1,722,043 research outputs found
Relative Left Ventricular Apical Sparing of Longitudinal Strain in Cardiac Amyloidosis: Is it Just Amyloid Infiltration?
No full textCardiac amyloidosis (CA) is a rare and phenotypically heterogeneous disease, with pathophysiological clinical and morphological variability. From an imaging point of view, CA is one of the cardiomyopathies with a hypertrophic phenotype and is frequently misdiagnosed on echocardiography as sarcomeric or idiopathic hypertrophic cardiomyopathy. However, there are morphological and functional features that could unravel the underlying myocardial infiltration, and relative apical sparing of longitudinal strain (LS) impairment is among the most specific and almost invariably reported findings
Standardising Care and Treatment of Transthyretin Amyloid Cardiomyopathy
Full text linkTransthyretin cardiac amyloidosis (ATTR-CA) has been traditionally considered a rare and inexorably fatal condition. ATTR-CA now is an increasingly recognised cause of heart failure and mortality worldwide with effective pharmacological treatments. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have transformed the diagnosis of ATTR-CA, which is now possible without recourse to endomyocardial biopsy in around 70% of cases. Many patients are now diagnosed at an earlier stage. Echocardiography and cardiac magnetic resonance have enabled identification of patients with possible ATTR-CA and more accurate prognostic stratification. Therapies able to slow or halt ATTR-CA progression and increase survival are now available and there is also evidence that patients may benefit from specific conventional heart failure medications. A wide horizon of possibilities is unfolding and awaits discovery
Wild-type transthyretin cardiac amyloidosis: the need for a comprehensive geriatric assessment beyond age
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Letter by Porcari et al Regarding Article, "Association Between Atrial Uptake on Cardiac Scintigraphy With Technetium-99m-Pyrophosphate Labeled Bone-Seeking Tracers and Atrial Fibrillation"
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Transthyretin cardiac amyloidosis
Full text linkTransthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized cause of heart failure (HF) and mortality worldwide. Advances in non-invasive diagnosis, coupled with the development of effective treatments, have shifted ATTR-CA from a rare and untreatable disease to a relatively prevalent condition that clinicians should consider on a daily basis. Amyloid fibril formation results from age-related failure of homoeostatic mechanisms in wild-type ATTR (ATTRwt) amyloidosis (non-hereditary form) or destabilizing mutations in variant ATTR (ATTRv) amyloidosis (hereditary form). Longitudinal large-scale studies in the United States suggest an incidence of cardiac amyloidosis in the contemporary era of 17 per 100 000, which has increased from a previous estimate of 0.5 per 100 000, which was almost certainly due to misdiagnosis and underestimated. The presence and degree of cardiac involvement is the leading cause of mortality both in ATTRwt and ATTRv amyloidosis, and can be identified in up to 15% of patients hospitalized for HF with preserved ejection fraction. Associated features, such as carpal tunnel syndrome, can preceed by several years the development of symptomatic HF and may serve as early disease markers. Echocardiography and cardiac magnetic resonance raise suspicion of disease and might offer markers of treatment response at a myocardial level, such as extracellular volume quantification. Radionuclide scintigraphy with 'bone' tracers coupled with biochemical tests may differentiate ATTR from light chain amyloidosis. Therapies able to slow or halt ATTR-CA progression and increase survival are now available. In this evolving scenario, early disease recognition is paramount to derive the greatest benefit from treatment
Cardiac Amyloidosis
No full textCardiac amyloidosis (CA) is considered the paradigm of the restrictive cardiomyopathies. Previously thought to be a rare disease, it is now emerging as an underdiagnosed cause of heart failure [1]. More than 30 proteins can form amyloid fibrils, but almost all clinical cases of CA are from either mis- folded monoclonal immunoglobulin light chains (AL amyloi- dosis) produced by an abnormal clonal proliferation of plasma cells in the bone marrow, or transthyretin (ATTR amyloido- sis), a protein synthetized by the liver normally involved in the transportation of the hormone thyroxine and retinol-binding protein [1]. ATTR amyloidosis may in turn be either heredi- tary (vATTR) arising from the misfolded mutated TTR, or non-hereditary, from misfolded wild-type TTR (wtATTR). According to available data, AL amyloidosis has a prevalence of 10–16 cases in 1 million/person/year [2]. The recognition of wtATTR amyloidosis has increased exponentially over the last few years [3]. Considered until very recently rare, current reports estimate a prevalence of 10–16% in some cohorts— particularly elderly (>80-year-old) patients with either heart failure, hypertrophy, aortic stenosis, or carpal tunnel (CT) syn- drome [3, 4]
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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