bluuebunny/crossref_metadata_2025_split huggingface dataset:
Thalassemia Major
Authors: Mohamed Said Y. 
Published: June 2017

Thalassemia is the most common monogenic hematologic disease that affects millions in the world and kills thousands of patients every year.
Without transfusion or transplantation, patients with thalassemia major are expected to die within months of diagnosis. However, long-term transfusion and chelation therapy is highly challenging for many developing countries where the disease is prevalent, representing a major and unsustainable health burden.
Stem cell transplantation is the only cure for thalassemia. It has witnessed major developments that have made it less toxic, more successful, and feasible for a larger number of patients with diverse comorbidities and from a wider range of donors. Advances in human leukocyte antigen typing have greatly refined alternate donor selection with results of matched unrelated donors similar to matched sibling donors.
Novel strategies such as haploidentical and cord blood transplantation have increased the possibility of patients with no healthy donor to get a better opportunity to survive and avoid chronic transfusion complications.
Cost-effectively, transplantation should be considered the primary treatment of choice in the presence of a suitable related or unrelated donor and at centers with a satisfactory experience in the field of transplantation and particularly, in managing those with thalassemia. Despite some complications such as graft-versus-host disease and late conditioning effects, the overall improvement in the quality of life of thalassemia is difficult to deny.
Unfortunately, the number of transplants for thalassemia represents only a minority of all transplants conducted globally and the essential requirement for transplants for thalassemia in limited-resources countries should mandate the transplant societies, including Worldwide Network for Blood and Marrow Transplantation, to collaborate to help initiate and support specialized transfusion and transplant programs for managing thalassemia.
A rare case of glial choristoma of the tongue associated with cleft palate
Authors: Yamada Tamaki , Matsuzawa Yusuke , Sogabe Izumi , Donen Masaki , Tei Kanchu , Mikoya Tadashi 
Published: June 2017

AbstractGlial choristoma is considered a developmental malformation characterized by brain heterotopias without connection to the central nervous system. In this article, we report a rare case of glial choristoma occurring on the dorsum of the tongue with cleft palate in a 3‐month‐old male infant. Complete surgical excision was performed prior to palatoplasty. There were no postoperative complications or evidence of recurrence after 5 years of follow‐up.
Variation in Empiric Coverage Versus Detection of Methicillin-ResistantStaphylococcus aureusandPseudomonas aeruginosain Hospitalizations for Community-Onset Pneumonia Across 128 US Veterans Affairs Medical Centers
Authors: Jones Barbara E. , Brown Kevin Antoine , Jones Makoto M. , Huttner Benedikt D. , Greene Tom , Sauer Brian C. , Madaras-Kelly Karl , Rubin Michael A. , Bidwell Goetz Matthew , Samore Matthew H. 
Published: June 2017

OBJECTIVETo examine variation in antibiotic coverage and detection of resistant pathogens in community-onset pneumonia.DESIGNCross-sectional study.SETTINGA total of 128 hospitals in the Veterans Affairs health system.PARTICIPANTSHospitalizations with a principal diagnosis of pneumonia from 2009 through 2010.METHODSWe examined proportions of hospitalizations with empiric antibiotic coverage for methicillin-resistantStaphylococcus aureus(MRSA) andPseudomonas aeruginosa(PAER) and with initial detection in blood or respiratory cultures. We compared lowest- versus highest-decile hospitals, and we estimated adjusted probabilities (AP) for patient- and hospital-level factors predicting coverage and detection using hierarchical regression modeling.RESULTSAmong 38,473 hospitalizations, empiric coverage varied widely across hospitals (MRSA lowest vs highest, 8.2% vs 42.0%; PAER lowest vs highest, 13.9% vs 44.4%). Detection rates also varied (MRSA lowest vs highest, 0.5% vs 3.6%; PAER lowest vs highest, 0.6% vs 3.7%). Whereas coverage was greatest among patients with recent hospitalizations (AP for anti-MRSA, 54%; AP for anti-PAER, 59%) and long-term care (AP for anti-MRSA, 60%; AP for anti-PAER, 66%), detection was greatest in patients with a previous history of a positive culture (AP for MRSA, 7.9%; AP for PAER, 11.9%) and in hospitals with a high prevalence of the organism in pneumonia (AP for MRSA, 3.9%; AP for PAER, 3.2%). Low hospital complexity and rural setting were strong negative predictors of coverage but not of detection.CONCLUSIONSHospitals demonstrated widespread variation in both coverage and detection of MRSA and PAER, but probability of coverage correlated poorly with probability of detection. Factors associated with empiric coverage (eg, healthcare exposure) were different from those associated with detection (eg, microbiology history). Providing microbiology data during empiric antibiotic decision making could better align coverage to risk for resistant pathogens and could promote more judicious use of broad-spectrum antibiotics.Infect Control Hosp Epidemiol2017;38:937–944
Activation of a small ephemeral lake in southern Jordan during the last full glacial period and its paleoclimatic implications
Authors: Catlett Gentry A. , Rech Jason A. , Pigati Jeffrey S. , Al Kuisi Mustafa , Li Shanying , Honke Jeffrey S. 
Published: June 2017

AbstractPlayas, or ephemeral lakes, are one of the most common depositional environments in arid and semiarid lands worldwide. Playa deposits, however, have mostly been avoided as paleoclimatic archives because they typically contain exceptionally low concentrations of organic material, making14C dating difficult. Here, we describe a technique for concentrating organic matter in sediments for radiocarbon dating and apply it to playa sediments recovered from a 2.35 m sediment core from a small playa in southern Jordan. Based on14C ages of the organic concentrate fraction, the playa was active from ~29 to 21 ka, coincident with the last major high stand of Paleolake Lisan and wet conditions recorded by other paleoclimatic proxies in the southernmost Levant during the last full glacial period (35–20 ka). The timing and spatial pattern of these records suggests that the increased moisture was likely derived from more frequent and deeper eastern Mediterranean (EM) cyclones associated with the intensification of the westerlies. The presence of full glacial pluvial deposits in southern Jordan (29°N), and the lack of similarly aged deposits in the northern Arabian Peninsula to the south, suggests that the southerly limit of the incursion of EM cyclones during last full glacial period was ~28°N.
Informing compensatory habitat creation with experimental trials: a 3-year study of a threatened amphibian
Authors: Valdez Jose W. , Klop-Toker Kaya , Stockwell Michelle P. , Fardell Loren , Clulow Simon , Clulow John , Mahony Michael J. 
Published: June 2017

AbstractThe creation or restoration of habitat to mitigate biodiversity loss is a common conservation strategy. Evidence-based research via an extensively monitored trial study should be undertaken prior to large-scale implementation to predict success and identify potential limiting factors. We constructed an experimental trial habitat for the threatened green and golden bell frogLitoria aurea, in Australia, to inform a broader programme of compensatory habitat creation. Individuals were released into the trial plot and a nearby natural wetland for comparison to determine if the created habitat would support their growth, survival and persistence. Half of the trial waterbodies were enclosed within an exclusion fence to separate the effects of habitat suitability from ecological processes. We found the habitat providedL. aureawith sufficient resources to grow, survive and persist for 3 years. However, no breeding occurred, and further investigations need to focus on understanding the drivers of reproduction. Although a disease outbreak occurred during the study, persistence continued for the next 2 years. This was attributed to the large number of individuals released, a strategy we recommend for future mitigation strategies to account for low survival and high turnover rates. Dispersal probably affected abundance in the unfenced areas, and landscape-level initiatives are suggested for this species. This study demonstrates that experimental trials are valuable, as they can inform future habitat management by identifying limitations that could hinder success prior to the implementation of large-scale initiatives.
Normative development of the Child Behavior Checklist Dysregulation Profile from early childhood to adolescence: Associations with personality pathology
Authors: Deutz Marike H. F. , Vossen Helen G. M. , De Haan Amaranta D. , Deković Maja , Van Baar Anneloes L. , Prinzie Peter 
Published: June 2017

AbstractThe Dysregulation Profile (DP) is a broad indicator of concurrent affective, behavioral, and cognitive dysregulation, often measured with the anxious/depressed, aggressive behavior, and attention problems syndrome scales of the Child Behavior Checklist. Despite an expanding body of research on the DP, knowledge of the normative developmental course of the DP from early childhood to adolescence is lacking. Furthermore, although we know that the DP longitudinally predicts personality pathology, no research yet has examined whether next to the DP in early childhood, the rate of change of the DP across development predicts personality pathology. Therefore, using cohort-sequential latent growth modeling in a population-based sample (N = 668), we examined the normative developmental course of mother-reported DP from ages 4 to 17 years and its associations with a wide range of adolescent-reported personality pathology dimensions 3 years later. The results showed that the DP follows a nonlinear developmental course with a peak in early adolescence. The initial level of the DP at age 4 and, to a lesser extent, the rate of change in the DP predicted a range of personality pathology dimensions in late adolescence. The findings suggest that the DP is a broad developmental precursor of personality pathology in late adolescence.
The relationship between community nutritionists’ use of policy, systems and environmental strategies to prevent obesity and its determinants depends on networking
Authors: Lu Angela H , Dickin Katherine L , Constas Mark A , Dollahite Jamie S 
Published: June 2017

AbstractObjectiveTo apply the Theory of Planned Behaviour to examine the relationship between the constructs of background factors and beliefs towards using policy, systems and environmental (PSE) strategies and reported use of PSE strategies to prevent obesity by a group of professional nutrition educators.DesignCross-sectional study using self-reported survey.SettingCooperative Extension in New York, USA.SubjectsNutrition educators (n58); survey response rate 100 %.ResultsNutrition educators’ reported use of PSE strategies to prevent obesity were positively associated with background factors of their community networking and number of staff they managed, their belief of other people’s expectations of them to make PSE changes and the belief that their communities were ready to use PSE strategies; and negatively associated with their belief that individual-level factors contributed to obesity. The relationships among these variables were complicated and their use of PSE strategies occurred only when they utilized their professional networks at a moderately high level (above mean of 5·3 on a scale of 1–7), given that their community was also ready to use PSE strategies.ConclusionsNutrition educators’ use of PSE strategies depends on several internal and external factors. Community networking needs to be emphasized as one of the most significant factors contributing to nutrition educators’ work in this area. Organizational and community support should be in place in order to facilitate nutrition educators’ effective use of PSE strategies.
Estimates of the willingness to pay for locally grown tree fruits in Cusco, Peru
Authors: Blare Trent , Donovan Jason , del Pozo Cesar 
Published: June 2017

AbstractUrbanization, changes in the retail sector and economic growth in developing countries may offer new opportunities to build connections between urban consumers and nearby farmers. The design of strategies to build such connections will require deeper insights into the food preferences of urban consumers. This paper presents a choice experiment of the preference of locally grown apples, avocados and pears vis-à-vis nonlocal equivalents with 300 consumers in a traditional market in Cusco, Peru. Willingness-to-pay estimates are derived from a multinomal logit analysis. We found that consumers who are younger and more educated and those with young children tend to be willing to pay more for locally produced apples, avocados and pears. The paper concludes with a discussion on the implications of the research for advancing efforts to localize food systems in developing countries and opportunities for future research.
Hypoxia-induced mobilization of NHE6 to the plasma membrane triggers endosome hyperacidification and chemoresistance
Authors: Lucien Fabrice , Pelletier Pierre-Paul , Lavoie Roxane R. , Lacroix Jean-Michel , Roy Sébastien , Parent Jean-Luc , Arsenault Dominique , Harper Kelly , Dubois Claire M. 
Published: June 2017

AbstractThe pH-dependent partitioning of chemotherapeutic drugs is a fundamental yet understudied drug distribution mechanism that may underlie the low success rates of current approaches to counter multidrug resistance (MDR). This mechanism is influenced by the hypoxic tumour microenvironment and results in selective trapping of weakly basic drugs into acidified compartments such as the extracellular environment. Here we report that hypoxia not only leads to acidification of the tumour microenvironment but also induces endosome hyperacidification. The acidity of the vesicular lumen, together with the alkaline pH of the cytoplasm, gives rise to a strong intracellular pH gradient that drives intravesicular drug trapping and chemoresistance. Endosome hyperacidification is due to the relocalization of the Na+/H+ exchanger isoform 6 (NHE6) from endosomes to the plasma membrane, an event that involves binding of NHE6 to the activated protein kinase C–receptor for activated C kinase 1 complex. These findings reveal a novel mechanism of hypoxia-induced MDR that involves the aberrant intracellular distribution of NHE6.
Single-molecule analysis of steroid receptor and cofactor action in living cells
Authors: Paakinaho Ville , Presman Diego M. , Ball David A. , Johnson Thomas A. , Schiltz R. Louis , Levitt Peter , Mazza Davide , Morisaki Tatsuya , Karpova Tatiana S. , Hager Gordon L. 
Published: June 2017

AbstractPopulation-based assays have been employed extensively to investigate the interactions of transcription factors (TFs) with chromatin and are often interpreted in terms of static and sequential binding. However, fluorescence microscopy techniques reveal a more dynamic binding behaviour of TFs in live cells. Here we analyse the strengths and limitations of in vivo single-molecule tracking and performed a comprehensive analysis on the intranuclear dwell times of four steroid receptors and a number of known cofactors. While the absolute residence times estimates can depend on imaging acquisition parameters due to sampling bias, our results indicate that only a small proportion of factors are specifically bound to chromatin at any given time. Interestingly, the glucocorticoid receptor and its cofactors affect each other’s dwell times in an asymmetric manner. Overall, our data indicate transient rather than stable TF-cofactors chromatin interactions at response elements at the single-molecule level.
Munc13-1 and Munc18-1 together prevent NSF-dependent de-priming of synaptic vesicles
Authors: He Enqi , Wierda Keimpe , van Westen Rhode , Broeke Jurjen H. , Toonen Ruud F. , Cornelisse L. Niels , Verhage Matthijs 
Published: June 2017

AbstractSynaptic transmission requires a stable pool of release-ready (primed) vesicles. Here we show that two molecules involved in SNARE-complex assembly, Munc13-1 and Munc18-1, together stabilize release-ready vesicles by preventing de-priming. Replacing neuronal Munc18-1 by a non-neuronal isoform Munc18-2 (Munc18-1/2SWAP) supports activity-dependent priming, but primed vesicles fall back into a non-releasable state (de-prime) within seconds. Munc13-1 deficiency produces a similar defect. Inhibitors of N-ethylmaleimide sensitive factor (NSF), N-ethylmaleimide (NEM) or interfering peptides, prevent de-priming in munc18-1/2SWAP or munc13-1 null synapses, but not in CAPS-1/2 null, another priming-deficient mutant. NEM rescues synaptic transmission in munc13-1 null and munc18-1/2SWAP synapses, in acute munc13-1 null slices and even partially in munc13-1/2 double null synapses. Together these data indicate that Munc13-1 and Munc18-1, but not CAPS-1/2, stabilize primed synaptic vesicles by preventing NSF-dependent de-priming.
miR-15a/miR-16 down-regulates BMI1, impacting Ub-H2A mediated DNA repair and breast cancer cell sensitivity to doxorubicin
Authors: Patel Nibedita , Garikapati Koteswara Rao , Pandita Raj K. , Singh Dharmendra Kumar , Pandita Tej K. , Bhadra Utpal , Bhadra Manika Pal 
Published: June 2017

AbstractThe B-lymphoma Moloney murine leukemia virus insertion region-1 protein (BMI1) acts as an oncogene in various cancers, including breast cancer. Recent evidence suggests that BMI1 is rapidly recruited to sites of DNA double strand breaks where it facilitates histone H2A ubiquitination and DNA double strand break repair by homologous recombination. Here we show that miR-15a and miR-16 expressionis decreased during the initial period after DNA damage where it would otherwise down-regulate BMI1, impairing DNA repair. Elevated miR-15a and miR-16 levels down-regulated BMI1 and other polycomb group proteins like RING1A, RING1B, EZH2 and also altered the expression of proteins associated with the BMI1 dependent ubiquitination pathway. Antagonizing the expression of miR-15a and miR-16, enhanced BMI1 protein levels and increased DNA repair. Further, overexpression of miR-15a and miR-16 sensitized breast cancer cells to DNA damage induced by the chemotherapeutic drug doxorubicin. Our results suggest that miR-15a and miR-16 mediate the down-regulation of BMI1, which impedes DNA repair while elevated levels can sensitize breast cancer cells to doxorubicin leading to apoptotic cell death. This data identifies a new target for manipulating DNA damage response that could impact the development of improved therapeutics for breast cancer.
High-throughput format for the phenotyping of fungi on solid substrates
Authors: Cánovas David , Studt Lena , Marcos Ana T. , Strauss Joseph 
Published: June 2017

AbstractFilamentous fungi naturally grow on solid surfaces, yet most genetic and biochemical analyses are still performed in liquid cultures. Here, we report a multiplexing platform using high-throughput photometric continuous reading that allows parallel quantification of hyphal growth and reporter gene expression directly on solid medium, thereby mimicking natural environmental conditions. Using this system, we have quantified fungal growth and expression of secondary metabolite GFP-based reporter genes in saprophytic Aspergillus and phytopathogenic Fusarium species in response to different nutrients, stress conditions and epigenetic modifiers. With this method, we provide not only novel insights into the characteristic of fungal growth but also into the metabolic and time-dependent regulation of secondary metabolite gene expression.
Dielectric Nanorod Scattering and its Influence on Material Interfaces
Authors: Mangalgiri Gauri M. , Manley Phillip , Riedel Wiebke , Schmid Martina 
Published: June 2017

AbstractThis work elaborates on the high scattering which dielectric nanorods exhibit and how it can be exploited to control light propagation across material interfaces. A detailed overview of how dielectric nanorods interact with light through a combination of dipolar scattering and leaky modes is performed via outward power flux calculations. We establish and account for design parameters that best result in light magnification owing to resonant behavior of nanorods. Impact of material parameters on scattering and their dispersion have been calculated to establish that low loss dielectric oxides like ZnO when nanostructured show excellent antenna like resonances which can be used to control light coupling and propagation. Interfacial scattering calculations demonstrate the high forward directivity of nanorods for various dielectric interfaces. A systematic analysis for different configurations of single and periodic nanorods on air dielectric interface emphasizes the light coupling tendencies exhibited by nanorods to and from a dielectric. Spatial characteristics of the localized field enhancement of the nanorod array on an air dielectric interface show focusing attributes of the nanorod array. We give a detailed account to tailor and selectively increase light propagation across an interface with good spectral and spatial control.
Anderson localization in synthetic photonic lattices
Authors: Vatnik Ilya D. , Tikan Alexey , Onishchukov Georgy , Churkin Dmitry V. , Sukhorukov Andrey A. 
Published: June 2017

AbstractSynthetic photonic lattices provide unique capabilities to realize theoretical concepts emerging in different fields of wave physics via the utilization of powerful photonic technologies. Here we observe experimentally Anderson localization for optical pulses in time domain, using a photonic mesh lattice composed of coupled fiber loops. We introduce a random potential through programmed electro-optic pulse phase modulation, and identify the localization features associated with varying degree of disorder. Furthermore, we present a practical approach to control the band-gap width in photonic lattices by varying the coupling between the fiber loops, and reveal that the strongest degree of localization is limited and increases in lattices with wider band-gaps. Importantly, this opens a possibility to enhance or reduce the effect of disorder and associated localization of optical pulses.
Peptides derived from MARCKS block coagulation complex assembly on phosphatidylserine
Authors: Kastelowitz Noah , Tamura Ryo , Onasoga Abimbola , Stalker Timothy J. , White Ormacinda R. , Brown Peter N. , Brodsky Gary L. , Brass Lawrence F. , Branchford Brian R. , Di Paola Jorge , Yin Hang 
Published: June 2017

AbstractBlood coagulation involves activation of platelets and coagulation factors. At the interface of these two processes resides the lipid phosphatidylserine. Activated platelets expose phosphatidylserine on their outer membrane leaflet and activated clotting factors assemble into enzymatically active complexes on the exposed lipid, ultimately leading to the formation of fibrin. Here, we describe how small peptide and peptidomimetic probes derived from the lipid binding domain of the protein myristoylated alanine-rich C-kinase substrate (MARCKS) bind to phosphatidylserine exposed on activated platelets and thereby inhibit fibrin formation. The MARCKS peptides antagonize the binding of factor Xa to phosphatidylserine and inhibit the enzymatic activity of prothrombinase. In whole blood under flow, the MARCKS peptides colocalize with, and inhibit fibrin cross-linking, of adherent platelets. In vivo, we find that the MARCKS peptides circulate to remote injuries and bind to activated platelets in the inner core of developing thrombi.