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Bhutan primarily relies on land transportation; hence, diverse technologies are crucial for ensuring the safety of the drivers. Dust, fog, smoke, and other dry particles often obstructs the road visibility. Furthermore; complex mountainous terrain and steep topography increase fog formation, while winding winds heighten accident risks. This study aims to reduce weather-related accidents by introducing an IoT-centric system designed to enhance visibility and provide timely alerts to drivers. The system employs the Dark Channel Prior (DCP) algorithm for real-time foggy video processing and refining of dehazed video using the Guided Filtering method. This research also focuses on developing an alert system using a time-of-flight (ToF) sensor with detection range of 15 m. Real-time testing and validation were conducted along the fog-prone Thimphu-Phuentsholing highway, specifically in the Jumja region. To evaluate the performance of the system; validation metrics, including the peak signal-to-noise ratio (PSNR) and Structural Similarity Index Measure (SSIM) were determined for quantitative analysis, and the original and dehazed videos were compared on the same screen for visual analysis. The effectiveness of obstacle detection system was evaluated by comparing the actual measured distance to the distance displayed on the screen. The results depicts that the proposed system significantly improves visibility and effectively sirens drivers to impending obstacles within the sufficient stopping sight distance through display of clear video in the dehazing system. The study highlights the potential of advanced IoT and computer vision technologies to enhance road safety and reduce accident rates under low-visibility conditions.

Abstract The post-surgical fluid leakage from the tubular tissues is a critical symptom after gastrointestinal or urinary tract surgeries. Elucidating the mechanism for such abnormalities is vital in surgical and medical science. The exposure of the fluid such as peritonitis due to urinary or gastrointestinal perforation has been reported to induce severe inflammation to the surrounding tissue. However, there have been no reports for the tissue responses by fluid extravasation and assessment of post-surgical and injury complication processes is therefore vital. The current model mouse study aims to investigate the effect of the urinary extravasation of the urethral injuries. Analyses on the urinary extravasation affecting both urethral mesenchyme and epithelium and the resultant spongio-fibrosis/urethral stricture were performed. The urine was injected from the lumen of urethra exposing the surrounding mesenchyme after the injury. The wound healing responses with urinary extravasation were shown as severe edematous mesenchymal lesions with the narrow urethral lumen. The epithelial cell proliferation was significantly increased in the wide layers. The mesenchymal spongio-fibrosis was induced by urethral injury with subsequent extravasation. The current report thus offers a novel research tool for surgical sciences on the urinary tract.

Background and objectivesTo clarify whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection cause acute encephalopathy in children and which are the most common syndromes that cause them and what are the outcomes.MethodsA nationwide web-based survey among all members of the Japanese Society of Child Neurology to identify pediatric patients aged < 18 years who developed acute encephalopathy in Japan between 1 January 2020 and 31 May 2022 associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection confirmed by polymerase chain reaction or antigen tests using pharyngeal swabs. Acute encephalopathy was defined as acute onset of impaired consciousness lasting > 24 h or an altered mental state; neurological symptoms arising within 2 weeks of onset of COVID-19 or multisystem inflammatory syndrome in children (MIS-C)/pediatric inflammatory multisystem syndrome (PIMS); evidence of SARS-CoV-2 infection; and reasonable exclusion of other diseases. Patients were divided into the known clinico-radiological acute encephalopathy syndrome group and unexplained or unclassifiable acute encephalopathy group. Outcomes were assessed by pediatric cerebral performance category (PCPC) score at hospital discharge.ResultsOf the 3,802 society members, 217 representing institutions responded, and 39 patients with suspected acute encephalopathy were reported, of which 31 met inclusion criteria. Of these patients, 14 were diagnosed with known clinico-radiological acute encephalopathy syndromes, with acute encephalopathy with biphasic seizures and late reduced diffusion (five patients) being the most common. Five developed acute encephalopathy associated with MIS-C/PIMS. Among 31 patients, 9 (29.0%) had severe sequelae or died (PCPC ≥ 4). Two of three patients with encephalopathy with acute fulminant cerebral edema and two with hemorrhagic shock and encephalopathy syndrome died. The PCPC scores were higher in the known clinico-radiological acute encephalopathy syndrome group than in the unexplained or unclassifiable acute encephalopathy group (P < 0.01).DiscussionAcute encephalopathy related to SARS-CoV-2 infection was demonstrated to be more severe than that caused by other viruses in Japan. Acute encephalopathy syndromes characterized by specific neuroradiological findings was associated with poor clinical outcomes.

Gene therapy using adeno-associated virus (AAV) is a rapidly developing technology with widespread treatment potential. AAV2 vectors injected directly into the brain by stereotaxic brain surgery have shown good results in treating aromatic l-amino acid decarboxylase deficiency. Moreover, gene therapy using the AAV9 vector, which crosses the blood–brain barrier, has been performed in more than 2000 patients worldwide as a disease-modifying therapy for spinal muscular atrophy. AAV vectors have been applied to the development of gene therapies for various pediatric diseases. Gene therapy trials for hemophilia and ornithine transcarbamylase deficiency are underway. Clinical trials are planned for glucose transporter I deficiency, Niemann-Pick disease type C, and spinocerebellar ataxia type 1. The genome of AAV vectors is located in the episome and is rarely integrated into chromosomes, making the vectors safe. However, serious adverse events such as hepatic failure and thrombotic microangiopathy have been reported, and ongoing studies are focusing on developing more efficient vectors to reduce required dosages.

Abstract Background Actin, alpha, skeletal muscle 1 (ACTA1) is one of the causative genes of nemaline myopathy (NM) and congenital fiber‐type disproportion (CFTD). CFTD is characterized by type 1 fiber atrophy and distinguished from NM in the absence of rods. Eight patients with CFTD, including one patient with dilated cardiomyopathy (DCM), have previously been reported. Herein, we report the case of a 10‐year‐old boy presenting with CFTD and DCM. Methods We performed exome sequencing and analyzed the effect of Met327Lys mutations on cultured C2C12 muscle cells compared with that seen in the wild type (WT, ACTA1) and previously identified Asp294Val mutations associated with a severe phenotype of CFTD without cardiomyopathy. Results Exome sequencing revealed a de novo mutation, c.980 T > A, p.(Met327Lys), in ACTA1 (NM_001100.4). C2C12 cells transfected with the WT plasmid expressed ACTA1 in the nucleus and cytoplasm. Cells with the Asp294Val mutant showed needle‐like structures in the cytoplasm, whereas the expression of the Met327Lys mutant resulted in few aggregations but many apoptotic cells. Conclusion Apoptosis induced in Met327Lys‐transfected muscle cells supports the pathogenicity of the mutation and can be implicated as one of the histopathological features associated with CFTD, as in NM.

Abstract Background Congenital central hypoventilation syndrome (CCHS) is a rare disease characterized by sleep apnea. Anoxia often occurs soon after birth, and it is important to prevent anoxia-mediated central nervous system complications; however, data on the relationship between respiratory management and the prognosis for intellectual development of patients with CCHS is not well yet investigate. Methods We performed a retrospective chart review cohort study of patients with CCHS in Japan. We investigated the risk and prognostic factors for developmental outcomes and examined the disease in terms of its symptoms, diagnosis, complications, and treatment. Results Of the 123 patients with CCHS included in the survey, 88 patients were 6 years old and older. They were divided into two group based on their intelligence quotient. Those treated using positive-pressure ventilation via tracheostomy in the first three months of life had a better developmental prognosis than those managed via tracheostomy after three months of age and those treated by ventilation using mask (OR = 3.80; 95% CI: 1.00–14.37, OR = 4.65; 95% CI: 1.11–19.37). There was no significant difference in physical development (P = 0.64). Conclusions The best respiratory treatment for patients with CCHS is ventilation via tracheostomy, initiated ideally before the age of three months.

BackgroundEarly intervention and prevention of psychiatric comorbidities of children with autism spectrum disorder (ASD) and attention-deficit/hyperactivity disorder (ADHD) are urgent issues. However, the differences in the diagnoses of ASD and ADHD and psychiatric comorbidities associated with age, long-term healthcare utilization trajectories, and its associated diagnostic features have not been fully elucidated in Japan.MethodWe conducted a retrospective observational study using the medical records. Member hospitals of three major consortiums of hospitals providing child and adolescent psychiatric services in Japan were recruited for the study. Children who accessed the psychiatry services of the participating hospitals in April 2015 were followed up for 5 years, and data on their clinical diagnoses, consultation numbers, and hospitalizations were collected. Non-hierarchical clustering was performed using two 10-timepoint longitudinal variables: consultation numbers and hospitalization. Among the major clusters, the differences in the prevalence of ASD, ADHD, comorbid intellectual disability, neurotic disorders, and other psychiatric disorders were assessed.ResultsA total of 44 facilities participated in the study (59.5%), and 1,003 participants were enrolled. Among them, 591 diagnosed with ASD and/or ADHD (58.9%) and 589 without missing data were assessed. The mean age was 10.1 years, and 363 (70.9%) were boys. Compared with the pre-schoolers, the school-aged children and adolescents had fewer ASD, more ADHD, and fewer comorbid intellectual disability diagnoses, as well as more diagnoses of other psychiatric disorders. A total of 309 participants (54.7%) continued consultation for 2 years, and 207 (35.1%) continued for 5 years. Clustering analysis identified three, two, and three major clusters among pre-schoolers, school-aged children, and adolescents, respectively. The largest cluster was characterized by early termination of the consultation and accounted for 55.4, 70.6, and 73.4% of pre-schoolers, school-aged children, and adolescents, respectively. Among the school-aged children, the diagnosis of ADHD was associated with a cluster that required longer periods of consultations. Among the adolescents, comorbid psychiatric disorders other than intellectual disability and neurotic disorders were associated with clusters that required hospitalization.ConclusionContinuous healthcare needs were common and psychiatric comorbidities were associated with complex trajectory among adolescents. The promotion of early intervention and prevention of comorbidities are important.

Short-chain enoyl-CoA hydratase (ECHS1) is involved in amino acid and fatty acid catabolism in mitochondria and its deficiency causes Leigh syndrome or exercise-induced dystonia. More than 60 patients with this condition have been reported till date. The accumulation of intermediate metabolites of valine is assumed to be responsible for the cytotoxicity. Since protein restriction, including valine reportedly improves neurological symptoms, it is essential to consider the possible incidence of and diagnose ECHS1 syndrome in the earlier stages. This study reported the liquid chromatography with tandem mass spectrometry (LC-MS/MS) urine and plasma metabolite analysis in six cases, including four new cases with ECHS1 deficiency. The values of urine cysteine/cysteamine conjugates from valine metabolites, S-(2-carboxypropyl) cysteine/cysteamine from methacrylyl-CoA, and S-(2-carboxyethyl) cysteine/cysteamine from acryloyl-CoA were separated between six patients and six normal controls. The LC-MS/MS analysis revealed that these metabolites can be used for the early diagnosis and evaluation of diet therapy.

With the continuous expansion of power grid capacity, the problem of short-circuit current exceeding the standard is becoming increasingly serious. Fault current limiter is a promising solution and is gradually becoming a research hotspot. In this study, fault current limiters are classified into four categories. And saturated-core fault current limiters are emphatically introduced, including its working principle and comparison with the other three categories. Saturated core fault current limiters are divided into four branches according to the ways leading the core saturated. A comprehensive review of the research activities and emerging technologies of saturated-core fault current limiters for AC power systems is presented in this study. The working principle and typical structure of DC-biased-, permanent-magnet-, superconducting- and hybrid-type saturated-core fault current limiters are introduced. The advantages and disadvantages of four types of saturated core fault current limiters are compared in detail from the aspects of current-limiting performance, iron core size and DC magnetomotive force. Real grid application examples of some types of devices are presented, as well as new progress in the techniques, are covered and discussed in detail. One may find the content of this study helpful as a detailed literature review or as practical technical guidance.

The large current generated by a direct start of the super-large-capacity high-voltage induction motor would have a huge impact on the power grid as well as the motor itself. Traditional soft starters have the shortcomings of discontinuous adjustment, voltage sags, sudden torque mutation, secondary current impact and high cost. To resolve this issue, the authors propose a novel coupled autotransformer and magnetic-control (CATMC) soft-start method. The structure of the new CATMC soft starter combines the functions of the autotransformer and magnetic control reactor via an innovative electric and magnetic circuit design. In this study, the authors analyse the magnetic circuit structure and working principles of the CATMC soft starter. Then, to validate its principle and performance, the authors conduct a simulation study using ANSYS software and design and test an 18 MW/10 kV CATMC soft starter prototype. The simulation results demonstrate that the CATMC soft starter effectively avoids secondary current impact and constrains the motor starting current to

In the magnetic field analysis of magnetic devices using a ferrite core, such as a pulse transformer, the frequency-domain analysis is often carried out using the measured complex permeability under different frequency range. However, the nonlinear magnetic characteristics cannot be considered in the frequency-domain analysis because of the harmonics caused by it cannot be represented. The nonlinear magnetic characteristics can be considered in the time-domain analysis, but suitable constant conductivity and permittivity taking account of the microstructure of ferrite core, which can represent the measured complex permeability under different frequencies, needs to be investigated for the time-domain analysis. In this paper, the effective permeability of a toroidal ferrite core is tried to be demonstrated by using the linear ac steady state magnetic field analysis taking account eddy currents and displacement currents. It is shown that the measured permeability can be realized roughly by using the modified constant conductivity and permittivity. The nonlinear time-domain magnetic field analysis can be carried out using the modified constant conductivity and permittivity obtained from this paper.

To achieve economical compensation for the huge-capacity negative sequence currents generated by high-speed railway load, an electromagnetic hybrid compensation system (EHCS) and control strategy is proposed. The EHCS is made up of a small-capacity railway static power conditioner (RPC) and a large-capacity magnetic static var compensator (MSVC). Compared with traditional compensation methods, the EHCS makes full use of the SVC's advantages of economy and reliability and of RPC's advantages of technical capability and flexibility. Based on the idea of injecting a negative sequence, the compensation principle of the EHCS is analyzed in detail. Then the minimum installation capacity of an EHCS is theoretically deduced. Furthermore, a constraint optimization compensation strategy that meets national standards, which reduces compensation capacity further, is proposed. An experimental platform based on a digital signal processor (DSP) and a programmable logic controller (PLC) is built to verify the analysis. Simulated and experimental results are given to demonstrate the effectiveness and feasibility of the proposed method.

In order to protect high voltage direct current (HVDC) system from destructive consequences caused by fault current, a novel concept of HVDC system fault current limiter (DCSFCL) was proposed previously. Since DCSFCL is based on saturable core reactor theory, iron core becomes the key to the final performance of it. Therefore, three typical kinds of soft magnetic materials were chosen to find out their impact on performances of DCSFCL. Different characteristics of materials were compared and their theoretical deductions were carried out, too. In the meanwhile, 3D models applying those three materials were built separately and finite element analysis simulations were performed to compare these results and further verify the assumptions. It turns out that materials with large saturation flux density value Bs like silicon steel and short demagnetization time like ferrite might be the best choice for DCSFCL, which can be a future research direction of magnetic materials.

To develop mechanical circuit breaker in high voltage direct current (HVDC) system, a fault current limiter is required. Traditional method to limit DC fault current is to use superconducting technology or power electronic devices, which is quite difficult to be brought to practical use under high voltage circumstances. In this paper, a novel concept of high voltage DC transmission system fault current limiter (DCSFCL) based on saturable core was proposed. In the DCSFCL, the permanent magnets (PM) are added on both up and down side of the core to generate reverse magnetic flux that offset the magnetic flux generated by DC current and make the DC winding present a variable inductance to the DC system. In normal state, DCSFCL works as a smoothing reactor and its inductance is within the scope of the design requirements. When a fault occurs, the inductance of DCSFCL rises immediately and limits the steepness of the fault current. Magnetic field simulations were carried out, showing that compared with conventional smoothing reactor, DCSFCL can decrease the high steepness of DC fault current by 17% in less than 10ms, which verifies the feasibility and effectiveness of this method.

Background: Mucus hypersecretion from airway epithelium is a characteristic feature of severe asthma. Glucocorticoids (GCs) may suppress mucus production and diminish the harmful airway obstruction. We investigated the ability of GCs to suppress mRNA expression and protein synthesis of a gene encoding mucin, MUC5AC, induced by transforming growth factor (TGF)-a in human mucoepidermoid carcinoma (NCI-H292) cells and the molecular mechanisms underlying the suppression. Methods: We determined if GCs such as dexamethasone (DEX), budesonide (BUD), and fluticasone (FP) could suppress MUC5AC production induced by a combination of TGF-α and double-strand RNA, polyinosinic- polycytidylic acid (polyI:C). MUC5AC mRNA expression and MUC5AC protein production were evaluated. The signaling pathways activated by TGF-α and their inhibition by GCs were tested using a phosphoprotein assay and MUC5AC promoter assay. Results: DEX significantly suppressed the expression of MUC5AC mRNA and MUC5AC protein induced by TGF-α. The activation of the MUC5AC promoter by TGF-α was significantly inhibited by DEX. DEX did not affect activation of downstream pathways of the EGF receptor or mRNA stability of MUC5AC transcripts. DEX, BUD, and FP suppressed MUC5AC protein expression induced by a combination of TGF-α and polyI:C in a dose-dependent manner. Conclusions: GCs inhibited MUC5AC production induced by TGF-α alone or a combination of TGF-α and polyI:C; the repression may be mediated at the transcriptional but not post-transcriptional level.