Abnormalities of the immune system are rarely reported in patients affected by RASopathies. Aim of the current study was to investigate the prevalence of immune system dysfunction in a cohort of ...patients affected by RASopathies.
A group of 69 patients was enrolled: 60 at the Federico II University, Naples, 7 at University Magna Graecia of Catanzaro, 2 at "Scuola Medica Salernitana", Salerno. An age- and sex-matched control group was also enrolled. Autoimmune disorders were investigated according to international consensus criteria. Immune framework was also evaluated by immunoglobulin levels, CD3, CD4, CD8, CD19, CD56 lymphocyte subpopulations, autoantibodies levels and panel of inflammatory molecules, in both patients and controls.
Frequent upper respiratory tract infections were recorded in 2 patients; pneumonia, psoriasis and alopecia in single patients. Low IgA levels were detected in 8/44 patients (18.18%), low CD8 T cells in 13/35 patients (37.14%). Anti-tg and anti-TPO antibodies were detected in 3/24 patients (12.5%), anti r-TSH in 2 cases (8.33%), all in euthyroidism. Serum IgA and CD8 levels were significantly lower in patients than in controls (p 0.00685; p 0.000656 respectively). All tested patients showed increased inflammatory molecules compared to controls. These findings may anticipate the detection of overt autoimmune disease.
Patients affected by RASopathies are at risk to develop autoimmune disorders. Routine screening for autoimmunity is recommended in patients with RASopathy.
We describe an innovative data transmission scheme exploiting optical vortices to multiplex and demultiplex independent data channels in a standard asynchronous laser link. We report extensive ...results of the proof of concept of the method, successfully used to transmit two parallel ASCII strings, demultiplexed and decoded in the far field of the radiation beam. A phase locked two arms interferometer is proved to be effective even accessing a small portion of the beam only. Results prove the robustness and reliability of the method to perform dense-code free space transmissions over long distances even in presence of wavefront distortions. Applications and the extension to a larger number of parallel channels are discussed.
We describe a method for effectively distinguishing the radiation endowed with optical angular momentum, also known as optical vortex, from ordinary light. We show that by detecting the inversion of ...the transverse intrinsic curvature sign (ITICS) an optical vortex can be locally recognized. The method is effective under conditions of huge importance for the exploitation of optical vortices, such as the far field of the source and access to a small fraction of the wavefront only. The validity of the method has been verified with table-top experiments with visible light, and the results show that a measurement performed over a transverse distance smaller than 4% of the beam diameter distinguishes a vortex from a Gaussian beam with a significance of 93.4%. New perspectives are considered for the characterization of vortices, with potential impact on the detection of extra-terrestrial radiation as well as on broadcast communication techniques.
•The dynamic model of the AC/DC shipboard microgrid comprises diesel engines or gas turbines that provide power for the rotation of synchronous or asynchronous generators. Next, the AC output voltage ...of the generators is turned into DC voltage with the use of AC to DC converters.•The dynamic model of the considered shipboard AC/DC microgrid, being initially expressed in a nonlinear and multivariable state-space form, undergoes approximate linearization around a temporary operating point that is recomputed at each time-step of the control method.•The linearization relies on first-order Taylor series expansion and on the computation of the associated Jacobian matrices. For the linearized state-space model of the shipboard AC/DC microgrid a stabilizing optimal (H-infinity) feedback controller is designed.•This controller stands for the solution to the nonlinear optimal control problem of the AC/DC microgrid under model uncertainty and external perturbations. To compute the controller’s feedback gains an algebraic Riccati equation is repetitively solved at each iteration of the control algorithm.•The global stability properties of the control method are proven through Lyapunov analysis. Finally, to implement state estimation-based control of the shipboard AC/DC microgrid, without the need to measure its entire state vector, the H-infinity Kalman Filter is used as a robust state estimator.•The article’s method provides one of the few algorithmically simple and computationally efficient solutions for the nonlinear optimal control problem of shipboard microgrids. Comparison to flatness-based control is provided.
Shipboard AC/DC microgrids are used for power supply and electric propulsion in vessels. An indicative form of such a microgrid comprises diesel engines or gas turbines that provide power for the rotation of synchronous or asynchronous generators. Next, the AC output voltage of the generators is turned into DC voltage with the use of AC to DC converters and is distributed through DC voltage buses to the ship’s compartments. Besides, with the use of DC to AC inverters voltage excitation is provided to synchronous or asynchronous motors which can be used in turn for the vessel’s propulsion. The dynamic model of the considered shipboard AC/DC microgrid, being initially expressed in a nonlinear and multivariable state-space form, undergoes approximate linearization around a temporary operating point that is recomputed at each time-step of the control method. The linearization relies on first-order Taylor series expansion and on the computation of the associated Jacobian matrices. For the linearized state-space model of the shipboard AC/DC microgrid a stabilizing optimal (H-infinity) feedback controller is designed. This controller stands for the solution to the nonlinear optimal control problem of the AC/DC microgrid under model uncertainty and external perturbations. To compute the controller’s feedback gains an algebraic Riccati equation is repetitively solved at each iteration of the control algorithm. The global stability properties of the control method are proven through Lyapunov analysis. Finally, to implement state estimation-based control of the shipboard AC/DC microgrid, without the need to measure its entire state vector, the H-infinity Kalman Filter is used as a robust state estimator. The article’s method provides one of the few algorithmically simple and computationally efficient solutions for the nonlinear optimal control problem of shipboard microgrids.
Among several approaches to tackle the problem of energy consumption in modern computing systems, two solutions are currently investigated: one consists of artificial neural networks (ANNs) based on ...photonic technologies, the other is a different paradigm compared to ANNs and it is based on random networks of non-linear nanoscale junctions resulting from the assembling of nanoparticles or nanowires as substrates for neuromorphic computing. These networks show the presence of emergent complexity and collective phenomena in analogy with biological neural networks characterized by self-organization, redundancy, and non-linearity. Starting from this background, we propose and formalize a generalization of the perceptron model to describe a classification device based on a network of interacting units where the input weights are non-linearly dependent. We show that this model, called “receptron”, provides substantial advantages compared to the perceptron as, for example, the solution of non-linearly separable Boolean functions with a single device. The receptron model is used as a starting point for the implementation of an all-optical device that exploits the non-linearity of optical speckle fields produced by a solid scatterer. By encoding these speckle fields we generated a large variety of target Boolean functions. We demonstrate that by properly setting the model parameters, different classes of functions with different multiplicity can be solved efficiently. The optical implementation of the receptron scheme opens the way for the fabrication of a completely new class of optical devices for neuromorphic data processing based on a very simple hardware.
FOCUS (Fast Monte CarlO approach to Coherence of Undulator Sources) is a new GPU‐based simulation code to compute the transverse coherence of undulator radiation from ultra‐relativistic electrons. ...The core structure of the code, which is written in the language C++ accelerated with CUDA, combines an analytical description of the emitted electric fields and massively parallel computations on GPUs. The combination is rigorously justified by a statistical description of synchrotron radiation based on a Fourier optics approach. FOCUS is validated by direct comparison with multi‐electron Synchrotron Radiation Workshop (SRW) simulations, evidencing a reduction in computation times by up to five orders of magnitude on a consumer laptop. FOCUS is then applied to systematically study the transverse coherence in typical third‐ and fourth‐generation facilities, highlighting peculiar features of undulator sources close to the diffraction limit. FOCUS is aimed at fast evaluation of the transverse coherence of undulator radiation as a function of the electron beam parameters, to support and help prepare more advanced and detailed numerical simulations with traditional codes like SRW.
FOCUS (Fast Monte CarlO approach to Coherence of Undulator Sources), a new GPU‐based code to compute the transverse coherence of X‐ray radiation from undulator sources as a function of the electron beam parameters, is described. FOCUS is validated with the Synchrotron Radiation Workshop (SRW) and SPECTRA codes. Examples of application to coherence studies in third‐ and fourth‐generation light sources are shown.
Endocrine complications have been described in patients affected by RASopathies but no systematic assessment has been reported. In this study, we investigate the prevalence of endocrine disorders in ...a consecutive unselected cohort of patients with RASopathies.
72 patients with a genetically confirmed RASopathy (Noonan syndrome NS, N=53; 29 LEOPARD syndrome LS, N=2; cardiofaciocutaneous syndrome CFCS, N=14; subjects showing co-occurring pathogenic variants in
and
, N=3) and an age- and sex-matched healthy controls were included in the study. Endocrine system involvement was investigated by assessing the thyroid function, pubertal development, auxological parameters, adrenal function and bone metabolism.
Short stature was detected in 40% and 64% of the NS and CFCS subcohorts, respectively. Patients showed lower Z-scores at DXA than controls (p<0.05) when considering the entire case load and both NS and CFCS groups. Vitamin D and Calcitonin levels were significantly lower (p< 0.01), Parathormone levels significantly higher (p<0.05) in patients compared to the control group (p<0.05). Patients with lower BMD showed reduced physical activity and joint pain. Finally, anti-TPO antibody levels were significantly higher in patients than in controls when considering the entire case load and both NS and CFCS groups.
The collected data demonstrate a high prevalence of thyroid autoimmunity, confirming an increased risk to develop autoimmune disorders both in NS and CFCS. Reduced BMD, probably associated to reduced physical activity and inflammatory cytokines, also occurs. These findings are expected to have implications for the follow-up and prevention of osteopenia/osteoporosis in both NS and CFCS.
A fascinating way of generating speckle patterns is by interfering the weak fields scattered by a disordered sample with the intense trans-illuminating beam. The resulting intensity fluctuations are ...known as Heterodyne Near Field Speckles. Thanks to the self-referencing layout, the intensity distribution allows direct assessment of the electric fields, thus preserving both amplitude and phase information. Originally observed with visible laser light, during the last years Heterodyne Near Field Speckles have been extended to partially coherent radiation and to X-ray beams. We give in this review a uniform argumentation of Heterodyne Near Field Speckles based on Fourier Optics, valid with both coherent and partially coherent illumination. Emphasis is given to the speckle size, a fundamental property of any speckle field and a basis for earlier and state-of-the-art development of the technique. We review the applications of Heterodyne Near Field Speckles in the fields of particle sizing, velocimetry, coherence measurements, X-ray wavefront sensing and X-ray phase-contrast imaging and tomography. Throughout the discussion, we also emphasize the common aspects shared with many different research areas, such as astronomical observations, holography and TEM imaging, thus evidencing the encompassing nature of the underlying physical principles.
We exploit the speckle field generated by scattering from a colloidal suspension to access both spatial and temporal coherence properties of broadband radiation. By applying the Wiener-Khinchine ...theorem to the retrieved temporal coherence function, information about the emission spectrum of the source is obtained in good agreement with the results of a grating spectrometer. Experiments have been performed with visible light. We prove more generally that our approach can be considered as a tool for modeling a variety of cases. Here we discuss how to apply such diagnostics to broad-spectrum betatron radiation produced in the laser-driven wakefield accelerator under development at SPARC LAB facility in Frascati.
As the quality and quantity of patient-centered care may be perceived differently by recipients and independent observers, assessment of humanization of pediatric care remains an elusive issue. ...Herein we aim to analyze differences between the degrees of verified existing vs. perceived humanization issues of a pediatric ward. Furthermore, we examine whether there is concurrence between the degrees of humanization perceived by users (parents/visitors) vs. staff members.
The study was conducted in the pediatric wards of seven medical centers of the Campania region (Italy) categorized as general (n = 4), children's (n = 1), and university (n = 2) hospitals. The degree of existing humanization was assessed by a multidisciplinary focus group for each hospital through a pediatric care-oriented checklist specifically developed to individuate the most critical areas (i.e., those with scores < 2.5). The degree of perceived humanization was assessed through four indicators: well-being, social aspects, safety and security, and health promotion.
The focus groups showed that critical areas common to all centers were mainly concerned with welfare, mediation, translation, and interpretation services. Specific critical issues were care and organizational processes oriented to the respect and specificity of the person and care of the relationship with the patient. Perceived humanization questionnaires revealed a lack of recreational facilities and mediation and translation services. As for specific features investigated by both tools, it was found that mediation and interpretation services were lacking in all facilities while patient perceptions and observer ratings for space, comfort, and orientation concurred only in the general hospital evaluations.
Future humanization interventions to ensure child- and family-friendly hospital care call for careful preliminary assessments, tailored to each pediatric ward category, which should consider possible differences between perceived and verified characteristics.
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