1,313 research outputs found

    Some tardigrades from Central Africa with the description of two new species: Macrobiotus ragonesei and M. priviterae (Eutardigrada Macrobiotidae)

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    Ten species of tardigrades are reported from Central Africa. Isohypsibius arbiter Binda 1980 is new for Africa; two species, Macrobiotus ragonesei and Macrobiotus priviterae are new to science. Macrobiotus ragonesei has two macroplacoids and microplacoid, and areolated eggs with conical processes sculptured in the basal portion and unsculptured in the terminal portion. Macrobiotus priviterae is similar to M. richtersi Murray 1911, M. peteri Pilato et al. 1989, and M. chieregoi Maucci et al. 1980 but differs from them by having eyes, and in the claw and egg characters; it is also similar to M. vanescens Pilato et al. 1991 and to M. danielae Pilato et al. 2001 but differs from them in claw and egg characters. The egg of Minibiotus africanus Binda & Pilato 1995, unknown so far, is described

    The inhomogeneous mechanical behaviour of Ascending Thoracic Aortic Aneurism (ATAA)

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    Surgical management of ascending thoracic aortic aneurysms (aTAAs) relies on maximum diameter, growth rate, and presence of connective tissue disorders. The surgical decision however is often not considering that dissection and rupture do occur in patients who do not meet criteria for surgical repair [1,2]. In this study the authors aim to investigate the mechanical properties of aTAAs to be implemented in computational biomechanics models for a preclinical risk evaluation. Additionally, in some recent studies, some data about the biomechanical properties of the aTAAs have been reported [3], but without any relation to bicuspidal or tricuspidal aTAA. The aim of this study was to investigate aTAA mechanical properties using a biaxial system to compare the circumferential and axial stress-strain relations for bicuspidal and tricuspidal aTAAs

    Precise Therapy for Thoracic Aortic Aneurysm in Marfan Syndrome: A Puzzle Nearing Its Solution.

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    Abstract Marfan Syndrome (MFS) is a rare connective tissue disorder, resulting from mutations in the fibrillin-1 gene, characterized by pathologic phenotypes in multiple organs, the most detrimental of which affects the thoracic aorta. Indeed, thoracic aortic aneurysms (TAA), leading to acute dissection and rupture, are today the major cause of morbidity and mortality in adult MFS patients. Therefore, there is a compelling need for novel therapeutic strategies to delay TAA progression and counteract aortic dissection occurrence. Unfortunately, the wide phenotypic variability of MFS patients, together with the lack of a complete genotype-phenotype correlation, have represented until now a barrier hampering the conduction of translational studies aimed to predict disease prognosis and drug discovery. In this review, we will illustrate available therapeutic strategies to improve the health of MFS patients. Starting from gold standard surgical overtures and the description of the main pharmacological approaches, we will comprehensively review the state-of-the-art of in vivo MFS models and discuss recent clinical pharmacogenetic results. Finally, we will focus on induced pluripotent stem cells (iPSC) as a technology that, if integrated with preclinical research and pharmacogenetics, could contribute in determining the best therapeutic approach for each MFS patient on the base of individual differences. Finally, we will suggest the integration of preclinical studies, pharmacogenetics and iPSC technology as the most likely strategy to help solve the composite puzzle of precise medicine in this condition

    Pseudomonas aeruginosa infection in cystic fibrosis caused by an epidemic metallo-β-lactamase-producing clone with a heterogeneous carbapenem resistance phenotype

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    AbstractAn epidemic IMP-13 metallo-β-lactamase (MBL)-producing Pseudomonas aeruginosa clone, causing infections and even large outbreaks in Italian critical care settings, was detected in a young cystic fibrosis patient. In this patient, the chronic infection was sustained by distinct clonal sub-populations of the MBL-producing P. aeruginosa clone, either susceptible or resistant to carbapenems. These findings underscore the importance of infection prevention practices in cystic fibrosis settings and pose an important diagnostic and therapeutic challenge

    Calcium as a key player in arrhythmogenic cardiomiopathy : adhesion disorder or intracellular alteration?

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    Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disease characterized by sudden death in young people and featured by fibro-adipose myocardium replacement, malignant arrhythmias, and heart failure. To date, no etiological therapies are available. Mutations in desmosomal genes cause abnormal mechanical coupling, trigger pro-apoptotic signaling pathways, and induce fibro-adipose replacement. Here, we discuss the hypothesis that the ACM causative mechanism involves a defect in the expression and/or activity of the cardiac Ca2+ handling machinery, focusing on the available data supporting this hypothesis. The Ca2+ toolkit is heavily remodeled in cardiomyocytes derived from a mouse model of ACM defective of the desmosomal protein plakophilin-2. Furthermore, ACM-related mutations were found in genes encoding for proteins involved in excitation\u2012contraction coupling, e.g., type 2 ryanodine receptor and phospholamban. As a consequence, the sarcoplasmic reticulum becomes more eager to release Ca2+, thereby inducing delayed afterdepolarizations and impairing cardiac contractility. These data are supported by preliminary observations from patient induced pluripotent stem-cell-derived cardiomyocytes. Assessing the involvement of Ca2+ signaling in the pathogenesis of ACM could be beneficial in the treatment of this life-threatening disease

    Transcatheter Mitral Valve-in-Valve Dislocation: A Rescue Strategy

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    In the transcatheter aortic valve replacement era, transapical valve-in-valve mitral valve implantation is considered an alternative treatment for high-risk patients with degenerated mitral bioprosthesis. We report an unusual strategy to remedy the potentially fatal complication of the mitral prosthesis migration into the aortic arch. The dislocated prosthesis was successfully stabilized in the aortic arch with a bare aortic stent, ensuring adequate perfusion of epiaortic vessels. \ua9 2018 The Society of Thoracic Surgeon

    Isolation and characterization of cardiac mesenchymal stromal cells from endomyocardial bioptic samples of arrhythmogenic cardiomyopathy patients

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    A normal adult heart is composed of several different cell types, among which cardiac mesenchymal stromal cells represent an abundant population. The isolation of these cells offers the possibility of studying their involvement in cardiac diseases, and, in addition, provides a useful primary cell model to investigate biological mechanisms. Here, the method for the isolation of C-MSC from arrhythmogenic cardiomyopathy patients\u2019 bioptic samples is described. The endomyocardial biopsy sampling is guided in the right ventricular areas adjacent to the scar visualized by electro-anatomical mapping. The digestion of the biopsies in collagenase and their plating on a plastic dish in culture medium to allow C-MSC growth is described. The isolated cells can be expanded in culture for several passages. To confirm their mesenchymal phenotype, the description of immuno-phenotypical characterization is provided. C-MSC are able to differentiate into several cell types like adipocytes, chondrocytes, and osteoblasts: in the context of ACM, characterized by adipocyte deposits in patients\u2019 hearts, the protocols for the adipogenic differentiation of C-MSC and the characterization of lipid droplet accumulation are described

    ALICE: An Automatic Design Flow for eFPGA Redaction

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    Fabricating an integrated circuit is becoming unaffordable for many semiconductor design houses. Outsourcing the fabrication to a third-party foundry requires methods to protect the intellectual property of the hardware designs. Designers can rely on embedded reconfigurable devices to completely hide the real functionality of selected design portions unless the configuration string (bitstream) is provided. However, selecting such portions and creating the corresponding reconfigurable fabrics are still open problems. We propose ALICE, a design flow that addresses the EDA challenges of this problem. ALICE partitions the RTL modules between one or more reconfigurable fabrics and the rest of the circuit, automating the generation of the corresponding redacted design
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