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Catecholaminergic polymorphic ventricular tachycardia is an ion channelopathy, caused by mutations in genes coding for calcium-handling proteins. It can coexist with left ventricular non-compaction. We aim to investigate the clinical and genetic characteristics of this co-phenotype.
Medical records of 24 patients diagnosed with catecholaminergic polymorphic ventricular tachycardia in two Chinese hospitals between September, 2005, and January, 2020, were retrospectively reviewed. We evaluated their clinical and genetic characteristics, including basic demographic data, electrocardiogram parameters, medications and survival during follow-up, and their gene mutations. We did structural analysis for a novel variant ryanodine receptor 2-E4005V.
The patients included 19 with catecholaminergic polymorphic ventricular tachycardia mono-phenotype and 5 catecholaminergic polymorphic ventricular tachycardia-left ventricular non-compaction overlap patients. The median age of onset symptoms was 9.0 (8.0,13.5) years. Most patients (91.7%) had cardiac symptoms, and 50% had a family history of syncope. Overlap patients had lower peak heart rate and threshold heart rate for ventricular tachycardia and ventricular premature beat during the exercise stress test (p < 0.05). Sudden cardiac death risk may be higher in overlap patients during follow-up. Gene sequencing revealed 1 novel ryanodine receptor 2 missense mutation E4005V and 1 mutation previously unreported in catecholaminergic polymorphic ventricular tachycardia, but no left ventricular non-compaction-causing mutations were observed. In-silico analysis showed the novel mutation E4005V broke down the interaction between two charged residues.
Catecholaminergic polymorphic ventricular tachycardia overlapping with left ventricular non-compaction may lead to ventricular premature beat/ventricular tachycardia during exercise stress test at lower threshold heart rate than catecholaminergic polymorphic ventricular tachycardia alone; it may also indicate a worse prognosis and requires strict follow-up. ryanodine receptor 2 mutations disrupted interactions between residues and may interfere the function of ryanodine receptor 2.
By combining the technique of energy selective surface and frequency selective rasorber, an energy selective rasorber is proposed, which performs selective energy protection in the low communication frequency band (0.8–2 GHz) and wave-absorbing property in the high-frequency band (6–18 GHz). The design consists of two layers, of which the bottom one contains a lumped diode structure for energy selection function in the transmission band, while together with the top layer, they perform a wideband wave absorbing function. The simulated and measured results agree well with each other, and both show good absorption in 6–18 GHz and energy-selective property around 1.86 GHz. That is, when the incident power changes from −30 to 14 dBm, the reflection coefficient changes from below −22 dB to above −2 dB, while the transmission coefficient changes from above −3 dB to below −17 dB.
This paper studies the synchronization control of the blanket remote maintenance robot (BRMR) of the China fusion engineering test reactor (CFETR). First, the general state space mathematical model of BRMR was established by using a physical-based method. Second, based on the receding horizon optimization of model predictive control (MPC) and cross-coupling error reduction in cross-coupling control (CCC), the innovative MPC-CCC controller was proposed to realize the single-system and multisystem error convergence and high accuracy transportation of blanket through the high accuracy synchronization control of BRMR. Third, to verify the control effectiveness of the MPC-CCC controller, two types of simulations and experiments were implied compared with the original proportional-integral (PI) controller in Mover. Results showed that simulation and experiments were highly consistent. It is found that the use of an MPC-CCC controller can result in up to a 70% reduction in displacement error and up to a 59% reduction in synchronization error compared to the PI controller. And the accuracy of the MPC-CCC controller satisfies the real requirement of the maintenance process of the blanket. This work provides the theoretical basis and practical experience for the highly stable, safe, and efficient maintenance of blankets in the future.
A high-power all polarization-maintaining (PM) chirped pulse amplification (CPA) system operating in the 2.0 μm range is experimentally demonstrated. Large mode area (LMA) thulium-doped fiber (TDF) with a core/cladding diameter of 25/400 μm is employed to construct the main amplifier. Through dedicated coiling and cooling of the LMA-TDF to manage the loss of the higher order mode and thermal effect, a maximum average power of 314 W with a slope efficiency of 52% and polarization extinction ratio of 20 dB is realized. The pulse duration is compressed to 283 fs with a grating pair, corresponding to a calculated peak power of 10.8 MW, considering the compression efficiency of 88% and the estimated Strehl ratio of 89%. Moreover, through characterizing the noise properties of the laser, an integrated relative intensity noise of 0.11% at 100 Hz−1 MHz is obtained at the maximum output power, whereas the laser timing jitter is degraded by the final amplifier from 318 to 410 fs at an integration frequency of 5 kHz to 1 MHz, owing to the self-phase modulation effect-induced spectrum broadening. The root-mean-square of long-term power fluctuation is tested to be 0.6%, verifying the good stability of the laser operation. To the best of our knowledge, this is the highest average power of an ultrafast laser realized from an all-PM-fiber TDF-CPA system ever reported.
The Helan Shan pika Ochotona argentata is an Endangered pika endemic to Ningxia Helan Shan National Nature Reserve in China. Little is known about the species, including regarding its population status, distribution, habitat requirements and even basic natural history. We conducted camera trapping in the Reserve during 13 January 2020–25 January 2022 and obtained two new records for this species. These, combined with five other new records obtained during the monitoring programme in the Reserve, represent a significant increase in the known range of the species and more than double the species’ known extent of occurrence from 107 to 223 km2. These records also provide the first evidence that this species is active both nocturnally and diurnally. However, this range increase does not alter the Endangered status of the pika, and the pika was not observed within its previously known range; future research should involve intensive camera trapping in the Reserve to ascertain whether this is a result of local extirpation, habitat loss or fragmentation or under-sampling on our part. Studies should also incorporate techniques used for other alpine pika species to collect baseline data on habitat use, population size and behaviour to determine the potential response of the Helan Shan pika to present and future threats.
Large gatherings of people on cruise ships and warships are often at high risk of COVID-19 infections. To assess the transmissibility of SARS-CoV-2 on warships and cruise ships and to quantify the effectiveness of the containment measures, the transmission coefficient (β), basic reproductive number (R0), and time to deploy containment measures were estimated by the Bayesian Susceptible-Exposed-Infected-Recovered model. A meta-analysis was conducted to predict vaccine protection with or without non-pharmaceutical interventions (NPIs). The analysis showed that implementing NPIs during voyages could reduce the transmission coefficients of SARS-CoV-2 by 50%. Two weeks into the voyage of a cruise that begins with 1 infected passenger out of a total of 3,711 passengers, we estimate there would be 45 (95% CI:25-71), 33 (95% CI:20-52), 18 (95% CI:11-26), 9 (95% CI:6-12), 4 (95% CI:3-5), and 2 (95% CI:2-2) final cases under 0%, 10%, 30%, 50%, 70%, and 90% vaccine protection, respectively, without NPIs. The timeliness of strict NPIs along with implementing strict quarantine and isolation measures is imperative to contain COVID-19 cases in cruise ships. The spread of COVID-19 on ships was predicted to be limited in scenarios corresponding to at least 70% protection from prior vaccination, across all passengers and crew.
Explosive dispersal of granular media widely occurs in nature across various length scales, also enabling engineering applications ranging from commercial or military explosive systems to the loss prevention industry. However, the complex particle–flow coupling makes the explosive dispersal behaviour of particles difficult to control or even characterize. Here, we study the central explosion-driven dispersal of dense particle layers using the coarse-grained computational fluid dynamics–discrete element method and present a comprehensive investigation of both macroscale dispersal behaviours and particle-scale pattern formation. Employing three independent dimensionless parameters that characterize the efficiency, homogeneity and completeness of explosive dispersal, we categorize the dispersal behaviours into ideal, partial, retarded and failed modes, and propose the corresponding thresholds. As the mass ratio of granular materials to central pressurized gases (M/C) spans four orders of magnitude, the dispersal mode transitions from ideal to partial, then to retarded and finally to failed mode. The transitions of dispersal modes correspond to the particle–flow coupling regime crossovers, which change from decoupling to weak, medium and finally to strong coupling as the dispersal mode undergoes corresponding transitions. We proceed to develop continuum models accounting for the shock compaction and the ensuing pulsation of the particle ring that are capable of identifying the ideal dispersal mode from various dispersal systems. We also provide insights into the origins of diverse particle-scale patterns that are strongly correlated with macroscale dispersal modes and critical for the accurate prediction of dispersal modes.
An all-fiber high-power linearly polarized chirped pulse amplification (CPA) system is experimentally demonstrated. Through stretching the pulse duration to a full width of approximately 2 ns with two cascaded chirped fiber Bragg gratings (CFBGs), a maximum average output power of 612 W is achieved from a high-gain Yb-doped fiber that has a core diameter of 20 μm with a slope efficiency of approximately 68% at the repetition rate of 80 MHz. At the maximum output power, the polarization degree is 92.5% and the M2 factor of the output beam quality is approximately 1.29; the slight performance degradations are attributed to the thermal effects in the main amplifier. By optimizing the B-integral of the amplifier and finely adjusting the higher-order dispersion of one of the CFBGs, the pulse width is compressed to 863 fs at the highest power with a compression efficiency of 72%, corresponding to a maximum compressed average power of 440.6 W, single pulse energy of 5.5 μJ and peak power of about 4.67 MW. To the best of our knowledge, this is the highest average power of a femtosecond laser directly generated from an all-fiber linearly polarized CPA system.
Aorto-right-atrial fistula is an uncommon condition with an unclear pathogenesis. We present the case of a 3-year-old girl with a giant omphalocele repaired days after birth and incidentally discovered with a celiacomesenteric trunk-to-right atrium fistula. Three-dimensional reconstruction CT unveiled its anatomical pattern, and the fistula was successfully closed using a Amplatzer vascular plug II percutaneously.
Multilayer dielectric gratings typically remove multiple-grating pillars after picosecond laser irradiation; however, the dynamic formation process of the removal is still unclear. In this study, the damage morphologies of multilayer dielectric gratings induced by an 8.6-ps laser pulse were closely examined. The damage included the removal of a single grating pillar and consecutive adjacent grating pillars and did not involve the destruction of the internal high-reflection mirror structure. Comparative analysis of the two damage morphological characteristics indicated the removal of adjacent pillars was related to an impact process caused by the eruption of localized materials from the left-hand pillar, exerting impact pressure on its adjacent pillars and eventually resulting in multiple pillar removal. A finite-element strain model was used to calculate the stress distribution of the grating after impact. According to the electric field distribution, the eruptive pressure of the dielectric materials after ionization was also simulated. The results suggest that the eruptive pressure resulted in a stress concentration at the root of the adjacent pillar that was sufficient to cause damage, corresponding to the experimental removal of the adjacent pillar from the root. This study provides further understanding of the laser-induced damage behavior of grating pillars and some insights into reducing the undesirable damage process for practical applications.
The mitochondrial genome provides important information for phylogenetic analysis and an understanding of evolutionary origin. In this study, the mitochondrial genomes of Ilisha elongata and Setipinna tenuifilis were sequenced, which are typical circular vertebrate mitochondrial genomes composed of 16,770 and 16,805 bp, respectively. The mitogenomes of I. elongata and S. tenuifilis include 13 protein-coding genes (PCGs), 22 transfer RNA (tRNA), two ribosomal RNA (rRNA) genes and one control region (CR). Both two species' genome compositions were highly A + T biased and exhibited positive AT-skews and negative GC-skews. The genetic distance and Ka/Ks ratio analyses indicated that 13 PCGs were affected by purifying selection and the selection pressures were different from certain deep-sea fishes, which were most likely due to the difference in their living environment. Results of phylogenetic analysis support close relationships among Chirocentridae, Denticipitidae, Clupeidae, Engraulidae and Pristigasteridae based on the nucleotide and amino acid sequences of 13 PCGs. Within Clupeoidei, I. elongata and S. tenuifilis were most closely related to the family Pristigasteridae and Engraulidae, respectively. These results will help to better understand the evolutionary position of Clupeiformes and provide a reference for further phylogenetic research on Clupeiformes species.
It has been suggested that added sugar intake is associated with non-alcoholic fatty liver disease (NAFLD). However, previous studies only focused on sugar-sweetened beverages; the evidence for associations with total added sugars and their sources is scarce. This study aimed to examine the associations of total added sugars, their physical forms (liquid v. solid) and food sources with risk of NAFLD among adults in Tianjin, China. We used data from 15 538 participants, free of NAFLD, other liver diseases, CVD, cancer or diabetes at baseline (2013–2018 years). Added sugar intake was estimated from a validated 100-item FFQ. NAFLD was diagnosed by ultrasonography after exclusion of other causes of liver diseases. Multivariable Cox proportional hazards models were fitted to calculate hazard ratios (HR) and corresponding 95 % CI for NAFLD risk with added sugar intake. During a median follow-up of 4·2 years, 3476 incident NAFLD cases were documented. After adjusting for age, sex, BMI and its change from baseline to follow-up, lifestyle factors, personal and family medical history and overall diet quality, the multivariable HR of NAFLD risk were 1·18 (95 % CI 1·06, 1·32) for total added sugars, 1·20 (95 % CI 1·08, 1·33) for liquid added sugars and 0·96 (95 % CI 0·86, 1·07) for solid added sugars when comparing the highest quartiles of intake with the lowest quartiles of intake. In this prospective cohort of Chinese adults, higher intakes of total added sugars and liquid added sugars, but not solid added sugars, were associated with a higher risk of NAFLD.
Identification of treatment-specific predictors of drug therapies for bipolar disorder (BD) is important because only about half of individuals respond to any specific medication. However, medication response in pediatric BD is variable and not well predicted by clinical characteristics.
A total of 121 youth with early course BD (acute manic/mixed episode) were prospectively recruited and randomized to 6 weeks of double-blind treatment with quetiapine (n = 71) or lithium (n = 50). Participants completed structural magnetic resonance imaging (MRI) at baseline before treatment and 1 week after treatment initiation, and brain morphometric features were extracted for each individual based on MRI scans. Positive antimanic treatment response at week 6 was defined as an over 50% reduction of Young Mania Rating Scale scores from baseline. Two-stage deep learning prediction model was established to distinguish responders and non-responders based on different feature sets.
Pre-treatment morphometry and morphometric changes occurring during the first week can both independently predict treatment outcome of quetiapine and lithium with balanced accuracy over 75% (all p < 0.05). Combining brain morphometry at baseline and week 1 allows prediction with the highest balanced accuracy (quetiapine: 83.2% and lithium: 83.5%). Predictions in the quetiapine and lithium group were found to be driven by different morphometric patterns.
These findings demonstrate that pre-treatment morphometric measures and acute brain morphometric changes can serve as medication response predictors in pediatric BD. Brain morphometric features may provide promising biomarkers for developing biologically-informed treatment outcome prediction and patient stratification tools for BD treatment development.
The paper deals with the workspace-based optimization of a novel humanoid robotic arm. The eight-degree-of-freedom hybrid manipulator that conforms to the kinematics characteristics of the human arm is briefly introduced. According to the structural features of this mechanism and the requirements of tasks in the complex environment, the workspace is divided into three parts, the orientation space of the humanoid shoulder joint, the position space of the humanoid elbow joint, and the active orientation space of the end-moving platform. Moreover, a multi-parameter planar model is proposed for the optimization problem with multidimensional parameters and highly nonlinear constraints. Based on the visualized optimization result, the coupling effect of each parameter on the corresponding workspace is clearly presented. Considering the compactness and the processing and assembling technology of this mechanism, a set of structural parameters satisfying the workspace-based optimization objective is obtained. Simulation results show that the corresponding workspace of the three parts has increased significantly by the factor of 1.45, 1.68, and 1.3, respectively.
Prospective cohort studies linking organ meat consumption and non-alcoholic fatty liver disease (NAFLD) are limited, especially in Asian populations. This study aimed to prospectively investigate the association between organ meat consumption and risk of NAFLD in a general Chinese adult population. This prospective cohort study included a total of 15 568 adults who were free of liver disease, CVD and cancer at baseline. Dietary information was collected at baseline using a validated FFQ. NAFLD was diagnosed by abdominal ultrasound after excluding other causes related to chronic liver disease. Cox proportional regression models were used to assess the association between organ meat consumption and risk of NAFLD. During a median of 4·2 years of follow-up, we identified 3604 incident NAFLD cases. After adjusting for demographic characteristics, lifestyle factors, vegetable, fruit, soft drink, seafood and red meat consumption, the multivariable hazard ratios (95 % CI) for incident NAFLD across consumption of organ meat were 1·00 (reference) for almost never, 1·04 (0·94, 1·15) for tertile 1, 1·08 (0·99, 1·19) for tertile 2 and 1·11 (1·01, 1·22) for tertile 3, respectively (Pfor trend < 0·05). Such association did not differ substantially in the sensitivity analysis. Our study indicates that organ meat consumption was related to a modestly higher risk of NAFLD among Chinese adults. Further investigations are needed to confirm this finding.
This paper investigates the shock-induced instability of the interfaces between gases and dense granular media with finite length via the coarse-grained compressible computational fluid dynamics–discrete parcel method. Despite generating a typical spike-bubble structure reminiscent of the Richtmyer–Meshkov instability (RMI), the shock-driven granular instability (SDGI) is governed by fundamentally different mechanisms. Unlike the RMI arising from baroclinic vorticity deposition on the interface, the SDGI is closely associated with the interfacial and bulk granular dynamics, which evolve with the transient coupling between particles and gases. Consequently, the SDGI follows a growth law distinctly different from that of the RMI, namely a semilinear slow regime followed by an exponentially expedited regime and a quadratic asymptotic regime. We further establish the instability criteria of the SDGI for granular media with infinite and finite lengths, which do not exist in the RMI. A scaling growth law of the SDGI for dense granular media with finite length is derived by normalizing the time with the rarefaction propagation time, which successfully collapses the data from cases with varying shock strength, particle column length and particle volume fraction and ought to hold for granular media with varying particle parameters. The effect of the initial perturbation magnitude can be properly considered in the scaling growth law by incorporating it into the length normalization.
As the corona virus disease 2019 (COVID-19) pandemic continues around the world, understanding the transmission characteristics of COVID-19 is vital for prevention and control. We conducted the first study aiming to estimate and compare the relative risk of secondary attack rates (SARs) of COVID-19 in different contact environments. Until 26 July 2021, epidemiological studies and cluster epidemic reports of COVID-19 were retrieved from SCI, Embase, PubMed, CNKI, Wanfang and CBM in English and Chinese, respectively. Relative risks (RRs) were estimated in pairwise comparisons of SARs between different contact environments using the frequentist NMA framework, and the ranking of risks in these environments was calculated using the surface under the cumulative ranking curve (SUCRA). Subgroup analysis was performed by regions. Thirty-two studies with 68 260 participants were identified. Compared with meal or gathering, transportation (RR 10.55, 95% confidence interval (CI) 1.43–77.85), medical care (RR 11.68, 95% CI 1.58–86.61) and work or study places (RR 10.15, 95% CI 1.40–73.38) had lower risk ratios for SARs. Overall, the SUCRA rankings from the highest to the lowest were household (95.3%), meal or gathering (81.4%), public places (58.9%), daily conversation (50.1%), transportation (30.8%), medical care (18.2%) and work or study places (15.3%). Household SARs were significantly higher than other environments in the subgroup of mainland China and sensitive analysis without small sample studies (<100). In light of the risks, stratified personal protection and public health measures need to be in place accordingly, so as close contacts categorising and management.
High dietary fibre intake has been associated with a lower risk of diabetes, but the association of dietary fibre with prediabetes is only speculative, especially in China, where the supportive data from prospective studies are lacking. This study aimed to examine the association between dietary fibre intake and risk of incident prediabetes among Chinese adults. We performed a prospective analysis in 18 085 participants of the Tianjin Chronic Low-grade Systemic Inflammation and Health cohort study who were free of diabetes, prediabetes, cancer and CVD at baseline. Dietary data were collected using a validated 100-item FFQ. Prediabetes was defined based on the American Diabetes Association diagnostic criteria. Cox proportional hazard models were used to estimate hazard ratios (HR) and 95 % CI. During 63 175 person-years of follow-up, 4139 cases of incident prediabetes occurred. The multivariable HR of prediabetes for the highest v. lowest quartiles were 0·85 (95 % CI 0·75, 0·98) (P for trend = 0·02) for total dietary fibre, 0·84 (95 % CI 0·74, 0·95) (P for trend < 0·01) for soluble fibre and 1·05 (95 % CI 0·93, 1·19) (P for trend = 0·38) for insoluble fibre. Fibre from fruits but not from cereals, beans and vegetables was inversely associated with prediabetes. Our results indicate that intakes of total dietary fibre, soluble fibre and fibre derived from fruit sources were associated with a lower risk of prediabetes.