Miracle-dz/UniFAR-FTUs huggingface dataset:
A new variant of Glanzmann's thrombasthenia (Strasbourg I). Platelets with functionally defective glycoprotein IIb-IIIa complexes and a glycoprotein IIIa 214Arg----214Trp mutation.

We describe a new variant of Glanzmann's thrombasthenia (variant Strasbourg I). The patient (M.S.) showed an absence of platelet aggregation to ADP, thrombin, and collagen, and a decreased clot retraction. Platelet fibrinogen was approximately 20% of normal levels. ADP-stimulated platelets bound markedly reduced amounts of soluble fibrinogen and platelet adhesion to surface-bound fibrinogen was defective. Normal to subnormal amounts of glycoprotein (GP) IIb-IIIa (alpha IIb beta 3) complexes, the platelet fibrinogen receptor, were revealed by SDS-PAGE, crossed immunoelectrophoresis, and antibody binding. However, the complexes were unusually sensitive to dissociation with EDTA at room temperature. Furthermore, flow cytometry showed that the platelets failed to bind the activation-dependent monoclonal antibody, PAC-1, after stimulation. In contrast, an RGDS-containing peptide induced significant binding of the anti-ligand-induced binding site antibody, D3GP3, suggesting the presence of a functional RGD binding domain on the patient's GPIIb-IIIa complex. Sequence analysis was performed after polymerase chain reaction amplification of selected patient's GPIIIa exons, and of the patient's platelet GPIIb and GPIIIa mRNAs. A point mutation (C to T) was localized in exon D (iv) of GPIIIa that resulted in an 214Arg to 214Trp amino acid substitution. The defect has been inherited from the parents who are heterozygous for the same mutation. This substitution points to an essential amino acid in a region of GPIIIa involved in the binding of fibrinogen and influencing the Ca(2+)-dependent stability of the GPIIb-IIIa complex.
Niclosamide sensitizes triple-negative breast cancer cells to ionizing radiation in association with the inhibition of Wnt/β-catenin signaling

Triple-negative breast cancer (TNBC) is one of the most difficult breast cancers to treat because there is no targeted treatment, and conventional cytotoxic chemotherapy followed by adjuvant radiation therapy is the standard of care for patients with TNBC. We herein reported that ionizing radiation (IR) induced Wnt3a, LRP6 and β-catenin expression and consequently activated Wnt/β-catenin signaling in TNBC MDA-MB-231, MDA-MB-468 and Hs578T cells. Moreover, depletion of β-catenin by shRNA sensitized TNBC cells to IR, whereas treatment of Wnt3a protein or overexpression of β-catenin resulted in radioresistance of TNBC cells. Niclosamide, a potent inhibitor of Wnt/β-catenin signaling, not only inhibited constitutive Wnt/β-catenin signaling, but also blocked IR-induced Wnt/β-catenin signaling in TNBC cells. In addition, niclosamide sensitized TNBC cells to IR, prevented Wnt3a-induced radioresistance, and overcame β-catenin-induced radioresistance in TNBC cells. Importantly, animals treated with the combination of niclosamide and γ-ray local tumor irradiation had significant inhibition of MDA-MB-231 tumor growth compared with treated with local tumor irradiation alone. These findings indicate that Wnt/β-catenin signaling pathway plays an important role in the development of radioresistance of TNBC cells, and that niclosamide had significant radiosensitizing effects by inhibiting Wnt/β-catenin signaling in TNBC cells. Our study also provides rationale for further preclinical and clinical evaluation of niclosamide in TNBC management.
The golden proportion in facial soft-tissues of Vietnamese females.

Objective of the current study was to evaluate proportions of frontal facial soft-tissues of Vietnamese females correspond to the golden proportion (GP). MATERIAL AND METHODS Sixty frontal facial photographs of Vietnamese female students aged 19 years were selected. The selected participants had symmetrical face, a Class I relationship occlusion, complete lip closure, and no history of trauma and orthodontic treatment. The photographic record was set-up with a white backdrop, fill light, a reflector, a camera Canon 650D and subjects were asked to sit in a standard position. Trichion (TR), Temporal soft tissue (TS), Lateral canthus (LC), Lateral nasal (LN), Chilion (CH), and Menton (ME) point were used for photometric measurements on CorelDRAW Graphic X3 software. RESULTS The vertical facial proportions (mean, percentage compared with GP) were significantly higher than the GP including: LC-CH:CH-ME (1.661, 102%), LN-ME:LC-LN (1.729, 106%), TR-ME:LC-ME (1.739, 107%), TR-LN:LN-ME (1.759, 108%). Whereas TR-ME:LN-ME; CH-ME:LN-CH; LC-LN:LN-CH had ratios of 84% - 92% and were significantly smaller in comparison with GP. The horizontal facial proportions of CHR-L:LNR-L, LCR-L:CHR-L and TSR-L:LCR-L deviated from the GP with mean values of 1.221, 1.922 and 1.229, respectively. The new mean values of TR-ME:LC-ME (1.733), TR-LN:LN-ME (1.732), LC-ME:TR-LC (1.374), CH-ME:LN-CH (1.524), which were predictable changes in proportions, can be significantly converged to the GP if LC-CH:CH-ME is equal to the GP. CONCLUSIONS Soft-tissue facial proportions of Vietnamese females did not correspond to the GP. Changing the lower third face may create harmony vertical facial proportions.
Influence of dead time on voltage harmonic spectrum of grid-connected PWM-VSC with LCL filter

In recent years, the three-phase pulse width modulation controlled voltage source converter (PWM-VSC) has found its way to wide range of applications, among which renewable energy sources and adjustable speed drives are the most common. In order to achieve low harmonic distortion of current at the point of common coupling (PCC), LCL filters are used for grid connection since they ensure higher damping of current harmonics than L-filters for the same volume. There are many different LCL filter design and optimization methods that are based on the assumption of an ideal converter. Since actual PWM-VSC includes many different sources of nonlinearities, their neglection can potentially lead to selection of inadequate LCL filter in terms of increased grid current harmonic distortion and decreased overall system performance. Research presented in this paper is part of ongoing effort to increase efficiency of adjustable speed drive for crane systems of which the PWM-VSC with LCL filter is a part. This paper focuses on influence of nonlinearities on voltage harmonic spectrum of PWM-VSC. Analysis is carried out by means of nonlinear models and it indicates that the dead time delay has the greatest impact on the system. Validation of simulation results by measurements suggest additional effects should be incorporated into the model. Such a model can then be used for development of more accurate non-iterative LCL filter design and optimization procedures as well as development of operating point optimization algorithms.
How Consumers Respond to the Behavior of Missing a Free Gift Promotion: Inaction Inertia Effect on Products Offered as Free Gifts

ABSTRACT Inaction inertia describes the phenomenon that an individual is unlikely to act on an attractive opportunity after having bypassed an even more attractive one. The results of two experiments indicate that after missing an initial opportunity to obtain a product as a free gift during a promotional period, the inaction inertia effect reduces the likelihood of consumers buying the product at a discounted price (second, inferior opportunity), particularly if the free gift has a high regular price. Additionally, according to the results of Experiment 2, those consumers are less likely to buy a product that has been offered previously as a free gift when a greater total quantity of the free gift is offered during a promotional period. Moreover, the mediation analysis results indicate that anticipated regret and valuation significantly impact the mediating role of inaction inertia.
Attitude synchronization of multiple 3-DOF helicopters without angular velocity measurements by bounded distributed control

The problem of designing distributed attitude synchronization control protocol is considered for multiple 3-DOF helicopters without angular velocity measurements. To explicitly take into account the actuator saturations constraint, the notion of local neighborhood synchronization error (LNSE) is extended to the nonlinear case using the hyperbolic tangent functions which are prior bounded. It is proved that under a connected communication topology, the convergence of attitude tracking errors can be achieved by forcing the extended LNSEs to zero. An auxiliary nonlinear filter and a continuous bounded distributed controller are designed for each helicopter to achieve attitude synchronization in both elevation and pitch channels, where only angular position signals are used. Numerical simulation results are provided to illustrate the effectiveness of the presented control scheme.
Assessment of an 18F-Labeled Phosphoramidate Peptidomimetic as a New Prostate-Specific Membrane Antigen–Targeted Imaging Agent for Prostate Cancer

Prostate-specific membrane antigen (PSMA) is a transmembrane protein commonly found on the surface of late-stage and metastatic prostate cancer and a well-known imaging biomarker for staging and monitoring therapy. Although 111In-labeled capropmab pendetide is the only approved agent available for PSMA imaging, its clinical use is limited because of its slow distribution and clearance that leads to challenging image interpretation. A small-molecule approach using radiolabeled urea-based PSMA inhibitors as imaging agents has shown promise for prostate cancer imaging. The motivation of this work is to explore phosphoramidates as a new class of potent PSMA inhibitors to develop more effective prostate cancer imaging agents with improved specificity and clearance properties. Methods: N-succinimidyl-4-18F-fluorobenzoate (18F-SFB) was conjugated to S-2-((2-(S-4-amino-4-carboxybutanamido)-S-2-carboxyethoxy)hydroxyphosphorylamino)-pentanedioic acid (Phosphoramidate (1)), yielding S-2-((2-(S-4-(4-18F-fluorobenzamido)-4-carboxybutanamido)-S-2-carboxyethoxy)hydroxyphosphorylamino)-pentanedioic acid (3). In vivo studies were conducted in mice bearing either LNCaP (PSMA-positive) or PC-3 (PSMA-negative) tumors. PET images were acquired at 1 and 2 h with or without a preinjection of a nonradioactive version of the fluorophosphoramidate. Tissue distribution studies were performed at the end of the 2 h imaging sessions. Results: Phosphoramidate (1) and its fluorobenzamido conjugate (2) were potent inhibitors of PSMA (inhibitory concentration of 50% [IC50], 14 and 0.68 nM, respectively). PSMA-mediated tumor accumulation was noted in the LNCaP versus the PC-3 tumor xenografts. The LNCaP tumor uptake was also blocked by the administration of nonradioactive (2) prior to imaging studies. With the exception of the kidneys, tumor-to-tissue and tumor-to-blood ratios were greater than 5:1 at 2 h. The strong kidney uptake may be due to the known PSMA expression in the mouse kidney, because significant reduction (>6-fold) in kidney activity was seen in mice injected with (2). Conclusion: 18F-labeled phosphoramidate (3) is a representative of a new class of PSMA targeting peptidomimetic molecules that shows great promise as imaging agents for detecting PSMA+ prostate tumors.
An Area-Efficient Noise-Adaptive Neural Amplifier in 130 nm CMOS Technology

Chronic recording of neural signals is indispensable in designing efficient brain-machine interfaces and to elucidate human neurophysiology. The advent of multichannel micro-electrode arrays has driven the need for electronics to record neural signals from many neurons. The dynamic range of the system can vary over time due to change in electrode-neuron distance and background noise. We propose a neural amplifier in UMC 130 nm, 1P8M complementary metal-oxide-semiconductor (CMOS) technology. It can be biased adaptively from 200 nA to 2 μA, modulating input referred noise from 9.92 μV to 3.9 μV. We also describe a low noise design technique which minimizes the noise contribution of the load circuitry. Optimum sizing of the input transistors minimizes the accentuation of the input referred noise of the amplifier and obviates the need of large input capacitance. The amplifier achieves a noise efficiency factor of 2.58. The amplifier can pass signal from 5 Hz to 7 kHz and the bandwidth of the amplifier can be tuned for rejecting low field potentials (LFP) and power line interference. The amplifier achieves a mid-band voltage gain of 37 dB. In vitro experiments are performed to validate the applicability of the neural low noise amplifier in neural recording systems.
Risk factors for visual loss in an Italian population-based cohort of patients with giant cell arteritis.

OBJECTIVE To evaluate the frequency of visual manifestations at presentation in an Italian population-based cohort of patients with biopsy-proven giant cell arteritis (GCA), and to investigate predictors for the development of permanent visual loss. METHODS We identified 136 Reggio Emilia (Italy) residents with biopsy-proven GCA diagnosed between 1986 and 2002. Medical records of these 136 patients were reviewed, and demographic, clinical, and laboratory data were collected.Multivariate analysis with multiple logistic regression models was performed to identify the best predictors of visual loss. RESULTS Visual manifestations developed in 41 patients (30.1%). Partial or total visual loss was observed in 26 patients (19.1%). Anterior ischemic optic neuropathy was seen in 24 patients, and 2 patients had central retinal artery occlusion. Unilateral vision loss occurred in 19 patients, and bilateral visual loss in 7. In 25 patients, visual loss developed before glucocorticoid therapy for GCA was started. The age at disease onset was significantly higher in patients with permanent visual loss compared with those without it. The frequency of systemic signs/symptoms and erythrocyte sedimentation rate (ESR) and C-reactive protein values at diagnosis were significantly lower in patients with permanent visual loss. By multivariate logistic regression, the only statistically significant predictor for the development of permanent visual loss was the absence of high levels of ESR at diagnosis (tertile 2: Odds ratio [OR] 0.08; tertile 3: OR 0.11). Other predictors included in the model were the absence of systemic manifestations (OR 0.24), an older age at disease diagnosis (quintile 5: OR 5.60), and the presence of an elevated platelet count at diagnosis (OR 4.99), however they were only of borderline statistical significance. CONCLUSION The proportion of Italian patients with GCA that developed visual loss was similar to that reported from other countries. The patients with low inflammatory response had a higher risk of visual loss.
Abnormal Resting-State Functional Connectivity of the Anterior Cingulate Cortex in Unilateral Chronic Tinnitus Patients

Purpose: The anterior cingulate cortex (ACC) has been suggested to be involved in chronic subjective tinnitus. Tinnitus may arise from aberrant functional coupling between the ACC and cerebral cortex. To explore this hypothesis, we used resting-state functional magnetic resonance imaging (fMRI) to illuminate the functional connectivity (FC) network of the ACC subregions in chronic tinnitus patients. Methods: Resting-state fMRI scans were obtained from 31 chronic right-sided tinnitus patients and 40 healthy controls (age, sex, and education well-matched) in this study. Rostral ACC and dorsal ACC were selected as seed regions to investigate the intrinsic FC with the whole brain. The resulting FC patterns were correlated with clinical tinnitus characteristics including the tinnitus duration and tinnitus distress. Results: Compared with healthy controls, chronic tinnitus patients showed disrupted FC patterns of ACC within several brain networks, including the auditory cortex, prefrontal cortex, visual cortex, and default mode network (DMN). The Tinnitus Handicap Questionnaires (THQ) scores showed positive correlations with increased FC between the rostral ACC and left precuneus (r = 0.507, p = 0.008) as well as the dorsal ACC and right inferior parietal lobe (r = 0.447, p = 0.022). Conclusions: Chronic tinnitus patients have abnormal FC networks originating from ACC to other selected brain regions that are associated with specific tinnitus characteristics. Resting-state ACC-cortical FC disturbances may play an important role in neuropathological features underlying chronic tinnitus.
Serum Lipids and Postprandial Glucose and Insulin Levels in Hyperlipidemic Subjects after Consumption of an Oat β-Glucan-Containing Ready Meal

Background/Aims: To investigate whether a daily intake of a nutrient-balanced ready meal containing 4 g of oat β-glucans lowers total cholesterol (T-C) and low-density-lipoprotein cholesterol (LDL-C) in hyperlipidemic subjects and to evaluate its effect on postprandial glucose and insulin concentrations. Methods: A parallel, placebo-controlled trial was carried out in 43 healthy men and women with elevated serum cholesterol levels. During the 3-week run-in period, all subjects consumed daily a ready-meal soup, low in energy and fat and high in fiber but without oat β-glucans. Subjects were then randomly assigned for the following 5-week intervention period to either continue consumption of the control soup or an equal soup supplemented with β-glucan. Results: Consumption of the β-glucan-enriched ready meal decreased T-C (0.22 ± 0.41 mmol/l) and LDL-C (0.16 ± 0.36 mmol/l), but the reductions were not significantly lower compared with control. After a meal with β-glucan soup, neither postprandial glucose nor insulin concentrations were significantly different from those after a control soup. Conclusion: A daily dose of 4 g of oat β-glucans incorporated into a healthy ready meal did not significantly lower T-C and LDL-C compared with an equal ready meal without β-glucans. Thus, if a food product fulfils general healthy dietary recommendations it may not necessarily be a candidate for supplementation with β-glucans.
Winqi: A System for 6D Localization and SLAM Augmentation Using Wideangle Optics and Coded Light Beacons

Simultaneous Localization and Mapping (SLAM) systems use commodity visible/near visible digital sensors coupled with processing units that detect, recognize and track image points in a camera stream. These systems are cheap, fast and make use of readily available camera technologies. However, SLAM systems suffer from issues of drift as well as sensitivity to lighting variation such as shadows and changing brightness. Beaconless SLAM systems will continue to suffer from this inherent drift problem irrespective of the improvements in on-board camera resolution, speed and inertial sensor precision. To cancel out destructive forms of drift, relocalization algorithms are used which use known detected landmarks together with loop closure processes to continually readjust the current location and orientation estimates to match "known" positions. However this is inherently problematic because these landmarks themselves may have been recorded with errors and they may also change under different illumination conditions. In this note we describe a unique beacon light coding system which is robust to desynchronized clock bit drift. The described beacons and codes are designed to be used in industrial or consumer environments for full standalone 6dof tracking or as known error free landmarks in a SLAM pipeline.
A randomised control trial of oral health education provided by a health visitor to parents of pre-school children.

OBJECTIVES To determine the effect of oral health education carried out by a specially trained health visitor on the dental health of young children. DESIGN AND SETTING Children, who were recruited during their 8-month distraction-hearing test, were randomly allocated to intervention and control groups. A home visit by the health visitor was arranged to parents in the intervention group who were given dental health advice. A second home visit, when the child was about 20 months old, focused on a completed diet record sheet and discussions about what and when the child was eating and drinking. Children in the intervention group received a toothbrush and toothpaste containing 440 ppm fluoride at both visits while those in the control group received the level of care usually provided by health visitors in the area. The children's teeth were examined when they were three years old and two years later as part of a census survey of 5-year-old children in the area. MAIN OUTCOME MEASURES The numbers of decayed, missing and filled tooth surfaces. RESULTS 251 children were recruited to the control group and 250 to the intervention group. At age three, they were examined; the mean dmfs scores were 2.19 (95% Confidence Interval: 1.41-2.97) in the control group (n = 171) and 2.03 (CI: 1.39-2.67) in the intervention group (n = 181). During the census survey 276 of the children in the study were examined at school. At this age the mean dmfs scores were 4.84 (CI: 3.39-6.29) in the control group (n = 129) and 3.99 (CI: 2.54-5.04) in the intervention group (n = 147). However, the mean dmfs of the remaining 2,253 children who were examined was 5.94 (CI: 5.55-6.33). CONCLUSIONS No statistically significant differences in mean dmfs scores were found between the control and intervention groups of children, although, as the children grew older, the gap between them widened. However, the mean dmfs score of other 5-year-olds in the area was significantly worse than that of children in the intervention group. Asking the control parents to take part in the study and examining their children at three years may have had an effect on their dental health status and have made it more difficult to detect any differences achieved by the programme.
Lower Airway Inflammation in Infants with Cystic Fibrosis Detected by Newborn Screening

Controversy exists over whether the lower airway inflammation that characterizes cystic fibrosis (CF) is initiated primarily by the genetic defect. To determine if inflammation precedes infection, we examined bronchoalveolar lavage (BAL) fluid cytology, cytokines (interleukin (IL)‐1β, IL‐4, IL‐5, IL‐6, IL‐8, IL‐10, and tumor necrosis factor‐α), and free neutrophil elastase activity from 70 CF (aged 1.5–71 months) children detected by newborn screening and 19 (aged 2.0–48 months) controls with chronic stridor. CF subjects were selected and categorized as pristine (13 aged ≤ 6 months, lacking prior respiratory symptoms and exposure to antibiotics, and without respiratory pathogens on BAL), infected (42 with viruses or ≥ 105 colony‐forming units/ml of pathogenic bacteria in BAL), and uninfected (15 aged > 6 months, asymptomatic, not taking antibiotics at bronchoscopy, and free of pathogens in their BAL). To further resolve if inflammation develops without infection, inflammatory mediators in paired annual BAL samples from 38 CF subjects were measured, and results were grouped according to whether BAL showed persistence (n = 6), acquisition (n = 8), clearance (n = 13), or absence (n = 11) of infection. While pristine, uninfected, and control subjects had similar BAL profiles, infected patients showed elevated inflammatory indices, including increased IL‐10 (P < 0.001). Pristine subjects had the fewest signs of inflammation. Analysis of BAL pairs found differences between the four infection groups for changes in neutrophil percentages, IL‐8 (P < 0.001), and free neutrophil elastase (P = 0.009). Infection was associated with elevated inflammatory mediators in BAL fluid. In contrast, minimal or reduced signs of inflammation accompanied absence of eradication of infection from BAL fluid. We conclude that in CF, infection initiates and sustains airway inflammation. Pediatr Pulmonol. © 2005 Wiley‐Liss, Inc.