bluuebunny/crossref_metadata_2025_split huggingface dataset:
Charcot-Marie-Tooth disease in Denmark: a nationwide register-based study of mortality, prevalence and incidence
Authors: Vaeth Signe , Vaeth Michael , Andersen Henning , Christensen Rikke , Jensen Uffe Birk 
Published: November 2017

ObjectivesCharcot-Marie-Tooth disease (CMT) is the most common inherited disorder of the peripheral nervous system, yet no studies have compared the mortality in patients with CMT with that of the general population, and prevalence estimates vary considerably. We performed a nationwide register-based study to investigate the prevalence, incidence and mortality of CMT in Denmark.DesignWe used the Danish National Patient Registry to select all records with primary diagnostic codes for CMT between 1977 and 2012 given at a neurological, neurophysiological, paediatric or clinical genetic clinic. The prevalence was estimated by 31 December 2012, and the incidence rate was calculated based on data from 1988 to 2012. We calculated a standardised mortality ratio (SMR) and an absolute excess mortality rate (AER) stratified according to age categories and disease duration.ResultsA total of 1534 patients (652 women) were identified. The prevalence proportion was 22.5 per 100 000 (95% CI 21.2 to 23.7) and the incidence rate was 0.98 (95% CI 0.93 to 1.04) per 100 000 person-years. The SMR was 1.36 (95% CI 1.21 to 1.53), and the AER was 4.87 per 1000 person-years (95% CI 2.77 to 6.96). We found a significantly higher SMR in cases below 50 years of age, and in cases with disease duration of more than 10 years.ConclusionsWe found a reduced life expectancy among patients diagnosed with CMT. To our knowledge, this is the first study of CMT to use nationwide register-based data, and the first to report an SMR and an AER.
How guiding coalitions promote positive culture change in hospitals: a longitudinal mixed methods interventional study
Authors: Bradley Elizabeth H , Brewster Amanda L , McNatt Zahirah , Linnander Erika L , Cherlin Emily , Fosburgh Heather , Ting Henry H , Curry Leslie A 
Published: November 2017

BackgroundQuality collaboratives are widely endorsed as a potentially effective method for translating and spreading best practices for acute myocardial infarction (AMI) care. Nevertheless, hospital success in improving performance through participation in collaboratives varies markedly. We sought to understand what distinguished hospitals that succeeded in shifting culture and reducing 30-day risk-standardised mortality rate (RSMR) after AMI through their participation in the Leadership Saves Lives (LSL) collaborative.ProceduresWe conducted a longitudinal, mixed methods intervention study of 10 hospitals over a 2-year period; data included surveys of 223 individuals (response rates 83%–94% depending on wave) and 393 in-depth interviews with clinical and management staff most engaged with the LSL intervention in the 10 hospitals. We measured change in culture and RSMR, and key aspects of working related to team membership, turnover, level of participation and approaches to conflict management.Main findingsThe six hospitals that experienced substantial culture change and greater reductions in RSMR demonstrated distinctions in: (1) effective inclusion of staff from different disciplines and levels in the organisational hierarchy in the team guiding improvement efforts (referred to as the ‘guiding coalition’ in each hospital); (2) authentic participation in the work of the guiding coalition; and (3) distinct patterns of managing conflict. Guiding coalition size and turnover were not associated with success (p values>0.05). In the six hospitals that experienced substantial positive culture change, staff indicated that the LSL learnings were already being applied to other improvement efforts.Principal conclusionsHospitals that were most successful in a national quality collaborative to shift hospital culture and reduce RSMR showed distinct patterns in membership diversity, authentic participation and capacity for conflict management.
Is quality important to our patients? The relationship between surgical outcomes and patient satisfaction
Authors: Lobo Prabhu Kristel , Cleghorn Michelle C , Elnahas Ahmad , Tse Alvina , Maeda Azusa , Quereshy Fayez A , Okrainec Allan , Jackson Timothy D 
Published: November 2017

BackgroundWith greater transparency in health system reporting and increased reliance on patient-centred outcomes, patient satisfaction has become a priority in delivering quality care. We sought to explore the relationship between patient satisfaction and short-term outcomes in patients undergoing general surgical procedures.MethodsSatisfaction surveys were distributed to patients following discharge from the general surgery service at an academic hospital between June 2012 and March 2015. Short-term clinical outcomes were obtained from the American College of Surgeons National Surgical Quality Improvement Program database. Patients rated their level of satisfaction on a 5-point Likert scale, and ordered logistic regression model was used to determine predictors of high patient satisfaction.Results757 patient satisfaction surveys were completed. The mean age of patients surveyed was 52.2 years; 60.0% of patients were female. The majority of patients underwent a laparoscopic procedure (85.9%) and were admitted as inpatients following surgery (72%). 91.5% of patients rated satisfaction of 4–5, and 95.0% said they would recommend the service. The odds of overall satisfaction were lower in patients who had complications (OR: 0.52, 95% CI 0.31 to 0.87) and 30-day readmission (OR: 0.35, 95% CI 0.17 to 0.70). Having elective surgery was associated with higher odds of satisfaction (OR: 1.62, 95% CI 1.07 to 2.47).ConclusionsWe found a significant association between patient satisfaction and both 30-day readmission and the occurrence of postoperative surgical complications. Given this association, further study is warranted to evaluate patient satisfaction as a healthcare quality indicator.
Phenotype-genotype correlations in Leigh syndrome: new insights from a multicentre study of 96 patients
Authors: Sofou Kalliopi , de Coo Irenaeus F M , Ostergaard Elsebet , Isohanni Pirjo , Naess Karin , De Meirleir Linda , Tzoulis Charalampos , Uusimaa Johanna , Lönnqvist Tuula , Bindoff Laurence Albert , Tulinius Már , Darin Niklas 
Published: November 2017

BackgroundLeigh syndrome is a phenotypically and genetically heterogeneous mitochondrial disorder. While some genetic defects are associated with well-described phenotypes, phenotype-genotype correlations in Leigh syndrome are not fully explored.ObjectiveWe aimed to identify phenotype-genotype correlations in Leigh syndrome in a large cohort of systematically evaluated patients.MethodsWe studied 96 patients with genetically confirmed Leigh syndrome diagnosed and followed in eight European centres specialising in mitochondrial diseases.ResultsWe found that ataxia, ophthalmoplegia and cardiomyopathy were more prevalent among patients with mitochondrial DNA defects. Patients with mutations in MT-ND and NDUF genes with complex I deficiency shared common phenotypic features, such as early development of central nervous system disease, followed by high occurrence of cardiac and ocular manifestations. The cerebral cortex was affected in patients with NDUF mutations significantly more often than the rest of the cohort. Patients with the m.8993T>G mutation in MT-ATP6 gene had more severe clinical and radiological manifestations and poorer disease outcome compared with patients with the m.8993T>C mutation.ConclusionOur study provides new insights into phenotype-genotype correlations in Leigh syndrome and particularly in patients with complex I deficiency and with defects in the mitochondrial ATP synthase.
Superficial white matter damage in anti-NMDA receptor encephalitis
Authors: Phillips Owen Robert , Joshi Shantanu H , Narr Katherine L , Shattuck David W , Singh Manpreet , Di Paola Margherita , Ploner Christoph J , Prüss Harald , Paul Friedemann , Finke Carsten 
Published: November 2017

BackgroundClinical brain MRI is normal in the majority of patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, extensive deep white matter damage wasrecently identifiedin these patients using diffusion weighted imaging. Here, our aim was to study a particularly vulnerable brain compartment, the late myelinating superficial white matter.MethodsForty-six patients with anti-NMDAR encephalitis were included. Ten out of these were considered neurologically recovered (modified Rankin scale of zero), while 36 patients were non-recovered. In addition, 30 healthy controls were studied. MRI data were collected from all subjects and superficial white matter mean diffusivity derived from diffusion tensor imaging was compared between groups in whole brain, lobar and vertex-based analyses. Patients underwent comprehensive cognitive testing, and correlation analyses were performed between cognitive performance and superficial white matter integrity.ResultsNon-recovered patients showed widespread superficial white matter damage in comparison to recovered patients and healthy controls. Vertex-based analyses revealed that damage predominated in frontal and temporal lobes. In contrast, the superficial white matter was intact in recovered patients. Importantly, persistent cognitive impairments in working memory, verbal memory, visuospatial memory and attention significantly correlated with damage of the superficial white matter in patients.ConclusionsAnti-NMDAR encephalitis is associated with extensive superficial white matter damage in patients with incomplete recovery. The strong association with impairment in several cognitive domains highlights the clinical relevance of white matter damage in this disorder and warrants investigations of the underlying pathophysiological mechanisms.
Content analysis of e-cigarette products, promotions, prices and claims on Internet tobacco vendor websites, 2013–2014
Authors: Williams Rebecca S , Derrick Jason , Liebman Aliza K , LaFleur Kevin 
Published: November 2017

ObjectiveTo identify the population of Internet e-cigarette vendors (IEVs) and conduct content analysis of products sold and IEVs’ promotional, claims and pricing practices.MethodsMultiple sources were used to identify IEV websites, primarily complex search algorithms scanning over 180 million websites. In 2013, 32 446 websites were manually screened, identifying 980 IEVs, with the 281 most popular selected for content analysis. This methodology yielded 31 239 websites for manual screening in 2014, identifying 3096 IEVs, with 283 selected for content analysis.ResultsWhile the majority of IEVs (71.9%) were US based in 2013, this dropped to 64.3% in 2014 (p<0.01), with IEVs located in at least 38 countries, and 12% providing location indicators reflecting two or more countries, complicating jurisdictional determinations.Reflecting the retail market, IEVs are transitioning from offering disposable and ‘cigalike’ e-cigarettes to larger tank and “mod” systems. Flavored e-cigarettes were available from 85.9% of IEVs in 2014, with fruit and candy flavors being most popular. Most vendors (76.5%) made health claims in 2013, dropping to 43.1% in 2014. Some IEVs featured conflicting claims about whether or not e-cigarettes aid in smoking cessation. There was wide variation in pricing, with e-cigarettes available as inexpensive as one dollar, well within the affordable range for adults and teens.ConclusionsThe number of Internet e-cigarette vendors grew threefold from 2013 to 2014, far surpassing the number of Internet cigarette vendors (N=775) at the 2004 height of that industry. New and expanded regulations for online e-cigarette sales are needed, including restrictions on flavors and marketing claims.
MAMMOTh: A new database for curated mathematical models of biomolecular systems
Authors: Kazantsev Fedor , Akberdin Ilya , Lashin Sergey , Ree Natalia , Timonov Vladimir , Ratushny Alexander , Khlebodarova Tamara , Likhoshvai Vitaly 
Published: November 2017

Motivation: Living systems have a complex hierarchical organization that can be viewed as a set of dynamically interacting subsystems. Thus, to simulate the internal nature and dynamics of the entire biological system, we should use the iterative way for a model reconstruction, which is a consistent composition and combination of its elementary subsystems. In accordance with this bottom-up approach, we have developed the MAthematical Models of bioMOlecular sysTems (MAMMOTh) tool that consists of the database containing manually curated MAMMOTh fitted to the experimental data and a software tool that provides their further integration. Results: The MAMMOTh database entries are organized as building blocks in a way that the model parts can be used in different combinations to describe systems with higher organizational level (metabolic pathways and/or transcription regulatory networks). The tool supports export of a single model or their combinations in SBML or Mathematica standards. The database currently contains 110 mathematical sub-models for Escherichia coli elementary subsystems (enzymatic reactions and gene expression regulatory processes) that can be combined in at least 5100 complex/sophisticated models concerning more complex biological processes as de novo nucleotide biosynthesis, aerobic/anaerobic respiration and nitrate/nitrite utilization in E. coli. All models are functionally interconnected and sufficiently complement public model resources. Availability: http://mammoth.biomodelsgroup.ru
Functional Role of a Novel Long Noncoding RNA TTN-AS1 in Esophageal Squamous Cell Carcinoma Progression and Metastasis
Authors: Lin Chenyu , Zhang Shengnan , Wang Ying , Wang Yuanshu , Nice Edouard , Guo Changying , Zhang Erhao , Yu Liting , Li Mengwei , Liu Chen , Hu Lirong , Hao Jingchao , Qi Weiyan , Xu Hanmei 
Published: November 2017

Abstract
Purpose: Emerging studies demonstrate that long noncoding RNAs (lncRNA) participate in the regulation of various cancers. In the current study, a novel lncRNA-TTN-AS1 has been identified and explored in esophageal squamous cell carcinoma (ESCC).
Experimental Design: To discover a new regulatory circuitry in which RNAs crosstalk with each other, the transcriptome of lncRNA-miRNA-mRNA from ESCC and adjacent nonmalignant specimens were analyzed using multiple microarrays and diverse bioinformatics platforms. The functional role and mechanism of a novel lncRNA-TTN-AS1 were further investigated by gain-of-function and loss-of-function assays in vivo and in vitro. An ESCC biomarker panel, consisting of lncRNA-TTN-AS1, miR-133b, and FSCN1, was validated by qRT-PCR and in situ hybridization using samples from 148 patients.
Results: lncRNA-TTN-AS1 as an oncogene is highly expressed in ESCC tissues and cell lines, and promotes ESCC cell proliferation and metastasis. Mechanistically, lncRNA-TTN-AS1 promotes expression of transcription factor Snail1 by competitively binding miR-133b, resulting in the epithelial–mesenchymal transition (EMT) cascade. Moreover, lncRNA-TTN-AS1 also induces FSCN1 expression by sponging miR-133b and upregulation of mRNA-stabilizing protein HuR, which further promotes ESCC invasion cascades. We also discovered and validated a clinically applicable ESCC biomarker panel, consisting of lncRNA-TTN-AS1, miR-133b, and FSCN1, that is significantly associated with overall survival and provides additional prognostic evidence for ESCC patients.
Conclusions: As a novel regulator, lncRNA-TTN-AS1 plays an important role in ESCC cell proliferation and metastasis. The lncRNA-TTN-AS1/miR133b/FSCN1 regulatory axis provides bona fide targets for anti-ESCC therapies. Clin Cancer Res; 24(2); 486–98. ©2017 AACR.
The Battle of Gibeah Reconsidered (Judges 20:29-48)
Authors: Naʼaman Nadav 
Published: November 2017

Abstract
In this article I discuss the episode of the Israelite-Benjaminite battle at Gibeah (Judges 20:29-48). The battle account suffers from many inconsistencies, and to date no satisfactory solution was suggested for its composition. I posit that the battle story has a unified framework (vv. 29-31a and 47-48), and this framework embraces two complete alternative accounts of the battle, each describing a different version of the event (vv. 31b-36a and 36b-46). Following a translation of the two accounts, I analyze each account seeking to show how it describes the related event. I then examine the diachronic and synchronic solutions offered for the way the text has emerged, seeking to clarify how it might have reached its garbled final form.
Synergie zwischen priesterlichem und göttlichem Handeln im Aaronitischen Segen (Num 6,22-27)
Authors: Geiger Michaela 
Published: November 2017

AbstractThe expression (יִשָּׂא יְהוָה פָּנָיו אֵלֶיךָNum 6,26) is unique in the Hebrew Bible and has not been fully explained yet. This article suggests that the preposition (אֶלNum 6,25.26) is the key to understanding, since it links Num 6,24-26 to Lev 9,22-24 (3 timesאֶל) as well as to the framing verses Num 6,22f.27 (3 timesאֶל). Semantical, redaction- and tradition-critical analyses prompt that the expressionיִשָּׂא יְהוָה פָּנָיו אֵלֶיךָwas created especially for the priestly blessing to evoke the imagination of being received before the divine king. The prepositionאֵלֶיךָin Num 6,25.26 emphasizes the relationship between God and the blessed individuals and alludes to the expressionוַיִּשָּׂא אַהֲרֹן אֶת־יָדוֹ אֶל־הָעָם וַיְבָרְכֵםin Lev 9,22: the priest’s gesture of blessing allows to perceive the presence of God. The liturgical formula was finally included into the Sinai pericope by a postpriestly redaction, along with the framing verses Num 6,22f.27.
Transboundary Waters, Infrastructure Development and Public Private Partnership
Authors: Paisley Richard , Denoon Riley , Etmanski Theressa , Weiler Patrick 
Published: November 2017

AbstractGovernments increasingly look to the private sector for the financing, design, construction, and operation of infrastructure projects, and as a result, public-private partnerships (ppps) have emerged as a valuable source for investment funds and expertise.ppps involving new uses of transboundary waters require giving particular attention to the huge potential for environmental and social impacts. This monograph examines whatppps are and how they, and environmental and social ‘safeguards,’ function in a transboundary waters context and with each other. This examination is undertaken through the prism of the Nam Theun 2 and Xayaburi hydroelectric power projects in Laopdr. This monograph discusses and draws some important lessons from theseppps contractual arrangements, costs, financing, and risk mitigation, forppps to be contemplated in other transboundary waters contexts.
The Inner-Core Temperature Structure of Hurricane Edouard (2014): Observations and Ensemble Variability
Authors: Munsell Erin B. , Zhang Fuqing , Braun Scott A. , Sippel Jason A. , Didlake Anthony C. 
Published: November 2017

Abstract
The inner-core thermodynamic structure of Hurricane Edouard (2014) is explored, primarily through an examination of both high-altitude dropsondes deployed during NASA’s Hurricane and Severe Storm Sentinel (HS3) and a 60-member convection-permitting ensemble initialized with an ensemble Kalman filter. The 7-day forecasts are initialized coincident with Edouard’s tropical depression designation and include Edouard’s significant intensification to a major hurricane. Ten-member ensemble groups are created based on timing of near–rapid intensification (RI) onset, and the associated composite inner-core temperature structures are analyzed. It is found that at Edouard’s peak intensity, in both the observations and the simulations, the maximum inner-core perturbation temperature (~10–12 K) occurs in the midlevels (~4–8 km). In addition, in all composite groups that significantly intensify, the evolution of the area-averaged inner-core perturbation temperatures indicate that weak to moderate warming (at most 4 K) begins to occur in the low to midlevels (~2–6 km) ~24–48 h prior to RI, and this warming significantly strengthens and deepens (up to ~8 km) ~24 h after RI has begun. Despite broad similarities in the evolution of Edouard’s warm core in these composites, variability in the height and strength of the maximum perturbation temperature and in the overall development of the inner-core temperature structure are present among the members of the composite groups (despite similar intensity time series). This result and concomitant correlation analyses suggest that the strength and height of the maximum perturbation temperature is not a significant causal factor for RI onset in this ensemble. Fluctuations in inner-core temperature structure occur either in tandem with or after significant intensity changes.
Association between monocyte chemoattractant protein-1 and blood pressure in smokers
Authors: Komiyama Maki , Takanabe Rieko , Ono Koh , Shimada Sayaka , Wada Hiromichi , Yamakage Hajime , Satoh-Asahara Noriko , Morimoto Tatsuya , Shimatsu Akira , Takahashi Yuko , Hasegawa Koji 
Published: November 2017

Objective The expression level of monocyte chemoattractant protein-1 (MCP-1) is increased in atherosclerotic regions, inducing monocyte migration to the blood vessel wall. Although the serum MCP-1 concentration is higher in patients with than without cardiovascular disease, the precise correlations between the serum MCP-1 concentration and factors associated with smoking and atherosclerosis are unknown. Methods The serum MCP-1 concentration was measured using an enzyme-linked immunosorbent assay in 207 consecutive smokers who visited our smoking cessation clinic. Results Sex-adjusted analysis of smokers revealed that the MCP-1 concentration was positively correlated with age ( β = 0.311), smoking duration ( β = 0.342), systolic blood pressure ( β = 0.225), and diastolic blood pressure ( β = 0.137) but not with the body mass index. Multivariate regression analysis showed that smoking duration and systolic blood pressure were independent determinants of the MCP-1 concentration. Conclusions The MCP-1 concentration was positively correlated with blood pressure among smokers. Long-term smokers with high blood pressure may be more susceptible to plaque rupture at atherosclerotic lesion sites.
Elevated Creatine Kinase due to Potential Drug Interaction With Ticagrelor and Atorvastatin
Authors: Beavers Janna C. 
Published: November 2017

Purpose: Ticagrelor and atorvastatin are commonly used medications in the management of acute coronary syndrome and percutaneous intervention. This is a report of a patient case of a potential drug interaction leading to the use of alternative therapy. Case Report: A 58-year-old male presented for cardiac catheterization following an abnormal stress test. He underwent placement of a drug-eluting stent and was started on ticagrelor. Three months later, he was noted to have elevated creatine kinase (CK), thought to be related to a potential drug–drug interaction between ticagrelor and atorvastatin. Ticagrelor was discontinued and he was successfully transitioned to clopidogrel. CK returned to normal within weeks of this change. Discussion: Pharmacokinetic studies have demonstrated a potential interaction between ticagrelor and atorvastatin but have not been deemed clinically significant. To date, only one other case report has been published discussing this interaction and consideration of alternative therapy. This case report is unique, with ticagrelor being the only new medication added prior to the abnormal CK finding. Conclusions: A probable drug–drug interaction occurred with concomitant ticagrelor and atorvastatin. While this interaction may not always be clinically significant, it is reasonable to consider in patients who present with signs and symptoms of adverse effects.