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Desminopathy is a rare heritable cardiac and skeletal muscle disease caused by variants in the DES gene, which encodes the primary muscle-specific intermediate filament protein, known as desmin. Childhood-onset is commonly associated with severe early-onset myopathy and early death. Here, we reported an 11-year-old Chinese girl presenting with complete atrioventricular block and cardiomyopathy, without skeletal muscle involvement. Genetic analysis identified a de novo variant (c.152C > T/p.Ser51Phe) in the DES gene.
Within Chinese legal studies, the construction of a distinctly Chinese knowledge system emphasising “Chinese subjective consciousness” (中国主体意识) has become a new important agenda. This introductory article responds to this methodological turn and growing interest in a broader intellectual history of Chinese law. Until recently, traditional Western scholarship on Chinese legal history has largely focused on written legal documents, not on their underlying processes of knowledge production. While Chinese scholars acknowledge the significance of (cultural) translation of foreign legal knowledge, the entanglements of Chinese and Western legal genealogies within a knowledge-historical framework remains underexplored. This article introduces a knowledge-historical approach to study Chinese legal history by narrating the history of administrative law through the lens of local knowledge production through cultural translation. It reveals compelling stories of local actors, who engaged with new knowledge of administrative law in multiple processes and layers of knowledge production from the late Qing dynasty to the late 1980s.
We investigate fully developed turbulent flow in curved channels to explore the interaction between turbulence and curvature-driven coherent structures. By focusing on two cases of mild and strong curvature, we examine systematically the effects of the Reynolds number through a campaign of direct numerical simulations, spanning flow regimes from laminar up to the moderately high Reynolds number – based on bulk velocity and channel height – of $87\,000$. Our analysis highlights the influence of curvature on the friction coefficient, showing that flow transition is anticipated by concave curvature and delayed by convex curvature. In the case of mild curvature, a frictional drag reduction compared with plane channel flow is found in the transitional regime. Spectral analysis reveals that the near-wall turbulence regeneration cycle is maintained in mildly curved channels, while it is absent or severely inhibited on the convex wall of strongly curved channels. Streamwise large-scale structures resembling Dean vortices are found to be weakly dependent on the Reynolds number and strongly affected by curvature: increasing curvature shifts these vortices towards the outer wall and reduces their size and coherence, limiting their contribution to streamwise velocity fluctuations and momentum transport. In the case of strong curvature, spanwise large-scale structures are also detected. These structures are associated with large pressure fluctuations and the suppression of turbulent stresses near the convex wall, where a region with negative turbulence production is observed and characterised via quadrant analysis.
We study the role of competition in customers’ reactions to litigation against firms, using anonymized mobile phone location data. A class action lawsuit filing is followed by a 4% average reduction in customer visits to target firms’ outlets in the following months. The effect strongly depends on competition. Outlets facing more competition experience significantly larger negative effects. Closer competition matters more, both in terms of geographic and industry proximity. Announcement returns and quarterly accounting revenues around lawsuit filings also strongly depend on competition. Our results suggest that competition is an important component in customers’ ability to discipline firms for misbehavior.
This article investigates the assumption that survival should form the goal of the politics both of nuclear weapons and of International Relations (IR). Rather than being a self-evident grounding upon which political contest then plays out, survival has its own implications and limitations in the thermonuclear age, in which survival has become premised on the threat of total annihilation. As such, nuclear weapons allow us to unpack ‘survival’ in a unique way because they have the power to destroy everything: to end all survival. Yet at the same time, nuclear weapons have become deeply embedded into our world. In an age of thermonuclear weaponry, survival through annihilation has thus become a paradox that structures the ambivalence of nuclear technology and the tensions of nuclear politics. The article first establishes the nature of assumptions of survival as a taken-for-granted goal of IR and nuclear weapons politics. It then argues that the paradoxical logic of survival as annihilation that accompanied the nuclear era has resulted in a politics of repetition in which both deterrence and disarmament actors have become trapped. The article ends by outlining the limits of understanding whose survival is at stake in nuclear weapons discourse and why this matters.
We introduce a wall model for large-eddy simulation (WMLES) applicable to rough surfaces with Gaussian and non-Gaussian distributions for both the transitionally and fully rough regimes. The model is applicable to arbitrary complex geometries where roughness elements are assumed to be underresolved, i.e. subgrid-scale roughness. The wall model is implemented using a multi-hidden-layer feedforward neural network, with the mean geometric properties of the roughness topology and near-wall flow quantities serving as input. The optimal set of non-dimensional input features is identified using information theory, selecting variables that maximize information about the output while minimizing redundancy among inputs. The model also incorporates a confidence score based on Gaussian process modelling, enabling the detection of potentially low model performance for untrained rough surfaces. The model is trained using a direct numerical simulation (DNS) roughness database comprising approximately 200 cases. The roughness geometries for the database are selected from a large repository through active learning. This approach ensures that the rough surfaces incorporated into the database are the most informative, achieving higher model performance with fewer DNS cases compared with passive learning techniques. The performance of the model is evaluated both apriori and aposteriori in WMLES of turbulent channel flows with rough walls. Over 550 channel flow cases are considered, including untrained roughness geometries, roughness Reynolds numbers and grid resolutions for both transitionally and fully rough regimes. Our rough-wall model offers higher accuracy than existing models, generally predicting wall shear stress within an accuracy range of 1%–15 %. The performance of the model is also assessed on a high-pressure turbine blade with two different rough surfaces. We show that the new wall model predicts the skin friction and the mean velocity deficit induced by the rough surface on the blade within 1%–10 % accuracy except the region with transition or shock waves. This work extends the building-block flow wall model (BFWM) introduced by Lozano-Durán & Bae (2023. J. Fluid Mech.963, A35) for smooth walls, expanding the BFWM framework to account for rough-wall scenarios.
Large-eddy simulations are analysed to determine the influence of suspended canopies, such as those formed in macroalgal farms, on ocean mixed layer (OML) deepening and internal wave generation. In the absence of a canopy, we show that Langmuir turbulence, when compared with wind-driven shear turbulence, results in a deeper OML and more pronounced internal waves beneath the OML. Subsequently, we examine simulations with suspended canopies of varying densities located in the OML, in the presence of a background geostrophic current. Intensified turbulence occurs in the shear layer at the canopy’s bottom edge, arising from the interaction between the geostrophic current and canopy drag. Structures resembling Kelvin–Helmholtz (KH) instability emerge as the canopy shear layer interacts with the underlying stratification, radiating internal waves beneath the OML. Both intensified turbulence and lower-frequency motions associated with KH-type structures are critical factors in enhancing mixing. Consequently, the OML depth increases by up to a factor of two compared with cases without a canopy. Denser canopies and stronger geostrophic currents lead to more pronounced KH-type structures and internal waves, stronger turbulence and greater OML deepening. Additionally, vertical nutrient transport is enhanced as the OML deepens due to the presence of the canopy. Considering that the canopy density investigated in this study closely represents offshore macroalgal farms, these findings suggest a mechanism for passive nutrient entrainment conducive to sustainable farming. Overall, this study reveals the intricate interactions between the suspended canopy, turbulent mixing and stratification, underscoring their importance in reshaping OML characteristics.
A collection of secondary instability calculations in streaky boundary layers is presented. The data are retrieved from well-resolved numerical simulations of boundary layers forced by free-stream turbulence (FST), considering different geometries and FST conditions. The stability calculations are performed before streak breakdown, taking place at various $Rey_x$ the Reynolds number based on the streamwise coordinate. Despite the rich streak population of various sizes, it is found that breaking streaks have similar aspect ratios, independently of the streamwise position where they appear. This suggests that wider streaks will break down further downstream than thinner ones, making the appearance of secondary instabilities somewhat independent of the streak’s wavelength. Moreover, the large difference in the integral length scale among the simulations suggests that this aspect ratio is also independent of the FST scales. An explanation for this behaviour is provided by showing that these breaking streaks are in the range of perturbations that can experience maximum transient growth according to optimal disturbance theory. This could explain why, at a given streamwise position, there is a narrow spanwise wavelength range where streak breakdown is more likely to occur.
Wave propagation in channels with area changes is a topic of significant practical interest that involves a rich set of coupled physics. While the acoustic wave problem has been studied extensively, the shock propagation problem has received less attention. In addition to its practical significance, this problem also introduces deep fundamental issues associated with how energy in propagating large-amplitude disturbances is redistributed upon interaction with inhomogeneities. This paper presents a study of shock scattering and entropy and vorticity coupling for shock wave propagation through discrete area changes. It compares results from computational fluid dynamics to those of one-dimensional quasi-steady calculations. The solution space is naturally divided into five ‘regimes’ based upon the incident shock strength and area ratio. This paper also presents perturbation methods to quantify the dimensionless scaling of physical effects associated with wave reflection/transmission and energy transfer to other disturbances. Finally, it presents an analysis of the ‘energetics’ of the interaction, quantifying how energy that initially resides in dilatational disturbances and propagates at the shock speed is redistributed into finite-amplitude reflected and transmitted waves as well as convecting vortical and entropy disturbances.
Studies on longitudinal associations between diet quality and lipid and amino acid metabolism in children and adolescents are limited. We studied associations between diet quality and serum markers of lipid and amino acid metabolism in the Physical Activity and Nutrition in Children (PANIC) study. These analyses included 403 children aged 6–9 years at baseline, 360 re-examined 2 years later at age 9–11 years and 219 eight years later at age 15–17 years. Food intake was recorded over 4 days, and diet quality was assessed using the Finnish Children Healthy Eating Index (FCHEI). Fasting serum fatty acids, amino acids, apolipoproteins and lipoprotein particle sizes were analysed via NMR spectroscopy. Linear mixed-effects models, adjusted for sex, age, body fat percentage, pubertal stage and physical activity were used to analyse the associations. Better diet quality was linked to increased serum PUFA and reduced saturated and MUFA, alanine and VLDL particle size. Consuming more vegetables, fruits, berries, vegetable oils and margarine with at least 60 % fat, fish and whole grains is associated with higher serum PUFA, lower SFA and smaller VLDL particles. Conversely, consuming higher-fat dairy products and sugary products is associated with higher saturated and MUFA, branched-chain and aromatic amino acids and larger VLDL particles. A diet rich in fruits, vegetables, unsaturated fats and fibre, with reduced sugar consumption, promotes favourable metabolic changes relevant to cardiometabolic health.
The formation process of a vortex pair generated by a two-dimensional starting jet has been investigated numerically over a range of Reynolds numbers from 500 to 2000. The effects of stroke ratio and nozzle configuration are examined. Only a single vortex pair can be observed in the vorticity field generated by small stroke ratios less than 10 while the leading vortex pair formed by larger stroke ratios eventually disconnects from the trailing jet. The formation numbers (13.6 and 9.3) for a straight nozzle and an orifice nozzle have been identified by the circulation criterion and they are further analysed by four other criteria. Using the contraction coefficient, formation numbers can be transformed into a universal value at about 16.5 for both nozzles. The effect of Reynolds number on the formation number is found to be within 12 % for parallel flow cases but it will increase up to 27 % for non-parallel flow cases due to shear-layer instability. A modified contraction-based slug model is proposed, and it can accurately predict the total invariants (e.g. circulation, hydrodynamic impulse and kinetic energy) shedding from the nozzle edge. Analytical estimation of the formation number is further conducted by matching the predicted total invariants to the Pierrehumbert model of steady vortex pairs. By assuming that pinch-off starts when the vortex pair achieves the steady state, two analytical models are proposed in terms of vortex impulse and translational velocity. The latter appears to be more appropriate to predict the formation number for two-dimensional flows.
This article presents a New Keynesian model to capture the linkages between macro fundamentals and the nominal yield curve. The model explains bond yields with a low level of news in expected inflation and plausible term premia. This implies that the slope of the yield curve predicts future bond yields and that risk-adjusted historical bond yields satisfy the expectations hypothesis. The model also explains the spanning puzzle, matches key moments for real bond yields, captures the evolution of the price-dividend ratio, and implies that the slope of the yield curve and the price-dividend ratio forecast excess equity returns.
This study aims to formulate a highly accurate numerical method, specifically a seventh-order Hermite technique with an error term of sixth order, to solve the Fisher and Burgers–Fisher equations. This technique employs a combination of orthogonal collocation on the finite element method and hepta Hermite basis functions. By ensuring continuity of the dependent variable and its first three derivatives across the entire solution domain, it achieves a remarkable level of accuracy and smoothness. The space discretization is handled through the application of hepta Hermite polynomials, while the time discretization is managed by the Crank–Nicholson scheme. The stability and convergence analysis of the scheme are discussed in detail. To validate the accuracy of the proposed technique, three examples are taken. The results obtained from these examples are thoroughly analysed and compared against the exact solutions and reliable data from the existing literature. It is established that the proposed technique is easy to implement and gives better results as compared with existing ones.
This study elucidates the litter dynamics including decomposition rate both in-situ and ex-situ, the initial acquisition traits (LATs), morphological traits (LMTs) and production rate of leaf litter of four economically important tree species viz. Terminalia arjuna (TA), Tectona grandis (TG), Eucalyptus citriodora (EC) and Psidium guajava (PG) with the major objective of restoration of degraded urban ecosystems in dry tropics. Annual litterfall production rates were observed as: TG > TA > EC > PG. LMTs, that is, specific leaf area followed the trend: TG > TA > PG > EC, whereas leaf mass per area followed the reverse trend. In TA, LATs involving carbon (C), nitrogen (N) and cellulose were highest but C/N and lignin/N ratios were lowest, whereas lignin, polyphenol, C/N and lignin/N ratios were highest in PG. In the leaf litter bag experiment, the decomposition rate followed the trend: TA > TG > EC > PG. In-situ and ex-situ rates of decomposition of all the four leaf litters were found to be similar. LATs especially lignin/N, N and C/N ratios rather than LMTs were found to be a better predictor of the litter decomposition rate. TA plantation having a higher litter decomposition rate, may be recommended for inclusion in the restoration strategies of degraded urban land.
The Circle of Security – Parenting (COSP™) is a psychoeducational intervention aiming at fostering secure child-parent attachment relationships. In a randomized controlled trial, we investigate the effect of COSP™ as an adjunct to care-as-usual compared to only care-as-usual for at-risk families. Mothers and their 2–12-month-old infants were randomized into COSP™ +care-as-usual (n = 197) for at-risk families in Copenhagen or only care-as-usual (n = 100). At-risk status was either mothers diagnosed with postpartum depression and/or infants showed social withdrawal. The primary outcome was maternal sensitivity which was coded with the Coding Interactive Behavior. Our secondary outcomes were maternal reflective functioning, assessed with the Parental Reflective Functioning Questionnaire – Infant Version, and child-mother attachment, assessed with the Strange Situation Procedure. Results showed no significant differences between the RCT groups on either the primary or secondary outcomes (all ps ≥ .146). We discuss these findings in relation to the applicability and targeted population who can benefit from COSP™, and whether alternative programs would be more effective for at-risk families with infants.
Turbulent transonic buffet is an aerodynamic instability causing periodic (albeit, often irregular) oscillations of lift/drag in aerospace applications. Involving complex coupling between inviscid and viscous effects, buffet is characterised by shock wave oscillations and flow separation/reattachment. Previous studies have identified both two-dimensional (2-D) chordwise shock-oscillation and three-dimensional (3-D) buffet-/stall-cell modes. While the 2-D instability has been studied extensively, investigations of 3-D buffet have been limited to only low-fidelity simulations or experiments. Due to computational cost, almost all high-fidelity studies to date have been limited to narrow span-widths around 5 % of aerofoil chord length (aspect ratio, ), which is insufficiently wide to observe large-scale three-dimensionality. In this work, high-fidelity simulations are performed up to , on an infinite unswept NASA Common Research Model (CRM) wing profile at $Re=5\times 10^{5}$. At , intermittent 3-D separation bubbles are observed at buffet conditions. While previous Reynolds-averaged Navier–Stokes (RANS)/stability-based studies predict quasi-simultaneous onset of 2-D- and 3-D-buffet, a case that remains essentially 2-D is identified here. Strongest three-dimensionality was observed near low-lift phases of the buffet cycle at maximum flow separation, reverting to essentially 2-D behaviour during high-lift phases. Buffet was found to become 3-D when extensive mean flow separation was present. At , multiple 3-D separation bubbles form in a spanwise wavelength range $\lambda =1c$ to $1.5c$. Spectral proper orthogonal decomposition (SPOD) was applied to analyse the spatio/temporal structure of 3-D buffet-cells. In addition to the 2-D chordwise shock-oscillation mode (Strouhal number $St \approx 0.07-0.1$), 3-D modal structures were observed at the shock wave/boundary layer interaction at $St \approx 0.002-0.004$.
The brachyuran crab fauna (Crustacea: Decapoda: Brachyura) collections in the extensive exploratory fishery survey cruises from the northeast coast of India was studied from June 2018 to March 2020. During the study period, 81 species of brachyuran crabs belonging to 19 families and 46 genera were recorded. Among these, 21 species were newly recorded from the northeast coast of India, and 1 species, Naxioides taurus (Pocock, 1890) represents a new record for the mainland coasts of India. This study serves as a baseline for understanding the diversity and distribution of brachyuran crabs in the region. It provides valuable insights for future research and conservation efforts.