Impaired pulmonary circulation hemodynamics are characteristic of pulmonary hypertension (PH). We therefore sought to evaluate possible correlations between endothelial function noninvasively ...assessed by brachial artery flow-mediated dilation (FMD) and hemodynamic parameters at right-sided cardiac catheterization in patients with clinically suspected PH. Consecutive outpatients with suspected PH were enrolled in the study. In all patients, endothelial function was assessed by FMD and hemodynamic parameters (pulmonary artery pressure PAP); pulmonary vascular resistances PVR) were derived by right-sided cardiac catheterization. For this study, 95 consecutive patients with suspected PH were enrolled (mean age 63 ± 13 years, 58% male) and included in the analysis. FMD values were significantly correlated with systolic (s)PAP levels (r = −0.29, p = 0.016); correlation with PVR was of borderline significance (r = −0.21, p = 0.78). After multivariable regression analysis including age, gender, tricuspid annular plane systolic excursion and peak tricuspid regurgitation velocity (peak TRV), and FMD, the latter remained significantly correlated with systolic pulmonary artery pressure (sPAP) values (B = −47, p = 0.02). After classifying patients according to median levels of peak TRV and FMD into 3 groups (neither, either, or both impaired), progressively increased levels of sPAP, mean PAP, and PVR were found (p for trend <0.001 in all cases). FMD values were inversely related to sPAP levels in a small population of patients with clinically suspected PH. In combination with peak TRV levels, FMD values noninvasively assessed were predictive of increased sPAP, mean PAP, and PVR.
Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular ...mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem to contribute to its onset. To further understand the complex relationship between endothelial and pulmonary hypertension and try to contribute to the development of future therapies, we report a comprehensive and updated review on endothelial function in pulmonary arterial hypertension.
In this article, we present an approach designed to extend the metadata schema of the Zenodo data management platform to strengthen the FAIRness of the published dataset. We focus on a bottom-up ...approach starting from a series of datasets ranging from the 3D digitalisation of monuments and sites to the creation of reconstructive records (including the scientific documentation they are based on), to the implementation of digital storytelling and to the development of open source-based web-apps. We propose the simplest possible set of metadata to be included in the Zenodo platform with the possibility, for the community, to adopt and further develop/modify them. This article will describe in detail the formalisation and the digital formats adopted providing the related metadata templates developed within the projects.
Abstract
Alterations in the DMD gene, which codes for the protein dystrophin, cause forms of dystrophinopathies such as Duchenne muscular dystrophy, an X-linked disease. Cardiomyopathy linked to DMD ...mutations is becoming the leading cause of death in patients with dystrophinopathy. Since phenotypic pathophysiological mechanisms are not fully understood, the improvement and development of new disease models, considering their relative advantages and disadvantages, is essential. The application of genetic engineering approaches on induced pluripotent stem cells, such as gene-editing technology, enables the development of physiologically relevant human cell models for in vitro dystrophinopathy studies. The combination of induced pluripotent stem cells-derived cardiovascular cell types and 3D bioprinting technologies hold great promise for the study of dystrophin-linked cardiomyopathy. This combined approach enables the assessment of responses to physical or chemical stimuli, and the influence of pharmaceutical approaches. The critical objective of in vitro microphysiological systems is to more accurately reproduce the microenvironment observed in vivo. Ground-breaking methodology involving the connection of multiple microphysiological systems comprised of different tissues would represent a move toward precision body-on-chip disease modelling could lead to a critical expansion in what is known about inter-organ responses to disease and novel therapies that have the potential to replace animal models. In this review, we will focus on the generation, development, and application of current cellular, animal, and potential for bio-printed models, in the study of the pathophysiological mechanisms underlying dystrophin-linked cardiomyopathy in the direction of personalized medicine.
Graphical Abstract
Graphical Abstract
The H2IOSC project aims to create a federated cluster of research infrastructures (RIs) in the domain of Cultural Heritage at the national level in Italy. Through four key Ris - DARIAH-IT, CLARIN, ...OPERAS, and E-RIHS - the project enables collaboration among researchers with interdisciplinary expertise. Within this context, DIGILAB emerges as a digital access platform for the Italian node of E-RIHS, providing data management, digital tools, and services to boost cultural heritage preservation, fruition, and study. A significant aspect of DIGILAB architecture is its capability to support geo-localization and real-time monitoring of cultural heritage sites in terms of environmental conditions, structural integrity, and diagnostics, leveraging on novel Internet of Things (IoT) systems and large-scale Wireless Sensor Networks (WSNs). By integrating WSNs into DIGILAB framework, the project enhances remote monitoring and control of cultural sites. These networks facilitate the collection of real-time data on factors such as temperature, humidity, and air quality, providing crucial insights for the Cultural Heritage research community. Moreover, WSNs enable proactive measures to be taken in response to emerging threats, mitigating risks and minimizing damage to cultural assets at a national level.
