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Amyloidosis of the Heart and Kidney
Amyloidosis encompasses a collection of disorders of pathological protein folding. The extracellular location where these “amyloid fibril” proteins are deposited determines the clinical presentation of the disease. The abnormal architecture of these fibrils makes them insoluble and not easily remove...
Autor principal: | |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Houston Methodist DeBakey Heart & Vascular Center
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9461691/ https://www.ncbi.nlm.nih.gov/pubmed/36132587 http://dx.doi.org/10.14797/mdcvj.1150 |
Sumario: | Amyloidosis encompasses a collection of disorders of pathological protein folding. The extracellular location where these “amyloid fibril” proteins are deposited determines the clinical presentation of the disease. The abnormal architecture of these fibrils makes them insoluble and not easily removed, leading to disruption of normal tissue structure and interference with normal physiology. Amyloidosis of the heart and kidney can be inherited, secondary to unrelated diseases, or due to a plasma cell disorder. This review will focus on immunoglobulin light chain amyloidosis, which is life-threatening and must be diagnosed as early as possible by employing precise and accurate typing to ensure timely and frequently curative therapy. |
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