bluuebunny/crossref_metadata_2025_split huggingface dataset:
Myotonia in a patient with a mutation in an S4 arginine residue associated with hypokalaemic periodic paralysis and a concomitant synonymous CLCN1 mutation
Authors: Thor Michael G. , Vivekanandam Vinojini , Sampedro-Castañeda Marisol , Tan S. Veronica , Suetterlin Karen , Sud Richa , Durran Siobhan , Schorge Stephanie , Kullmann Dimitri M. , Hanna Michael G. , Matthews Emma , Männikkö Roope 
Published: November 2019

AbstractThe sarcolemmal voltage gated sodium channel NaV1.4 conducts the key depolarizing current that drives the upstroke of the skeletal muscle action potential. It contains four voltage-sensing domains (VSDs) that regulate the opening of the pore domain and ensuing permeation of sodium ions. Mutations that lead to increased NaV1.4 currents are found in patients with myotonia or hyperkalaemic periodic paralysis (HyperPP). Myotonia is also caused by mutations in the CLCN1gene that result in loss-of-function of the skeletal muscle chloride channel ClC-1. Mutations affecting arginine residues in the fourth transmembrane helix (S4) of the NaV1.4 VSDs can result in a leak current through the VSD and hypokalemic periodic paralysis (HypoPP), but these have hitherto not been associated with myotonia. We report a patient with an Nav1.4 S4 arginine mutation, R222Q, presenting with severe myotonia without fulminant paralytic episodes. Other mutations affecting the same residue, R222W and R222G, have been found in patients with HypoPP. We show that R222Q channels have enhanced activation, consistent with myotonia, but also conduct a leak current. The patient carries a concomitant synonymous CLCN1 variant that likely worsens the myotonia and potentially contributes to the amelioration of muscle paralysis. Our data show phenotypic variability for different mutations affecting the same S4 arginine that have implications for clinical therapy.
Early pregnancy loss in 15-hydroxyprostaglandin dehydrogenase knockout (15-HPGD−/−) mice due to requirement for embryo 15-HPGD activity
Authors: Roizen Jeffrey D. , Asada Minoru , Tong Min , Tai Hsin-Hsiung , Muglia Louis J. 
Published: November 2019

AbstractProstaglandins (PGs) have critical signaling functions in a variety of processes including the establishment and maintenance of pregnancy, and the initiation of labor. Most PGs are non-enzymatically degraded, however, the two PGs most prominently implicated in the termination of pregnancy, including the initiation of labor, prostaglandin E2 (PGE2) and prostaglandin F2α (PGF2α), are enzymatically degraded by 15-hydroxyprostaglandin dehydrogenase (15-HPGD). The role of PG metabolism by 15-HPGD in the maintenance of pregnancy remains largely unknown, as direct functional studies are lacking. To test the hypothesis that 15-PGDH-mediated PG metabolism is essential for pregnancy maintenance and normal labor timing, we generated and analyzed pregnancy in 15-HPGD knockout mice (Hpgd−/−). We report here that pregnancies resulting from matings between 15-HPGD KO mice (Hpgd−/− X Hpgd−/−KO mating) are terminated at mid gestation due to a requirement for embryo derived 15-HPGD. Aside from altered implantation site spacing, pregnancies from KO matings look grossly and histologically normal at days post coitum (dpc) 6.5 and 7.5 of pregnancy. However, virtually all of these pregnancies are resorbed by dpc 8.5. This resorption is preceded by elevation of PGF2∝ but is not preceded by a decrease in circulating progesterone, suggesting that pregnancy loss is a local inflammatory phenomenon rather than a centrally mediated phenomena. This pregnancy loss can be temporarily deferred by indomethacin treatment, but treated pregnancies are not maintained to term and indomethacin treatment increases maternal mortality. We conclude that PG metabolism to inactive products by embryo derived 15-HPGD is essential for pregnancy maintenance in mice, and may serve a similar function during human pregnancy.
Thromboinflammatory changes in plasma proteome of pregnant women with PCOS detected by quantitative label-free proteomics
Authors: Arffman R. K. , Saraswat M. , Joenväärä S. , Khatun M. , Agarwal R. , Tohmola T. , Sundström-Poromaa I. , Renkonen R. , Piltonen T. T. 
Published: November 2019

AbstractPolycystic ovary syndrome (PCOS) is the most common endocrinological disorder of fertile-aged women. Several adverse pregnancy outcomes and abnormalities of the placenta have been associated with PCOS. By using quantitative label-free proteomics we investigated whether changes in the plasma proteome of pregnant women with PCOS could elucidate the mechanisms behind the pathologies observed in PCOS pregnancies. A total of 169 proteins with ≥2 unique peptides were detected to be differentially expressed between women with PCOS (n = 7) and matched controls (n = 20) at term of pregnancy, out of which 35 were significant (p-value < 0.05). A pathway analysis revealed that networks related to humoral immune responses, inflammatory responses, cardiovascular disease and cellular growth and proliferation were affected by PCOS. Classification of cases and controls was carried out using principal component analysis, orthogonal projections on latent structure-discriminant analysis (OPLS-DA), hierarchical clustering, self-organising maps and ROC-curve analysis. The most significantly enriched proteins in PCOS were properdin and insulin-like growth factor II. In the dataset, properdin had the best predictive accuracy for PCOS (AUC = 1). Additionally, properdin abundances correlated with AMH levels in pregnant women.
Obesity during pregnancy results in maternal intestinal inflammation, placental hypoxia, and alters fetal glucose metabolism at mid-gestation
Authors: Wallace Jessica G. , Bellissimo Christian J. , Yeo Erica , Fei Xia Yu , Petrik Jim J. , Surette Michael G. , Bowdish Dawn M. E. , Sloboda Deborah M. 
Published: November 2019

AbstractWe investigated whether diet-induced changes in the maternal intestinal microbiota were associated with changes in bacterial metabolites and their receptors, intestinal inflammation, and placental inflammation at mid-gestation (E14.5) in female mice fed a control (17% kcal fat, n = 7) or a high-fat diet (HFD 60% kcal fat, n = 9; ad libitum) before and during pregnancy. Maternal diet-induced obesity (mDIO) resulted in a reduction in maternal fecal short-chain fatty acid producing Lachnospiraceae, lower cecal butyrate, intestinal antimicrobial peptide levels, and intestinal SCFA receptor Ffar3, Ffar2 and Hcar2 transcript levels. mDIO increased maternal intestinal pro-inflammatory NFκB activity, colonic CD3+ T cell number, and placental inflammation. Maternal obesity was associated with placental hypoxia, increased angiogenesis, and increased transcript levels of glucose and amino acid transporters. Maternal and fetal markers of gluconeogenic capacity were decreased in pregnancies complicated by obesity. We show that mDIO impairs bacterial metabolite signaling pathways in the mother at mid-gestation, which was associated with significant structural changes in placental blood vessels, likely as a result of placental hypoxia. It is likely that maternal intestinal changes contribute to adverse maternal and placental adaptations that, via alterations in fetal hepatic glucose handling, may impart increased risk of metabolic dysfunction in offspring.
Optimising the dielectric property of carbon nanotubes/P(VDF‐CTFE) nanocomposites by tailoring the shell thickness of liquid crystalline polymer modified layer
Authors: Zhou Yang , Luo Hang , Chen Sheng , Han Xianghui , Zhang Dou 
Published: November 2019

Interfacial modification is a good approach to improve the dielectric properties of nanocomposites. However, there are few reports to study the influence of interfacial modified thickness. In this study, the graft‐from strategy was adopted to introduce the rigid liquid crystalline fluoropolymer (poly[2,5‐bis[(4‐trifluoromethoxyphenyl)‐oxycarbonyl]] styrene (PTFMS) onto the surface of carbon nanotubes (CNT). The shell thickness increased from 20 ± 3 to 62 ± 6 nm via controlling the ratio of monomer to the initiator. The results indicated that the dielectric properties of the P(VDF‐CTFE) nanocomposites with the modified CNT were significantly enhanced compared with those original CNT, and the maximum dielectric constant of nanocomposites with 1.25 vol% CNT@PTFMS containing the thickest thickness reached 36 at 40 Hz due to the highest micro‐capacitors, which was 360% than the neat P(VDF‐CTFE). This work provides the perspective to understand the relationship between the shell thickness and dielectric property of polymer nanocomposites based on conductive fillers.
Die homöopathische Behandlung von chronischen und Autoimmunkrankheiten
Authors: Ilhan Fulya , Saltık Gülbin 
Published: November 2019

ZusammenfassungBei der Behandlung von Autoimmunerkrankungen und chronischen Erkrankungen stellt die Homöopathie eine ganzheitliche und günstige Therapieoption dar. Eine homöopathische Behandlung kann bei vielen Krankheiten zumindest die Häufigkeit und Anzahl der Krankheitsphasen und -schübe reduzieren. Hier werden 7 Fälle mit unterschiedlichen Diagnosen präsentiert, die erfolgreich homöopathisch behandelt wurden. So kann dies ein Referenzartikel für den homöopathischen Behandlungsansatz in der Türkei sein.
Verifikationen – Plädoyer für einen Neustart
Authors: Lucae Christian 
Published: November 2019

ZusammenfassungDie Rubrik „Verifikationen“ wurde in der Zeitschrift für Klassische Homöopathie (ZKH) seit 1987 regelmäßig geführt und soll in der AHZ ab 2020 neu aufgenommen werden. Damit haben alle Leserinnen und Leser die Möglichkeit, zur Verbesserung der Qualität der homöopathischen Materia medica beizutragen.
Evaluation of 18F-FDG uptake in lung parenchyma compensating for tissue fraction: Comparison between non-enhanced low dose CT and intravenous contrast-enhanced diagnostic CT
Authors: Garpered Sabine , Minarik David , Frantz Sophia , Valind Sven , Wollmer Per 
Published: November 2019

Abstract
Aim To determine how the presence of intravenous (IV) contrast-enhanced CT influences SUV measurements corrected for both attenuation and tissue fraction.
Material and Methods Eighteen patients with different malignancies, free from lung disorders, lung cancer or metastasis, were prospectively recruited when referred for staging with combined 18F-FDG-PET/CT examination. A non-enhanced low-dose CT over the chest was immediately followed by a whole-body IV contrast-enhanced diagnostic CT and finally the PET acquisition. PET data were reconstructed with attenuation correction based on the two CT data sets. The lungs were segmented in the CT images and lung density was measured. Uptake of 18F-FDG in lung parenchyma was recorded using both non-enhanced and IV contrast-enhanced CT as well as with and without compensation for lung aeration. A comparison of SUV values of corrected and uncorrected PET images was performed.
Results There was no significant difference between low dose PET/CT and IV contrast-enhanced PET/CT when removing the impact of air fraction (p = 0.093 for the right lung and p = 0.085 for the left lung). When tissue fraction was not corrected for, there was a significant difference between low dose PET/CT and IV contrast enhanced PET/CT used for attenuation correction (p = 0.006 for the right lung and p = 0.015 for the left lung).
Conclusion There was only a marginal effect on the assessement of SUV in the lung tissue when using IV contrast enhanced CT for attenuation correction when the air fraction was accounted for.
3 Diboron Compounds: Synthesis and Applications
Authors: Yoshida H. 
Published: November 2019

AbstractThis chapter describes the synthesis of symmetrical and unsymmetrical diboron compounds, and their synthetic applications for installing boron into organic molecules via activation of the boron–boron σ-bond under transition-metal/transition-metal-free catalysis. Because of the vast scale of these diboron-based boron-installing reactions, this chapter focuses mainly on the reactions with unsaturated carbon–carbon bonds such as alkynes and alkenes, α,β-unsaturated carbonyl componds, organic halides, and Caryl–H bonds. A discussion of the regio-, stereo-, and enantioselectivities of the boron-installing reactions is also included.
5 anti Boron Addition to Alkynes
Authors: Sawamura M. , Ohmiya H. 
Published: November 2019

AbstractBoron additions, such as carboboration, silaboration, diboration, or hydroboration of alkynes, offer efficient strategies for the synthesis of alkenylborons, which are versatile synthetic intermediates. Most of the reported reactions occur in syn addition mode. Accordingly, anti-selective boron addition to alkynes is an important challenge in modern organic synthesis. This chapter describes successful examples of anti-selective boron additions.
Ontological and epistemological challenges of measuring the effectiveness of urban counterterrorism measures
Authors: Jore Sissel H. 
Published: November 2019

AbstractCities and their citizens are increasingly seen as vulnerable targets for terrorist attacks, and nowadays city planners have become important actors in making decisions about urban security. Multiple urban counterterrorism measures that have affected the urban landscape have been implemented in recent years. Simultaneously, new legislation requires security measures to be effective. This article outlines and discusses the epistemological and ontological challenges of acquiring knowledge about the effectiveness of urban counterterrorism measures from a local city planner’s perspective. This piece of work is a discussion paper based on a literature review. We conclude that, despite the knowledge limitations regarding the threat of urban terrorism and associated countermeasures, local planners should refrain from just uncritically implementing urban countermeasures without considering the effectiveness of such measures. Without knowledge on what constitutes the effectiveness of urban security measures, the city might end up infringing the same values that it aims to protect, without achieving security.
Shaping the zebrafish myotome by intertissue friction and active stress
Authors: Tlili S. , Yin J. , Rupprecht J.-F. , Mendieta-Serrano M. A. , Weissbart G. , Verma N. , Teng X. , Toyama Y. , Prost J. , Saunders T. E. 
Published: November 2019

Significance
How do tissues self-organize to generate the complex organ shapes observed in vertebrates? Organ formation requires the integration of chemical and mechanical information, yet how this is achieved is poorly understood for most organs. Muscle compartments in zebrafish display a V shape, which is believed to be required for efficient swimming. We investigate how this structure emerges during zebrafish development, combining live imaging and quantitative analysis of cellular movements. We use theoretical modeling to understand how cell differentiation and mechanical interactions between tissues guide the emergence of a specific tissue morphology. Our work reveals how spatially modulating the mechanical environment around and within tissues can lead to complex organ shape formation.
Artificial turf infill associated with systematic toxicity in an amniote vertebrate
Authors: Xu Elvis Genbo , Lin Nicholas , Cheong Rachel S. , Ridsdale Charlotte , Tahara Rui , Du Trina Y. , Das Dharani , Zhu Jiping , Peña Silva Laura , Azimzada Agil , Larsson Hans C. E. , Tufenkji Nathalie 
Published: November 2019

Significance
Athletes and children are playing on artificial turfs. However, the health risk associated with exposure to crumb rubber from artificial turfs is unknown for higher vertebrates. Here, we employed chicken embryo as a developing amniote vertebrate model to show that toxic leachate from artificial athletic turf infill impairs the early development of chicken, notably brain and cardiovascular system. This study triggers a scientific discussion as to whether crumb rubber is an appropriate infill material for artificial fields.
Fresnel drag in space–time-modulated metamaterials
Authors: Huidobro Paloma A. , Galiffi Emanuele , Guenneau Sébastien , Craster Richard V. , Pendry J. B. 
Published: November 2019

Significance
Space–time-dependent electromagnetic media, whose constitutive parameters are directionally biased in space and time, are a topic of current enormous interest across physics. Here we address a problem of fundamental relevance: the similarities and differences between such modulated materials and the classic problem of moving media. We establish that, surprisingly, the Fresnel drag effect of light in moving media, dictated by special relativity, is also present in modulated media, fully resolving this paradox with an analytical approach. We discuss how nonreciprocity can emerge at vanishing frequency. While we focus on electromagnetism, our findings can be extended to other wave systems.
Topology optimization through stiffness/weight ratio analysis for a three-point bending test of additive manufactured parts
Authors: Garcia-Granada A A , Catafal-Pedragosa J , Lemu H G 
Published: November 2019

Abstract
Topology Optimization (TO) is a technique that allows for increasingly efficient designs and its objective is to maximize the performance of mechanical systems or structure in a variety of fields. Attempts to employ TO for parts manufactured with conventional methods such as casting, forging, injection moulding, CNC machining and the like could not lead to desired optimum results due to the existing manufacturing constraints regarding geometrical complexity. Currently, additive manufacturing (AM) techniques allow the fabrication of more complex shapes which in principle will lead to improved performances through application of the TO concept. This study focuses on structural optimization of additive manufactured parts of thermoplastic parts based on analysis of the stiffness/weight (mass) ratio for a beam subjected to a three-point bending load. The experimental work is done on optimization of parts manufactured by Fused Deposition Modelling (FDM) technology and finally compared with an identical model manufactured using Polyjet 3D printer. Different TO software are compared to conduct the optimization, and a module of SolidWorks 2018 from Dassault Systems is chosen for the topology optimization for the final experiment. The study focuses on the results on stiffness/mass ratios, paying attention to the influence of different printing parameters on the test results. An increase of stiffness/weight ratio of 31.7% was predicted by software while experiments showed an increase of just 27.04%.
State-of-the-art of crack propagation modelling in tubular joints
Authors: Atteya M , Mikkelsen O , Lemu H G 
Published: November 2019

Abstract
Offshore structures are conventionally designed for a service life between 20-30 years and mostly used beyond their service life. They are operated up to the point where costs of operations, maintenance and repair exceed the revenue generated by the offshore structure. The cyclic nature of environmental loading induces multi-axial stresses with high gradients in welded tubular joints that lead to fatigue cracks emanating from imperfections in the welded materials. Tubular joints may exhibit significant residual life after fatigue cracks formation due to crack propagating around the weld circumference. It is important to understand tubular joints behaviour and estimate the residual life beyond crack initiation as it promotes the safe operation of the offshore installation, inspection planning and life extension of the asset. This paper presents an overview of the residual life estimation of cracked tubular joints using numerical methods. The recent developments and possible enhancements to the modelling methods of cracked tubular joints are presented.
Impact from ice floes and icebergs on ships and offshore structures in Polar Regions
Authors: Amdahl J 
Published: November 2019

Abstract
The principles for ULS design and ALS design for offshore structures are discussed. The use of pressure-area curve versus force-area curves for analysis for estimation of ALS impacts is deliberated. Aspects of local and global shape of the ice feature are discussed in view of external mechanics (demand for energy dissipation) and internal mechanics (local damage). Simplified methods for structural damage assessment are reviewed for ice loads that may move both transverse to and along the shell plating. Material modelling of ice for nonlinear finite element analysis (NLFEA) of ice-structure interaction is reviewed. The material models must be calibrated against design curves and the sensitivity of the material parameters with respect to mesh size is investigated. Results from simulation of impacts of ice with different shapes on the column of a floating platform are presented, and the critical shape for penetration of the front plating for the present structural configuration is identified.
Remote inspection by the magnetic tomography method (MTM) to prevent the risks imposed by exploitation of Arctic offshore pipelines
Authors: Kamaeva S S , Kolesnikov I S , Eremin N A , Khusnutdinov L A 
Published: November 2019

Abstract
Cold climate areas that provide opportunities for the remote inspection of pipelines include the Barents Sea, the Russian Arctic, the Alaskan Chukchi Sea, the Beaufort Sea and the Canadian Arctic offshore. First, an analysis of several actual projects of contactless diagnostics using the magnetic tomography method of pipelines in Arctic conditions is done. Second, the Risk-Based Inspection methodology for Arctic offshore pipelines is discussed. It involves ensuring pipeline reliability on the basis of data on the technical condition of the metal in actual operating conditions. The magnetic tomography method allows not only to remotely identify areas of anomalies with metal defects, but also to register mechanical stress levels taking into account actual loads. This reduces the risk for the structure to come to the critical state in terms of exceeding local loads. Finally, magnetic tomography technology allows managing risks in cases of local corrosion, stress cracking or loss of stability of underwater pipelines in areas with free spanning. The qualitative indicators of the inspection include the probabilities of identifying, interpreting the degree of danger, missing a dangerous defect. The pipeline diagnostics report provides the parameters of reliability forecasting: the period of incident-free operation, safe working pressure, and pressure coefficient.
About a method of acoustic impact on high viscosity oil fields
Authors: Alisheva Z N , Eremin N A , Metaksa G P 
Published: November 2019

Abstract
The report describes the method of the acoustic impact on the oil reservoir. Chemical decomposition of formation water molecules occurs with the help of energy-accumulating substances. Water becomes a supplier of hydrogen and oxygen. This allows the processes of hydrogenation and synthesis of new compounds. The decomposition of water is carried out using activated aluminum. As a result of the reaction, hydrogen is released, which provides the reaction of hydrogenation of all fractions of heavy oils. Industrial tests of the new method of acoustic impact were carried out at one of the fields with high viscosity of the Embamunaigas company in 2016. Pilot tests revealed an increase in the flowability of heavy oil during the hydrogenation of its components. The increase in well productivity was recorded during the first days of testing a new method. The negative side of the tests was the colmatation of the well with the released aluminum oxide. Physical and chemical processes on the surface of the reacting components were studied. In the course of the research, the state of the phase interface was studied. The spectral composition of the responses at the phase interface was measured. The report presents the results of measurements of the spectral response of water to external influences. The test results of the new acoustic method showed the need for additional research.