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Published in 2018 at "JAMA Cardiology"
DOI: 10.1001/jamacardio.2018.2093
Abstract: Importance Cardiac amyloidosis is an underdiagnosed disease and is highly fatal when untreated. Early diagnosis and treatment with the emerging novel therapies significantly improve survival. A comprehensive analysis of amyloidosis-related mortality is critical to appreciate…
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Keywords:
death;
1979 2015;
mortality;
amyloidosis ... See more keywords
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Published in 2022 at "JAMA dermatology"
DOI: 10.1001/jamadermatol.2022.2124
Abstract: This case report describes 2 patients with iatrogenic amyloidosis secondary to subcutaneous injections of anakinra to manage neonatal-onset multisystem inflammatory disease.
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Keywords:
anakinra associated;
dermatology;
amyloidosis;
associated amyloidosis ... See more keywords
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Published in 2022 at "Annals of Clinical and Translational Neurology"
DOI: 10.1002/acn3.51565
Abstract: Light chain (AL) amyloidosis is a life‐threatening disorder characterised by extracellular deposition of amyloid leading to dysfunction of multiple organs. Peripheral nerve involvement, particularly small fibre neuropathy, may be associated with poorer survival. Corneal confocal…
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Keywords:
small nerve;
corneal confocal;
microscopy;
nerve ... See more keywords
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Published in 2023 at "Annals of Clinical and Translational Neurology"
DOI: 10.1002/acn3.51741
Abstract: Amyloidosis due to the transthyretin Ser77Tyr mutation (ATTRS77Y) is a rare autosomal‐dominant disorder, characterized by carpal‐tunnel syndrome, poly‐ and autonomic‐neuropathy, and cardiomyopathy. However, related symptoms and signs are often nonspecific and confirmatory tests are required.…
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Keywords:
diagnostic features;
israel initial;
ser77tyr transthyretin;
amyloidosis israel ... See more keywords
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Published in 2023 at "American Journal of Hematology"
DOI: 10.1002/ajh.26866
Abstract: Patient‐reported outcomes in AL amyloidosis have not been well‐studied. We analyzed health‐related quality of life (HRQOL) and AL amyloidosis symptoms data from the phase 3 TOURMALINE‐AL1 trial (NCT01659658) (ixazomib‐dexamethasone, n = 85; physician's choice of…
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Keywords:
ixazomib dexamethasone;
physician choice;
patients relapsed;
quality life ... See more keywords
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Published in 2017 at "Annals of Neurology"
DOI: 10.1002/ana.24914
Abstract: We aimed to assess the possibility of using a noninvasive screening method for hereditary transthyretin amyloidosis by means of abdominal fat ultrasonography. Quantitative analysis of ultrasound B‐mode images demonstrated a significant increase in mean echogenicity…
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Keywords:
novel screening;
transthyretin amyloidosis;
amyloidosis;
hereditary transthyretin ... See more keywords
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Published in 2019 at "Brain and Behavior"
DOI: 10.1002/brb3.1371
Abstract: Amyloid transthyretin amyloidosis (ATTR) is a progressive and often fatal disease caused by the buildup of mutated (hereditary ATTR [hATTR]; also known as ATTR variant [ATTRv]) or normal transthyretin (wild‐type ATTR) throughout the body. Two…
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Keywords:
transthyretin amyloidosis;
amyloidosis;
advances treatment;
attr ... See more keywords
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Published in 2018 at "ESC Heart Failure"
DOI: 10.1002/ehf2.12203
Abstract: The aim of this study was to evaluate the diagnostic utility of high‐sensitivity cardiac troponin T (hs‐cTnT) levels in discriminating cardiac amyloidosis from patients with cardiac hypertrophy caused by aetiologies other than cardiac amyloidosis.
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Keywords:
patients cardiac;
cardiac troponin;
amyloidosis;
diagnostic utility ... See more keywords
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Published in 2019 at "ESC Heart Failure"
DOI: 10.1002/ehf2.12361
Abstract: Mutant transthyretin (ATTRm) amyloidosis is a systemic disease caused by the deposition of amyloid fibrils derived from mutated transthyretin. Although cardiac involvement impacts the prognosis of patients with ATTRm amyloidosis, the incidence of cardiac events,…
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Keywords:
attrm amyloidosis;
mutation septal;
amyloidosis;
non val30met ... See more keywords
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Published in 2020 at "ESC Heart Failure"
DOI: 10.1002/ehf2.13130
Abstract: Cardiac scintigraphy, a non‐invasive technique for diagnosing ATTR cardiac amyloidosis, lacks specificity in patients with concomitant monoclonal gammopathy (up to 40% of cases). For these patients, amyloid type is often established by endomyocardial biopsy (EMB),…
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Keywords:
non cardiac;
biopsy;
frequency;
amyloidosis ... See more keywords
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Published in 2022 at "ESC Heart Failure"
DOI: 10.1002/ehf2.13792
Abstract: Cardiac involvement in systemic amyloidosis is a marker of particularly poor prognosis. Cardiac amyloidosis (CA) is characterized by extracellular amyloid deposits inducing heart failure and symptoms of cardiac microvascular disease. While amyloid deposition is most…
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Keywords:
heart failure;
cardiac amyloidosis;
amyloidosis;
dysfunction ... See more keywords