc/article/view/662/1017
https://journals.edizioniseed.it/index.php/rhc/article/view/662/1018
 
Coverage Worldwide


 
Rights Copyright (c) 2013 Seed
http://creativecommons.org/licenses/by-nc/4.0
 

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