A human neuronal model of Niemann Pick C disease developed from stem cells isolated from patient's skin.

Niemann Pick C (NPC) disease is a neurovisceral lysosomal storage disorder due to mutations in NPC1 or NPC2 genes, characterized by the accumulation of endocytosed unesterified cholesterol, gangliosides and other lipids within the lysosomes/late endosomes. Even if the neurodegeneration is the main feature of the disease, the analysis of the molecular pathways linking the lipid accumulation and cellular damage in the brain has been challenging due to the limited availability of human neuronal models.The aim of this study was to develop a human neuronal model of NPC disease by inducing neuronal differentiation of multipotent adult stem cells (MASC) isolated from NPC patients.Stem cells were isolated from 3 NPC patients and 3 controls both from skin biopsies and previously established skin fibroblast cultures. Cells were induced to differentiate along a neuronal fate adapting methods previously described by Beltrami et al, 2007. The surface immunophenotype of stem cells was analyzed by FACS. Stem cell and neuronal markers expression were evaluated by immunofluorescence. Intracellular accumulation of cholesterol and gangliosides were assessed by filipin staining and immunofluorescence, respectively. A morphometric analysis was performed using a Neurite outgrowth image program.After 3 passages in selective medium, MASC isolated either from skin biopsies or previously established skin fibroblast cultures displayed an antigenic pattern characteristic of mesenchymal stem cells and expressed the stem cell markers Oct-4, Nanog, Sox-2 and nestin. A massive lysosomal accumulation of cholesterol was observed only in cells isolated from NPC patients. After the induction of neural differentiation, remarkable morphologic changes were observed and cells became positive to markers of the neuronal lineage NeuN and MAP2. Differentiated cells from NPC patients displayed characteristic features of NPC disease, they showed intracellular accumulation of unesterified cholesterol and GM2 ganglioside and presented morphological differences with respect to cells derived from healthy donors.In conclusion, we generated a human neuronal model of NPC disease through the induction of differentiation of stem cells obtained from patient's easily accessible sources. The strategy described here may be applied to easily generate human neuronal models of other neurodegenerative diseases

Self-Organized Criticality of Wildfires Ecologically Revisited.

Wildfire cumulative frequency¿area distributions of Mediterranean landscapes are examined for agreement with self-similar (fractal) behavior. Our results support landscape-specific restricted scaling regions of 1.5¿3.5 orders of magnitude in size, which are delimited by breakpoints or `cut-offs¿. By identifying the extent of such regions in the fractal frequency¿area distribution of wildfires, fractal statistics may give a deeper insight into the scale-invariant dynamics of fire spread, whereas the observed cut-offs may be related to changes in the process¿pattern interactions that control wildfire propagation at the landscape scale.Peer Reviewe

Role of Interleukin-1 in Radiation-Induced Cardiomyopathy

Thoracic X-ray therapy (XRT), used in cancer treatment, is associated with increased risk of heart failure. XRT-mediated injury to the heart induces an inflammatory response leading to cardiomyopathy. The aim of this study was to determine the role of interleukin (IL)-1 in response to XRT injury to the heart and on the cardiomyopathy development in the mouse. Female mice with genetic deletion of the IL-1 receptor type I (IL-1R1 knockout mice [IL-1R1 KO]) and treatment with recombinant human IL-1 receptor antagonist anakinra, 10 mg/kg twice daily for 7 d, were used as independent approaches to determine the role of IL-1. Wild-type (wt) or IL-1R1 KO mice were treated with a single session of XRT (20 or 14 gray [Gy]). Echocardiography (before and after isoproterenol challenge) and left ventricular (LV) catheterization were performed to evaluate changes in LV dimensions and function. Masson’s trichrome was used to assess myocardial fibrosis and pericardial thickening. After 20 Gy, the contractile reserve was impaired in wt mice at d 3, and the LV ejection fraction (EF) was reduced after 4 months when compared with sham-XRT. IL-1R1 KO mice had preserved contractile reserve at 3 d and 4 months and LVEF at 4 months after XRT. Anakinra treatment for 1 d before and 7 d after XRT prevented the impairment in contractile reserve. A significant increase in LV end-diastolic pressure, associated with increased myocardial interstitial fibrosis and pericardial thickening, was observed in wt mice, as well as in IL-1R1 KO–or anakinra-treated mice. In conclusion, induction of IL-1 by XRT mediates the development of some, such as the contractile impairment, but not all aspects of the XRT-induced cardiomyopathy, such as myocardial fibrosis or pericardial thickening

e-Tools: an agent coordination layer to support the mobility of persons with disabilities

This paper outlines the development and integration of an agent coordination layer with a robotic platform to support senior citizens or persons with disabilities. This platform is situated in a given context (such as a Hospital) and it is intended to enhance user's mobility and autonomy. This objective is performed in a safe and sound fashion that meets the sets of laws, norms or protocols which rule the selected context.IFIP International Conference on Artificial Intelligence in Theory and Practice - Agents 2Red de Universidades con Carreras en Informática (RedUNCI

Prognostic role of endocarditis in isolated tricuspid valve surgery. A propensity-weighted study

Objectives The role of the underlying etiology in isolated tricuspid valve surgery has not been investigated extensively in current literature. Aim of this study was to analyse outcomes of patients undergoing surgery due to endocarditis compared to other pathologies. Methods The SURTRI study is a multicenter study enrolling adult patients who underwent isolated tricuspid valve surgery (n = 406, 55 ± 16 y.o.; 56% female) at 13 international sites. Propensity weighted analysis was performed to compare groups (IE group n = 107 vs Not-IE group n = 299). Results No difference was found regarding the 30-day mortality (Group IE: 2.8% vs Group Not-IE = 6.8%; OR = 0.45) and major adverse events. Weighted cumulative incidence of cardiac death was significantly higher for patients with endocarditis (p = 0.01). The composite endpoint of cardiac death and reoperation at 6 years was reduced in the Group IE (63.2 ± 6.8% vs 78.9 ± 3.1%; p = 0.022). Repair strategy resulted in an increased late survival even in IE cases. Conclusions Data from SURTRI study report acceptable 30-day results but significantly reduced late survival in the setting of endocarditis of the tricuspid valve. Multi-disciplinary approach, repair strategy and earlier treatment may improve outcomes. © 2022 The Author

Study of Oxidation and Combustion Characteristics of Iron Nanoparticles under Idealized and Enginelike Conditions

This document is the Accepted Manuscript version of a Published Work that appeared in final form in Energy Fuels, copyright © American Chemical Society after peer review and technical editing by the publisher.[EN] The present work includes findings from proof-of-principle feasibility studies on iron nanopowder combustion under idealized, enginelike, and real engine conditions. The study was conducted under the scope of recent interest in metallic nanoparticles as alternative fuels for internal combustion engines. More specifically, Fe nanoparticles with different morphologies and average primary particle sizes ranging from 25 to 85 nm were studied with respect to their oxidation characteristics via thermogravimetric analysis as well as in customized shock tube, constant-volume vessel, and compression-ignition (CI) engine configurations. Combusted powder samples were in all cases examined via in situ and ex situ techniques for the identification of combustion products and their morphologies. The findings facilitated the determination of the main phenomena involved during oxidation. The results verified that combustion of Fe nanoparticles in a slightly modified CI engine is feasible, albeit with various technological challenges related to ignition and scavenging that inhibit combustion quality.The authors thank the European Commission for partial funding of this work through the Project “COMETNANO” (FP7-NMP4-SL-2009-229063).Mandilas, C.; Karagiannakis, G.; Konstandopoulos, AG.; Beatrice, C.; Lazzaro, M.; Di Blasio, G.; Molina, S.... (2016). Study of Oxidation and Combustion Characteristics of Iron Nanoparticles under Idealized and Enginelike Conditions. Energy and Fuels. 30(5):4318-4330. https://doi.org/10.1021/acs.energyfuels.6b00121S4318433030

Validity and reliability of the Structured Clinical Interview for the Trauma and Loss Spectrum (SCI-TALS)

Background. DSM-IV identifies three stress response disorders (acute stress Disorder (ASD), post-traumatic stress Disorder (PTSD) and adjustment disorders (AD)) that derive from specific life events. An additional condition of complicated grief (CG), well described in the literature, is triggered by bereavement. This paper reports on the reliability and validity of the Structured Clinical Interview for Trauma and Loss Spectrum (SCI-TALS) developed to assess the spectrum of stress response. The instrument is based on a spectrum model that emphasizes soft signs, low-grade symptoms, subthreshold syndromes, as well as temperamental and personality traits comprising clinical and subsyndromal manifestations. Methods. Study participants, enrolled at 6 Italian Departments of Psychiatry located at six sites, included consecutive patients with PTSD, 44 with CG and a comparative group of 48 unselected controls. Results. We showed good reliability and validity of the SCI-TALS. Domain scores were significantly higher in participants with PTSD or CG compared to controls. There were high correlations between specific SCI-TALS domains and corresponding scores on established measures of similar constructs. Participants endorsing grief and loss events reported similar scores on all instruments, except those with CG who scored significantly higher on the domain of grief reactions. Conclusion. These findings provide strong support for the internal consistency, the discriminant validity and the reliability of the SCI-TALS. These results also support the existence of a specific grief-related condition and the proposal that different forms of stress response have similar manifestations

Broncalt®, class ii medical device, in patients with chronic relapsed upper airways disease: A survey in clinical practice

Chronic respiratory otorhinolaryngological (ORL) diseases may exacerbate. Broncalt® is a class II Medical Device containing: thermal water (Medesano, PR, Italy), hyaluronic acid, and grapefruit seed extract. It could exert a safe and effective anti-inflammatory, washing, and antimicrobial activity. The current survey, conducted in clinical practice of 84 Italian ORL centers, evaluated its safety and efficacy in the treatment of patients with exacerbated chronic upper airways disease. The 459 (254 males, mean age 44.7 years) patients were evaluated at baseline (T0) and after a 2-week treatment (T1), treated or not-treated with Broncalt®. Signs and symptoms severity were measured by visual analogue scale. Broncalt® significantly, quickly, and safely diminished the clinical features in all sub-groups (p<0.001 for all). In conclusion, Broncalt® is a class II Medical Device able to exert a safe, quick, and effective activity in patients with relapsed chronic ORL disorders. (www.actabiomedica.it)

Broncalt®, class II medical device, in patients with acute upper airways disease: A survey in clinical practice

Inflammation is a common pathogenic mechanism involved in many otorhinolaryngological (ORL) disorders. Broncalt® is a class II Medical Device containing: Thermal water (Medesano, PR, Italy), hyaluronic acid, and grapefruit seed extract. It has been reported that it exerted a safe and effective anti-inflammatory, washing, and antimicrobial activity by virtue of these components. Therefore, the aim of the current survey, conducted in clinical practice of 84 Italian ORL centers, was to evaluate its safety and efficacy in the treatment of patients with acute upper airways disease. The 3,533 (1,797 males, mean age 43.5 years) patients were evaluated at baseline (T0) and after a 2-week treatment (T1) with or without Broncalt®. Signs and symptoms severity were measured by visual analogue scale. Broncalt® significantly and safely diminished the clinical features in all sub-groups (p<0.001 for all). Interestingly, Broncalt® significantly induced a faster symptom relief already within 3 days after the start of the treatment. In conclusion, Broncalt® is a class II Medical Device able to exert a safe and effective activity in patients with acute ORL disorders. (www.actabiomedica.it)