Miracle-dz/UniFAR-FTUs huggingface dataset:
Hypochondroplasia gain-of-function mutation in FGFR3 causes defective bone mineralization in mice

Hypochondroplasia (HCH) is a mild dwarfism caused by missense mutations in fibroblast growth factor receptor 3 (FGFR3), with the majority of cases resulting from a heterozygous p.Asn540Lys gain-of-function mutation. Here, we report the generation and characterization of the first mouse model (Fgfr3Asn534Lys/+) of HCH to our knowledge. Fgfr3Asn534Lys/+ mice exhibited progressive dwarfism and impairment of the synchondroses of the cranial base, resulting in defective formation of the foramen magnum. The appendicular and axial skeletons were both severely affected and we demonstrated an important role of FGFR3 in regulation of cortical and trabecular bone structure. Trabecular bone mineral density (BMD) of long bones and vertebral bodies was decreased, but cortical BMD increased with age in both tibiae and femurs. These results demonstrate that bones in Fgfr3Asn534Lys/+ mice, due to FGFR3 activation, exhibit some characteristics of osteoporosis. The present findings emphasize the detrimental effect of gain-of-function mutations in the Fgfr3 gene on long bone modeling during both developmental and aging processes, with potential implications for the management of elderly patients with hypochondroplasia and osteoporosis.
Synthesis, thermal evolution and magnetic investigations of SBA-15 silica functionalized with anchored iron phosphonate molecules

In the current work, we report on the synthesizing of a series of novel nanocomposite materials obtained by functionalizing the SBA-15 silica matrix with anchored iron phosphonate molecules and the following thermal treatment. The obtained results reveal the formation of a unique amorphic layer of Fe-based compounds on the surface of silica walls of SBA-15 channels as a result of the organic groups’ decomposition after moderate thermal treatment. Due to their unique structure, represented in an active Fe-containing amorphous coating spread over a large surface area, these materials are of great interest for their potential applications in fields such as catalysis, adsorption, and non-linear optics. The obtained materials remain amorphous, preserving the SBA-15 mesoporous structure up to temperatures of approximately 800 °C, after which the partial melting of the silica backbone is observed with the simultaneous formation of nanocrystals inside the newly-formed glassy mass. All obtained materials were characterized using such techniques as thermogravimetry, transmission and scanning electron microscopy combined with energy dispersive x-ray spectroscopy mapping, Raman spectroscopy, N2 sorption analysis, x-ray diffraction, x-ray photoelectron spectroscopy, Mössbauer spectroscopy, and SQUID measurements.
Calprotectin, a sensitive marker of inflammation, is robustly assessed in plasma from patients with early or established rheumatoid arthritis by use of different laboratory methods

Abstract Calprotectin (S100A8/S100A9, MRP8/MRP14) is a major leukocyte protein found to be more sensitive than C-Reactive Protein (CRP) and Erythrocyte Sedimentation Rate (ESR) as a marker of inflammation in patients with rheumatoid arthritis (RA). The present objective was to explore the robustness of calprotectin assessments by comparing two different laboratory methods assessing calprotectin in plasma samples from patients with early or established RA. A total of 212 patients with early RA (mean (SD) age 52(13.3) years, disease duration 0.6(0.5) years) and 177 patients with established RA (mean (SD) age 52.9(13.0) years, disease duration 10.0(8.8) years) were assessed by clinical, laboratory, and ultrasound examinations. Frozen plasma samples (−80 °C) were analysed for calprotectin levels at baseline, 1, 2, 3, 6 and 12 months by use of either enzyme-linked immunosorbent assay (ELISA) or fluoroenzyme immunoassay (FEIA). The ELISA technique used kits from Calpro AS and the FEIA technology was assessed on an automated Thermo Fisher Scientific instrument. The results showed high correlations between the two methods at baseline and during follow-up, with Spearman correlation at baseline 0.93 (p < 0.001) in the early and 0.96 (p < 0.001) in the established RA cohorts. The correlations between each of the two calprotectin assessments and clinical examinations had similar range. Calprotectin correlated well with clinical examinations, with at least as high correlations as CRP and ESR. The present study showed similar results for the two analytical methods, supporting the robustness of calprotectin analyses, and suggest calprotectin in plasma to be included in the assessments offered by clinical routine laboratories.
Influence of wave normal angles on hiss‐electron interaction in Earth's slot region

Wave‐particle interaction which occurs in the radiation belts is generally determined by variations in wave normal angles. Using the Gaussian wave normal angle ( X=tanθ ) distribution, we study the influence of peak wave normal angle ( Xm=tanθm ) on gyroresonance between plasmaspheric hiss waves and energetic electrons in the slot regions L = 2.5, 3.0, and 3.5. The bounce‐averaged diffusion coefficients are calculated for different Xm = 0,1,3,5, and then the phase space density (PSD) evolutions of energetic electrons driven by hiss waves are simulated over a continuous energy range 0.2–5 MeV. As Xm increases, diffusion coefficients basically decrease mainly from the medium to a critical pitch angle αc for Ek≤1.0 MeV but increase at lower pitch angles for Ek>1.0 MeV. Differences of diffusion coefficients between Xm = 0 and 1 are close but become substantial as Xm≥3. Hiss can cause substantial drops in electron PSDs for Ek≤0.5 MeV and Xm≤1 from the loss cone αL to αc. For Ek≥1.0 MeV, such PSD drops become much smaller and confined in the lower pitch angles close to αL for each Xm. In contrast, electron PSD increases for Ek≤1.0 MeV above αc at L = 2.5 and 3.0, probably because momentum diffusion coefficients increase steeply above αc. The current results demonstrate that gyroresonance between plasmaspheric hiss and energetic electrons in the slot region is strongly associated with variations of peak wave normal angles, which should be integrated into future global modeling of radiation belt dynamics.
Classification of Epileptic and Psychogenic Non-Epileptic Seizures Using Electroencephalography and Electrocardiography

Patients with psychogenic non-epileptic seizures (PNES) may exhibit similar clinical features to patients with epileptic seizures (ES). Misdiagnosis of PNES and ES can lead to inappropriate treatment and significant morbidity. This study investigates the use of machine learning techniques for classification of PNES and ES based on electroencephalography (EEG) and electrocardiography (ECG) data. Video-EEG-ECG of 150 ES events from 16 patients and 96 PNES from 10 patients were analysed. Four preictal periods (time before event onset) in EEG and ECG data were selected for each PNES and ES event (60-45 min, 45–30 min, 30–15 min, 15–0 min). Time-domain features were extracted from each preictal data segment in 17 EEG channels and 1 ECG channel. The classification performance using k-nearest neighbour, decision tree, random forest, naive Bayes, and support vector machine classifiers were evaluated. The results showed the highest classification accuracy was 87.83% using the random forest on 15–0 min preictal period of EEG and ECG data. The performance was significantly higher using 15–0 min preictal period data than 30–15 min, 45–30 min, and 60–45 min preictal periods ( $\text{p} < 0.001$ ). The classification accuracy was improved from 86.37% to 87.83% by combining ECG data with EEG data ( $\text{p} < 0.001$ ). The study provided an automated classification algorithm for PNES and ES events using machine learning techniques on preictal EEG and ECG data.
In-season autoregulation of one weekly strength training session maintains physical and external load match performance in professional male football players

ABSTRACT The aim of this study was to compare the effects of autoregulating strength training volume based on an objective (external load match performance) versus a subjective (self-selected) method in professional male football players. Sixteen players completed a 10-week strength training programme where the number of sets was regulated based on football match high-intensity running distance (HIR >19.8 km/h, AUTO, n = 7), or self-selected (SELF, n = 9). In addition to traditional physical performance assessments (30-m sprint, countermovement jump, leg-strength, and body composition), external load match performance was assessed with five matches in the beginning and in the end of the study period. Both groups performed ~ 1 weekly bout of ~ 6 sets in leg extensor exercises during the 10-week period, and maintained physical performance during the competitive season, with no group differences detected after the training period. Non-overlap of all pairs (NAP) analysis showed weak-to-moderate effects in external load match performance from before to after the study period, suggesting that players maintained or improved their performance. In conclusion, no group differences were observed, suggesting that both external load autoregulated and self-selected, low-volume in-season strength training maintained physical, and external load match performance in professional male football players.
SARS-CoV-2 Variants and Age-Dependent Infection Rates among Household and Nonhousehold Contacts

To determine the effects of age and variants of concern on transmission of SARS-CoV-2, we analyzed infection rates among close contacts over 4 periods in Toyama Prefecture, Japan. Among household contacts, odds of infection were 6.2 times higher during the period of the Omicron variant than during previous periods, particularly among children and adolescents.
Singular Riccati theory via extended symplectic pencils

A Riccati like equation, termed as the generalized (discrete-time) algebraic Riccati equation, which incorporates as special cases both the standard and the constrained discrete-time algebraic Riccati equations is introduced and investigated under the weakest possible assumptions imposed on the initial data. A complete characterization of the conditions under which such an equation of general form has a stabilizing solution is presented in terms of the so called proper deflating subspace of the extended symplectic pencil. An evaluation of an associated quadratic index along constrained stable trajectories is given in terms of the stabilizing solution to the generalized Riccati equation. Possible applications of the developed theory range from nonstandard spectral and inner-outer factorizations to singular H/sup 2/ and H/sup /spl infin// control. The results exposed in the present paper could be seen as an extension to singular cases of the discrete-time algebraic Riccati equation theory of indefinite sign.
Predictive value of motor-evoked potentials for motor recovery in patients with hemiparesis secondary to acute ischemic stroke

Abstract Background Motor recovery following a stroke is related to the initial stroke severity and corticospinal tract integrity. One of the outcomes representing corticospinal tract integrity is the motor evoked potential (MEP). This study aimed to investigate the predictive value of MEP for motor recovery in patients with acute ischemic stroke. Patients and Methods Patients with hemiparesis secondary to initial acute ischemic stroke were enrolled. MEPs of the upper limb were assessed as preserved (MEP+) or absent (MEP-) response ≤10 days post-stroke. Fugl-Meyer assessment (FMA) was performed at baseline and post-stroke at 30 and 90 days. A modified Rankin scale (mRS) was conducted at 90 days post-stroke. Patients were divided into two groups according to the highest FMA score of MEP- patients. Generalized estimating equations and logistic regression were used for our study analysis. Results Sixty-one participants were included in this study. The highest FMA score of MEP- patients ≤10 days after stroke was 38. Among patients with an initial FMA score ≤38, FMA scores at 30 and 90 days post-stroke were significantly higher in MEP + patients than in MEP- patients. Proportional recovery at 30 and 90 days post-stroke was significantly higher in MEP + patients than in MEP- patients. MEP + patients had a higher percentage of good functional outcomes than MEP- patients, without statistical difference. Among patients with initial FMA score >38, FMA scores were 60.4 ± 4.8 and 63.9 ± 2.9 and proportional recovery was 65.2 ± 27.0% and 83.7 ± 24.6% at 30 and 90 days post-stroke, respectively. Conclusions Among patients with moderate-to-severe ischemic stroke, MEP + patients had better motor recoveries (approximately 70%) than MEP– patients at 90 days post-stroke. MEP + patients had better functional outcomes than MEP- patients. Key Messages Among patients with moderate-to-severe ischemic stroke, those with positive motor-evoked potentials (MEPs) had better motor recovery than those with negative MEPs at 90 days post-stroke. Assessment of motor-evoked potentials is a reliable method for predicting motor recovery in patients with moderate-to-severe ischemic stroke. Corticospinal tract function of patients with acute ischemic stroke was tested with transcranial magnetic stimulation.
The Growing Interest in the Combined Hepatocellular-intrahepatic Cholangiocarcinoma (cHCC-CCA).

Combined hepatocellularcholangiocarcinoma (cHCCCCA) is a rare primary liver cancer displaying both hepatocytic and cholangiocytic differentiation. This combined tumor has about 1% incidence among primary hepatic cancers, a more aggressive behaviour and a poorer prognosis as compared to hepatocellular carcinoma (HCC). While keeping the proportion of 1% among primary liver cancers, the cHCCCCA incidence is increasing. A systematic review and metaanalysis of 53 epidemiological studies performed both in Western and Asian countries between 2008 and 2019 demonstrated that cHHC-CCA incidence had increased in this period with an annual percentage change (APC) of + 4% as compared to HCC (APC + 2.6%) [1]. The increase occurred mainly in Western countries, whereas trends decreased in the Asian region, although still remaining high. The etiology of the combined tumor is the same as that of its components. Geographic variations in CCA incidence are related to the variations in risk factors. Classical risk factors for HCC and CCA seem also to predispose to the development of cHCC-CCA. In the context of a better control of viral B and C infections, the increased risk of primary liver cancers should probably be related to the worldwide increase in the prevalence of metabolic EDITORIAL DOI: http://dx.doi. org/10.15403/jgld-5033
Rapid granular flows down inclined planar chutes. Part 1. Steady flows, multiple solutions and existence domains

The highly agitated flow of grains down an inclined chute is modelled using a kinetic theory for inelastic collisions. Solutions corresponding to steady, fully developed flows are obtained by solving numerically a nonlinear system of ordinary differential equations using a highly accurate pseudospectral method based on mapped Chebyshev polynomials. The solutions are characterized by introducing macroscopic, depth-integrated variables representing the mass flux of flowing material per unit width, its centre-of-mass and the mass supported within the flowing layer, and the influence of the controlling parameters on these solutions is investigated. It is shown that, in certain regions of parameter space, multiple steady solutions can be found for a specified mass flux of material. An asymptotic analysis of the governing equations, appropriate to highly agitated flows, is also developed and these results aid in the demarcation of domains in parameter space where steady solutions can be obtained.
Development and Application of a Nanobody-Based Competitive ELISA for Detecting Antibodies against Hepatitis E Virus from Humans and Domestic Animals

HEV is thought to be a zoonotic infection and is widespread worldwide; it is beneficial to establish a more convenient and spectral method for HEV antibody detection. In this study, a convenient, time-saving, reproducible, highly sensitive, specific, and novel nanobody-based cELISA was developed and can be used to detect IgG antibodies against mammalian HEV. It provides a new technique for serological evaluation and ELISA-based diagnosis of HEV infection. ABSTRACT Hepatitis E virus (HEV) is a zoonotic pathogen that is widespread worldwide. At present, most enzyme-linked immunosorbent assay (ELISA) kits only detect antibodies against human HEV. In this study, a nanobody-horseradish peroxidase (HRP) fusion protein-based competitive ELISA (cELISA) with more convenience and spectral characteristics for HEV antibody detection was developed and used to detect HEV IgG in various species. First, 6 anti-swine HEV capsid protein nanobodies were screened using phage display technology from an immunized Bactrian camel. Then, HEV-Nb67-HRP fusions were expressed and used as a probe for developing a cELISA. The cutoff value of the cELISA was 17.8%, and there was no cross-reaction with other anti-swine virus antibodies, suggesting that the cELISA had good specificity. The intra-assay and interassay coefficients of variation (CVs) were 1.33 to 5.06% and 1.52 to 6.84%, respectively. The cELISA and Western blot showed a higher coincidence rate (97.14%, kappa value = 0.927) than cELISA and indirect ELISA (95.00%, kappa value = 0.876) in clinical swine serum samples. Finally, the seroprevalence of HEV IgG in humans, pigs, rabbits, cows, and goats was 30.67%, 19.26%, 8.75%, 27.59%, and 18.08%, respectively, suggesting that cELISA may have a broader scale for mammalian HEV antibody detection. These results suggest that the newly developed cELISA was rapid, low-cost, reliable, and useful for the serological evaluation of current HEV. IMPORTANCE HEV is thought to be a zoonotic infection and is widespread worldwide; it is beneficial to establish a more convenient and spectral method for HEV antibody detection. In this study, a convenient, time-saving, reproducible, highly sensitive, specific, and novel nanobody-based cELISA was developed and can be used to detect IgG antibodies against mammalian HEV. It provides a new technique for serological evaluation and ELISA-based diagnosis of HEV infection.
Edge strengthening and phonetic variability in Spanish /l/: an ultrasound study

Abstract Previous research has shown that /l/ in Spanish displays patterns of articulatory variability that are determined by a complex interaction of phonetic, phonological and dialectal factors. In this study, we report the results of an experiment using Ultrasound Tongue Imaging (UTI) that tests /l/-articulations in a dialectal cross-section of Spanish speakers. We show that lengthening of /l/ in phrase-edge contexts is accompanied by articulatory distinctions (e.g. root/dorsum retraction) for some speakers, whereas others produce lengthened realisations of /l/ in these contexts without observable differences in tongue position. We also find acoustic evidence for reduction in utterance-medial intervocalic and preconsonantal environments (duration, intensity, F1 frequency measures are discussed). However, articulatory correlates of reduction are not consistently observed across speakers in these contexts. As well as relating the results to prosodically-driven strengthening and reduction patterns, our findings are of relevance to debates about resyllabification in Spanish. Specifically, we argue that our results cannot be straightforwardly accommodated under phonological analysis assuming that word-final consonants regularly resyllabify across word boundaries prevocalically.
Systematic Review of Hansen Disease Attributed to Mycobacterium lepromatosis

In 2008, bacilli from 2 Hansen disease (leprosy) cases were identified as a new species, Mycobacterium lepromatosis. We conducted a systematic review of studies investigating M. lepromatosis as a cause of HD. Twenty-one case reports described 27 patients with PCR–confirmed M. lepromatosis infection (6 dual M. leprae/M. lepromatosis): 10 case-patients in the United States (7 originally from Mexico), 6 in Mexico, 3 in the Dominican Republic, 2 each in Singapore and Myanmar, and 1 each in Indonesia, Paraguay, Cuba, and Canada. Twelve specimen surveys reported 1,098 PCR–positive findings from 1,428 specimens, including M. lepromatosis in 44.9% (133/296) from Mexico, 3.8% (5/133) in Colombia, 12.5% (10/80) in Brazil, and 0.9% (2/224) from the Asia-Pacific region. Biases toward investigating M. lepromatosis as an agent in cases of diffuse lepromatous leprosy or from Mesoamerica precluded conclusions about clinicopathologic manifestations and geographic distribution. Current multidrug treatments seem effective for this infection.
Efficacy of Allisartan Isoproxil in the Treatment of Mild-to-Moderate Essential Hypertension.

BACKGROUND Allisartan isoproxil is a selective nonpeptide angiotensin II (AT1) receptor blocker developed by China, this study aimed to assess its clinical efficacy for essential hypertension(EH). METHODS Patients with mild-to-moderate EH, selected at 44 sites in China from September 9, 2016, to December 7, 2018, were administered 240 mg allisartan isoproxil daily for 4 weeks. Patients with controlled blood pressure(BP) continued monotherapy for 8 weeks, others were randomly assigned (1:1) to A + D group (allisartan isoproxil 240 mg + indapamide 1.5 mg) or A + C group (allisartan isoproxil + amlodipine besylate 5 mg) for 8 weeks. BP were measured at week 4, 8 and 12. RESULTS 2126 patients were included. After 12 weeks of treatment, systolic blood pressure(SBP) and diastolic blood pressure(DBP) decreased by 19.24 ±12.02 and 10.63 ±8.89 mmHg, respectively, and the overall BP control rate was 78.56%. The sitting blood pressures (SBP/DBP) decreased by 19.12 ±11.71/10.84 ±8.73 mmHg in patients with 12 weeks allisartan isoproxil monotherapy (both p < 0.0001). The BP reductions and control rates were comparable between A + D and A + C groups. 48 patients with monotherapy-controlled BP underwent ambulatory blood pressure monitoring, with a mean decrease in ambulatory blood pressure of 10.04 ±10.87/5.50 ±8.07 mmHg after 12 weeks of treatment, and consistent reductions between day and night. SBP and DBP had trough-to-peak ratios of 64.64% and 62.63% and smoothness indices of 3.82 and 2.92, respectively. CONCLUSIONS An allisartan-isoproxil-based antihypertensive regimen can effectively control BP in patients with mild-to-moderate EH.
Delay-Independent Stability of Switched Linear Systems with Unbounded Time-Varying Delays

This paper is focused on delay-independent stability analysis for a class of switched linear systems with time-varying delays that can be unbounded. When the switched system is not necessarily positive, we first establish a delay-independent stability criterion under arbitrary switching signal by using a new method that is different from the methods to positive systems in the literature. We also apply this method to a class of time-varying switched linear systems with mixed delays.