gebhart/patrepeval huggingface dataset:
Psychosocial Functioning in Youth With Barth Syndrome (2009)

This pilot study assessed the quality of life and psychosocial functioning of pediatric patients with Barth Syndrome. Thirty-four boys with Barth Syndrome and 22 healthy male controls were administered a measure of verbal ability and completed measures of quality of life, loneliness, perceived peer support, and sibling relationship quality. Parents completed measures of parental distress, parenting stress, child academic functioning, child adaptive behavior, and child emotional and behavioral functioning. Quality of life ratings were consistently lower in youth with Barth Syndrome relative to both healthy controls and a previously reported sample of youth with cardiac disease. Compared to healthy controls, children with Barth Syndrome were rated as having more internalizing and externalizing symptoms, social problems, loneliness, and lower independent functioning. Parents of boys with Barth Syndrome reported greater distress and parenting stress relative to healthy controls. In addition, parents reported a significant need for academic accommodations, given their son's illness and associated impairments. Boys with Barth Syndrome and their parents appear to be affected by the presence of the illness in numerous ways. Results suggest the need for interventions aimed at helping children and families cope with illness-related stressors to enhance quality of life and overall functioning.
Penetrance of Parkinson's Disease in LRRK2 p.G2019S Carriers Is Modified by a Polygenic Risk Score (2020)

Our results suggest that there is a genetic contribution for penetrance of PD among p.G2019S carriers. These results have important etiological consequences and potential impact on the selection of subjects for clinical trials. © 2020 International Parkinson and Movement Disorder Society.
The Ada (Trade Name) Compiler Validation Capability Implementers' Guide. Version 1, (1986)

Abstract : The primary purpose of the Ada Compiler Validation Capability (ACVC ) is to help decide whether Ada translators conform to ANSI/MIL-STD-1815A-1983, 'The Reference Manual for the Ada Language' (the RM). The ACVC has three main components: An Implementers' Guide, which describes implementation implications of the RM and the conditions to be checked by validation tests, test programs to be submitted to a compiler, and validation support tools, which assist in preparing tests for execution and in analyzing the results of execution.
Development of thermoelectric thin films and characterization methods (2019)

Abstract This work reports on the fabrication of thin films of inorganic thermoelectric materials like borides, germanides, manganese compounds, and characterization of their thermoelectric properties. We have utilized a unique high temperature molecular beam epitaxy (MBE) apparatus to grow hexaboride thin films and manganese germanides. Magnetic manganese based alloy thin films were also grown using sputtering, since we are interested in the possibilities of utilizing magnetism to develop thermoelectric materials.
Inpatient magnetic resonance cholangiopancreatography: does it increase the efficiency in emergency hepatopancreaticobiliary surgery services? (2016)

INTRODUCTION Magnetic resonance cholangiopancreatography (MRCP) is commonly used to evaluate the biliary tree, although indications for patients who require inpatient imaging are not fully defined. The aim of this study was to evaluate inpatient MRCP performed on surgical patients and to devise a treatment pathway for these patients. MATERIAL AND METHODS All adult inpatient MRCP examinations between January 2012 and December 2013 were reviewed. Demographic, clinical and radiological data were collated. RESULTS During the study period, 271 inpatient MRCP were requested, of which 234 examinations were included. The majority of patients were female (n=140) and the median age was 63 years (range 16-93 years). Surgical admissions accounted for 171 (73%) of cases. Indications for inpatient MRCP include gallstone-related complications (n=173; 74%), malignant process (n=17; 7%) and other indications (n=44; 19%). Overall, inpatient MRCP led to further inpatient interventions in 22% (gallstone group, n=32, 18%; patients with malignancy, n=8, 47%; other indications, n=12, 27%). The median duration of inpatient MRCP from request to examination was 2 days (range 0-15 days) and median reporting after examination was 1 day (range 0-14 days). DISCUSSION AND CONCLUSION Improved access and timely reporting of iMRCP may reduce length of hospital stay. Inpatient MRCP also led to further inpatient interventions, in particular, in patients with malignancy.
Good Low-Temperature Properties of Nitrogen-Enriched Porous Carbon as Sulfur Hosts for High-Performance Li–S Batteries (2016)

Despite the increased attention devoted to exploring cathode construction based on various nitrogen-enriched carbon scaffolds at room temperature, the low-temperature behaviors of Li-S cathodes have yet to be studied. Herein, we demonstrate the good low-temperature electrochemical performances of nitrogen-enriched carbon/sulfur composite cathodes. Electrochemical evaluation indicates that a reversible capacity of 368 mAh g(-1) (0.5 C) over 100 cycles is achieved at -20 °C. After returning to 25 °C, a capacity of 620 mAh g(-1) (0.5 C) is achieved over 350 cycles with a low-capacity attenuation rate (0.071% per cycle) and an initial capacity of 1151 mAh g(-1) (0.1C). This positive electrochemical property was speculated to result from the good surface chemistry of the various amine groups in the nitrogen-enriched carbon materials with enhanced polysulfide immobilization.
Bi‐allelic CLPB mutations cause cataract, renal cysts, nephrocalcinosis and 3‐methylglutaconic aciduria, a novel disorder of mitochondrial protein disaggregation (2015)

Whole exome sequencing was used to investigate the genetic cause of mitochondrial disease in two siblings with a syndrome of congenital lamellar cataracts associated with nephrocalcinosis, medullary cysts and 3-methylglutaconic aciduria. Autosomal recessive inheritance in a gene encoding a mitochondrially targeted protein was assumed; the only variants which satisfied these criteria were c.1882C>T (p.Arg628Cys) and c.1915G>A (p.Glu639Lys) in the CLPB gene, encoding a heat shock protein/chaperonin responsible for disaggregating mitochondrial and cytosolic proteins. Functional studies, including quantitative PCR (qPCR) and Western blot, support pathogenicity of these mutations. Furthermore, molecular modelling suggests that the mutations disrupt interactions between subunits so that the CLPB hexamer cannot form or is unstable, thus impairing its role as a protein disaggregase. We conclude that accumulation of protein aggregates underlies the development of cataracts and nephrocalcinosis in CLPB deficiency, which is a novel genetic cause of 3-methylglutaconic aciduria. A common mitochondrial cause for 3-methylglutaconic aciduria appears to be disruption of the architecture of the mitochondrial membranes, as in Barth syndrome (tafazzin deficiency), Sengers syndrome (acylglycerol kinase deficiency) and MEGDEL syndrome (impaired remodelling of the mitochondrial membrane lipids because of SERAC1 mutations). We now propose that perturbation of the mitochondrial membranes by abnormal protein aggregates leads to 3-methylglutaconic aciduria in CLPB deficiency.
Rehabilitation and nutrition protocols for optimising return to play from traditional ACL reconstruction in elite rugby union players: A case study (2019)

Current nutrition and exercise focus during rehabilitation periods has been on reducing muscle atrophy associated with immobilisation. This case report outlines a best practice anterior cruciate ligament (ACL) rehabilitation programme undertaken by two professional rugby athletes, with the addition of an evidence-based supplementation (gelatine and vitamin C) and exercise protocol focused on collagenous tissue. Both players ruptured their left ACL and were repaired with a traditional hamstring graft. Players undertook a structured rehabilitation programme for 34 weeks before being clinically assessed ready to play. Players saw minimal changes in body composition in the early rehabilitation period (P1 - 0.8 kg; P2 - 0.4 kg). Leg lean mass reduced in both legs of Player 1 (Injured - 0.8 kg, Non-injured - 0.6 kg) at 17 weeks, with Player 2 only experiencing a loss of 0.3 kg of lean tissue in the injured leg. Both players returned to baseline body compositions after 24 weeks. Leg strength returned to a maximum at 24 and 15 weeks, respectively, with knee function returning to baseline by 30 weeks. This case report provides evidence that nutrition and rehabilitation programmes targeted at minimising the effects of disuse in both muscle and connective tissue may assist return to play after ACL injury.
Production of $$\mathbf {\Sigma (1385)^{\pm }}$$ Σ ( 1385 ) ± and $$\mathbf {\Xi (1530)^{0}}$$ Ξ ( 1530 ) 0 in proton–proton collisions at $$\mathbf {\sqrt{s}=}$$ s = 7 TeV (2015)

The production of the strange and double-strange baryon resonances ([Formula: see text], [Formula: see text]) has been measured at mid-rapidity ([Formula: see text][Formula: see text]) in proton-proton collisions at [Formula: see text] [Formula: see text] 7 TeV with the ALICE detector at the LHC. Transverse momentum spectra for inelastic collisions are compared to QCD-inspired models, which in general underpredict the data. A search for the [Formula: see text] pentaquark, decaying in the [Formula: see text] channel, has been carried out but no evidence is seen.
Analysis of Axial Leakage in High-Speed Slotless PM Motors for Industrial Hand Tools (2009)

This paper presents an analysis of axial-leakage effects in high-speed slotless permanent-magnet (PM) motors for industrial hand-tool applications. Analytical predictions based on a 2-D model and results from a 3-D finite-element (3DFEM) simulation model are compared with experimental results from three series-produced PM motors. The reduction in flux density (resulting from 3DFEM simulations and measurements) outside the motor's axial ends is substantial; the flux density has practically vanished only a few millimeters outside the stator lamination stack. To predict the resulting flux linkage, a simple winding model is presented where the winding is made up of a discrete number of winding layers. Using this winding model, it is verified that the reduction in flux linkage, manifested as a reduction in the back electromotive force, is not only dependent on the motor's axial length but also that the effect is minor for slotless PM motors with dimensions suitable for hand-tool applications. The results are also supported by no-load line-to-line voltage measurements on the three PM motors.
GaAs Flip Chip Evaluation in the 3 to 110 GHz Range (2004)

In the European MEDEA+ packaging project HIMICRO (Novel Packaging Technologies for Highly Integrated MICRO modules for Next Generation Telecom and Automotive Products), we address the problem to flip chip mount unbumped chips for prototyping and low volume production as a step on the way towards higher volumes. Two different flip chip (FC) interconnect technologies have been evaluated by measurements of the scattering parameters after initial electromagnetic (EM) simulation of the general structure: 1.Thermo compression (TC) flip chip bonding of the MMICs to gold ball bumps bonded on the substrate2.TC flip chip bonding of the MMICs to electrolytically plated gold pillars on the substrate Also, the possible occurrence of parasitic parallel plate (PPL) modes in flip chip assemblies with microstrip (MS), coplanar waveguide (CPW) and backside plated coplanar waveguide (BPCPW) MMICs (Microwave Monolithic Integrated Circuits) is investigated and compared. In addition, the effect of adding a resistive layer on the thin film substrate is evaluated.
Deletion of Lkb1 in Pro-Opiomelanocortin Neurons Impairs Peripheral Glucose Homeostasis in Mice (2011)

Our findings demonstrate that LKB1 in hypothalamic POMC neurons plays a key role in the central regulation of peripheral glucose metabolism but not body-weight control. This phenotype contrasts with that seen in mice lacking AMPK in POMC neurons with defects in body-weight regulation but not glucose homeostasis, which suggests that LKB1 plays additional functions distinct from activating AMPK in POMC neurons.
Multi - Antenna Systems for UWB Communications - A Review (2009)

This article focuses on multi-antenna systems for Ultra Wideband (UWB) communications. Conceptual antenna arrangements and specific designs are reviewed according to the aimed applications. Some specific parameters to characterize antennas for MIMO-UWB applications are discussed. The perspectives in sense of future developments of UWB multi-antennas and their functionalities are also suggested. In this article patent based information on multiple antenna for UWB communication is also discussed. Keywords: Array antennas, multi-antennas, multiple-input multiple-output (MIMO), ultra wideband (UWB) antenna
Defect-Induced Charge Freezing on Epitaxial Fe3O4(001) Film Surfaces Studied by Spin-Polarized Scanning Tunneling Microscopy (2005)

We report the role of atom defects on the charge freezing of Fe 3 O 4 (001) surfaces studied by spin-polarized scanning tunneling microscopy (SP-STM) using a Ni tip. Epitaxially grown Fe 3 O 4 (001) films on a MgO(001) substrate were used as samples. Atomically flat surfaces are obtained by annealing in an ultrahigh vacuum and in oxygen. The surfaces exhibit a (√2×√2) R 45° reconstruction as revealed by STM with a W tip. STM images indicate surface termination at B-sites. An atomic structure with a 0.3 nm periodicity is observed within the cation rows that are aligned along the [110] direction. SP-STM images show a pronouncedly different periodicity of 1.2 nm on areas having surface defects such as cation vacancies. This corrugation with a 1.2 nm periodicity can be attributed to a charge localization of Fe 3+ and Fe 2+ ions that are trapped by cation vacancies and then isolated from the electron hopping process. The results also indicate the important role of oxygen vacancies in modifying the 1.2 nm periodicity.
Finite element modeling of electrode mass loading effects in longitudinal leaky SAW resonators (2008)

Abstract A finite element (FE) model is developed for investigating effects of metal electrode mass loading in longitudinal leaky surface acoustic wave (SAW) resonators on YZ‐LiNbO 3 . Variation of resonator admittance with electrode height and electrode metallization ratio is observed. FE modeling is promising for the prediction and optimization of LLSAW performance, especially sensitive to electrode mass loading effects. © 2008 Wiley Periodicals, Inc. Microwave Opt Technol Lett 51: 390–395, 2009; Published online in Wiley InterScience (www.interscience.wiley.com). DOI 10.1002/mop.24042
Series Bosch System Development (2012)

State-of-the-art (SOA) carbon dioxide (CO2) reduction technology for the International Space Station produces methane as a byproduct. This methane is subsequently vented overboard. The associated loss of hydrogen ultimately reduces the mass of oxygen that can be recovered from CO2 in a closed-loop life support system. As an alternative to SOA CO2 reduction technology, NASA is exploring a Series-Bosch system capable of reducing CO2 with hydrogen to form water and solid carbon. This results in 100% theoretical recovery of oxygen from metabolic CO2. In the past, Bosch-based technology did not trade favorably against SOA technology due to a high power demand, low reaction efficiencies, concerns with carbon containment, and large resupply requirements necessary to replace expended catalyst cartridges. An alternative approach to Bosch technology, labeled "Series-Bosch," employs a new system design with optimized multi-stage reactors and a membrane-based separation and recycle capability. Multi-physics modeling of the first stage reactor, along with chemical process modeling of the integrated system, has resulted in a design with potential to trade significantly better than previous Bosch technology. The modeling process and resulting system architecture selection are discussed.
Case study of concealed weapons detection at stand-off distances using a compact, large field-of-view THz camera (2014)

The detection of concealed weapons in crowd situations is a critical need and solutions are being sought after by security agencies at the federal, state and municipal levels. Millimeter waves have been evaluated for these kinds of applications, but the currently available technologies are typically too large and bulky to allow for widespread deployment. Alternatively soft X-rays have been considered but safety issues hinder their acceptance. Terahertz technology is ideally suited for such an application as it has the ability to see through clothing, and offers higher resolution than in the millimeter band, also being more compact. THz photons have lower energy than infrared and do not show the ionizing properties of X-ray radiation. The longer Terahertz waves penetrate deeper into various materials then their visible and infrared counterparts. Though the wavelength is longer it has been shown that high resolution in a small form factor can be obtained in the THz wavebands thanks to the use of small pixel pitch detectors. In this paper, a case study for the use of a compact THz camera for active see-through imaging at stand-off distances is presented. More specifically, the cases of seeing through packages and clothing are analyzed in the perspective of concealed weapons detection. The paper starts with a review of the characteristics of a high resolution THz camera exhibiting small pixel size and large field-of-view. Some laboratory results of concealed object imaging along with details of a concept for live surveillance using a compact see-through imaging system are reviewed.
Abstract 5140: Discovery of biomarkers predicting response to radiation and anti PD1 therapy in head and neck squamous cell cancer (2019)

Abstract Introduction: Treatment of head and neck squamous cell cancer (HNSCC) frequently includes surgery, radiation, chemotherapy, targeted agents, and increasingly combination of the former with immune checkpoint inhibition. An understanding of the mechanisms mediating response to these therapies as well as biomarkers that can better tailor therapy on an individualized basis and reduce treatment-related toxicity would benefit patients. Methods: To uncover these mechanisms and biomarkers, we analyzed whole transcriptome RNA-Seq data of two different cohorts of head and neck squamous cell cancer patients: pre and post-treatment samples from radiation therapy and pre-treatment samples from anti-PD1 therapy. We evaluated the abundance of eight immune and two stromal cell populations and pathway activity changes by using Gene Set Variation Analysis (GSVA). Results and Conclusions: Following radiation genes involved in epithelial to mesenchymal transition, extracellular matrix remodeling, and wound healing are upregulated. Additionally, there is evidence of tumor microenvironment remodeling post-radiation consistent with increased fibroblast abundance and decreased immune cell infiltration. Furthermore, innately resistant tumors to anti-PD1 also display similar signatures of gene regulation, suggesting a common mechanism mediating response to both types of treatment. Notably, these signatures are strongly correlated to activation of the transforming growth factor (TGF-β) signaling pathway and suggest that attenuating its signaling may improve anti-PD-1 response and also response to radiation treatment in head and neck squamous cell cancer. Citation Format: Joon Sang Lee, Michele Sanicola-Nadel, Alexei Protopopov, Emma Wang, Joachim Theilhaber, Tun Tun Lin, Michael Korrer, Sohini Roy, Young Jun Kim, Jack Pollard. Discovery of biomarkers predicting response to radiation and anti PD1 therapy in head and neck squamous cell cancer [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 5140.
2′-Chloro,2′-fluoro Ribonucleotide Prodrugs with Potent Pan-genotypic Activity against Hepatitis C Virus Replication in Culture (2017)

Pan-genotypic nucleoside HCV inhibitors display a high genetic barrier to drug resistance and are the preferred direct-acting agents to achieve complete sustained virologic response in humans. Herein, we report, the discovery of a β-d-2'-Cl,2'-F-uridine phosphoramidate nucleotide 16, as a nontoxic pan-genotypic anti-HCV agent. Phosphoramidate 16 in its 5'-triphosphate form specifically inhibited HCV NS5B polymerase with no marked inhibition of human polymerases and cellular mitochondrial RNA polymerase. Studies on the intracellular half-life of phosphoramidate 16-TP in live cells demonstrated favorable half-life of 11.6 h, suggesting once-a-day dosing. Stability in human blood and favorable metabolism in human intestinal microsomes and liver microsomes make phosphoramidate 16 a prospective candidate for further studies to establish its potential value as a new anti-HCV agent.
Cationized dextran nanoparticle-encapsulated CXCR4-siRNA enhanced correlation between CXCR4 expression and serum alkaline phosphatase in a mouse model of colorectal cancer (2012)

CXCR4 siRNA/dextran-spermine nanoparticles appear to be highly effective, and may be suitable for further in vivo applications. Further research evaluation will be needed to determine the effect of CXCR4 silencing on serum ALP levels, which may be a useful marker to predict liver metastasis in colorectal cancer.
Biased expression of JH-proximal VH genes occurs in the newly generated repertoire of neonatal and adult mice. (1990)

We have previously demonstrated a dramatic preference for utilization of the most JH-proximal VH gene segments in the newborn liver versus adult spleen. We now examine in detail the relative expression of different VH gene families throughout ontogeny and in immunodeficient mice to gain insight into factors that cause the shift in VH usage. We find that the relative expression of VH gene families remains constant and biased throughout fetal and neonatal liver development. In addition, the primary VH repertoire expressed in neonatal spleen displays a similarly biased, position-dependent VH repertoire. The pattern of VH gene expression begins to change at 5-7 d postnatally and reaches the adult randomized pattern at approximately 2 wk of age. We also find biased expression of JH-proximal VH gene families in adult bone marrow and in spleens of adult leaky scid mice, suggesting that the spontaneously generated repertoire of adult mice is similar to that observed in neonates. Together, these data suggest that a position-dependent repertoire is generated in differentiating pre-B cells at all stages of ontogeny, at least in part, as a result of preferential rearrangement of proximal VH gene segments. Therefore, mechanisms subsequent to V gene rearrangement, such as regulatory interactions and antigen selection, must play a major role in normalizing the repertoire.