1,721,188 research outputs found
The use of mass spectrometry for the diagnosis of plasma cell dyscrasias: focus on light chain (AL) amyloidosis patterns.
Get PDFIndividualized Approach to Management of Light Chain Amyloidosis
No full textSystemic light chain (AL) amyloidosis is caused by a B-cell (most commonly plasma cell) clone that produces a toxic light chain that forms amyloid fibrils in tissues and causes severe, progressive organ dysfunction. The clinical presentation is protean, and patients are usually extremely frail, thus requiring careful adaptation of the treatment approach. However, the severity of organ involvement can be accurately assessed with biomarkers that allow a sharp prognostic stratification and precise tailoring of the treatment strategy. Moreover, the availability of biomarker-based response criteria also allows adjustment of the treatment approach over time. The recent completion of 3 large randomized clinical trials has offered new evidence for designing appropriate treatments. All this information has recently been integrated in the joint guidelines of the International Society of Amyloidosis and the European Hematology Association for the treatment of AL amyloidosis. Other clinical trials are underway testing new agents directed against the amyloid clone and the amyloid deposits. Our understanding of the peculiarities of the amyloid clone, as well as our ability to detect residual clonal disease and improve organ dysfunction, are also being refined and will result in more precise personalization of the treatment approach
Novel strategies for the diagnosis and treatment of cardiac amyloidosis
No full texthttps://www.ncbi.nlm.nih.gov/pubmed/2649623
Rethinking the Role of Co-morbidities in Transthyretin Cardiac Amyloidosis: Cinderella or Princess? The Case of Anemia and Iron Deficiency
No full textOptions for Rescue Treatment of Patients with AL Amyloidosis Exposed to Upfront Daratumumab
No full textPurpose of Review: This review aims to assess the therapeutic strategies available for relapsed/refractory patients with immunoglobulin light chain (AL) amyloidosis who received upfront daratumumab-based regimens. Recent Findings: The treatment landscape of AL amyloidosis has changed radically thanks to the introduction in the upfront setting of daratumumab in combination with bortezomib, cyclophosphamide and dexamethasone (DaraCyBorD) which improved patients’ outcomes increasing the rate of hematologic and organ responses. However, many patients eventually relapse or are refractory to daratumumab and the best salvage therapy is not well defined yet. In this contest, we reviewed the available therapeutic options after daratumumab failure, and we look towards the current advances in Bcl-2 inhibitors, novel immunotherapeutic agents as chimeric antigen receptor (CAR-T) therapy and bispecific antibodies (bsAbs). Summary: Relapsed/refractory AL amyloidosis represent an unmet clinical need and novel targeted drugs require urgent prospective assessment
Serum-free light-chain analysis in diagnosis and management of multiple myeloma and related conditions
No full texthttps://www.ncbi.nlm.nih.gov/pubmed/2746789
Influence of the nanostructure on the electric transport properties of resistive switching cluster-assembled gold films
Get PDFThe use of Au clusters produced in the gas phase and deposited at low kinetic energy on a substrate allows the bottom-up fabrication of nanostructured metallic thin films with an extremely large number of interfaces, grain boundary junctions and crystal lattice defects. Cluster-assembled gold films exhibit non-ohmic electrical transport properties with a complex resistive switching behavior, exploring discrete resistance states which depend on their structural features (average thickness, resistance value reached on the percolation curve). Their electric conduction properties can be modelled in terms of complex networks of nanojunctions and used to perform binary classification of Boolean functions. The fabrication of devices based on cluster-assembled Au films and exploiting emergent complexity and collective phenomena requires a deep understanding of the influence of the nanoscale structure on the fundamental mechanisms of electrical conduction. Here we present a detailed study of the correlation between the nanostructure and the electrical properties of cluster-assembled gold films by a systematic characterization of the film growth from sub-monolayer to continuous layer beyond the electrical percolation threshold. The influence of different cluster size distributions on the onset of the electrical conduction and the role of defects is investigated by combining in situ and ex situ electrical and structural characterizations
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe 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
Get PDF“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
- …
