Formation of X-ray emitting stationary shocks in magnetized protostellar jets

X-ray observations of protostellar jets show evidence of strong shocks heating the plasma up to temperatures of a few million degrees. In some cases, the shocked features appear to be stationary. They are interpreted as shock diamonds. We aim at investigating the physics that guides the formation of X-ray emitting stationary shocks in protostellar jets, the role of the magnetic field in determining the location, stability, and detectability in X-rays of these shocks, and the physical properties of the shocked plasma. We performed a set of 2.5-dimensional magnetohydrodynamic numerical simulations modelling supersonic jets ramming into a magnetized medium and explored different configurations of the magnetic field. The model takes into account the most relevant physical effects, namely thermal conduction and radiative losses. We compared the model results with observations, via the emission measure and the X-ray luminosity synthesized from the simulations. Our model explains the formation of X-ray emitting stationary shocks in a natural way. The magnetic field collimates the plasma at the base of the jet and forms there a magnetic nozzle. After an initial transient, the nozzle leads to the formation of a shock diamond at its exit which is stationary over the time covered by the simulations (~ 40 - 60 yr; comparable with time scales of the observations). The shock generates a point-like X-ray source located close to the base of the jet with luminosity comparable with that inferred from X-ray observations of protostellar jets. For the range of parameters explored, the evolution of the post-shock plasma is dominated by the radiative cooling, whereas the thermal conduction slightly affects the structure of the shock.Comment: Accepted for publication in Astronomy and Astrophysic

Controlled Fault-Tolerant Power Converters for Power Quality Enhancement

Power quality depends generally on the interaction of electrical power with electrical equipments. If electrical equipments operate correctly and reliably without being damaged or stressed, a suitable level of power quality is assured. On the other hand, if the electrical equipment malfunctions, is unreliable, or is damaged during normal usage, power quality is poor and probably the economical loss could be important like the technical one. In the scenario of the Distributed Generation, power quality issues will be moreover important because an higher dissemination of power conditioning equipment will be requested and this obviously increases the sources of vulnerability of the electrical system. In this paper fault tolerant power converters are considered as a viable solution of power quality problems and a suitable control algorithm of them is presented. The control proposed in the paper is based on the model of the power converter reformulated in terms of healthy leg binary variable and the paper shows how this control is able to save the aspect of power quality when the converter works in the linear range. The effectiveness of such an algorithm and of the fault tolerant power converters are finally verified by means of simulations

Modeling of the Vela complex including the Vela supernova remnant, the binary system gamma2 Velorum, and the Gum nebula

We study the geometry and dynamics of the Vela complex including the Vela supernova remnant (SNR), the binary system gamma2 Velorum and the Gum nebula. We show that the Vela SNR belongs to a subclass of non-Sedov adiabatic remnants in a cloudy interstellar medium (ISM), the dynamics of which is determined by the heating and evaporation of ISM clouds. We explain observable characteristics of the Vela SNR with a SN explosion with energy 1.4 x 10^50 ergs near the step-like boundary of the ISM with low intercloud densities (~ 10^{-3} cm^{-3}) and with a volume-averaged density of clouds evaporated by shock in the north-east (NE) part about four times higher than the one in the south-west (SW) part. The observed asymmetry between the NE and SW parts of the Vela SNR could be explained by the presence of a stellar wind bubble (SWB) blown by the nearest-to-the Earth Wolf-Rayet (WR) star in the gamma2 Velorum system. We show that the size and kinematics of gamma2 Velorum SWB agree with predictions of numerical calculations for the evolution of the SWB of M_ini = 35M* star. The low initial mass of the WR star in gamma2 Velorum implies that the luminosity of the nuclear line of 26Al, produced by gamma2 Velorum, is below the sensitivity of existing gamma-ray telescopes.Comment: 8 pages, 2 figures, accepted for publication in A&

Anderson-Fabry disease: a multiorgan disease.

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A . FD causes glycolipids, such as globotriaosylceramide (Gb3), to accumulate in the vascular endothelium of several organs (fig.2), including the skin, kidneys, nervous system, and heart, thereby triggering inflammation and fibrosis . These processes generally result in organ dysfunction, which is usually the first clinical evidence of FD. Patients with classic FD have various symptoms, eg, acroparesthesias, hypohidrosis, angiokeratomas, corneal opacities, cerebrovascular lesions, cardiac disorders, andrenal dysfunction.However, evolving knowledge about the natural course of disease suggests that it is more appropriate to describe FD as a disease with a wide spectrum of heterogeneously progressive clinical phenotypes. Indeed, most female heterozygotes develop symptoms due to yet undetermined mechanisms and a high percentage of females develops vital organ involvement including the kidneys, heart and/or brain about a decade later than males . Renal failure is a serious complication of this disease. Fabry nephropathy lesions are present and progress in childhood while the disease commonly remains silent by routine clinical measures. Early and timely diagnosis of Fabry nephropathy is crucial since late initiation of enzyme replacement therapy may not halt progressive renal dysfunction. This may be challenging due to difficulties in diagnosis of Fabry disease in children and absence of a sensitive non-invasive biomarker of early Fabry nephropathy. Accurate measurement of glomerular filtration rate and regular assessment for proteinuria and microalbuminuria are useful, though not sensitive enough to detect early lesions in the kidney. The principal clinical manifestationsin Fabry disease consist of artery associated complications (such as cerebral disease and nephropathy), but the pathophysiology of this specific vasculopathy is unclear. Several studies indicate that the specific vascular lesions that are present in Fabry disease occur as a result of vascular dysfunction with major components being endothelial dysfunction, alterations in cerebral perfusion and a pro-thrombotic phenotype. Fabry cardiac involvement has several clinical manifestations (table 10): concentric left ventricular hypertrophy without left ventricular dilation and severe loss of left ventricular systolic function, mitral and aortic valvulopathy, disorders of the atrioventricular conduction or repolarization, and compromised diastolic function. The neurological manifestations of Fabry disease include both peripheral nervous system and CNS involvement, with globotriaosylceramide accumulation found in Schwann cells and dorsal root ganglia together with deposits in CNS neurones. The main involvement of the CNS is attributable to cerebrovasculopathy, with an increased incidence of stroke. The abnormal neuronal accumulation of glycosphingolipid appears to have little clinical effect on the natural history of Fabry disease, with the possible exception of some reported mild cognitive abnormalities. The pathogenesis of Fabry vasculopathy remains poorly understood, but probably relates, in part, to abnormal functional control of the vessels, secondary to endothelial dysfunction as a consequence of α-galactosidase A deficiency. The diagnosis of Fabry disease is made in hemizygous males after the detection of the presence of angiokeratomas (fig 19 A, B), irregularities in sweating, edema, scant body hair, painful sensations, and of cardiovascular, gastrointestinal, renal, ophthalmologic, phlebologic, and respiratory involvement. A deficiency of alpha-gal A in serum, leukocytes, tears, tissue specimens, or cultured skin fibroblasts further supports the diagnosis in male patients. Since heterozygous women show angiokeratomas in only about 30% of cases and may have alpha-gal A levels within normal range, genetic analysis is recommended. The resultant storage of undegraded glycolipids leads to the progressive development of potentially life-threatening manifestations affecting multiple organ systems in the body. The Mainz Severity Score Index (MSSI) (table 12), a scoring system for patients with Fabry disease has been proven to be representative in patients with 'classic' Fabry disease and may be useful for monitoring clinical improvement in patients receiving enzyme replacement therapy. The MSSI of patients with AFD was significantly higher than that of patients with other severe debilitating diseases

Neurological complications of Anderson-Fabry disease

Characteristic clinical manifestations of AFD such as acroparesthesias, angiokeratoma, corneal opacity, hypo/ and anhidrosis, gastrointestinal symptoms, renal and cardiac dysfunctions can occur in male and female patients, although heterozygous females with AFD usually seems to be less severely affected. The most prominent CNS manifestations consist of cerebrovascular events such as transient ischaemic attacks (TIAs) and (recurrent) strokes . For the most part, CNS complications in AFD have been attributed to cerebral vasculopathy, including anatomical abnormalities. The natural history of Fabry patients includes transitory cerebral ischaemia and strokes, even in very young persons of both genders. The mechanism is partly due to vascular endothelial accumulation of Gb-3. White matter lesions (WML) on MRI occur. Both males and females can be safely treated with enzyme replacement; and thus screening for Fabry disease of young stroke populations should be considered. There are, however, no hard data of treatment effect on mortality and morbidity. Stroke in Anderson-Fabry disease study of 721 patients with cryptogenic stroke, aged 18-55 years, showed a high prevalence of Fabry disease in this group: 5% (21/432) of men and 3% (7/289) of women. Combining results for both sexes showed that 4% of young patients with stroke of previously unknown cause had Fabry disease, corresponding to about 1-2% of the general population of young stroke patients. Cerebral micro- and macro-vasculopathy have been described in Fabry disease. Neuronal globotriaosylceramide accumulation in selective cortical and brain stem areas including the hippocampus has been reported by autopsy studies in FD, but clinical surrogates as well as the clinical relevance of these findings have not been investigated so far. Another Neurologic hallmarks of Fabry disease (FD) include small fiber neuropathy as well as cerebral micro- and macroangiopathy with premature stroke. Cranial MRI shows progressive white matter lesions (WML) at an early age, increased signal intensity in the pulvinar, and tortuosity and dilatation of the larger vessels. Conventional MRI shows a progressive load of white matter lesions (WMLs) due to cerebral vasculopathy in the course of FD. Another study has been conducted to quantify brain structural changes in clinically affected male and female patients with FD. The peripheral neuropathy in Fabry disease manifests as neuropathic pain, reduced cold and warm sensation and possibly gastrointestinal disturbances. Patients with Fabry disease begin having pain towards the end of the first decade of life or during puberty. Children as young as 6 years of age have complained of pain often associated with febrile illnesses with reduced heat and exercise tolerance. The patients describe the pain as burning that is often associated with deep ache or paresthesiae. Some patients also have joint pain. A high proportion of patients with Fabry disease is at increased risk of developing neuropsychiatric symptoms, such as depression and neuropsychological deficits. Due to both somatic and psychological impairment, health-related quality of life (QoL) is considerably reduced in patients with Fabry disease. Targeted screening for Fabry disease among young individuals with stroke seems to disclose unrecognized cases and may therefore very well be recommended as routine in the future. Furthermore, ischemic stroke is related to inflammation and arterial stiffness [and no study had addressed this relationship in patients with AF disease and cerebrovascular disease, so this topic could represent a possible future research line

Pathogenesis and molecular mechanisms of anderson–fabry disease and possible new molecular addressed therapeutic strategies

Anderson–Fabry disease (AFD) is a rare disease with an incidenceof approxi-mately 1:117,000 male births. Lysosomal accumulation of globotriaosylceramide (Gb3) is the element characterizing Fabry disease due to a hereditary deficiency α-galactosidase A (GLA) enzyme. The accumulation of Gb3 causes lysosomal dysfunction that compromises cell signaling pathways. Deposition of sphingolipids occurs in the autonomic nervous system, dorsal root ganglia, kidney epithelial cells, vascular system cells, and myocardial cells, resulting in organ failure. This manuscript will review the molecular pathogenetic pathways involved in Anderson–Fabry disease and in its organ damage. Some studies reported that inhibition of mitochondrial function and energy metabolism plays a signif-icant role in AFD cardiomyopathy and in kidney disease of AFD patients. Furthermore, mitochondrial dysfunction has been reported as linked to the dysregulation of the au-tophagy–lysosomal pathway which inhibits the mechanistic target of rapamycin kinase (mTOR) mediated control of mitochondrial metabolism in AFD cells. Cerebrovascular complications due to AFD are caused by cerebral micro vessel stenosis. These are caused by wall thickening resulting from the intramural accumulation of glycolipids, luminal oc-clusion or thrombosis. Other pathogenetic mechanisms involved in organ damage linked to Gb3 accumulation are endocytosis and lysosomal degradation of endothelial calcium-activated intermediate-conductance potassium ion channel 3.1 (KCa3.1) via a clathrin-de-pendent process. This process represents a crucial event in endothelial dysfunction. Several studies have identified the deacylated form of Gb3, globotriaosylsphingosine (Lyso-Gb3), as the main catabolite that increases in plasma and urine in patients with AFD. The mean concentrations of Gb3 in all organs and plasma of Galactosidase A knockout mice were significantly higher than those of wild-type mice. The distributions of Gb3 isoforms vary from organ to organ. Various Gb3 isoforms were observed mainly in the kidneys, and kidney-specific Gb3 isoforms were hydroxylated. Furthermore, the action of Gb3 on the KCa3.1 channel suggests a possible contribution of this interaction to the Fabry disease process, as this channel is expressed in various cells, including endothelial cells, fibro-blasts, smooth muscle cells in proliferation, microglia, and lymphocytes. These molecular pathways could be considered a potential therapeutic target to correct the enzyme in ad-dition to the traditional enzyme replacement therapies (ERT) or drug chaperone therapy

Predicted gamma-ray image of SN 1006 due to inverse Compton emission

We propose a method to synthesize the inverse Compton (IC) γ-ray image of a supernova remnant starting from the radio (or hard X-ray) map and using results of the spatially resolved X-ray spectral analysis. The method is successfully applied to SN 1006. We found that synthesized IC γ-ray images of SN 1006 show morphology in nice agreement with that reported by the High Energy Stereoscopic System (HESS) collaboration. The good correlation found between the observed very high energy γ-ray and X-ray/radio appearance can be considered as evidence of the fact that the γ-ray emission of SN 1006 observed by HESS is leptonic in origin, although a hadronic origin may not be excluded.Fil: Petruk, O.. Institute for Applied Problems in Mechanics and Mathematics; UcraniaFil: Bocchino, F.. Istituto Nazionale Di Astrofísica. Osservatorio Astronómico Di Palermo; ItaliaFil: Miceli, M.. Istituto Nazionale Di Astrofísica. Osservatorio Astronómico Di Palermo; ItaliaFil: Dubner, Gloria Mabel. Consejo Nacional de Investigaciónes Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Astronomía y Física del Espacio. - Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Astronomía y Física del Espacio; ArgentinaFil: Castelletti, Gabriela Marta. Consejo Nacional de Investigaciónes Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Astronomía y Física del Espacio. - Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Astronomía y Física del Espacio; ArgentinaFil: Orlando, S.. Istituto Nazionale Di Astrofísica. Osservatorio Astronómico Di Palermo; ItaliaFil: Iakubovskyi, D.. Bogolyubov Institute for Theoretical Physics; UcraniaFil: Telezhinsky, I.. Kiev National Taras Shevchenko University; Ucrani

Model for the low-temperature magnetic phases observed in doped YBa_2Cu_3O_{6+x}

A classical statistical model for the antiferromagnetic (AFM) ordering of the Cu-spins in the CuO_2 planes of reduced YBa_2Cu_3O_{6+x} type materials is presented. The magnetic phases considered are the experimentally observed high-temperature AFI phase with ordering vector Q_I=(1/2,1/2,0), and the low-temperature phases: AFII with Q_II=(1/2,1/2,1/2) and intermediate TA (Turn Angle) phases TAI, TAII and TAIII with components of both ordering vectors. It is shown that the AFII and TA phases result from an effective ferromagnetic (FM) type coupling mediated by free spins in the CuO_x basal plane. Good agreement with experimental data is obtained for realistic model parameters.Comment: 11 pages, 2 Postscript figures, Submitted to Phys.Rev.Let

A new dedicated clinic for HCWs' counseling and vaccination: experience of an academic hospital

Abstract Issue Despite low healthcare workers (HCWs) vaccination coverage being a risk for hospital outbreaks, vaccine hesitancy is not unusual among HCWs. In Italy vaccinations are strongly recommended for HCWs, but there are few occasions for a dedicated counseling. Aim of the study is to evaluate the effectiveness of a new vaccination service in the academic hospital of Udine (northern Italy) in tackling vaccine hesitancy among HCWs. Description of the problem Available data on HCWs specific antibody titers revealed that in high-risk units, 25% of HCWs were certainly unprotected for at least 1/6 of the vaccine preventable diseases (VPDs): measles, rubella, mumps, varicella, pertussis, hepatitis B; only varicella coverage reached the herd immunity target. Periodic occupational health visit was the only moment to screen for VPDs protection and suggest vaccination, but the following inconvenient procedure of HCWs contacting the vaccination office outside the hospital, often lead to delays or loss. In order to improve vaccination adherence, since June 2019 a dedicated clinic has been set up inside the hospital, making vaccination counseling and administration available every two weeks, with appointments directly given by the occupational doctor. Results From June 2019 to February 2020, a total of 362 appointments were booked for the dedicated vaccination clinic, 69.7% of which actually took place as 252 HCWs actually accessed the service. Hours dedicated to the service activity were 76 hours, distributed over 19 days. Administered vaccination were 322, including 107 MMR (measles, rubella, mumps), 4 MMRV (MMR+varicella), 20 varicella, 64 hepatitis B, 127 DTPa (diphtheria, tetanus, pertussis). Lessons Making the access to vaccination more convenient in term of service location within the hospital and giving the appointment when performing the occupational health visit seems to be helpful in filling the VPDs protection among HCWs gap. Key messages Monitoring immunological status of HCWs and promoting vaccination at occupational health visit would sustain herd immunity protection for susceptible individuals in healthcare settings. The dedicated hospital vaccination clinic and the effective procedure of giving the appointment during the occupational health visit could be helpful in improving HCWs vaccine adherence