Articles with "amyloidosis" as a keyword



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Geographic Disparities in Reported US Amyloidosis Mortality From 1979 to 2015: Potential Underdetection of Cardiac Amyloidosis

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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… read more here.

Keywords: death; 1979 2015; mortality; amyloidosis ... See more keywords
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Anakinra-Associated Amyloidosis.

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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. read more here.

Keywords: anakinra associated; dermatology; amyloidosis; associated amyloidosis ... See more keywords
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Corneal confocal microscopy to detect early immune‐mediated small nerve fibre loss in AL amyloidosis

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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… read more here.

Keywords: small nerve; corneal confocal; microscopy; nerve ... See more keywords
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Ser77Tyr transthyretin amyloidosis in Israel: Initial manifestations and diagnostic features

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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.… read more here.

Keywords: diagnostic features; israel initial; ser77tyr transthyretin; amyloidosis israel ... See more keywords
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Quality of life and symptoms among patients with relapsed/refractory AL amyloidosis treated with ixazomib‐dexamethasone versus physician's choice

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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… read more here.

Keywords: ixazomib dexamethasone; physician choice; patients relapsed; quality life ... See more keywords
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Novel screening for transthyretin amyloidosis by using fat ultrasonography

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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… read more here.

Keywords: novel screening; transthyretin amyloidosis; amyloidosis; hereditary transthyretin ... See more keywords
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Advances in the treatment of hereditary transthyretin amyloidosis: A review

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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… read more here.

Keywords: transthyretin amyloidosis; amyloidosis; advances treatment; attr ... See more keywords
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Diagnostic utility of cardiac troponin T level in patients with cardiac amyloidosis

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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. read more here.

Keywords: patients cardiac; cardiac troponin; amyloidosis; diagnostic utility ... See more keywords
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Non‐Val30Met mutation, septal hypertrophy, and cardiac denervation in patients with mutant transthyretin amyloidosis

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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,… read more here.

Keywords: attrm amyloidosis; mutation septal; amyloidosis; non val30met ... See more keywords
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Non‐cardiac biopsy sites with high frequency of transthyretin amyloidosis

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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),… read more here.

Keywords: non cardiac; biopsy; frequency; amyloidosis ... See more keywords
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Eyes on amyloidosis: microvascular retinal dysfunction in cardiac amyloidosis

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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… read more here.

Keywords: heart failure; cardiac amyloidosis; amyloidosis; dysfunction ... See more keywords