Results 11 to 20 of about 98,806 (295)
Postgraduate Medical Journal, 2001 Abstract Amyloidosis is not a single disease but a series of diseases in which there is extracellular deposition of a protein which, although it may be derived from different and unrelated sources, folds into a β pleated sheet. There have recently been significant advances in elucidating the pathogenesis and in the ...
M F, Khan, R H, Falk
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Annals of Clinical Biochemistry: International Journal of Laboratory Medicine, 2012 The term amyloid describes the deposition in the extracellular space of certain proteins in a highly characteristic, insoluble fibrillar form. Amyloidosis describes the various clinical syndromes that occur as a result of damage by amyloid deposits in tissues and organs throughout the body.
Jennifer H, Pinney, Philip N, Hawkins
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Blood Cancer Journal, 2022 Effective systemic therapies suppress toxic light chain production leading to an increased proportion of patients with light chain (AL) amyloidosis who survive longer albeit with end-stage renal disease.
Andrea Havasi +21 more
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Navigating the Complex Web of Prescribing Amyloidosis Therapeutics: A Primer
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2022 Advancement in the diagnosis and treatment of transthyretin amyloid cardiomyopathy has made great strides in recent years. Novel therapeutics for transthyretin amyloidosis such as tafamidis, patisiran, and inotersen have shown significant benefits in a ...
Hongya Chen +6 more
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Clinical Amyloid Typing by Proteomics: Performance Evaluation and Data Sharing between Two Centres
Molecules, 2021 Amyloidosis is a relatively rare human disease caused by the deposition of abnormal protein fibres in the extracellular space of various tissues, impairing their normal function. Proteomic analysis of patients’ biopsies, developed by Dogan and colleagues
Diana Canetti +12 more
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Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study
Blood Cancer Journal, 2021 The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival
Andrew Staron +6 more
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Proteomic Analysis for the Diagnosis of Fibrinogen Aα-chain Amyloidosis
Kidney International Reports, 2019 Introduction: Hereditary fibrinogen Aα-chain (AFib) amyloidosis is a relatively uncommon renal disease associated with a small number of pathogenic fibrinogen Aα (FibA) variants; wild-type FibA normally does not result in amyloid deposition.
Graham W. Taylor +11 more
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International Journal of Cardiology: Heart & Vasculature, 2020 Objectives: Left atrial (LA) function is an important marker of hemodynamic status in cardiac amyloidosis (CA), and its characterization may provide relevant prognostic information.
Giovanni Donato Aquaro +10 more
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Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease, 2023 Background Transthyretin cardiac amyloidosis (ATTR‐CM), found in 6% to 15% of cohorts with heart failure with preserved ejection fraction, has long been considered a rare disease with poor prognosis.
Morris M. Kim +7 more
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Renal Amyloidosis Associated With 5 Novel Variants in the Fibrinogen A Alpha Chain Protein
Kidney International Reports, 2017 Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK.
Dorota Rowczenio +18 more
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