Search

Halide perovskites (HPs) recently have emerged as one class of competitive scintillators for X‐ray detection and imaging owing to its high quantum efficiency, short decay time, superior X‐ray absorption capacity, low cost, and ease of crystal growth. The tunable structure and versatile chemical compositions of halide perovskites provide distinguishable advantages over traditional inorganic scintillators for optimizing scintillation performance. Since the first observation of the scintillation phenomenon in HPs, substantial efforts have been devoted to expanding the inventory of HP scintillators and regulating material properties. Understanding the relationship between the structure and scintillation properties of HP scintillators is essential for developing materials with improved X‐ray detection and imaging capacities. This review summarizes strategies for improving the light yield of HP scintillators and provides a roadmap for improving the X‐ray imaging performance. Additionally, methods for controlling the light propagation direction in HP scintillators are highlighted for improving X‐ray imaging resolution. Finally, we highlight the current challenge in HP scintillators and provide a perspective on the future development of this emerging scintillator. [ABSTRACT FROM AUTHOR]

A great deal of evidence has revealed an important link between gut microbiota and the heart. In particular, the gut microbiota plays a key role in the onset of cardiovascular (CV) disease, including heart failure (HF). In HF, splanchnic hypoperfusion causes intestinal ischemia resulting in the translocation of bacteria and their metabolites into the blood circulation. Among these metabolites, the most important is Trimethylamine N-Oxide (TMAO), which is responsible, through various mechanisms, for pathological processes in different organs and tissues. In this review, we summarise the complex interaction between gut microbiota and CV disease, particularly with respect to HF, and the possible strategies for influencing its composition and function. Finally, we highlight the potential role of TMAO as a novel prognostic marker and a new therapeutic target for HF. [ABSTRACT FROM AUTHOR]

We realize single-mode, waveguide-coupled, electrically driven silicon light emitting diodes in commercial, unmodified silicon photonics foundry processes and develop a model of both the electrical and optical behavior to understand the performance limitations. We measure a center wavelength of 1130 nm, a 90 nm 3 dB optical bandwidth, and 200 pW of optical power propagating in each direction. We show on-chip modulation and detection of the generated light using native resonant photodetectors integrated in the same chip. Our work unveils a new native light source available in silicon photonics processes, which can find applications ranging from device screening and fabrication quality assessment to imaging, refractive index sensing, or intra-chip communication. [ABSTRACT FROM AUTHOR]

With the advancements in mobile technology, a large amount of data is generated by end devices, which has created a renewed interest in developing new AI-based applications for gaining insights into these data. However, most of these distributed applications need data aggregated at a central server, which has posed severe bandwidth, latency, security, and privacy issues. This paper presents OSGAN (One-Shot distributed learning algorithm using Generative Adversarial Networks), a generic framework that trains a generative adversarial network (GAN) at each client and uses GAN's generative capabilities to create sample data at the server. The server aggregates these data from various clients, builds a deep learning model and sends its parameters back to the clients in one communication round, i.e. the exchange of information between the clients and the server happens only once. In this paper, we present the design and implementation of OSGAN and evaluate its performance by comparing it with the state-of-the-art federated learning (FL) and central training algorithms for both IID and non-IID distribution of data. Our experiments on multiple datasets show that our proposed approach achieves a similar accuracy when compared with both FL and central training algorithms. Specifically, the accuracy drop with OSGAN is maintained within 2% for multiple datasets and multiple numbers of clients. Our results show that the proposed approach reduces the amount of data transfer by almost 98% when compared with federated learning and close to 80% when compared with the central learning approach thereby providing substantial benefit in terms of saving the bandwidth. [ABSTRACT FROM AUTHOR]

Simple Summary: Pancreatic ductal adenocarcinoma remains one of the most serious malignancies and a leading cause of cancer-related deaths worldwide. There are no effective screening methods available so far, even for high-risk individuals. At the time of diagnosis, impaired glucose metabolism is present in about 3/4 of all patients. Several types of diabetes mellitus can be found in pancreatic cancer; however, type 2, pancreatic-cancer-associated type 3c, and diabetes mellitus associated with non-malignant diseases of the exocrine pancreas (with a reduction or loss of islet-cell mass) are the most frequent ones. This paper proposed a distinct approach to older subjects with new-onset diabetes mellitus with possible pancreatic cancer. It could improve the current unsatisfactory situation in diagnostics and subsequent poor outcomes of treatment of pancreatic ductal adenocarcinoma. Background: Pancreatic ductal adenocarcinoma (PDAC) is associated with a very poor prognosis, with near-identical incidence and mortality. According to the World Health Organization Globocan Database, the estimated number of new cases worldwide will rise by 70% between 2020 and 2040. There are no effective screening methods available so far, even for high-risk individuals. The prognosis of PDAC, even at its early stages, is still mostly unsatisfactory. Impaired glucose metabolism is present in about 3/4 of PDAC cases. Methods: Available literature on pancreatic cancer and diabetes mellitus was reviewed using a PubMed database. Data from a national oncology registry (on PDAC) and information from a registry of healthcare providers (on diabetes mellitus and a number of abdominal ultrasound investigations) were obtained. Results: New-onset diabetes mellitus in subjects older than 60 years should be an incentive for a prompt and detailed investigation to exclude PDAC. Type 2 diabetes mellitus, diabetes mellitus associated with chronic non-malignant diseases of the exocrine pancreas, and PDAC-associated type 3c diabetes mellitus are the most frequent types. Proper differentiation of particular types of new-onset diabetes mellitus is a starting point for a population-based program. An algorithm for subsequent steps of the workup was proposed. Conclusions: The structured, well-differentiated, and elaborately designed approach to the elderly with a new onset of diabetes mellitus could improve the current situation in diagnostics and subsequent poor outcomes of therapy of PDAC. [ABSTRACT FROM AUTHOR]

Copyright of Nova Tellus is the property of Instituto de Investigaciones Filologicas - UNAM and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Adenylyl cyclase 5 (ADCY5)‐related movement disorder (ADCY5‐RMD) is a rare, childhood‐onset disease resulting from pathogenic variants in the ADCY5 gene. The clinical features, diagnostic options, natural history, and treatments for this disease are poorly characterized and have never been established through a structured approach. Objective: This scoping review attempts to summarize all available clinical literature on ADCY5‐RMD. Methods: Eighty‐seven articles were selected for inclusion in this scoping review. The majority of articles identified were case reports or case series. Results: These articles demonstrate that patients with ADCY5‐RMD suffer from permanent and/ or paroxysmal hyperkinetic movements. The paroxysmal episodes can be worsened by environmental triggers, in particular the sleep–wake transition phase in the early morning. Occurrence of nocturnal paroxysmal dyskinesias and perioral twitches are highly suggestive of the diagnosis when present. In the majority of patients intellectual capacity is preserved. ADCY5‐RMD is considered a non‐progressive disorder, with inter‐individual variations in evolution with aging. Somatic mosaicism, mode of inheritance and the location of the mutation within the protein can influence phenotype. Conclusions: The current evidence for therapeutic options for ADCY5‐RMD is limited: caffeine, benzodiazepines and deep brain stimulation have been consistently reported to be useful in case reports and case series. [ABSTRACT FROM AUTHOR]

Copyright of Archives of Industrial Hygiene & Toxicology / Arhiv za Higijenu Rada I Toksikologiju is the property of Sciendo and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Clarithromycin (CLA) has been widely used in the treatment of bacterial infection. Research reveals the adverse effects on the central nervous system among patients receiving CLA treatment; whereas, a relevant underlying mechanism remains considerably unclear. According to our research, an integrated lipidomic and transcriptomic analysis was applied to explore the effect of CLA on neurobehavior. CLA treatment caused anxiety-like behaviors dose-dependently during open field as well as elevated plus maze trials on mice. Transcriptomes and LC/MS–MS–based metabolomes were adopted for investigating how CLA affected lipidomic profiling as well as metabolic pathway of the cerebral cortex. CLA exposure greatly disturbed glycerophospholipid metabolism and the carbon chain length of fatty acids. By using whole transcriptome sequencing, we found that CLA significantly downregulated the mRNA expression of CEPT1 and CHPT1, two key enzymes involved in the synthesis of glycerophospholipids, supporting the findings from the lipidomic profiling. Also, CLA causes changes in neuronal morphology and function in vitro, which support the existing findings concerning neurobehavior in vivo. We speculate that altered glycerophospholipid metabolism may be involved in the neurobehavioral effect of CLA. Our findings contribute to understanding the mechanisms of CLA-induced adverse effects on the central nervous system. 1. Clarithromycin treatment caused anxiety-like behavior with dose-dependent response both in the open field and elevated plus maze test in mice; 2. Clarithromycin exposing predominately disturbed the metabolism of glycerophospholipids in the cerebral cortex of mice; 3. Clarithromycin application remarkably attenuated CEPT1 and CHPT1 gene expression, which participate in the last step in the synthesis of glycerophospholipids; 4. The altered glycerophospholipid metabolomics may be involved in the abnormal neurobehavior caused by clarithromycin. [ABSTRACT FROM AUTHOR]

The discovery of prions has challenged dogmas and has revolutionized our understanding of protein-misfolding diseases. The concept of self-propagation via protein conformational changes, originally discovered for the prion protein (PrP), also applies to other proteins that exhibit similar behavior, such as alpha-synuclein (aSyn), a central player in Parkinson's disease and in other synucleinopathies. aSyn pathology appears to spread from one cell to another during disease progression, and involves the misfolding and aggregation of aSyn. How the transfer of aSyn between cells occurs is still being studied, but one important hypothesis involves receptor-mediated transport. Interestingly, recent studies indicate that the cellular prion protein (PrP C ) may play a crucial role in this process. PrP C has been shown to act as a receptor/sensor for protein aggregates in different neurodegenerative disorders, including Alzheimer's disease and amyotrophic lateral sclerosis. Here, we provide a comprehensive overview of the current state of knowledge regarding the interaction between aSyn and PrP C and discuss its role in synucleinopathies. We examine the properties of PrP and aSyn, including their structure, function, and aggregation. Additionally, we discuss the current understanding of PrP C 's role as a receptor/sensor for aSyn aggregates and identify remaining unanswered questions in this area of research. Ultimately, we posit that exploring the interaction between aSyn and PrP C may offer potential treatment options for synucleinopathies., (© 2023 The Authors. Journal of Neurochemistry published by John Wiley & Sons Ltd on behalf of International Society for Neurochemistry.)

Coronary artery disease (CAD) is the leading cause of mortality worldwide. In chronic and myocardial infarction (MI) states, aberrant levels of circulating microRNAs compromise gene expression and pathophysiology. We aimed to compare microRNA expression in chronic-CAD and acute-MI male patients in peripheral blood vasculature versus coronary arteries proximal to a culprit area. Blood from chronic-CAD, acute-MI with/out ST segment elevation (STEMI/NSTEMI, respectively), and control patients lacking previous CAD or having patent coronary arteries was collected during coronary catheterization from peripheral arteries and from proximal culprit coronary arteries aimed for the interventions. Random coronary arterial blood was collected from controls; RNA extraction, miRNA library preparation and Next Generation Sequencing followed. High concentrations of microRNA-483-5p (miR-483-5p) were noted as 'coronary arterial gradient' in culprit acute-MI versus chronic-CAD (p = 0.035) which were similar to controls versus chronic-CAD (p < 0.001). Meanwhile, peripheral miR-483-5p was downregulated in acute-MI and chronic-CAD, compared with controls (1.1 ± 2.2 vs. 2.6 ± 3.3, respectively, p < 0.005). A receiver operating characteristic curve analysis for miR483-5p association with chronic CAD demonstrated an area under the curve of 0.722 (p < 0.001) with 79% sensitivity and 70% specificity. Using in silico gene analysis, we detected miR-483-5p cardiac gene targets, responsible for inflammation (PLA2G5), oxidative stress (NUDT8, GRK2), apoptosis (DNAAF10), fibrosis (IQSEC2, ZMYM6, MYOM2), angiogenesis (HGSNAT, TIMP2) and wound healing (ADAMTS2). High miR-483-5p 'coronary arterial gradient' in acute-MI, unnoticed in chronic-CAD, suggests important local mechanisms for miR483-5p in CAD in response to local myocardial ischemia. MiR-483-5p may have an important role as a gene modulator for pathologic and tissue repair states, is a suggestive biomarker, and is a potential therapeutic target for acute and chronic cardiovascular disease. [ABSTRACT FROM AUTHOR]

Background: In opaque orthographies, such as English, children with low reading skills tend to rely more on semantic information due to their inadequate acquisition of sub‐lexical knowledge. This tendency has also been reported for kanji, a non‐alphabetic and opaque Japanese orthography. However, previous studies on this phenomenon have had methodological limitations, such as a small number of stimuli in reading tests and insufficient investigation of a consistency effect. This study addressed these limitations and aimed to clarify whether Japanese children with low reading accuracy are characterised by a stronger reliance on semantic information and a smaller contribution to the reading process based on character‐to‐sound correspondences to the kanji word‐reading performance than children with high reading accuracy in reading kanji words. Methods: A total of 129 Japanese students in the fifth and sixth grades participated in this study, and 11 of them had been previously diagnosed with developmental dyslexia. They read an experimenter‐created list of kanji words. We tested how frequency, imageability and consistency of character‐to‐sound correspondences affected children's reading accuracy and error types and how the effects of these variables were modulated by reading accuracy level. Results: For children with lower reading accuracy, the frequency and consistency effects on reading accuracy decreased, whereas the imageability effect was stronger. Children with low reading accuracy frequently did not respond, whereas children with high reading accuracy made word substitution and legitimate alternative reading of component (LARC) errors frequently. Conclusions: The reading processing of children with low reading accuracy is characterised by a stronger reliance on semantic information and a smaller contribution of a reading process based on character‐to‐sound correspondences to reading performance than that of children with high reading accuracy. Reading characteristics of children with low reading accuracy might be due to their inadequate lexical and sub‐word knowledge. Highlights: What is already known about this topicJapanese children with developmental dyslexia show a larger imageability effect than children without reading deficits, thus suggesting a heavier reliance on semantic information when reading kanji words (Akashi et al., 2013).Compared with Japanese children without reading deficits, Japanese children with developmental dyslexia frequently make two types of errors: word substitution errors (i.e. reading a target word as another word) and no response (Akashi et al., 2013).The percentage of legitimate alternative reading of component (LARC) errors in Japanese children with developmental dyslexia was lower compared with their peers without reading deficits, which suggests a lack of sub‐word knowledge, that is, character‐to‐sound correspondences (Akashi et al., 2013). What this paper addsIn addition to a larger imageability effect and fewer LARC errors, the reading performance of children with low reading scores was characterised by smaller effects of frequency and consistency on reading accuracy compared with children with high reading scores.In addition to LARC errors, children with high reading scores made word substitution errors more frequently than those with low reading scores, which is inconsistent with Akashi et al. (2013).This study showed that a process using semantic information and one based on character‐to‐sound correspondences could affect reading accuracy in Japanese children. Implications for theory, policy or practiceThis study elucidated the kanji word‐reading characteristics of children with low reading scores. The results will enable teachers to identify children who have difficulty reading kanji words based on their reading characteristics.Our findings suggest that we should develop kanji word‐reading interventions to improve sub‐word and lexical reading skills in Japanese children with low reading accuracy because their reading characteristics are mostly affected by a lack of orthography–phonology correspondences at the word and sub‐word levels. To improve sub‐word reading skills, teachers can teach multiple pronunciations of each character and which pronunciation of a character is the most frequent among compound words with the character. To improve lexical reading skills, focusing on orthography–phonology and orthography–semantics associations at the word level by looking at a printed word, pronouncing it and saying its meaning multiple times may be helpful. [ABSTRACT FROM AUTHOR]

In catalysis, determining the relationship between the dynamic electronic and atomic structure of the catalysts and the catalytic performance under actual reaction conditions is essential to gain a deeper understanding of the reaction mechanism since the structure evolution induced by the absorption of reactants and intermediates affects the reaction activity. Hard x-ray spectroscopy methods are considered powerful and indispensable tools for the accurate identification of local structural changes, for which the development of suitable in situ reaction cells is required. However, the rational design and development of spectroscopic cells is challenging because a balance between real rigorous reaction conditions and a good signal-to-noise ratio must be reached. Here, we summarize the in situ cells currently used in the monitoring of thermocatalysis, photocatalysis, and electrocatalysis processes, focusing especially on the cells utilized in the BL14W1-x-ray absorption fine structure beamline at the Shanghai Synchrotron Radiation Facility, and highlight recent endeavors on the acquisition of improved spectra under real reaction conditions. This review provides a full overview of the design of in situ cells, aiming to guide the further development of portable and promising cells. Finally, perspectives and crucial factors regarding in situ cells under industrial operating conditions are proposed. [ABSTRACT FROM AUTHOR]

SESAME (Synchrotron-light for Experimental Science and Applications in the Middle East) is a third-generation synchrotron light source with the capacity to develop experimental facilities on bending magnet and insertion devices. Officially opened in Allan (Jordan) on 16 May 2017, SESAME is a cooperative venture by scientists and governments of the region, the first synchrotron light source in the Middle East and neighbouring countries, and the region's first major international centre of excellence for research. [ABSTRACT FROM AUTHOR]

Underwater acoustic communications are limited by the following channel impairments: time variability, narrow bandwidth, multipath, frequency selective fading and the Doppler effect. Orthogonal Frequency Division Modulation (OFDM) is recognized as an effective solution to such impairments, especially when optimally designed according to the propagation conditions. On the other hand, OFDM implementation requires accurate channel knowledge atboth transmitter and receiver sides. Long propagation delay may lead to outdated channel information. In this work, we present an adaptive OFDM scheme where channel state information is predicted through a Kalman-like filter so as to optimize communication parameters, including the cyclic prefix length. This mechanism aims to mitigate the variability of channel delay spread. This is cast in a protocol where channel estimation/prediction are jointly considered, so as to allow efficiency. The performance obtained through extensive simulations using real channels and interference show the effectiveness of the proposed scheme, both in terms of rate and reliability, at the expense of an increasing complexity. However, this solution is significantly preferable to the conventional mechanism, where channel estimation is performed only at the receiver, with channel coefficients sent back to the transmit node by means of frequent overhead signaling. [ABSTRACT FROM AUTHOR]

The processes of biotransformation of pantothenic acid (Pan) in the biosynthesis and hydrolysis of CoA, key role of pantothenate kinase (PANK) and CoA synthetase (CoASY) in the formation of the priority mitochondrial pool of CoA, with a high metabolic turnover of the coenzyme and limited transport of Pan across the blood-brain barrier are considered. The system of acetyl-CoA, a secondary messenger, which is the main substrate of acetylation processes including formation of N-acetyl aspartate and acetylcholine, post-translational modification of histones, predetermines protection of the neurons against degenerative signals and cholinergic neurotransmission. Biochemical mechanisms of neurodegenerative syndromes in the cases of PANK and CoASY defects, and the possibility of correcting of CoA biosynthesis in the models with knockouts of these enzymes have been described. The data of a post-mortem study of the brains from the patients with Huntington's and Alzheimer's diseases are presented, proving Pan deficiency in the CNS, which is especially pronounced in the pathognomonic neurostructures. In the frontal cortex of the patients with Parkinson's disease, combined immunofluorescence of anti-CoA- and anti-tau protein was detected, reflecting CoAlation during dimerization of the tau protein and its redox sensitivity. Redox activity and antioxidant properties of the precursors of CoA biosynthesis were confirmed in vitro with synaptosomal membranes and mitochondria during modeling of aluminum neurotoxicity accompanied by the decrease in the level of CoA in CNS. The ability of CoA biosynthesis precursors to stabilize glutathione pool in neurostructures, in particular, in the hippocampus, is considered as a pathogenetic protection mechanism during exposure to neurotoxins, development of neuroinflammation and neurodegeneration, and justifies the combined use of Pan derivatives (for example, D-panthenol) and glutathione precursors (N-acetylcysteine). Taking into account the discovery of new functions of CoA (redox-dependent processes of CoAlation of proteins, possible association of oxidative stress and deficiency of Pan (CoA) in neurodegenerative pathology), it seems promising to study bioavailability and biotransformation of Pan derivatives, in particular of D-panthenol, 4′-phospho-pantetheine, its acylated derivatives, and compositions with redox pharmacological compounds, are promising for their potential use as etiopathogenetic agents. [ABSTRACT FROM AUTHOR]

Dystonia Responding to Levodopa in A Child with Myoclonus-Dystonia Keywords: myoclonus; dystonia; SGCE; levodopa; DYT11 EN myoclonus dystonia SGCE levodopa DYT11 687 690 4 04/18/23 20230401 NES 230401 Myoclonus-dystonia (M-D) is an inherited childhood-onset movement disorder characterized by the combination of myoclonus and dystonia. It is worth noting that dopa-responsive dystonia may also present with a phenotype mimicking M-D.[11] Our case emphasizes an overlap between the two dystonia syndromes that is not clearly reflected in current classifications, leading to a lag in genetic diagnosis but not in clinical management. [Extracted from the article]

The aim of this research was to characterize cognitive abilities in patients with Glut1-Deficiency syndrome (Glut1DS) following ketogenic diet therapy (KDT). Methods: The cognitive profiles of eight children were assessed using the Wechsler Intelligence Scale (WISC-IV). The effect of ketogenic diet therapy (KDT) on individual subareas of intelligence was analyzed considering the potential influence of speech motor impairments. Results: Patients with Glut1DS showed a wide range of cognitive performance levels. Some participants showed statistically and clinically significant discrepancies between individual subdomains of intelligence. Both variables, KDT initiation as well as duration, had a positive effect on the overall IQ score. Significant correlations were partially found between the time of KDT initiation and the level of IQ scores, depending on the presence of expressive language test demands of the respective subtests of the WISC-IV. Accordingly, the participants benefited les in the linguistic cognitive domain. The discrepancies in cognitive performance profiles of patients with Glut1DS can be attributed to the possibility of a negative distortion of the results due to the influence of speech motor impairments. Conclusions: The individual access skills of test persons should be more strongly considered in test procedures for the assessment of intelligence to reduce the negative influence of motor deficits on test performance. Specific characterization and systematization of the speech disorder are indispensable for determining the severity of speech motor impairment in Glut1DS. Therefore, a stronger focus on dysarthria during diagnosis and therapy is necessary. [ABSTRACT FROM AUTHOR]

The performance of the imaging systems such as CT, SPECT and digital radiography may be investigated by the modulation transfer function (MTF) in which the spatial resolution properties have been addressed. All physical aspects and technical subjects in the imaging system may be related to the proposed function, for instance, the amount of the X-ray energy varied the MTF. In this study, an analytical-parametric function is introduced to characterize the MTF together with the estimation of the cutoff frequency (fc) that it is able to obtain the information on the technical specifications of the system. The MTF curves at the various spatial frequencies were modified by the different parameters included in the proposed function to optimize the system toward prediction and design of the affective factors to improve the spatial resolution as well as the qualified maps. The simulated results were at agreement to the experimental ones published before. For the same spatial frequency, while LSF widened the MTF dropped and cutoff decreased. Also, the best LSF-MTF pair was at E of 65 keV plus fc of 22.86 lp/mm. The MTF was improved by increasing energy threshold because the probability of distinguishing events happening far away from a given pixel is diminished. [ABSTRACT FROM AUTHOR]

Rule-enabled Internet of Things (IoT) systems operate autonomous and dynamic service scenarios through real-time events and actions based on deployed rules. For handling the increasing events and actions in the IoT networks, the computational ability can be distributed and deployed to the edge of networks. However, operating a consistent rule to provide the same service scenario in heterogeneous IoT networks is difficult because of the difference in the protocols and rule models. In this paper, we propose a transparent rule deployment approach based on the rule translator by integrating the interworking proxy to IoT platforms for operating consistent service scenarios in heterogeneous IoT networks. The rule-enabled IoT architecture is proposed to provide functional blocks in the layers of the client, rule service, IoT service, and device. Additionally, the interworking proxy is used for translating and transferring rules between IoT platforms in different IoT networks. Based on the interactions between the IoT platforms, the same service scenarios are operated in the IoT environment. Moreover, the integrated interworking proxy enables the heterogeneity of IoT frameworks in the IoT platform. Therefore, rules are deployed on IoT platforms transparently, and consistent rules are operated in heterogeneous IoT networks without considering the underlying IoT frameworks. [ABSTRACT FROM AUTHOR]

Aim: To evaluate early dystonic features in children and adolescents with SGCE‐myoclonus–dystonia. Method: In this cross‐sectional study, 49 patients (26 females and 23 males) with SGCE‐myoclonus–dystonia (aged 15y 2mo, SD 12y) with childhood‐onset (2y 10mo, SD 1y 10mo) dystonia were examined using a standardized video recorded protocol. Dystonia was rated using the Writer's Cramp and Gait Dystonia Rating Scales. Disability and impairment for handwriting and walking were also rated. Results: Dystonia was present at rest (n=1), posture (n=12), and during specific motor tasks (n=45) such as writing (n=35), walking (n=23), and running (n=20). Most children reported disability while performing these tasks. Early dystonic patterns were identified for writer's cramp and gait dystonia, the latter named the 'circular shaking leg', 'dragging leg', and 'hobby‐horse gait' patterns. Sensory tricks were used by five and eight children to improve dystonia and myoclonus during writing and walking respectively. The rating scales accurately measured the severity of action dystonia and correlated with self‐reported disability. Interpretation: Children with SGCE‐myoclonus–dystonia show recognizable dystonic patterns and sensory tricks that may lead to an early diagnosis and timely therapeutic approach. Isolated writer's cramp is a key feature in childhood and should prompt SCGE analysis. The proposed action dystonia scales could be used to monitor disease course and response to treatment. What this paper adds: Most children with SGCE‐myoclonus–dystonia got writer's cramp and had walking and running dystonia.Writer's cramp was a key feature and should prompt SGCE genetic investigation.'Circular shaking leg', 'dragging leg', and 'hobby‐horse gait' were recognized as early gait patterns.Children used sensory tricks to improve myoclonus and dystonia, suggesting common pathophysiological mechanisms.Action dystonia rating scales are valid tools to assess severity in children. [ABSTRACT FROM AUTHOR]

Inherited disorders of biogenic amine metabolism are genetically determined conditions resulting in dysfunctions or lack of enzymes involved in the synthesis, degradation, or transport of dopamine, serotonin, adrenaline/noradrenaline, and their metabolites or defects of their cofactor or chaperone biosynthesis. They represent a group of treatable diseases presenting with complex patterns of movement disorders (dystonia, oculogyric crises, severe/hypokinetic syndrome, myoclonic jerks, and tremors) associated with a delay in the emergence of postural reactions, global development delay, and autonomic dysregulation. The earlier the disease manifests, the more severe and widespread the impaired motor functions. Diagnosis mainly depends on measuring neurotransmitter metabolites in cerebrospinal fluid that may address the genetic confirmation. Correlations between the severity of phenotypes and genotypes may vary remarkably among the different diseases. Traditional pharmacological strategies are not disease-modifying in most cases. Gene therapy has provided promising results in patients with DYT-DDC and in vitro models of DYT/PARK-SLC6A3. The rarity of these diseases, combined with limited knowledge of their clinical, biochemical, and molecular genetic features, frequently leads to misdiagnosis or significant diagnostic delays. This review provides updates on these aspects with a final outlook on future perspectives. [ABSTRACT FROM AUTHOR]

Accurate state-of-charge (SOC) estimation is essential for maximizing the lifetime of battery-powered wireless sensor networks (WSNs). Lightweight estimation methods are widely used in WSNs due to their low measurement and computation requirements. However, accuracy of existing lightweight methods is not high, and their adaptability to different batteries and working conditions is relatively poor. This paper proposes a lightweight SOC estimation method, which applies Peukert's Law to estimate the effective capacity of the battery and then calculates the SOC by subtracting the cumulative current consumption from the estimated capacity. In order to evaluate the proposed method comprehensively, different primary batteries and working conditions (constant current, constant resistance, and emulated duty-cycle loads) are employed. Experimental results show that the proposed method is superior to existing methods for different batteries and working conditions, which mainly benefits from the ability of Peukert's Law to better model the rate-capacity effect of the batteries. [ABSTRACT FROM AUTHOR]

An acute, transient episode of left ventricular dysfunction characterizes Takotsubo syndrome. It represents about 2% of all cases of acute coronary syndrome (ACS), and occurs predominantly in postmenopausal women, generally following a significant physical or emotional stressor. It can be diagnosed based on clinical symptoms and the absence of coronary artery disease on angiography. Ventriculography remains the gold standard for the diagnosis. Despite its transitory characteristic Takotsubo syndrome should not be considered a benign condition since complications occur in almost half of the patients, and the mortality rate reaches 4-5%. Lately, it has been revealed that Takotsubo syndrome can also lead to permanent myocardial damage due to the massive release of catecholamines that leads to myocardial dysfunction. Different mechanisms have been advanced to explain this fascinating syndrome, such as plaque rupture and thrombosis, coronary spasm, microcirculatory dysfunction, catecholamine toxicity, and activation of myocardial survival pathways. Here are still several issues with Takotsubo syndrome that need to be investigated: the complex relationship between the heart and the brain, the risk of permanent myocardial damage, and the impairment of cardiomyocyte. Our review aims to elucidate the pathophysiology and the mechanisms underlying this complex disease to manage the diagnostic and therapeutic algorithms to create a functional synergy between physicians and patients. [ABSTRACT FROM AUTHOR]

Dynamics of the population of the excited Ce states responsible for the luminescence response time in Gd3Al2Ga3O12:Ce scintillating crystals is studied by revealing the dynamics of nonequilibrium carriers in the picosecond domain. Optical pump and probe technique exploiting selective excitation of structural units of the crystal and probing the induced absorption as a function of time and spectral position is exploited. A fast response within a few picoseconds due to the absorption by holes at Gd ions and by electrons occupying the first excited state of Ce ions with the intracenter relaxation time of 500 fs are identified. Trapping of nonequilibrium electrons during their migration through the matrix to the emitting Ce ions are shown to be responsible for the slow component in the population of the excited Ce state. Elimination of the slow component is evidenced even at Mg codoping as low as 10 ppm. The elimination correlates with the acceleration of the response in coincidence time resolution experiments showing potential of GAGG:Ce, Mg in medical and high-energy physics applications. [ABSTRACT FROM AUTHOR]

Photius' lexicon contains an entry on the rare adjective ἀναλφάβητος ('illiterate, ignorant') that cites Phrynichus Atticista. Based on this testimony, the whole passage has been edited as fr. 19 of Phrynichus' Praeparatio sophistica. This article demonstrates that in this lemma Photius conflates material which comes from Phrynichus and one other source, hypothetically identified with the anonymous Antiatticist lexicon, which preserves an abridged entry on ἀναλφάβητος and which Photius employed in the compilation of his lexicon. The article also explores the possibility that the work in which Phrynichus dealt with ἀναλφάβητος was not the Praeparatio sophistica but the Eclogue. This hypothesis requires challenging some assumptions concerning the transmission of Atticist lexicography in the Byzantine era, chiefly the assumption that material from the Eclogue did not circulate at Byzantium before the fourteenth century. [ABSTRACT FROM AUTHOR]

We present a spectroscopic system constituted by a Silicon Drift Detector (SDD) coupled to a CMOS charge sensitive preamplifier, named SIRIO, specifically designed to reach ultimate low noise levels. The SDD, with an active area of \13~mm^2, has been manufactured by optimizing the production processes in order to reduce the anode current, successfully reaching current densities between \17~pA/cm^2 and \25~pA/cm^2 at + \20~^\circ C for drift fields ranging from 100 V/cm to 500 V/cm. The preamplifier shows minimum intrinsic noise levels of 1.27 and 1.0 electrons r.m.s. at +\20~^\circC and -\30~^\circC, respectively. At room temperature ( + 20^\circ C) the ^55Fe 5.9 keV and the pulser lines have 136 eV and 64 eV FWHM, respectively, corresponding to an equivalent noise charge of 7.4 electrons r.m.s.; the noise threshold is at 165 eV. The energy resolution, as measured on the pulser line, ranges from 82 eV FWHM (9.4 electrons r.m.s.) at + \30~^\circ C down to 29 eV FWHM (3.3 electrons r.m.s.) at - \30~^\circ C. [ABSTRACT FROM AUTHOR]

The aim of this paper is to conduct a statistical analysis of the effects of the fillet radii on the dynamics of the falling plate using the nonintrusive spectral projection (NISP) method. The free fall of two-dimensional cards immersed in a fluid was studied using a deterministic and stochastic numerical approach. The motion is characterized by the fluid-body interaction described by coupling the Navier-Stokes and rigid body dynamic equations. The model's predictions have been validated using both experimental and numerical data available in the literature. In the stochastic simulations, the fillet radius of the plate was considered a random variable characterized by a uniform probability density function introducing, in this way, some uncertainties in the plate's trajectory. To take into account the uncertainties, we employed the NISP method based on polynomial chaos expansion. The analysis was focused on finding the ensemble mean trajectory and error bar for a confidence interval of 95 % for both tumbling and fluttering regimes. [ABSTRACT FROM AUTHOR]

Summary: The cognitive radio network (CRN) is an emerging technology where communication among secondary users (SUs) takes place in an opportunistic manner. Since, a backbone eases the routing, reduces communication overhead, and efficiently handles the dynamic changes in the network. Therefore, we intend to propose a backbone formation algorithm in CRN. However, finding optimal backbone is equivalent to find minimum connected dominating set (CDS) for the given network, which is NP‐complete. Hence, the backbone construction problem can be better handled by optimization algorithms than the deterministic ones. The bio‐inspired is an interesting category of optimization algorithms. Therefore, it has been popularly used to handle various optimization problems in wireless networks. However, it has never been used for backbone construction in CRN. The ant colony optimization (ACO) is a promising bio‐inspired method because it has been effectively used to handle many problems in wireless networks, e.g. cluster head selection, leader election, routing etc. Therefore, we use ant colony system (ACS) for backbone formation in CRN. Also, the approach helps us to find optimized path between any pair of nodes to ensure efficient communication. To the best of our knowledge, it is the first bio‐inspired backbone construction algorithm in CRN. [ABSTRACT FROM AUTHOR]

Hereditary neurological disorders (HNDs) are a clinically and genetically heterogeneous group of disorders. These disorders arise from the impaired function of the central or peripheral nervous system due to aberrant electrical impulses. More than 600 various neurological disorders, exhibiting a wide spectrum of overlapping clinical presentations depending on the organ(s) involved, have been documented. Owing to this clinical heterogeneity, diagnosing these disorders has been a challenge for both clinicians and geneticists and a large number of patients are either misdiagnosed or remain entirely undiagnosed. Contribution of genetics to neurological disorders has been recognized since long; however, the complete picture of the underlying molecular bases are under-explored. The aim of this study was to accurately diagnose 11 unrelated Pakistani families with various HNDs deploying NGS as a first step approach. Using exome sequencing and gene panel sequencing, we successfully identified disease-causing genomic variants these families. We report four novel variants, one each in, ECEL1, NALCN, TBR1 and PIGP in four of the pedigrees. In the rest of the seven families, we found five previously reported pathogenic variants in POGZ, FA2H, PLA2G6 and CYP27A1. Of these, three families segregate a homozygous 18 bp in-frame deletion of FA2H, indicating a likely founder mutation segregating in Pakistani population. Genotyping for this mutation can help low-cost population wide screening in the corresponding regions of the country. Our findings not only expand the existing repertoire of mutational spectrum underlying neurological disorders but will also help in genetic testing of individuals with HNDs in other populations. [ABSTRACT FROM AUTHOR]

Background: Global developmental delay (GDD) has a heterogeneous clinical profile among patients, accounting for approximately 1%–3% of cases in children. An increasing number of gene defects have been demonstrated to be associated with GDD; up to now, only limited studies have reported developmental disorders driven by WDR45B. Methods: Trio‐whole exome sequencing (Trio‐WES) was performed for the patient and her family. All variants with a minor allele frequency <0.01 were selected for further interpretation according to the ACMG guidelines. Candidate pathogenic variants were validated by Sanger sequencing in her family. Results: A homozygous nonsynonymous variant in WDR45B [NM_019613.4: c.677G>C (p. Arg226Thr)] was identified from the proband. The variant was absent in published databases such as gnomAD and Exome Aggregation Consortium (ExAC). The variant was predicted to be damaging for proteins and classified as VUS according to the ACMG guidelines. We reviewed the literature, and the development delay level in our case was less severe than the other reported cases. Conclusion: We reported another case with a novel homozygous variant of WDR45B and showed the heterogeneity of clinical features. [ABSTRACT FROM AUTHOR]

Scintillators display radioluminescent properties when irradiated by high‐energy photons and therefore play an essential role in radiation detection. The current inventory of scintillators is overwhelmingly represented by achiral structures, where the radioluminescence propagates isotropically after generation. Herein, we demonstrate that chiral perovskites of (R‐3AP)PbBr3Cl⋅H2O (R‐3APP) and (S‐3AP)PbBr3Cl⋅H2O (S‐3APP) emerge as a new type of scintillator displaying a distinct property of circularly polarized radioluminescence (CPRL). A high quantum yield of 27.6 %, high luminescence dissymmetric factors (glum) of 4×10−2, and high X‐ray absorption coefficients were observed for these compounds. As proof of concept, we fabricated a polarized scintillator pair by assembling chiral scintillator crystals, which provides a new strategy to attenuate optical crosstalk between the scintillators by precisely controlling the radioluminescence propagation and improves the X‐ray imaging quality at the boundary region. [ABSTRACT FROM AUTHOR]

In this paper, we present the first tests performed after the full installation of the SIDDHARTA-2 experimental apparatus on the interaction region of the DAΦNE collider at the INFN National Laboratories of Frascati. Before starting the first measurement of the kaonic deuterium 2p → 1s transition, an accurate evaluation of the background rejection was required, mainly achieved with the kaon trigger system. This run, performed in the period 04--26/05/2022 with a 4He gaseous target, confirmed the 105 order of magnitude of the rejection factor obtained with the reduced version of the setup and different machine conditions in 2021. This important outcome motivated the filling of the target cell with deuterium and the start of the measurement campaign of the kaonic deuterium 2p → 1s transition. [ABSTRACT FROM AUTHOR]

The VIP Collaboration is performing high sensitivity tests of the Pauli exclusion principle for electrons in the extremely-low cosmic background environment of the Gran Sasso underground National Laboratory of INFN. In its open-systems configuration, the experiment checks the continuously renewed symmetry state of the conductive target, constantly supplied with electrons through a direct current. Consequently, VIP is operating the sole experiment challenging the spin-statistics connection in compliance with the Messiah-Greenberg superselection rule. The strongest bounds set by the VIP-2 experiment on the Pauli exclusion principle violation probability, by exploiting a copper target, will be reviewed. The future VIP-3 experiment will be presented, the aim of which is to map the Pauli exclusion principle violation probability as a function of the atomic number of the target under test. [ABSTRACT FROM AUTHOR]

Traditional healthcare services have changed into modern ones in which doctors can diagnose patients from a distance. All stakeholders, including patients, ward boy, life insurance agents, physicians, and others, have easy access to patients' medical records due to cloud computing. The cloud's services are very cost-effective and scalable, and provide various mobile access options for a patient's electronic health records (EHRs). EHR privacy and security are critical concerns despite the many benefits of the cloud. Patient health information is extremely sensitive and important, and sending it over an unencrypted wireless media raises a number of security hazards. This study suggests an innovative and secure access system for cloud-based electronic healthcare services storing patient health records in a third-party cloud service provider. The research considers the remote healthcare requirements for maintaining patient information integrity, confidentiality, and security. There will be fewer attacks on e-healthcare records now that stakeholders will have a safe interface and data on the cloud will not be accessible to them. End-to-end encryption is ensured by using multiple keys generated by the key conclusion function (KCF), and access to cloud services is granted based on a person's identity and the relationship between the parties involved, which protects their personal information that is the methodology used in the proposed scheme. The proposed scheme is best suited for cloud-based e-healthcare services because of its simplicity and robustness. Using different Amazon EC2 hosting options, we examine how well our cloud-based web application service works when the number of requests linearly increases. The performance of our web application service that runs in the cloud is based on how many requests it can handle per second while keeping its response time constant. The proposed secure access scheme for cloud-based web applications was compared to the Ethereum blockchain platform, which uses internet of things (IoT) devices in terms of execution time, throughput, and latency. [ABSTRACT FROM AUTHOR]

Infantile neuroaxonal dystrophy (INAD) is a rare paediatric neurodegenerative condition caused by mutations in the PLA2G6 gene, which is also the causative gene for PARK14-linked young adult-onset dystonia parkinsonism. INAD patients usually die within their first decade of life, and there are currently no effective treatments available. GLP1 receptor (GLP-1R) agonists are licensed for treating type 2 diabetes mellitus but have also demonstrated neuroprotective properties in a clinical trial for Parkinson's disease. Therefore, we evaluated the therapeutic efficacy of a new recently licensed GLP-1R agonist diabetes drug in a mouse model of INAD. Systemically administered high-dose semaglutide delivered weekly to juvenile INAD mice improved locomotor function and extended the lifespan. An investigation into the mechanisms underlying these therapeutic effects revealed that semaglutide significantly increased levels of key neuroprotective molecules while decreasing those involved in pro-neurodegenerative pathways. The expression of mediators in both the apoptotic and necroptotic pathways were also significantly reduced in semaglutide treated mice. A reduction of neuronal loss and neuroinflammation was observed. Finally, there was no obvious inflammatory response in wild-type mice associated with the repeated high doses of semaglutide used in this study. [ABSTRACT FROM AUTHOR]

In semiconductor manufacturing, black silicon (bSi) has traditionally been considered as a sign of unsuccessful etching. However, after more careful consideration, many of its properties have turned out to be so superior that its integration into devices has become increasingly attractive. In devices where bSi covers the whole wafer surface, such as solar cells, the integration is already rather mature and different bSi fabrication technologies have been studied extensively. Regarding the integration into devices where bSi should cover only small selected areas, existing research focuses on device properties with one specific bSi fabrication method. Here, we fabricate bSi patterns with varying dimensions ranging from millimeters to micrometers using three common bSi fabrication techniques, i.e., plasma etching, metal-assisted chemical etching (MACE) and femtosecond-laser etching, and study the corresponding fabrication characteristics and resulting material properties. Our results show that plasma etching is the most suitable method in the case of $\mu \text{m}$ -scale devices, while MACE reaches surprisingly almost the same performance. Femtosecond-laser has potential due to its maskless nature and capability for hyperdoping, however, in this study its moderate accuracy, large silicon consumption and spreading of the etching damage outside the bSi region leave room for improvement. [ABSTRACT FROM AUTHOR]

Dopa-responsive dystonia (DRD) comprises a group of rare but treatable dystonias that exhibit diurnal fluctuation. The GCH1 gene encodes GTP cyclohydrolase-1 (GTPCH-I), a protein that catalyzes the first rate-limiting step of tetrahydrobiopterin biosynthesis. Pathogenic variants in GCH1 are the most common causes of DRD. However, the autosomal recessive form of DRD caused by biallelic GCH1 variants is very rare. Homozygous GCH1 variants have been associated with two clinically distinct human diseases: hyperphenylalaninemia, and DRD with or without hyperphenylalaninemia. Here, we describe one patient who presented during infancy with severe truncal hypotonia and motor developmental regression but without diurnal fluctuation and hyperphenylalaninemia. Treatment with levodopa/carbidopa resulted in the complete and persistent remission of clinical symptoms without any side effects. This was accompanied by age-appropriate neurological development during follow-up. A homozygous GCH1 variant (c.604G>A/p.V202I) was identified in the patient. To our knowledge, this is the first Chinese case of DRD caused by a homozygous GCH1 variant, thus expanding the spectrum of DRD phenotypes. Autosomal recessive DRD that is associated with homozygous GCH1 variants should be considered in patients with severe truncal hypotonia, with or without diurnal fluctuation, even if there is an absence of limb dystonia and hyperphenylalaninemia. [ABSTRACT FROM AUTHOR]