Miracle-dz/UniFAR-FTUs huggingface dataset:
Cysteamine bitartrate delayed‐release capsules control leukocyte cystine levels and promote statural growth and kidney health in an open‐label study of treatment‐naïve patients <6 years of age with nephropathic cystinosis

Nephropathic cystinosis is a rare autosomal recessive lysosomal storage disease that is characterized by accumulation of cysteine and formation of crystals within cells of different organs and tissues causing systemic manifestations in childhood that include poor linear growth, ocular involvement, hypothyroidism, and progressive kidney disease. This study was a long‐term, prospective open‐label evaluation of twice‐daily delayed release (DR) cysteamine capsules in cystinosis patients <6 years of age who were naïve to any form of cysteamine treatment. Fifteen treatment‐naïve patients <6 years old (mean age 2.2 ± 1.0 years, 53% male, 73% White) were enrolled and treated with DR‐cysteamine capsules for up to 18 months. Patients had clinically meaningful decreases in WBC cysteine concentration during treatment (3.2 ± 3.0 nmol ½ cystine/mg protein at Day 1 to 0.8 ± 0.8 nmol ½ cystine/mg protein at study exit), and anthropometric data improvements were consistently observed in height, weight and body surface area. Additionally, estimated glomerular filtration rate increased from 55.93 ± 22.43 ml/min/1.73 m2 at baseline to 63.79 ± 21.44 ml/min/1.73 m2 at study exit. Pharmacokinetic/Pharmacodynamic results support the use of the same starting, escalation, and maintenance doses according to body surface for children aged <6 years that are currently recommended in adults and older children. All patients experienced ≥1 adverse event(s) with vomiting (80%) and upper respiratory tract infection (53%) most frequently reported. Based on our study, patients <6 years of age with nephropathic cystinosis without prior treatment can safely and effectively initiate treatment with DR‐cysteamine, a delayed‐release form of cysteamine bitartrate that can be given every 12 h.
Accounting for Cryptocurrency Value

Information pertaining to new addresses is highly value-relevant in the cryptocurrency market. Innovations to the number of new addresses explain eight percent of the variations in cryptocurrency returns. Unlike traditional markets, we do not ﬁnd evidence of pre- or post-drift around the release of new address information. The presence of strong market reactions at the release of new address information and the absence of drifts around the release highlight the distinct features of cryptocurrencies and provide a benchmark case where information is released publicly and continuously. Lastly, we construct the price-to-new address ratios and show that they negatively predict future cryptocurrency returns, which we refer to as the cryptocurrency value effect.
Plant Gravitropism and Signal Conversion under a Stress Environment of Altered Gravity

Humans have been committed to space exploration and to find the next planet suitable for human survival. The construction of an ecosystem that adapts to the long-term survival of human beings in space stations or other planets would be the first step. The space plant cultivation system is the key component of an ecosystem, which will produce food, fiber, edible oil and oxygen for future space inhabitants. Many plant experiments have been carried out under a stimulated or real environment of altered gravity, including at microgravity (0 g), Moon gravity (0.17 g) and Mars gravity (0.38 g). How plants sense gravity and change under stress environment of altered gravity were summarized in this review. However, many challenges remain regarding human missions to the Moon or Mars. Our group conducted the first plant experiment under real Moon gravity (0.17 g) in 2019. One of the cotton seeds successfully germinated and produced a green seedling, which represents the first green leaf produced by mankind on the Moon.
An Exploratory Analysis of Factors Affecting Participation in Air Force Knowledge Now Communities of Practice

Abstract : As the AF continues to lose its knowledge base through retirements and downsizing, the need to get maximum use from the remaining knowledge base becomes increasingly important. In their efforts to help the Department of Defense and the Air Force Chief Information Officer (AFCIO) meet their knowledge management goals, Air Force Material Command (AFMC) has been working to implement the use of communities of practice. A primary goal of AFMC/DRW , the AF Knowledge Now program office, and the office of the AFCIO is to increase effectiveness and participation within communities of practice. The goal of this research is to identify factors from the literature that may affect knowledge transfer, information sharing, and technology acceptance, and compare those factors with AFKN hosted communities of practice (CoPs) exhibiting high and low levels of participation. Additionally, factors of interest identified in interviews with AFKN personnel will be researched. This research used a cross-sectional research instrument to survey CoP members within all AFKN hosted CoPs containing 20 or more members. This research suggests there are differences in the way members within high and low use CoPs perceive these factors: trust, willingness to share information, job fit, outcome expectations, social factors, facilitating conditions, anonymity, security constraints, knowledge champion, facilitator. The results of these findings may allow AFKN to focus on these factors when the goal is to improve participation in future CoPs.
Local Dynamics of Offshore Wind Turbine Jacket Sub-structures

The scope of this thesis has been to investigate the existence of out-of-plane vibration of jacket sub-structures for offshore wind turbines. This is done by making the program code JAC (Jacket Analysis Code), which is capable of solving dynamic problems in both time and frequency domain. The code is also able to do damage analysis as it has been a major goal to quantify the change in damage caused by the out-of-plane vibrations and to see how this is influenced by dierent parameters. It is shown that the out-of-plane vibrations are caused by rotation of the intersection node, between tower and jacket, exciting a global-for-aft and global side-side eigenmode of the jacket structure. The rotation of the intersection node is probably caused by the higher harmonic components of load from the rotor. The out-of-plane vibration gives a significant contribution to the damage at some parts of the structure. In particular this applies to the face of the jacket perpendicular to the main load direction. However for the analysis performed by the authors, the damage generated by the in-plane stresses is governing. The infjuence of marine growth, structural damping and incremental time step is investigated. It is shown how these parameters will have significant influence on the damage of the structure. The analysis is done using a sequential approach where calculations on wind turbine and sub-structure are separated. The thesis shows that this method could highly overestimate the response of out-of-plane vibrations if the structure is exposed to a widely scattered load. This is due to coupling of modes.
Perceived threats and the trade-off between security and human rights

It is well established that exposure to threats causes citizens to prioritize security considerations and accept restrictions on civil liberties. Yet most studies on which these findings are based come from longstanding democracies and do not distinguish among types of threat. This article argues that the effects of internal and external threats are conditional on regime type. It tests the argument via an experiment embedded in an original survey of Georgia and Kazakhstan, countries that vary in regime type but face similar levels of threat. In authoritarian Kazakhstan, there is no difference in attitudes by threat type, whereas external threats produce greater support for security than internal ones in more pluralistic Georgia. Contrary to previous research, security preferences are not mediated by the triggering of anxiety. The findings contribute to literatures on the link between threats and authoritarian preferences, the rally-round-the-flag effect, and the ways that political institutions mediate psychological processes.
Cohesin Mutations Induce Chromatin Conformation Perturbation of the H19/IGF2 Imprinted Region and Gene Expression Dysregulation in Cornelia de Lange Syndrome Cell Lines

Traditionally, Cornelia de Lange Syndrome (CdLS) is considered a cohesinopathy caused by constitutive mutations in cohesin complex genes. Cohesin is a major regulator of chromatin architecture, including the formation of chromatin loops at the imprinted IGF2/H19 domain. We used 3C analysis on lymphoblastoid cells from CdLS patients carrying mutations in NIPBL and SMC1A genes to explore 3D chromatin structure of the IGF2/H19 locus and evaluate the influence of cohesin alterations in chromatin architecture. We also assessed quantitative expression of imprinted loci and WNT pathway genes, together with DMR methylation status of the imprinted genes. A general impairment of chromatin architecture and the emergence of new interactions were found. Moreover, imprinting alterations also involved the expression and methylation levels of imprinted genes, suggesting an association among cohesin genetic defects, chromatin architecture impairment, and imprinting network alteration. The WNT pathway resulted dysregulated: canonical WNT, cell cycle, and WNT signal negative regulation were the most significantly affected subpathways. Among the deregulated pathway nodes, the key node of the frizzled receptors was repressed. Our study provides new evidence that mutations in genes of the cohesin complex have effects on the chromatin architecture and epigenetic stability of genes commonly regulated by high order chromatin structure.
How explicit instruction improves phonological awareness and perception of L2 sound contrasts in younger and older
 adults

Despite the importance of conscious awareness in second language acquisition theories, little is known about how L2 speech perception can be improved by explicit phonetic instruction. This study examined the relationship between phonological awareness and perception in Dutch younger and older adult L2 listeners, focusing on English contrasts of two types: a familiar contrast in an unfamiliar position (word-final /t/-/d/) and an unfamiliar contrast (/æ/-/ε/). Awareness was assessed with a task in which written minimal pairs and homophone pairs had to be judged as sounding the same or different. Perception was assessed with a two-alternative forced-choice identification task with auditorily presented words from minimal pairs. We investigated whether listeners’ awareness and perception improved after a video-based explicit instruction that oriented their attention to one of these contrasts, and we tested whether including information about the phonetic cue of vowel duration increased learning. Awareness and perception of each contrast were shown to be moderately correlated at the study’s outset. Furthermore, awareness and perception for each contrast generally improved more after the instruction drawing attention to that contrast. However, the effectiveness of explicit phonetic instruction varied depending on the combination of the contrast, cue information, and listener age group.
Solid-boundary treatment for moment systems

Solid-boundary treatment for moment systems by Loc Huu Khieu Chair: Bram van Leer Hyperbolic-relaxation systems for describing fluid flow, such as Grad’s 13-moment (13M) system, are an alternative to the systems generated by the Chapman-Enskog expansion, which include derivatives of increasing order. The former have a number of numerical advantages stemming from the compactness of their spatial operator. They have been demonstrated to be suited for subsonic and transonic flow. In this dissertation we explore the solid-wall boundary conditions that have to supplement these moment systems. Starting from the boundary treatment proposed by Grad, we first remove an inconsistency in his theory that would lead to ambiguous results. We formulate two boundary conditions, 1–C and 2–C , containing one and two parameters, respectively; these are used to satisfy the non-penetration condition in 1–C and 2–C , and an additional normalization condition in 2–C . The boundary conditions are implemented in a computational code based on the 13M system. In order to study the accuracy of the new boundary condition we also develop a benchmark code based on the Boltzmann equation with Bhatnagar–Gross–Krook collision model (BGK equation), and implement the same boundary conditions in
Acute Effects of Dermal Suction on Passive Muscle and Joint Stiffness

The aim of the present study was to examine the acute effects of dermal suction on the passive mechanical properties of specific muscles and joints. Dermal suction was applied to the calves of 24 subjects. Passive plantar flexion torque was measured with the right knee fully extended and the right ankle positioned at 20°, 10°, 0°, and −10° angles, where 0° represents the ankle neutral position, and positive values correspond to the plantar flexion angle. The shear wave velocity (SWV) (m/s) of the medial gastrocnemius was measured in the same position using ultrasound shear wave elastography. The relationship between the joint angle and passive torque at each 10° angle was defined as passive joint stiffness (Nm/°). Passive muscle and joint stiffness were measured immediately before and after the dermal suction protocol. When the ankle joint was positioned at 20° (r = 0.53, P = 0.006), 10° (r = 0.43, P = 0.030), and −10° (r = 0.60, P = 0.001), the SWV was significantly higher after dermal suction than that before dermal suction. Regarding joint stiffness, we found no significant difference between the pre- and post-dermal suction values (partial η2 = 0.093, P > 0.05). These findings suggest that dermal suction increases passive muscle stiffness and has a limited impact on passive joint stiffness.
ExoMars TGO/NOMAD‐UVIS Vertical Profiles of Ozone: 1. Seasonal Variation and Comparison to Water

We present ∼1.5 Mars Years (MY) of ozone vertical profiles, covering LS = 163° in MY34 to LS = 320° in MY35, a period which includes the 2018 global dust storm. Since April 2018, the Ultraviolet and Visible Spectrometer channel of the Nadir and Occultation for Mars Discovery (NOMAD) instrument aboard the ExoMars Trace Gas Orbiter has observed the vertical, latitudinal and seasonal distributions of ozone. Around perihelion, the relative abundance of both ozone and water (from coincident NOMAD measurements) increases with decreasing altitude below ∼40 km. Around aphelion, localized decreases in ozone abundance exist between 25 and 35 km coincident with the location of modeled peak water abundances. High‐latitude (>±55°), high altitude (40–55 km) equinoctial ozone enhancements are observed in both hemispheres (LS ∼350°–40°) and discussed in the companion article to this work (Khayat et al., 2021). The descending branch of the main Hadley cell shapes the observed ozone distribution at LS = 40°–60°, with the possible signature of a northern hemisphere thermally indirect cell identifiable from LS = 40°–80°. Morning terminator observations show elevated ozone abundances with respect to evening observations, with average ozone abundances between 20 and 40 km an order of magnitude higher at sunrise compared to sunset, attributed to diurnal photochemical partitioning along the line of sight between ozone and O or fluctuations in water abundance. The ozone retrievals presented here provide the most complete global description of Mars ozone vertical distributions to date as a function of season and latitude.
Molecular Consequences of Depression Treatment: A Potential In Vitro Mechanism for Antidepressants-Induced Reprotoxic Side Effects

The incidence of depression among humans is growing worldwide, and so is the use of antidepressants. However, our fundamental understanding regarding the mechanisms by which these drugs function and their off-target effects against human sexuality remains poorly defined. The present study aimed to determine their differential toxicity on mouse spermatogenic cells and provide mechanistic data of cell-specific response to antidepressant and neuroleptic drug treatment. To directly test reprotoxicity, the spermatogenic cells (GC-1 spg and GC-2 spd cells) were incubated for 48 and 96 h with amitriptyline (hydrochloride) (AMI), escitalopram (ESC), fluoxetine (hydrochloride) (FLU), imipramine (hydrochloride) (IMI), mirtazapine (MIR), olanzapine (OLZ), reboxetine (mesylate) (REB), and venlafaxine (hydrochloride) (VEN), and several cellular and biochemical features were assessed. Obtained results reveal that all investigated substances showed considerable reprotoxic potency leading to micronuclei formation, which, in turn, resulted in upregulation of telomeric binding factor (TRF1/TRF2) protein expression. The TRF-based response was strictly dependent on p53/p21 signaling and was followed by irreversible G2/M cell cycle arrest and finally initiation of apoptotic cell death. In conclusion, our findings suggest that antidepressants promote a telomere-focused DNA damage response in germ cell lines, which broadens the established view of antidepressants’ and neuroleptic drugs’ toxicity and points to the need for further research in this topic with the use of in vivo models and human samples.
A High Risk of Missing Congenital Cytomegalovirus-Associated Hearing Loss through Newborn Hearing Screening in Japan

It remains unclear to what extent newborn hearing screening (NHS) detects congenital cytomegalovirus (cCMV)-associated sensorineural hearing loss (SNHL) in Japan. This study aimed to clarify the NHS results and audiological characteristics of patients with cCMV-associated SNHL. A total of 541 individuals with unilateral or bilateral hearing loss of unknown etiology were examined for cCMV infection. cCMV infection was defined by the presence of CMV DNA in the dried umbilical cord detected using real-time quantitative PCR. NHS results and audiological data were retrospectively obtained from medical records. Forty-four cases (8.1%) were positive for cCMV infection. Of them, 33 cases underwent NHS and 13 cases (39.4%) passed NHS bilaterally. The pure-tone audiograms of 21 patients were obtained. There were seven cases of unilateral SNHL, five cases of asymmetric bilateral SNHL, and nine cases of symmetric bilateral SNHL. cCMV-related hearing loss is highly heterogeneous, and there is a high risk of missing this condition through NHS.
Copper(ii) complexes based on tripodal pyridyl amine derivatives as efficient anticancer agents

The in vitro cytotoxicity of a series of chlorido-Cu(ii) complexes based on tripod pyridyl N4-donor derivatives revealed significant-to-moderate cytotoxicity against human cancer cell lines with the best results obtained for [Cu(BQPA)Cl]ClO4/PF6 (5-ClO4/PF6) with IC50 values of 4.7–10.8 μM.
Health QR Code Application in the Novel Containment Strategy and Healthcare Plan for Pregnant Women and Children Under Quarantine During the Summer Outbreak of SARS-CoV-2 Delta Variant in Chengdu, China: An Observational Study

Purpose To report the experience of health QR code application in Chengdu’s anti-epidemic measures including circle-layer management, hospital triage system and healthcare plan for quarantined pregnant women and children during the summer outbreak of SARS-CoV-2 Delta strain in 2021 and to evaluate these measures. Methods We comprehensively summarized Chengdu’s health code application in the circle-layer management (a set of stringent confinement measures of places confirmed cases and close contacts have recently been to and less strict quarantine measures of surrounding areas), hospital triage system, and healthcare plan for quarantined pregnant women and children. We also assessed the effectiveness or efficiency of these measures by analyzing the number of different cases with confirmed COVID-19 infections or epidemiological history, the attitude of quarantined pregnant women toward the summer outbreak and healthcare services, as well as the time needed for obtaining epidemiological history and accuracy of health-code-based hospital triage system. Results The circle-layer management lasted 15 days and ended with no community or nosocomial transmission happened. Approximately 70 pregnant women and 600 children below 6-year-old were quarantined. Four home visits and two patient transfers were performed. Online survey indicated that about 80% of quarantined women felt satisfactory about the healthcare service. The novel triage system identified 137/221 (61.99%) patients with epidemiological history from patients with yellow health code, and 71/4504 (1.57%) patients from patients with green health code in our hospital (p < 0.001). The health QR code markedly outperformed the traditional methods in the efficiency experiment of obtaining epidemiological history (3.52 ± 0.98 vs 78.91 ± 23.18 seconds, P < 0.001). Conclusion The circle-layer management has successfully and precisely prevented the spread of the summer outbreak of COVID-19 in Chengdu. The health-code-based triage system showed great effectiveness and efficiency in triaging patients with epidemiological history. The healthcare services for quarantined pregnant women has basically met their needs.
Violence among State House Candidates during the COVID‐19 Pandemic

Although there is an established comparative literature addressing electoral violence, and an emerging literature on violence against US officeholders, research has not examined levels of violence perpetrated by constituents against US candidates, nor how a crisis, such as the COVID‐19 pandemic, including the political polarization connected to it, may affect violence. This note presents findings from a survey of state house candidates from 18 US states in the 2020 electoral cycle. The findings focus on psychological violence and suggest that it is a common occurrence, and that the COVID‐19 pandemic and polarization may have affected it. Specifically, most candidates reported that: they believe that the pandemic led to violence; those in states with larger increases in COVID‐19 cases and those running in states with higher levels of COVID‐19 public political events reported more violence. In addition, candidates running in more polarized states experienced more violence.
A test validated model of plates with constrained viscoelastic materials

Surface damping treatments by viscoelastic materials are often considered nowadays to enhance dissipation in sheet metal and laminated glass parts. Constraining layers are needed to increase shear levels in the viscoelastic which is the primary source of dissipation. The damping behavior of such sandwich types structures is strongly influenced by the characteristics of the constraining layer and the deformation modes of the structure. It is first shown that finite element models based on classical laminated plate theory (CLPT) do not allow a proper representation of boundary conditions of the viscoelastic and the constraining layer which leads to an inaccurate prediction of the behavior of the damped structure. A layered shell/solid/shell model is introduced and shown to correct these shortcomings. The validity of the proposed model is demonstrated by correlation with experimental results obtained on the GARTEUR SM-AG-19 testbed. The damping treatment of this structure is a layer of ISD112 (a viscoelastic manufactured by 3M) constrained by an aluminum plate. Test and analysis damping levels are first shown to be very well correlated. Temperature dependence and computational times are finally discussed to give further insight in the proposed methodology.