bluuebunny/crossref_metadata_2025_split huggingface dataset:
Inhibitors in Nonsevere Hemophilia A: What Is Known and Searching for the Unknown
Authors: Abdi Amal , Linari Silvia , Pieri Lisa , Voorberg Jan , Fijnvandraat Karin , Castaman Giancarlo 
Published: February 2018

AbstractNonsevere hemophilia A (NSHA) is an inherited X-linked bleeding disorder, caused by mutations of the F8 gene, leading to decreases of clotting factor VIII (FVIII) levels to 1 to 40 IU/dL. Desmopressin is the first therapeutic option for NSHA, but 40 to 50% of patients fail to attain adequate postinfusion FVIII levels. Thus, in these cases, FVIII concentrates remain the mainstay of treatment. The development of neutralizing FVIII antibodies (inhibitors) is a major challenge with replacement therapy. In contrast to severe disease, NSHA patients have a lifelong risk of inhibitor development. Recent data indicate that inhibitors are associated with a deterioration of clinical outcome, illustrated by an increase in bleeding and mortality rate. F8 genotype is an important risk factor for inhibitor occurrence together with surgical interventions and a high dose of FVIII concentrate. Adequate prevention and treatment of inhibitors in NSHA patients is limited by a lack of understanding of the underlying immunological mechanisms. Elucidation of the immunology driving inhibitor development is required to identify high-risk patients, to understand the association between clinical risk factors and inhibitor occurrence, and to provide the opportunity to develop new preventive and therapeutic strategies.
Sjögren-Syndrom bei Kindern und Jugendlichen
Authors: Haas Johannes-Peter 
Published: February 2018

ZusammenfassungDas Sjögren Syndrom ist bei jungen Erwachsenen eine seltene, jedoch oft zu spät erkannte Autoimmunopathie. Es manifestiert sich als primäres juveniles Sjögren Syndrom (jSS) oder im Rahmen anderer Autoimmunerkrankungen als sekundäres jSS. Die Klinik weicht in einigen Aspekten von der des SS beim Adulten ab. Differentialdiagnosen sind andere Formen der Parotitis, die bei Kindern und Jugendlichen häufiger vorkommen. Schwere systemische Verläufe eines jSS und selbst die Entwicklung von B-Zell-Lymphomen werden jedoch auch bei jungen Patienten beobachtet. Daher sollte auch bei Jugendlichen mit entsprechenden Erstsymptomen an ein jSS gedacht und eine entsprechende Diagnostik veranlasst werden. Patienten mit einem jSS bedürfen einer regelmäßigen kinderrheumatologischen Betreuung.
Deutschsprachige Präventionsprogramme für Essstörungen – Ein systematisches Review
Authors: Pickhardt Mara , Adametz Luise , Richter Felicitas , Strauß Bernhard , Berger Uwe 
Published: February 2018

ZusammenfassungIm deutschsprachigen Raum existiert mittlerweile eine Vielzahl primärer und sekundärer Präventionsprogramme für Essstörungen, jeweils entwickelt für unterschiedliche Zielgruppen und Kontexte. Ziel dieser Arbeit war eine systematische Zusammenfassung der Inhalte und Ergebnisse primärer und sekundärer deutschsprachiger Präventionsprogramme sowie die Integration der Studienergebnisse. Dies soll medizinischem Fachpersonal aber auch Psychologen, Lehrkräften und Sozialarbeitern einen umfassenden Überblick bei der Auswahl wirksamer und gut evaluierter Programme bieten. Die systematische Literaturrecherche ergab 22 primär- und sekundärpräventive Präventionsprogramme für Essstörungen, davon waren 11 wissenschaftlich evaluiert. Die Programme sind entweder für Schulen, das Internet oder ambulante Gruppen konzipiert. Die Mehrzahl der Studien weist auf eine Reduktion von Gewichts- und Figursorgen und Schlankheitsstreben sowie auf eine Erhöhung des (Körper-) Selbstwerts in der Gesamtstichprobe oder in der Risikogruppe hin. Eine Reduktion des auffälligen Essverhaltens hingegen scheint nur bei Risikogruppen oder sekundärpräventiven Programmen möglich. Alle Programme verändern mind. eine Zielvariable bedeutsam. Sekundäre Präventionsprogramme erzielten höhere Effektstärken als primärpräventive. Limitationen der Studien und Implikationen für zukünftige Forschung werden diskutiert.
Psychische Komorbidität in der Gastroenterologie und Hepatologie: Prävalenz und psychosozialer Versorgungsbedarf in der Tertiärversorgung
Authors: Niecke Alexander , Lemke Hanna , Goeser Tobias , Hellmich Martin , Vitinius Frank , Albus Christian 
Published: February 2018

Zusammenfassung
Hintergrund Im Gebiet der Gastroenterologie und Hepatologie sind Zusammenhänge und Wechselwirkungen mit komorbiden psychischen Störungen vielfach beschrieben, allerdings fehlen aktuelle und methodisch hochwertige Studien zu Prävalenz und Versorgungsbedarf. Ziel der vorliegenden Studie war es, diesen Mangel zu beheben.
Methodik Prospektive, monozentrische Querschnittsuntersuchung einer repräsentativen Stichprobe stationärer Patienten einer Universitätsklinik. Insgesamt wurden 308 Teilnehmer mit einem validierten Fragebogen (PHQ-D) auf das Vorliegen 8 häufiger psychischer Syndrome untersucht. Zusätzlich wurden 63 Teilnehmer auf das Vorliegen psychischer Störungen gemäß ICD-10 mit einem diagnostischen Interview (SKID-I) untersucht, wobei hier ein größeres Diagnosespektrum erfasst wurde. Der psychosoziale Versorgungsbedarf wurde aus Sicht der internistischen Behandler und aus Sicht der psychosomatischen Experten erhoben.
Ergebnisse Die Prävalenz für mind. 1 psychisches Syndrom in der Fragebogen-Stichprobe (PHQ-D exkl. PHQ-15) beträgt 39,6%, wobei unter diesen bei 23,8% mehr als ein Syndrom vorliegt. Am häufigsten kommen depressive Syndrome und Angstsyndrome vor. Es existieren teilweise deutliche Unterschiede in den verschiedenen Subgruppen (Geschlecht, Art und Schwere der somatischen Erkrankung). Die 4-Wochen-Prävalenz für irgendeine psychische Störung in der Interview-Stichprobe (SKID-I) beträgt 52,4%. Es dominieren affektive und somatoforme Störungen sowie Anpassungstörungen. Psychosozialer Versorgungsbedarf bestand in 23,1% aus Behandlersicht und in 30,2% aus Expertensicht.
Schlussfolgerungen Patienten der gastroenterologisch-hepatologischen Tertiärversorgung zeigen hohe Prävalenzen behandlungswertiger psychischer Syndrome respektive Störungen. Im Vergleich mit der Prävalenz psychischer Störungen in der deutschen Allgemeinbevölkerung ist der Anteil aktuell vorliegender psychischer Störungen fast doppelt so hoch (52,4 vs. 27,1%, p<0,001). Zur Deckung des Versorgungsbedarfs sollten die Einrichtung psychosomatischer Liaison-Dienste sowie die Stärkung der psychosomatischen Grundversorgung in der Gastroenterologie/Hepatologie erfolgen.
Nuclear quantum effect with pure anharmonicity and the anomalous thermal expansion of silicon
Authors: Kim D. S. , Hellman O. , Herriman J. , Smith H. L. , Lin J. Y. Y. , Shulumba N. , Niedziela J. L. , Li C. W. , Abernathy D. L. , Fultz B. 
Published: February 2018

Significance
Silicon has a peculiar negative thermal expansion at low temperature. This behavior has been understood with a “quasiharmonic” theory where low-energy phonons decrease in frequency with volume contraction. We report inelastic neutron scattering measurements of phonon dispersions over a wide range of temperatures. These measurements cast doubt upon quasiharmonic theory, which predicts the wrong sign for most phonon shifts with temperature. Fully anharmonic ab initio calculations correctly predict the phonon shifts and thermal expansion. Crystal structure, anharmonicity, and nuclear quantum effects all play important roles in the thermal expansion of silicon, and a simple mechanical explanation is inappropriate. The quantum effect of nuclear vibrations is also expected to be important for thermophysical properties of many materials.
Bayesian selection of misspecified models is overconfident and may cause spurious posterior probabilities for phylogenetic trees
Authors: Yang Ziheng , Zhu Tianqi 
Published: February 2018

SignificanceThe Bayesian method is widely used to estimate species phylogenies using molecular sequence data. While it has long been noted to produce spuriously high posterior probabilities for trees or clades, the precise reasons for this overconfidence are unknown. Here we characterize the behavior of Bayesian model selection when the compared models are misspecified and demonstrate that when the models are nearly equally wrong, the method exhibits unpleasant polarized behaviors, supporting one model with high confidence while rejecting others. This provides an explanation for the empirical observation of spuriously high posterior probabilities in molecular phylogenetics.
Maximizing the information learned from finite data selects a simple model
Authors: Mattingly Henry H. , Transtrum Mark K. , Abbott Michael C. , Machta Benjamin B. 
Published: February 2018

SignificanceMost physical theories are effective theories, descriptions at the scale visible to our experiments which ignore microscopic details. Seeking general ways to motivate such theories, we find an information theory perspective: If we select the model which can learn as much information as possible from the data, then we are naturally led to a simpler model, by a path independent of concerns about overfitting. This is encoded as a Bayesian prior which is nonzero only on a subspace of the original parameter space. We differ from earlier prior selection work by not considering an infinite quantity of data. Having finite data is always a limit on the resolution of an experiment, and in our framework this selects how complicated a theory is appropriate.
Adaptive evolution of genomically recoded
Escherichia coli
Authors: Wannier Timothy M. , Kunjapur Aditya M. , Rice Daniel P. , McDonald Michael J. , Desai Michael M. , Church George M. 
Published: February 2018

Significance
The construction of an organism with an altered genetic code negatively impacted its fitness. We evolved this organism for ∼1,100 generations in the laboratory to recover fitness and learn what changes would accumulate during evolutionary trajectories toward faster growth rates. We observed several selective mutations that helped alleviate insufficient translation termination or that corrected for unintended mutations that accumulated when we originally altered the genetic code. Further observed mutations were generally adaptive in a nonrecoded background. This work bolsters our understanding of the pliability of the genetic code and will help guide future efforts seeking to recode genomes. Finally, it results in a useful strain for nonstandard amino acid incorporation in numerous contexts relevant for research and industry.
Time-spliced X-ray diffraction imaging of magnetism dynamics in a NdNiO
3
thin film
Authors: Beyerlein Kenneth R. 
Published: February 2018

Significance
Imaging the atomic-scale dynamics of thin films is important to develop the next generation of computer technology. Coherent diffraction imaging can provide this information for other dimensionalities, but is unreliable when applied to thin-film measurements. This paper describes an approach to solving this problem using many measurements on a system that is changing in time. As an example, a demagnetization front is imaged as it sweeps through an antiferromagnetic film at twice the speed of sound, leaving a paramagnetic state in its wake. This fast switching is initiated by a midinfrared pulse tuned to the substrate. The recovered magnetization evolution then shows the potential for control of optoelectronic switching devices by driving interface lattice dynamics.
Transcriptome dynamics at
Arabidopsis
graft junctions reveal an intertissue recognition mechanism that activates vascular regeneration
Authors: Melnyk Charles W. , Gabel Alexander , Hardcastle Thomas J. , Robinson Sarah , Miyashima Shunsuke , Grosse Ivo , Meyerowitz Elliot M. 
Published: February 2018

Significance
Plant grafting is an ancient and agriculturally important technique. Despite its widespread use, little is known about how plants graft. Here, we perform a genome-wide transcriptome analysis of tissues above and below graft junctions. We observed a sequential activation of genes important for vascular development including cambium-, phloem-, and xylem-related genes. Massive changes in gene expression that rapidly differentiate the top of the graft from the bottom occur. These changes disappear as the graft heals and the vasculature reconnects. Many genes below the junction rapidly respond to the presence of attached tissues including genes involved in vascular differentiation and cell division. This intertissue communication process occurs independently of functional vascular connections and acts as a signal to activate vascular regeneration.
Polo-like kinase 4 inhibition produces polyploidy and apoptotic death of lung cancers
Authors: Kawakami Masanori , Mustachio Lisa Maria , Zheng Lin , Chen Yulong , Rodriguez-Canales Jaime , Mino Barbara , Kurie Jonathan M. , Roszik Jason , Villalobos Pamela Andrea , Thu Kelsie L. , Silvester Jennifer , Cescon David W. , Wistuba Ignacio I. , Mak Tak W. , Liu Xi , Dmitrovsky Ethan 
Published: February 2018

Significance
Despite current treatments, lung cancers remain a major public health problem. Innovative ways are needed to treat or prevent these cancers. Centrosomes are critical for fidelity of mitosis. Abnormal centrosome numbers can cause aberrant mitosis and cell death. Polo-like kinase 4 (PLK4) regulates centriole duplication, and its deregulation alters centrosome number and mitosis. The potent PLK4 inhibitor CFI-400945 is reported here to exert marked antineoplastic effects against lung cancers. CDK2 inhibition also deregulates mitosis and was found to cooperate with PLK4 antagonism. CFI-400945 is now undergoing phase I clinical trial testing (NCT01954316). Taken together, targeting PLK4 for inhibition holds promise in lung cancer therapy either as a single agent or when combined with an agent that deregulates mitosis.
Origami-inspired, on-demand deployable and collapsible mechanical metamaterials with tunable stiffness
Authors: Zhai Zirui , Wang Yong , Jiang Hanqing 
Published: February 2018

Significance
Origami has been employed to build deployable mechanical metamaterials through folding and unfolding along the crease lines. These deployable structures are flexible in their deployment direction so that they can be easily collapsed along the same path they are deployed. Here we create an origami-inspired mechanical metamaterial with on-demand deployability and selective collapsibility: autonomous deployability from the collapsed state and selective collapsibility along two different paths, with low stiffness for one path and substantially high stiffness for another path. The created mechanical metamaterial yields load-bearing capability in the deployed direction while still possessing great deployability and collapsibility. The principle in this work can be utilized to design and create versatile origami-inspired mechanical metamaterials that can find many applications.
To be or not to be your son’s father/mother
Authors: Salamh Sami Ben , Maalej Zouheir , Alghbban Mohammed 
Published: February 2018

AbstractThe current article offers a comparative account of the address system of two dialects of Arabic, Najdi and Tunisian Arabic. Capitalizing on the theory of Idealized Cognitive Model, the article defends the view that the two systems display Idealized models, which are central to the system, and non-Idealized models, which are peripheral to it. Najdi Arabic includes Idealized terms such as first names, teknonyms, and kinship terms while non-Idealized models include a battery of terms of address. Tunisian Arabic Idealized models hinge on Si/Lalla + first names, first names, and kinship terms while non-Idealized models make use of endeared first names, kinship terms, and diminished kinship terms. The two systems are shown to differ at the level of types of encounter (including formality, closeness, and deference), availability of address options, socialhorizontality-verticality, and use of metaphor and metonymy.
Farmers Markets and Food‐Borne Illness*
Authors: Bellemare Marc F. , Nguyen Ngoc (Jenny) 
Published: February 2018

AbstractUsing longitudinal administrative data on all U.S. states and the District of Columbia for the years 2004, 2006, and 2008–2013, we study the relationship between farmers markets and food‐borne illness. We find a positive relationship between the number of farmers markets per million individuals and the number per million of reported (i) total outbreaks and cases of food‐borne illness, (ii) outbreaks and cases of norovirus, and (iii) outbreaks of campylobacter in the average state‐year. Our estimates indicate that doubling the number of farmers markets in the average state‐year would be associated with 2.6 additional outbreaks of food‐borne illness, 0.8 additional outbreaks of norovirus, and 0.3 additional outbreaks of campylobacter per million, as well as with 34.5 additional total cases of food‐borne illness, 22.9 additional cases of norovirus, and 1.5 additional cases of campylobacter per million in the same state‐year. Our core results are robust to different specifications as well as to deleting outliers and leverage points.
What Works and Does Not Work in a Self-Management Intervention for People With Chronic Pain? Qualitative Systematic Review and Meta-Synthesis
Authors: Devan Hemakumar , Hale Leigh , Hempel Dagmar , Saipe Barbara , Perry Meredith A 
Published: February 2018

Abstract

Background
Self-management interventions fostering self-efficacy improve the well-being of people with chronic pain.

Purpose
The purpose of this study was to synthesize the enablers (what works) and barriers (what does not) of incorporating self-management strategies for people in everyday life after completion of a pain self-management intervention.

Data Sources
Major electronic databases (MEDLINE, AMED, PsycINFO, Cochrane Library, PubMed, CINAHL, Scopus, and Google Scholar) were searched from inception to July 2016.

Study Selection
Study selection included qualitative and mixed-method studies that explored the perceptions of individuals with chronic pain after completion of a self–management intervention.

Data Extraction
A thematic analysis approach was used to synthesize the review findings, and a Confidence in the Evidence from Reviews of Qualitative Research (CERQual) Approach was used to assess the level of confidence.

Data Synthesis
Thirty-three studies with 512 participants were included. Enablers to self-management included self-discovery—the ability to distinguish self (ie, body, thoughts, and feelings) from pain; feeling empowered by incorporating self-management strategies into practice; and supportive ambience via collaborative relationships with clinicians and support from family and friends. Barriers to self-management included difficulty with sustaining motivation for pain self-management; distress experienced from ongoing pain, anxiety, and depression; and unsupportive relationships with clinicians, family, and friends.

Limitations
This review only included interventions that involved at least 4 self–management skills; thus, informative studies may have been missed. The follow-up period varied from immediately after the intervention to 72 months following the intervention; therefore, it is uncertain which of the key enablers and barriers were most influential long term. Only articles published in the English language were included; studies conducted in low- and middle-income countries could not be located.

Conclusions
The sustained effort to self-manage chronic pain could be exhausting, and motivation could wane over time following intervention. Providing intermittent support in the form of booster sessions and peer support groups may be important. Person-centered care via shared decision making and guided problem solving is essential to facilitating ongoing self-management.
Clusters of health behaviours in Queensland adults are associated with different socio-demographic characteristics
Authors: Hobbs M , Duncan M J , Collins P , Mckenna J , Schoeppe S , Rebar A L , Alley S , Short C , Vandelanotte C 
Published: February 2018

Abstract

Background
The co-occurrence of unhealthy lifestyles, calls for interventions that target multiple health behaviours. This study investigates the clustering of health behaviours and examines demographic differences between each cluster.

Methods
In total, 934 adults from Queensland, Australia completed a cross-sectional survey assessing multiple health behaviours. A two-step hierarchical cluster analysis using multiple iterations identified the optimal number of clusters and the subset of distinguishing health behaviour variables. Univariate analyses of variance and chi-squared tests assessed difference in health behaviours by socio-demographic factors and clusters.

Results
Three clusters were identified: the ‘lower risk’ cluster (n = 436) reported the healthiest profile and met all public health guidelines. The ‘elevated risk’ cluster (n = 105) reported a range of unhealthy behaviours such as excessive alcohol consumption, sitting time, fast-food consumption, smoking, inactivity and a lack of fruit and vegetables. The ‘moderate risk behaviour’ cluster (n = 393) demonstrated some unhealthy behaviours with low physical activity levels and poor dietary outcomes. The ‘elevated risk’ cluster were significantly younger and more socio-economically disadvantaged than both the ‘lower and moderate risk’ clusters.

Discussion
Younger people who live in more deprived areas were largely within the ‘elevated risk’ cluster and represent an important population for MHBC interventions given their wide range of unhealthy behaviours.
Identifying Pain and Effects on Quality of Life from Chronic Wounds Secondary to Lower-Extremity Vascular Disease: An Integrative Review
Authors: Newbern Stacy 
Published: February 2018

GENERAL PURPOSE:
To provide information to enable better assessment and management of patients with lower extremity vascular disease (LEVD) chronic wound pain.

TARGET AUDIENCE:
This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care.

LEARNING OBJECTIVES/OUTCOMES:
After completing this continuing education activity, you will be able to:

ABSTRACT
A lack of wound pain validation and provider understanding about the effect of pain on quality of life are the biggest barriers to pain management for patients with chronic wound pain. There is also a need for a holistic, validated pain assessment tool for these patients. This is an integrative review of the literature intended to identify gaps in pain identification and (re)assessment for patients with chronic wounds related to lower-extremity vascular disease.