bluuebunny/crossref_metadata_2025 huggingface dataset:
Have ozone effects on carbon sequestration been over-estimated? A new biomass response function for wheat
Authors: Pleijel H. , Danielsson H. , Simpson D. , Mills G.
Published: April 2014

Abstract. Elevated levels of tropospheric ozone can significantly impair the growth of crops. The reduced removal of CO2 by plants leads to higher atmospheric concentrations of CO2, enhancing radiative forcing. Ozone effects on economic yield, e.g. the grain yield of wheat (Triticum aestivum L.) are currently used to model effects on radiative forcing. However, changes in grain yield do not necessarily reflect changes in total biomass. Based on analysis of 21 ozone exposure experiments with field-grown wheat, we investigated whether use of effects on grain yield as a~proxy for effects on biomass under- or over-estimates effects on biomass. First, we confirmed that effects on partitioning and biomass loss are both of significant importance for wheat yield loss. Then we derived ozone dose response functions for biomass loss and for harvest index (the proportion of above-ground biomass converted to grain) based on twelve experiments and recently developed ozone uptake modelling for wheat. Finally, we used a European scale chemical transport model (EMEP MSC-West) to assess the effect of ozone on biomass (−9%) and grain yield (−14%) loss over Europe. Based on yield data per grid square, we estimated above ground biomass losses due to ozone in 2000 in Europe totalling 22.2 million tonnes. Incorrectly applying the grain yield response function to model effects on biomass instead of the biomass response function of this paper would have indicated total above ground biomass losses totalling 38.1 million (i.e. overestimating effects by 15.9 million tonnes). A key conclusion from our study is that future assessments of ozone induced loss of agroecosystem carbon storage should use response functions for biomass, such as that provided in this paper, not grain yield, to avoid overestimation of the indirect radiative forcing from ozone effects on crop biomass accumulation.
The generic MESSy submodel TENDENCY (v1.0) for process-based analyses in Earth System Models
Authors: Eichinger R. , Jöckel P.
Published: April 2014

Abstract. The tendencies of prognostic variables in Earth System Models are usually only accessible, e.g., for output, as sum over all physical, dynamical and chemical processes at the end of one time integration step. Information about the contribution of individual processes to the total tendency is lost, if no special precautions are implemented. The knowledge on individual contributions, however, can be of importance to track down specific mechanisms in the model system. We present the new MESSy (Modular Earth Submodel System) infrastructure submodel TENDENCY and use it exemplarily within the EMAC (ECHAM/MESSy Atmospheric Chemistry) model to trace process-based tendencies of prognostic variables. The main idea is the outsourcing of the tendency accounting for the state variables from the process operators (submodels) to the TENDENCY submodel itself. In this way, a record of the tendencies of all process-prognostic variable pairs can be stored. The selection of these pairs can be specified by the user, tailor-made for the desired application, in order to minimise memory requirements. Moreover a standard interface allows the access to the individual process tendencies by other submodels, e.g., for on-line diagnostics or for additional parameterisations, which depend on individual process tendencies. An optional closure test assures the correct treatment of tendency accounting in all submodels and thus serves to reduce the models susceptibility. TENDENCY is independent of the time integration scheme and therefore applicable to other model systems as well. Test simulations with TENDENCY show an increase of computing time for the EMAC model (in a setup without atmospheric chemistry) of 1.8 ± 1% due to the additional subroutine calls when using TENDENCY. Exemplary results reveal the dissolving mechanisms of the stratospheric tape recorder signal in height over time. The separation of the tendency of the specific humidity into the respective processes (large-scale clouds, convective clouds, large-scale advection, vertical diffusion and methane-oxidation) show that the upward propagating water vapour signal dissolves mainly because of the chemical and the advective contribution. The TENDENCY submodel is part of version 2.42 or later of MESSy.
Non-linear water waves generated by impulsive motion of submerged obstacles
Authors: Makarenko N. I. , Kostikov V. K.
Published: April 2014

Abstract. A fully non-linear problem on unsteady water waves generated by an impulsively moving obstacle is studied analytically. Our method involves reduction of the Euler equations to the integral-differential system for the wave elevation together with normal and tangential fluid velocities at a free surface. Exact model equations are derived in explicit form in a case where an isolated obstacle is presented by a totally submerged elliptic cylinder. A small-time asymptotic solution is constructed for a cylinder which starts with constant acceleration from rest. It is demonstrated that the leading-order solution terms describe several wave regimes such as the formation of non-stationary splash jets by vertical rising or vertical submersion of the obstacle; the generation of diverging waves is also observed.
Fracture-induced softening for large-scale ice dynamics
Authors: Albrecht T. , Levermann A.
Published: April 2014

Abstract. Floating ice shelves can exert a retentive and hence stabilizing force onto the inland ice sheet of Antarctica. However, this effect has been observed to diminish by the dynamic effects of fracture processes within the protective ice shelves, leading to accelerated ice flow and hence to a sea-level contribution. In order to account for the macroscopic effect of fracture processes on large-scale viscous ice dynamics (i.e., ice-shelf scale) we apply a continuum representation of fractures and related fracture growth into the prognostic Parallel Ice Sheet Model (PISM) and compare the results to observations. To this end we introduce a higher order accuracy advection scheme for the transport of the two-dimensional fracture density across the regular computational grid. Dynamic coupling of fractures and ice flow is attained by a reduction of effective ice viscosity proportional to the inferred fracture density. This formulation implies the possibility of non-linear threshold behavior due to self-amplified fracturing in shear regions triggered by small variations in the fracture-initiation threshold. As a result of prognostic flow simulations, sharp across-flow velocity gradients appear in fracture-weakened regions. These modeled gradients compare well in magnitude and location with those in observed flow patterns. This model framework is in principle expandable to grounded ice streams and provides simple means of investigating climate-induced effects on fracturing (e.g., hydro fracturing) and hence on the ice flow. It further constitutes a physically sound basis for an enhanced fracture-based calving parameterization.
Auteur and Style in National Cinema: A Reframing of Metin Erksan's Time To Love
Authors: Akser Murat
Published: April 2014

This essay will hunt down and classify the concept of the national as a discourse in Turkish cinema that has been constructed back in 1965, by the film critics, by filmmakers and finally by today’s theoretical standards. So the questions we will be constantly asking throughout the essay can be: Is what can be called part of national film culture and identity? Is defining a film part of national heritage a modernist act that is also related to theories of nationalism? Is what makes a film national a stylistic application of a particular genre (such as melodrama)? Does the allure of the film come from the construction of a hero-cult after a director deemed to be national?
Gerenciamento de Projetos na Construção Civil como Ferramenta Chave de Marketing no Serviço Público
Authors: França Freitas Thyago Phellip , De Sousa Ruberval Rodrigues
Published: April 2014

O Governo Federal tem a responsabilidade sobre a administração pública do Brasil por meio das pastas que estão sob sua jurisdição. Este investe recursos financeiros na execução e gerenciamento de projetos que, não são aceitos pela população. Contudo, investe em campanhas de marketing, para reverter este quadro, bem como suprir as metas e alcançar os índices de assistência à comunidade. Entretanto, devido às falhas no gerenciamento acaba influenciando diretamente o projeto macro das estratégias de marketing, pois este é uma parte no processo das ações. A abordagem sobre gerenciamento de projetos na construção civil ganha força no serviço público, pois este processo se torna oneroso pela dificuldade de aplicação do método coerente, tornando difícil, assim, o processo das estratégias de marketing na organização.DOI:10.5585/gep.v4i2.100
Contributions of the PMBok to the Project Management of an ERP System Implementation
Authors: Da Silva Gomes Rui Manuel
Published: April 2014

The implementation of Enterprise Resource Planning (ERP) Systems has become generalized in companies and organizations, as a way to achieve the supply chain integration, to improve productivity and gain competitive advantages. Those implementations, more than simple technology projects, have revealed to be complex and time consuming business projects due to the structural and behaviour changes involved. This article analyzes, through the Project Coordinator’s point of view, two real cases of ERP implementation projects at Ibico Portuguesa, comparing the project management methodology adopted by Ibico with the Project Management Body of Knowledge (PMBoK), coming to the conclusion that the use of the PMBoK norms and best practices by the Project Manager could have been a success factor in those implementations, particularly referring to the elements that have been pointed as the most critical: planning, involvement and commitment of top management as well as stakeholders’ management.DOI:10.5585/gep.v4i2.37
Tissue-resident natural killer (NK) cells are cell lineages distinct from thymic and conventional splenic NK cells
Authors: Sojka Dorothy K , Plougastel-Douglas Beatrice , Yang Liping , Pak-Wittel Melissa A , Artyomov Maxim N , Ivanova Yulia , Zhong Chao , Chase Julie M , Rothman Paul B , Yu Jenny , Riley Joan K , Zhu Jinfang , Tian Zhigang , Yokoyama Wayne M
Published: April 2014

Natural killer (NK) cells belong to the innate immune system; they can control virus infections and developing tumors by cytotoxicity and producing inflammatory cytokines. Most studies of mouse NK cells, however, have focused on conventional NK (cNK) cells in the spleen. Recently, we described two populations of liver NK cells, tissue-resident NK (trNK) cells and those resembling splenic cNK cells. However, their lineage relationship was unclear; trNK cells could be developing cNK cells, related to thymic NK cells, or a lineage distinct from both cNK and thymic NK cells. Herein we used detailed transcriptomic, flow cytometric, and functional analysis and transcription factor-deficient mice to determine that liver trNK cells form a distinct lineage from cNK and thymic NK cells. Taken together with analysis of trNK cells in other tissues, there are at least four distinct lineages of NK cells: cNK, thymic, liver (and skin) trNK, and uterine trNK cells.
Delivery of endocytosed proteins to the cell–division plane requires change of pathway from recycling to secretion
Authors: Richter Sandra , Kientz Marika , Brumm Sabine , Nielsen Mads Eggert , Park Misoon , Gavidia Richard , Krause Cornelia , Voss Ute , Beckmann Hauke , Mayer Ulrike , Stierhof York-Dieter , Jürgens Gerd
Published: April 2014

Membrane trafficking is essential to fundamental processes in eukaryotic life, including cell growth and division. In plant cytokinesis, post-Golgi trafficking mediates a massive flow of vesicles that form the partitioning membrane but its regulation remains poorly understood. Here, we identify functionally redundant Arabidopsis ARF guanine-nucleotide exchange factors (ARF-GEFs) BIG1–BIG4 as regulators of post-Golgi trafficking, mediating late secretion from the trans-Golgi network but not recycling of endocytosed proteins to the plasma membrane, although the TGN also functions as an early endosome in plants. In contrast, BIG1-4 are absolutely required for trafficking of both endocytosed and newly synthesized proteins to the cell–division plane during cytokinesis, counteracting recycling to the plasma membrane. This change from recycling to secretory trafficking pathway mediated by ARF-GEFs confers specificity of cargo delivery to the division plane and might thus ensure that the partitioning membrane is completed on time in the absence of a cytokinesis-interphase checkpoint.
A frontal cortex event-related potential driven by the basal forebrain
Authors: Nguyen David P , Lin Shih-Chieh
Published: April 2014

Event-related potentials (ERPs) are widely used in both healthy and neuropsychiatric conditions as physiological indices of cognitive functions. Contrary to the common belief that cognitive ERPs are generated by local activity within the cerebral cortex, here we show that an attention-related ERP in the frontal cortex is correlated with, and likely generated by, subcortical inputs from the basal forebrain (BF). In rats performing an auditory oddball task, both the amplitude and timing of the frontal ERP were coupled with BF neuronal activity in single trials. The local field potentials (LFPs) associated with the frontal ERP, concentrated in deep cortical layers corresponding to the zone of BF input, were similarly coupled with BF activity and consistently triggered by BF electrical stimulation within 5–10 msec. These results highlight the important and previously unrecognized role of long-range subcortical inputs from the BF in the generation of cognitive ERPs.
Illuminating traffic control for cell–division planes
Authors: Robatzek Silke
Published: April 2014

When a plant cell divides, four related proteins control the trafficking of vesicles and ensure that cargo that is normally recycled to the plasma membrane is instead re-routed to the plane of cell division.
Stimulated Raman spectral microscope using synchronized Er- and Yb-fiber lasers
Authors: Nose Keisuke , Kishi Tatsuya , Ozeki Yasuyuki , Kanematsu Yasuo , Takata Hideaki , Fukui Kiichi , Takai Yoshizo , Itoh Kazuyoshi
Published: April 2014

We report our development of a stimulated Raman scattering (SRS) spectral microscope employing synchronized fiber-laser (FL)-based pulse sources, which generate both pump and Stokes pulses. The pump pulses are obtained through the second-harmonic generation of Er-FL pulses. The high-frequency noise in the photocurrent of the pump pulses is successfully suppressed to the shot-noise limit by the collinear balanced detection (CBD) technique using a compact fiber delay line. Fast wavelength-tunable Stokes pulses are generated through the spectral filtering of broadband Yb-FL pulses and post amplification. To achieve a wide wavelength tunability, we introduce a double-pass spectral filter and confirm that the wavelength tunability can be extended to ∼280 cm−1, while the wavelength tunability with a single-pass spectral filter is ∼190 cm−1. The spectral filter is also used to compensate for the group delay dispersion (GDD) of Stokes pulses, which is induced by fiber amplifiers. We show that the negative dispersion can be introduced to the pulses simply by shifting the position of the diffraction grating in the spectral filter. Although the wavelength tunability is slightly decreased to 230 cm−1 after the compensation, it is sufficient for covering the CH-stretching vibration region. The SRS imaging and microspectroscopy of polymer beads and HeLa cells are successfully demonstrated.
Pursuing Professional Interests at the Local Policy Level: A Comparative Case Study
Authors: Kjær Joensen Anne Sofie , Kousgaard Marius Brostrøm , Burau Viola
Published: April 2014

The relationship between the state and organized occupational groups is a salient issue in the sociology of professions, but which has been changing considerably over recent decades. This paper revisits state-professions dynamics at the local level especially in relation to welfare service professions. The contribution is two-fold: empirically, we investigate how welfare professionals attempt to influence local policies; theoretically, we adapt the notion of social closure introduced by the sociology of professions to local institutional contexts. The analysis is based on a comparative case study of two groups of welfare professionals in two Danish municipalities. Empirically, our study shows that welfare professionals actively consider the local institutional context when acting to promote the interests of their own professional groups. Theoretically, our study identifies three dimensions of local closure strategies; the strategies involve both administrative and practising professionals and within that offensive/defensive as well as direct/indirect means.
Australia plays ‘catch-up’ with Fetal Alcohol Spectrum Disorders
Authors: Elliott Elizabeth Jane
Published: April 2014

Elliott, E. (2014). Australia plays ‘catch-up’ with Fetal Alcohol Spectrum Disorders. The International Journal Of Alcohol And Drug Research, 3(1), 121-125. doi:http://dx.doi.org/10.7895/ijadr.v3i1.177Australians are amongst the highest consumers of alcohol worldwide, and "risky" drinking is increasing in young women. Contrary to the advice in national guidelines, drinking in pregnancy is common. Many women don’t understand the potential for harm to the unborn child and 20% have a "tolerant" attitude to drinking during pregnancy. As attitude, rather than knowledge, predicts risk of drinking in a future pregnancy, this presents a challenge for public health campaigns. Alcohol is teratogenic, crosses the placenta, and contributes to a range of physical, developmental, learning and behavioural problems, including fetal alcohol spectrum disorders (FASD). As nearly half of all pregnancies in Australia are unplanned, inadvertent exposure to alcohol is common. Good-quality prevalence data on FASD are lacking in Australia, although alcohol use at "risky" levels is well documented in some disadvantaged communities. In the last decade, clinicians, researchers, governments and non-governmental organizations have shown renewed interest in addressing alcohol use in pregnancy and FASD. This has included a parliamentary inquiry into FASD, provision of targeted funding for FASD, and development of educational materials for health professionals and the general public. Key challenges for the future are to prevent FASD and to offer timely diagnosis and help to children and families living with FASD. The implementation of evidence-based interventions known to decrease access to, and excessive use of, alcohol in our society will aid in the prevention of FASD. The development of national diagnostic tools for screening and diagnosis, and the training of health professionals in the management of FASD, are urgently needed.
Linear mode conversion of Langmuir/z mode waves to radiation: Averaged energy conversion efficiencies, polarization, and applications to Earth's continuum radiation
Authors: Schleyer Fiona , Cairns Iver H. , Kim Eun‐Hwa
Published: April 2014

Linear mode conversion (LMC) is the linear transfer of energy from one wave mode to another in a density gradient. It is relevant to planetary continuum radiation, type II and III radio bursts, and ionospheric radio emissions. This paper analyzes LMC by calculating angle‐averaged energy (ε) and power (εp) conversion efficiencies in both 2‐D and 3‐D for Langmuir/z mode waves (including upper hybrid waves for perpendicular wave vectors) converting to free‐space radiation in turbulent plasmas. The averages are over the distributions of the incoming Langmuir/z mode wave vectors k, density scale lengths L, and angles α and δ, where α is the angle between k and the background magnetic field B0 and δ is the angle between the density gradient ∇N0 and B0. The results show that the averaged and unaveraged conversion efficiencies are dependent on γβ, where γ is the adiabatic index and β is related to the electron temperature Te by β = Te/mec2. The averaged energy conversion efficiencies are proportional to γβ in 2‐D and to (γβ)3/2 in 3‐D, whereas the power conversion efficiencies are proportional to (γβ)1/2 in 2‐D and γβ in 3‐D. The special case of a perpendicular density gradient (δ≈90°) is considered and used to predict the conversion efficiencies of terrestrial continuum radiation (TCR) in three known source regions: the plasmapause, magnetopause, and the plasma sheet. The observed energy conversion efficiencies are estimated and are found to be consistent with the 2‐D and 3‐D predicted efficiencies; importantly, these results imply that LMC is a possible generation mechanism for TCR. The polarization of TCR is also predicted: TCR should be produced primarily in the o mode at the plasmapause and in both the o and x modes at the magnetopause and plasma sheet. These predictions are consistent with previous independent predictions and observations.
Large‐scalequantization from local correlations in space plasmas
Authors: Livadiotis George , McComas David J.
Published: April 2014

This study examines the large‐scale quantization that can characterize the phase space of certain physical systems. Plasmas are such systems where large‐scale quantization,ħ*, is caused by Debye shielding that structures correlations between particles. The value ofħ*is constant—some 12 orders of magnitude larger than the Planck constant—across a wide range of space plasmas, from the solar wind in the inner heliosphere to the distant plasma in the inner heliosheath and the local interstellar medium. This paper develops the foundation and advances the understanding of the concept of plasma quantization; in particular, we (i) show the analogy of plasma to Planck quantization, (ii) show the key points of plasma quantization, (iii) construct some basic quantum mechanical concepts for the large‐scale plasma quantization, (iv) investigate the correlation between plasma parameters that implies plasma quantization, when it is approximated by a relation between the magnetosonic energy and the plasma frequency, (v) analyze typical space plasmas throughout the heliosphere and show the constancy of plasma quantization over many orders of magnitude in plasma parameters, (vi) analyze Advanced Composition Explorer (ACE) solar wind measurements to develop another measurement of the value ofħ*, and (vii) apply plasma quantization to derive unknown plasma parameters when some key observable is missing.
Simulations of pickup ion mediated quasi‐perpendicular shocks: Implications for the heliospheric termination shock
Authors: Matsukiyo Shuichi , Scholer Manfred
Published: April 2014

The microstructure of the heliospheric termination shock and the accompanied local acceleration processes of both ions and electrons are investigated by utilizing one‐dimensional full particle‐in‐cell simulations for a variety of parameters. The relative pickup ion density is assumed to be 20–30%. The magnetic field and the shock potential profiles exhibit significant differences, since the former mostly reflects the dynamics of solar wind ions, whereas the latter is mainly sustained by the bulk motion of the reflected pickup ions in the extended foot. The discrepancy between the magnetic field profile and the potential profile increases with Alfvén Mach number. Most of the downstream thermal energy is gained by the pickup ions, while some heating of the solar wind ions and electrons occurs through the modified two‐stream instability excited in the extended foot. Self‐reformation can occur when the relative pickup ion density is 20% but is blurred when it becomes as large as 30%. Reformation is also suppressed if the local solar wind ion temperature in the extended foot is high, which can either be due to heating by the modified two‐stream instability or is already determined by the solar wind temperature far upstream. In all runs presented in this study no evidence for shock surfing acceleration of pickup ions could be found. Nonthermal particle acceleration occurs for oblique shocks. Electron (pickup ion) shock drift acceleration is evidenced when the shock angle is below 80° (60°).
Temperature dependence of the water vapor self‐continuum by cavity ring‐down spectroscopy in the 1.6 µm transparency window
Authors: Mondelain D. , Manigand S. , Kassi S. , Campargue A.
Published: April 2014

The very weak water vapor self‐continuum has been investigated by high‐sensitivity cavity ring‐down spectroscopy in the 1.6 µm window at five temperatures between 302 K and 340 K. The absorption cross sections, Cs(ν, T), were retrieved for 10 selected wave numbers from a fit of the absorption coefficients measured in real time during pressure ramps, after subtraction of the contributions of the local water monomer lines and of water adsorbed on the cell mirrors. The values measured between 5875 and 6665 cm−1 range between 1.5 × 10−25 and 2 × 10−24 cm2 mol−1 atm−1 with a minimum around 6300 cm−1. At 302 K, an agreement within 50% is observed over the whole window with the cross sections provided by the MT_CKD V2.5 model. Nevertheless, while our measurements show that the Cs(ν, T) decrease from 302 K to 340 K is no more than 50% for all our selected wave numbers, the MT_CKD V2.5 model predicts a much more pronounced temperature dependence in the center of the window, the agreement being better on the edges of the window. The obtained results are discussed in relation with theoretical modeling of the water vapor self‐continuum as far wings of monomer lines or water dimer absorption. For potential atmospheric applications, cross sections are provided at each temperature with a sampling step of 10 cm−1 for the entire 5850–6700 cm−1 range.
Characterization of runoff‐storage relationships by satellite gravimetry and remote sensing
Authors: Riegger J. , Tourian M. J.
Published: April 2014

GRACE observations of the time‐dependent gravity field provide a direct measurement of the monthly state of mass and thus monthly total water storage in a catchment. This for the first time allows for a direct comparison of monthly runoff and water storage. Investigations of global scale Runoff‐Storage (R‐S) relationships for different climatic conditions show distinct periodic characteristics with hysteresis for total water storage. For fully humid tropical catchments, hysteresis reveals a time invariant temporal delay from storage to runoff. Our spectral analysis supports the fact that the R‐S relationships can be characterized as a Linear Time Invariant (LTI) System. As a consequence in time domain an adjustment of time lag leads to correlation of 0.98 between runoff and storage. Based hereon, the hypothesis of a R‐S relationship characterized by the superposition of linear contributions from coupled/liquid storage and nonlinear contributions from uncoupled storages is investigated by means of remote sensing. For boreal catchments MODIS snow coverage is used to separate total storage into coupled/liquid and uncoupled/solid components either directly by assigning frozen solid storage to the snow‐covered areas or indirectly by a model‐based aggregation of snow and liquid according to snow coverage. Both methods show that the nonlinear part of the R‐S relationship can be fully assigned to the uncoupled/solid storage while the relationship of runoff and liquid storage can also be characterized as an LTI system. This system behavior thus allows for a direct determination of river runoff from GRACE mass and vice versa for unmanaged catchments, provided that the coupled/uncoupled storage components can be quantified remote sensing.
USGS grants
Authors: Wendel JoAnna
Published: April 2014

The U.S. Geological Survey (USGS) has announced that it will award up to $5 million in grants for earthquake hazards research in 2015. The USGS Earthquake Hazards program awards research grants to universities, state geological surveys, and private institutions every year for new and innovative proposals. Interested researchers can apply online at http://www.grants.gov under funding opportunity number G14AS00036. Applications are due 22 May 2014.
Cassini sheds light on Titan's second largest lake, Ligeia Mare
Authors: Wendel JoAnna
Published: April 2014

Saturn's largest moon, Titan, is known for its dense, planet‐like atmosphere and large lakes most likely made of methane and ethane. It has been suggested that Titan's atmosphere and surface are a model of early Earth. Since the early 2000s, NASA's Cassini space probe has been unlocking secrets of the distant moon.
Intense Pulsed Light Sintering of Electrodeposited CdS Thin Films
Authors: Dharmadasa Ruvini , Dharmadasa I.M. , Druffel Thad
Published: April 2014

In order to manufacture market competitive solar cells, material processing methods with low capital and running costs are required. One method for achieving this goal would be to use atmospheric processing techniques with a high throughput. Here, we report the use of intense pulsed light (IPL) to process cadmium sulfide (CdS) thin films. IPL is an ultra‐fast and facile technique for the heat treatment of materials. Pulses of light from the UV to IR wavelengths are absorbed by the material, leading to localized heating and sintering. IPL has been used to successfully make Ag and Cu electrical contacts for use in electronic circuits, however little work exists on the use of IPL in the processing of semiconductor materials. CdS was selected due to its importance in solar cell manufacturing. This n‐type semiconductor has been used as a “window” material in high efficiency CdTe and Cu In(1−x)GaxSe2‐based solar cells. The effect of energy input and the energy density of the pulse on the films optical, compositional and morphological properties of the films was investigated using UV–Vis spectroscopy, XRD, and SEM. IPL sintering resulted in significant improvement to the CdS crystallinity with processing times <2 mins.
Additive Manufacturing of Ceramic‐Based Materials
Authors: Travitzky Nahum , Bonet Alexander , Dermeik Benjamin , Fey Tobias , Filbert‐Demut Ina , Schlier Lorenz , Schlordt Tobias , Greil Peter
Published: April 2014

This paper offers a review of present achievements in the field of processing of ceramic‐based materials with complex geometry using the main additive manufacturing (AM) technologies. In AM, the geometrical design of a desired ceramic‐based component is combined with the materials design. In this way, the fabrication times and the product costs of ceramic‐based parts with required properties can be substantially reduced. However, dimensional accuracy and surface finish still remain crucial features in today's AM due to the layer‐by‐layer formation of the parts. In spite of the fact that significant progress has been made in the development of feedstock materials, the most difficult limitations for AM technologies are the restrictions set by material selection for each AM method and aspects considering the inner architectural design of the manufactured parts. Hence, any future progress in the field of AM should be based on the improvement of the existing technologies or, alternatively, the development of new approaches with an emphasis on parts allowing the near‐net formation of ceramic structures, while optimizing the design of new materials and of the part architecture.
Bosutinib efficacy and safety in chronic phase chronic myeloid leukemia after imatinib resistance or intolerance: Minimum 24‐month follow‐up
Authors: Gambacorti‐Passerini Carlo , Brümmendorf Tim H. , Kim Dong‐Wook , Turkina Anna G. , Masszi Tamas , Assouline Sarit , Durrant Simon , Kantarjian Hagop M. , Khoury H. Jean , Zaritskey Andrey , Shen Zhi‐Xiang , Jin Jie , Vellenga Edo , Pasquini Ricardo , Mathews Vikram , Cervantes Francisco , Besson Nadine , Turnbull Kathleen , Leip Eric , Kelly Virginia , Cortes Jorge E.
Published: April 2014

Bosutinib is an orally active, dual Src/Abl tyrosine kinase inhibitor for treatment of chronic myeloid leukemia (CML) following resistance/intolerance to prior therapy. Here, we report the data from the 2‐year follow‐up of a phase 1/2 open‐label study evaluating the efficacy and safety of bosutinib as second‐line therapy in 288 patients with chronic phase CML resistant (n = 200) or intolerant (n = 88) to imatinib. The cumulative response rates to bosutinib were as follows: 85% achieved/maintained complete hematologic response, 59% achieved/maintained major cytogenetic response (including 48% with complete cytogenetic response), and 35% achieved major molecular response. Responses were durable, with 2‐year estimates of retaining response >70%. Two‐year probabilities of progression‐free survival and overall survival were 81% and 91%, respectively. The most common toxicities were primarily gastrointestinal adverse events (diarrhea [84%], nausea [45%], vomiting [37%]), which were primarily mild to moderate, typically transient, and first occurred early during treatment. Thrombocytopenia was the most common grade 3/4 hematologic laboratory abnormality (24%). Outcomes were generally similar among imatinib‐resistant and imatinib‐intolerant patients and did not differ with age. The longer‐term results of the present analysis confirm that bosutinib is an effective and tolerable second‐line therapy for patients with imatinib‐resistant or imatinib‐intolerant chronic phase CML. ClinicalTrials.gov Identifier: NCT00261846. Am. J. Hematol. 89:732–742, 2014. © 2014 The Authors American Journal of Hematology Published by Wiley Periodicals, Inc.
Bone marrow 18F‐fluoro‐2‐deoxy‐d‐glucose positron emission tomography/computed tomography cannot replace bone marrow biopsy in diffuse large B‐cell lymphoma
Authors: Adams Hugo J.A. , Kwee Thomas C. , Fijnheer Rob , Dubois Stefan V. , Nievelstein Rutger A.J. , de Klerk John M.H.
Published: April 2014

This study aimed to investigate whether visual and quantitative 18F‐fluoro‐2‐deoxy‐d‐glucose positron emission tomography/computed tomography (FDG‐PET/CT)‐based bone marrow assessment can replace blind bone marrow biopsy (BMB) in newly diagnosed diffuse large B‐cell lymphoma (DLBCL). This retrospective study included 78 patients with newly diagnosed DLBCL who had undergone both FDG‐PET/CT and BMB. FDG‐PET/CT images were visually evaluated for bone marrow involvement. Patient‐based sensitivity of visual FDG‐PET/CT assessment was calculated using BMB as the reference standard. Metabolically active volume, maximum standardized uptake value, 3D partial volume corrected mean standardized uptake value, and 3D partial volume corrected mean metabolic volume product (cMVPmean) of FDG‐avid bone marrow lesions were measured. Cox regression analysis was used to determine the influence of (potential) prognostic factors (BMB status, visual [dichotomous] FDG‐PET/CT bone marrow status, metabolically active volume, maximum standardized uptake value, 3D partial volume corrected mean standardized uptake value, 3D partial volume corrected mean metabolic volume product, and International Prognostic Index score) on progression‐free survival and overall survival. FDG‐PET/CT detected bone marrow involvement in 34 (43.6%) cases and BMB in 16 (20.5%) of 78 cases, of whom 11 were also detected by FDG‐PET/CT, resulting in a patient‐based sensitivity of 68.8% (95% confidence interval = 44.2%–86.1%) for FDG‐PET/CT. In the multivariate Cox proportional hazards model, only BMB status was an independent predictive factor of progression‐free survival (P = 0.016) and overall survival (P = 0.004). In conclusion, FDG‐PET/CT misses bone marrow involvement that has been detected by BMB in a non‐negligible proportion of patients. Furthermore, both visual and quantitative FDG‐PET/CT‐based bone marrow assessments are prognostically inferior to BMB. Therefore, FDG‐PET/CT cannot replace BMB in newly diagnosed DLBCL. Am. J. Hematol. 89:726–731, 2014. © 2014 Wiley Periodicals, Inc.
Phenotypic variability in Waardenburg syndrome resulting from a 22q12.3‐q13.1 microdeletion involving SOX10
Authors: Jelena Brezo , Christina Lam , Eric Vilain , Fabiola Quintero‐Rivera
Published: April 2014

Waardenburg syndrome (WS) is a neurocristopathy characterized by pigmentation abnormalities of the skin, hair, and iris, as well as sensorineural hearing loss. Contiguous gene deletions encompassing SOX10 are rare, which limits conclusions about genotype–phenotype correlation regarding patient prognosis and management. This study adds to the existing body of knowledge by characterizing a 2.4 Mb deletion [arr[hg19] 22q12.3‐q13.1 (36467502–38878207)x1] encompassing SOX10 and 53 additional RefSeq genes in a 15‐year‐old female with atypical WS. The patient presented with developmental delay, profound bilateral sensorineural hearing loss, heterochromia iridis, hypotonia, and bilateral finger contractures. Published genomic and phenotypic profiles of patients with SOX10‐encompassing deletions point toward several plausible candidate gene that could account for the considerable clinical heterogeneity. These studies suggest the existence of modifiers among the co‐deleted, dosage‐sensitive genes (e.g., MYH9) and among genes whose effect may depend on the unmasking of recessive mutations (e.g., PLA2G6). Finally, we highlight evidence illustrating extensive interconnectivity of SOX10‐hypothesizing that haploinsufficiency of SOX10 may “unmask” subtler effects on expression or epistasis associated with variants in SOX10 targets (e.g., DHH), in its partners (e.g., PAX3, EGR2), and in genes with functional overlap (e.g., SOX8, SOX9). © 2014 Wiley Periodicals, Inc.
Expanding the phenotypic profile of boys with 47, XXY: The impact of familial learning disabilities
Authors: Samango‐Sprouse Carole A. , Stapleton Emily J. , Mitchell Francie L. , Sadeghin Teresa , Donahue Thomas P. , Gropman Andrea L.
Published: April 2014

The aim of the study was to examine the impact of familial learning disabilities (FLD) on the phenotypic profile of 47, XXY males and the possibility that 47, XXY males with more severe cognitive deficits may be partially a consequence of familial dyslexia/reading disorder. We wondered if FLD could pose an additional risk for complex neurodevelopmental differences in 47, XXY. The neurodevelopmental profile of males with 47, XXY has been characterized by developmental dyspraxia, language‐based learning disorders, executive dysfunction, reading, and attentional deficits. One hundred eighteen boys with 47, XXY diagnosed prenatally who did not receive early hormonal treatment were divided into two groups based on positive histories of FLD and given comprehensive neurodevelopmental evaluations between 36 and 108 months. The assessments included intelligence (nonverbal and verbal), neuromotor (fine and gross), speech, and language. The group with FLD performed significantly lower in multiple neurodevelopmental domains of the Wechsler of VIQ P = 0.015, FSIQ P = 0.0005, the Brief IQ P = 0.0525 of the Leiter, in Auditory Comprehension P = 0.0505, Expressive Communication P = 0.0055, and neuromotor domains of Manual Coordination P = 0.0032, Fine Motor Control P = 0.0378, and Motor Coordination P = 0.008. Our study demonstrates the influence of FLD on neurodevelopment and expands the phenotypic profile of 47, XXY, suggesting some neurodevelopmental variability is attributable to other factors than the additional X. FLD may increase the vulnerability of the 47, XXY children and anticipatory guidance should be provided to families. © 2014 Wiley Periodicals, Inc.
Axenfeld‐Rieger syndrome: Further clinical and array delineation of four unrelated patients with a 4q25 microdeletion
Authors: Titheradge Hannah , Togneri Fiona , McMullan Dominic , Brueton Louise , Lim Derek , Williams Denise
Published: April 2014

Axenfeld‐Rieger syndrome (ARS) is an autosomal dominant disorder with variable expressivity. It is characterized by dysgenesis of the anterior segment of the eye together with dental, cardiac, and umbilical anomalies. There is a high incidence of secondary high tension glaucoma. It is a genetically heterogeneous condition due to deletion or mutations of FOXC1 (6p25) or PITX2 (4q25). We report on four unrelated patients with overlapping microdeletions encompassing PITX2 at 4q25. We compare the genotypes and phenotypes of these newly described ARS patients and discuss the involvement of contiguous genes. Patients 1, 2, and 3 had mild learning difficulties, not typically seen in patients with ARS. We implicate the adjacent neuronally expressed genes; NEUROG2, UGT8, NDST3, and PRSS12 as potentially causal. Our findings support the use of microarray analysis in ARS patients for full prognostic information in infants presenting with ARS‐like phenotypes. © 2014 Wiley Periodicals, Inc.
Complex mosaic CDKL5 deletion with two distinct mutant alleles in a 4‐year‐old girl
Authors: Boutry‐Kryza Nadia , Ville Dorothée , Labalme Audrey , Calender Alain , Dupont Jean‐Michel , Touraine Renaud , Edery Patrick , des Portes Vincent , Sanlaville Damien , Lesca Gaetan
Published: April 2014

Mutations of the CDKL5 gene cause early epileptic encephalopathy. Patients manifest refractory epilepsy, beginning before the age of 3 months, which is associated with severe psychomotor delay and features that overlap with Rett syndrome. We report here a patient with mosaicism for CDKL5 exonic deletion, with the presence of two mutant alleles. The affected 4‐year‐old girl presented with infantile spasms, beginning at the age of 9 months, but subsequent progression of the disease was consistent with the classical CDKL5‐related phenotype. A deletion of exons 17 and 18 was suspected on the basis of Multiplex Ligation Probe Amplification analysis, but unexpected results for cDNA analysis, which showed the presence of an abnormal transcript with the deletion of exon 18 only, led us to suspect that two distinct events might have occurred. We used custom array‐CGH to determine the size and breakpoints of these deletions. Exon 18 was deleted from one of the abnormal alleles, and exon 17 was deleted from the other. A Fork Stalling and Template Switching (FoSTeS) mechanism was proposed to explain the two events, given the presence of regions of microhomology at the breakpoints. We propose here an original involvement of the FoSTeS mechanism to explain the co‐occurrence of these two events in the CDKL5 gene in a single patient. This patient highlights the difficulties involved in the detection of such abnormalities, particularly when they occur in a mosaic state and involve two distinct mutational events in a single gene. © 2014 Wiley Periodicals, Inc.
Screening children with neurofibromatosis type 1 for autism spectrum disorder
Authors: Tinker Jade , Carbone Paul S. , Viskochil David , Mathiesen Amber , Ma Khe‐Ni , Stevenson David A.
Published: April 2014

Autism spectrum disorder (ASD) is reported to be increased in neurofibromatosis type 1 (NF1), but it's unknown if ASD screening tools are sensitive and specific for NF1. This study compared the rate at which children with NF1 screen‐positive for two ASD screening tools [Modified Checklist for Autism in Toddlers (M‐CHAT) and Childhood Autism Spectrum Test (CAST)] to the screen‐positive rate of the general population. A retrospective cross‐sectional observational design to investigate the association between children with NF1 and at risk status for ASD was used. Medical records of children between 16 months and 11 years of age seen in an NF Clinic were reviewed for an ASD screening questionnaire. There were no statistically significant differences in the screen‐positive rate for ASD in NF1 compared to published controls, but mean CAST scores were higher in NF1. © 2014 Wiley Periodicals, Inc.
Upper airway surgery of obstructive sleep apnea in pycnodysostosis: Case report and literature review
Authors: Testani Elisa , Scarano Emanuele , Leoni Chiara , Dittoni Serena , Losurdo Anna , Colicchio Salvatore , Gnoni Valentina , Vollono Catello , Zampino Giuseppe , Paludetti Gaetano , Marca Giacomo Della
Published: April 2014

Pycnodysostosis is an autosomal recessive disorder due to a mutation in the cathepsin K gene, which causes a decrease of the bone turnover; a review of the literature suggests that pycnodysostosis is frequently associated with severe respiratory obstruction, which needs surgical treatment. The aim of this paper is to describe the surgical treatment of a 3½‐year‐old girl affected by Pycnodysostosis complicated by a severe sleep‐related respiratory disorder. The surgical treatment, consisting of adenotonsillectomy and palatoplasty, resulted in a striking amelioration of respiratory parameters and increased posterior airway space, and allowed the patient to avoid tracheotomy while awaiting for maxillo‐mandibular surgery. © 2014 Wiley Periodicals, Inc.
7p22.3 microdeletion disrupting SNX8 in a patient presenting with intellectual disability but no tetralogy of Fallot
Authors: Rendu John , Satre Véronique , Testard Hervé , Devillard Francoise , Vieville Gaëlle , Fauré Julien , Amblard Florence , Jouk Pierre‐Simon , Coutton Charles
Published: April 2014

© 2014 Wiley Periodicals, Inc.
Hepatomegaly and hyperammonemia in a girl with Silver–Russell syndrome caused by maternal uniparental isodisomy of chromosome 7
Authors: Li Cheng‐Fang , Lin Hsiang‐Yu , Liu Hao‐Chuan , Lee Sheng‐Hung , Lo Ming‐Yu , Lin Shuan‐Pei , Lo Fu‐Sung , Niu Dau‐Ming
Published: April 2014

© 2014 Wiley Periodicals, Inc.
Structures and Reactivities of Iminium Ions Derived from Substituted Cinnamaldehydes and Various Chiral Imidazolidin‐4‐ones
Authors: An Feng , Paul Shyeni , Ammer Johannes , Ofial Armin R. , Mayer Peter , Lakhdar Sami , Mayr Herbert
Published: April 2014

A series of α,β‐unsaturated iminium ions derived from substituted cinnamaldehydes and C2‐ and C5‐substituted chiral imidazolidin‐4‐ones were isolated and characterized in solution and in the solid state. The kinetics of the reactions of the iminium ions with silyl ketene acetals were determined in dichloromethane at 20 °C. The resulting second‐order rate constants were used to estimate the electrophilicity E of the iminium ions according to the linear free energy relationship log k2(20 °C)=sN(N+E). The kinetics for the reactions of two of the iminium ions with tributylphosphine were studied by laser flash spectroscopy and their second‐order rate constants were found to agree within a factor of 2.2 with those calculated by using the linear free energy relationship above.
Geminate Recombination as a Photoprotection Mechanism for Fluorescent Dyes
Authors: Holzmeister Phil , Gietl Andreas , Tinnefeld Philip
Published: April 2014

Despite common presumption due to fast photodestruction pathways through higher excited states, we show that further improvement of photostability is still achievable with diffusion‐limited photoprotection formulas. Single‐molecule fluorescence spectroscopy reveals that thiolate ions effectively quench triplet states of dyes by photoinduced electron transfer. Interestingly, this reaction rarely yields a radical anion of the dye, but direct return to the ground state is promoted by an almost instantaneous back electron transfer (geminate recombination). This type of mechanism is not detected for commonly used reductants such as ascorbic acid and trolox. The mechanism avoids the formation of radical cations and improves the photostability of single fluorophores. We find that a combination of β‐mercaptoethanol and classical reducing and oxidizing systems yields the best results for several dyes including Atto532 and Alexa568.
Hydrocarbation of CC Bonds: Quantification of the Nucleophilic Reactivity of Ynamides
Authors: Laub Hans A. , Evano Gwilherm , Mayr Herbert
Published: April 2014

Donor‐substituted diarylcarbenium ions Ar2CH+ react with ynamides to give 1‐amido‐substituted allyl cations (α,β‐unsaturated iminium ions). Kinetic studies show that these adducts, which correspond to the addition of a CH bond across the CC bond, are formed stepwise with initial formation of keteniminium ions and subsequent 1,3‐hydride shifts. The linear correlations between the second‐order rate constants (lg k2, 20 °C) with the electrophilicity parameters E of the diarylcarbenium ions allow us to include ynamides in our comprehensive nucleophilicity scale and thus predict potential electrophilic reaction partners.