1,720,958 research outputs found
Cardiac scintigraphy for transthyretin amyloidosis: a decade of progress, a future of precision
TTR amyloidosi
Case report: recurrent chest pain as initial manifestation of rapidly progressing light-chain cardiac amyloidosis with microvascular infiltration-a novel red flag associated with poor outcomes
Background: Angina due to microvascular amyloid infiltration is an under-recognized early red flag of infiltrative disease and should be investigated as a predictor of poor outcomes. Case summary: A 73-year-old man with cardiovascular risk factors and a history of treated obstructive coronary artery disease presented 10 months later with recurrent angina. His condition progressively worsened, with the development of severe dyspnoea. Within 6 months, he succumbed to cardiogenic shock in the presence of severe biventricular dysfunction and unobstructed coronary arteries. Post-mortem diagnosis revealed AL cardiac amyloidosis with massive myocardial infiltration and extensive microvascular amyloid deposits. Discussion: This case underscores chest pain as a critical clinical marker, potentially indicating microvascular amyloid infiltration in patients with light-chain cardiac amyloidosis, particularly those with unobstructed coronary arteries. Its assessment may also have implications for future therapies
Amyloidosis and Amyloidogenesis: One Name, Many Diseases
Amyloidosis is a heterogenous group of disorders, caused by the deposition of insoluble fibrils derived from misfolded proteins in the extracellular space of various organs. These proteins have an unstable structure that causes them to misfold, aggregate, and deposit as amyloid fibrils with the pathognomonic histologic property of green birefringence when viewed under cross-polarized light after staining with Congo red. Amyloid fibrils are insoluble and degradation-resistant; resistance to catabolism results in progressive tissue amyloid accumulation. The outcome of this process is organ disfunction independently from the type of deposited protein, however there can be organ that are specifically targeted from certain proteins
Flexible approaches based on multi-state models and microsimulation to perform real-world cost-effectiveness analyses: an application to pcsk9-inhibitors
Objectives: This study aims to show the application of flexible statistical methods in real-world cost-effectiveness analyses applied in the cardiovascular field, focusing specifically on the use of PCSK9 inhibitors for hyperlipidaemia. Methods: The proposed method allowed us to use an electronic health database to emulate a target trial for cost-effectiveness analysis using multi-state modelling and microsimulation. We formally established the study design and provided precise definitions of the causal measures of interest, while also outlining the assumptions necessary for accurately estimating these measures using the available data. Additionally, we thoroughly considered goodness-of-fit assessments and sensitivity analyses of the decision model, which are crucial to capture the complexity of individuals' healthcare pathway and to enhance the validity of this type of health economic models. Results: In the disease model, the Markov assumption was found to be inadequate, and a "time-reset" timescale was implemented together with the use of a time-dependent variable to incorporate past hospitalization history. Furthermore, the microsimulation decision model demonstrated a satisfying goodness-of-fit, as evidenced by the consistent results obtained in the short-term horizon compared to a non-model-based approach. Notably, only in the long-term follow-up PCSK9 inhibitors revealed their favorable cost-effectiveness, with a minimum willingness-to-pay of 39,000 Euro/LY gained. Conclusions: The approach demonstrated its significant utility in several ways. Unlike non-model based or alternative model-based methods, it enabled to 1) investigate long-term cost-effectiveness comprehensively, 2) employ an appropriate disease model that aligns with the specific problem under study, and 3) conduct subgroup-specific cost-effectiveness analyses to gain more targeted insights
Going Beyond Counting First Authors in Author Co-citation Analysis
The 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
“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
Appropriate Similarity Measures for Author Cocitation Analysis
We provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Dispelling the Myths Behind First-author Citation Counts
We conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
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