bluuebunny/crossref_metadata_2025 huggingface dataset:
The Spectrum of Movement Disorders in Childhood‐onset Lysosomal Storage Diseases
Authors: Ebrahimi‐Fakhari Darius , Hildebrandt Clara , Davis Peter E. , Rodan Lance H. , Anselm Irina , Bodamer Olaf
Published: December 2017

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Background

Movement disorders are a significant clinical problem in lysosomal storage diseases (LSD) and account for substantial morbidity. The spectrum of movement disorders in childhood‐onset LSD, however, remains poorly defined.

Objectives

To define the spectrum of movement disorders in a well‐characterized cohort of children with LSD.

Methods

A retrospective chart review at a single tertiary care center (Boston Children's Hospital). Patients up to the age of 18 years with a clinical, genetic, and/or biochemical diagnosis of an LSD and at least one predefined movement disorder (parkinsonism, dystonia, ataxia, tremor, chorea, myoclonus, ballism, restless leg syndrome) were included.

Results

Ninety‐six patients were identified and 76 patients had a sufficiently document biochemical and/or genetic diagnosis. Of these, 18 patients met inclusion criteria (mean age: 10.3 ± 5.8 [SD] years, range: 3–18 years; 72% male). The most common LSD associated with a movement disorder was Niemann‐Pick disease type C (NPC), followed by several types of neuronal ceroid lipofuscinosis (NCL), and different mucopolysaccharidoses. The most common movement disorder was ataxia followed by rest tremor, dystonia, and myoclonus. The other predefined movement disorders were rare. The majority of patients presented with more than one movement disorder. The movement disorder was slowly progressive in all patients. Brain MRI changes included diffuse cerebral volume loss, white matter abnormalities with thinning of the corpus callosum, and cerebellar atrophy.

Conclusions

Movement disorders develop in a significant number of LSD patients. Ataxia, often in patients with NPC and NCL, is the most common phenotype but significant heterogeneity exists within and between different LSD.
Deleterious variants in DCHS1 are prevalent in sporadic cases of mitral valve prolapse
Authors: Clemenceau Alisson , Bérubé Jean‐Christophe , Bélanger Paméla , Gaudreault Nathalie , Lamontagne Maxime , Toubal Oumhani , Clavel Marie‐Annick , Capoulade Romain , Mathieu Patrick , Pibarot Philippe , Bosse Yohan
Published: December 2017

Background

A recent study identified DCHS1 as a causal gene for mitral valve prolapse. The goal of this study is to investigate the presence and frequency of known and novel variants in this gene in 100 asymptomatic patients with moderate to severe organic mitral regurgitation.

Methods

DNA sequencing assays were developed for two previously identified functional missense variants, namely p.R2330C and p.R2513H, and all 21 exons of DCHS1. Pathogenicity of variants was evaluated in silico.

Results

p.R2330C and p.R2513H were not identified in this cohort. Sequencing all coding regions revealed eight missense variants including six considered deleterious. This includes one novel variant (p.A2464P) and two rare variants (p.R2770Q and p.R2462Q). These variants are predicted to be deleterious with combined annotation‐dependent depletion (CADD) scores greater than 25, which are in the same range as p.R2330C (CADD = 28.0) and p.R2513H (CADD = 24.3). More globally, 24 of 100 cases were carriers of at least one in silico‐predicted deleterious missense variant in DCHS1, suggesting that this single gene may account for a substantial portion of cases.

Conclusion

This study reveals an important contribution of germline variants in DCHS1 in unrelated patients with mitral valve prolapse and supports genetic testing of this gene to screen individuals at risk.
3D printed phantoms mimicking cortical bone for the assessment of ultrashort echo time magnetic resonance imaging
Authors: Rai Robba , Manton David , Jameson Michael G. , Josan Sonal , Barton Michael B. , Holloway Lois C. , Liney Gary P.
Published: December 2017

Purpose

Human cortical bone has a rapid decay, and it can be visualized using ultrashort echo time (UTE) techniques in magnetic resonance imaging (MRI). These sequences operate at the limits of gradient and transmit‐receive signal performance. Development of multicompartment anthropomorphic phantoms that can mimic human cortical bone can assist with quality assurance and optimization of UTE sequences.

The aims of this study were to (a) characterize the MRI signal properties of a photopolymer resin that can be 3D printed, (b) develop multicompartment phantoms based on the resin, and (c) demonstrate the feasibility of using these phantoms to mimic human anatomy in the assessment of UTE sequences.

Methods

A photopolymer resin (Prismlab China Ltd, Shanghai, China) was imaged on a 3 Tesla MRI system (Siemens Skyra) to characterize its MRI properties with emphasis on signal and longevity. Two anthropomorphic phantoms, using the 3D printed resin to simulate skeletal anatomy, were developed and imaged using UTE sequences. A skull phantom was developed and used to assess the feasibility of using the resin to develop a complex model with realistic morphological human characteristics. A tibia model was also developed to assess the suitability of the resin at mimicking a simple multicompartment anatomical model and imaged using a three‐dimensional UTE sequence (PETRA). Image quality measurements of signal‐to‐noise ratio (SNR) and contrast factor were calculated and these were compared to in vivo values.

Results

The and T1 (mean ± standard deviation) of the photopolymer resin was found to be 411 ± 19 μs and 74.39 ± 13.88 ms, respectively, and demonstrated no statistically significant change during 4 months of monitoring. The resin had a similar decay to human cortical bone; however, had lower T1 properties. The bone water concentration of the resin was 59% relative to an external water reference phantom, and this was higher than in vivo values reported for human cortical bone. The multicompartment anthropomorphic head phantom was successfully produced and able to simulate realistic air cavities, bony anatomy, and soft tissue. Image quality assessment in the tibia phantom using the PETRA sequence showed the suitability of the resin to mimic human anatomy with high SNR and contrast making it suitable for tissue segmentation.

Conclusions

A solid resin material, which can be 3D printed, has been found to have similar magnetic resonance signal properties to human cortical bone. Phantoms replicating skeletal anatomy were successfully produced using this resin and demonstrated their use for image quality and segmentation assessment of ultrashort echo time sequences.
Multiple Autologous Bone Marrow-Derived CD271+ Mesenchymal Stem Cell Transplantation Overcomes Drug-Resistant Epilepsy in Children
Authors: Milczarek Olga , Jarocha Danuta , Starowicz–Filip Anna , Kwiatkowski Stanislaw , Badyra Bogna , Majka Marcin
Published: December 2017

There is a need among patients suffering from drug-resistant epilepsy (DRE) for more efficient and less toxic treatments. The objective of the present study was to assess the safety, feasibility, and potential efficacy of autologous bone marrow cell transplantation in pediatric patients with DRE. Two females and two males (11 months to 6 years) were enrolled and underwent a combined therapy consisting of autologous bone marrow nucleated cells (BMNCs) transplantation (intrathecal: 0.5 × 109; intravenous: 0.38 × 109–1.72 × 109) followed by four rounds of intrathecal bone marrow mesenchymal stem cells (BMMSCs) transplantation (18.5 × 106–40 × 106) every 3 months. The BMMSCs used were a unique population derived from CD271-positive cells. The neurological evaluation included magnetic resonance imaging, electroencephalography (EEG), and cognitive development assessment. The characteristics of BMMSCs were evaluated. Four intravenous and 20 intrathecal transplantations into the cerebrospinal fluid were performed. There were no adverse events, and the therapy was safe and feasible over 2 years of follow-up. The therapy resulted in neurological and cognitive improvement in all patients, including a reduction in the number of epileptic seizures (from 10 per day to 1 per week) and an absence of status epilepticus episodes (from 4 per week to 0 per week). The number of discharges on the EEG evaluation was decreased, and cognitive improvement was noted with respect to reactions to light and sound, emotions, and motor function. An analysis of the BMMSCs' characteristics revealed the expression of neurotrophic, proangiogenic, and tissue remodeling factors, and the immunomodulatory potential. Our results demonstrate the safety and feasibility of BMNCs and BMMSCs transplantations and the considerable neurological and cognitive improvement in children with DRE.
Concise Review: The Cellular Conspiracy of Amyotrophic Lateral Sclerosis
Authors: Serio Andrea , Patani Rickie
Published: December 2017

Amyotrophic lateral sclerosis (ALS) is incurable and devastating. A dearth of therapies has galvanized experimental focus onto the cellular and molecular mechanisms that both initiate and subsequently drive motor neuron degeneration. A traditional view of ALS pathogenesis posits that disease-specific injury to a subtype of neurons is mechanistically cell-autonomous. This “neuron-centric” view has biased past research efforts. However, a wealth of accumulating evidence now strongly implicates non-neuronal cells as being major determinants of ALS. Although animal models have proven invaluable in basic neuroscience research, a growing number of studies confirm fundamental interspecies differences between popular model organisms and the human condition. This may in part explain the failure of therapeutic translation from rodent preclinical models. It follows that integration of a human experimental model using patient-specific induced pluripotent stem cells may be necessary to capture the complexity of human neurodegeneration with fidelity. Integration of enriched human neuronal and glial experimental platforms into the existing repertoire of preclinical models might prove transformational for clinical trial outcomes in ALS. Such reductionist and integrated cross-modal approaches allow systematic elucidation of cell-autonomous and non-cell-autonomous mechanisms of disease, which may then provide novel cellular targets for therapeutic intervention.
The Worried Well? Characteristics of Cognitively Normal Patients Presenting to a Rural and Remote Memory Clinic
Authors: Verity Ryan , Kirk Andrew , O’Connell Megan E. , Karunanayake Chandima , Morgan Debra G.
Published: December 2017

Introduction: In an effort to better understand why cognitively normal patients were referred to a memory clinic, we sought to identify features of “worried well” patients to better identify those more likely to be cognitively normal. Methods: In total, 375 consecutive patients referred by primary care practitioners to a Rural and Remote Memory Clinic were categorized into two groups based on their neurologic diagnosis, “worried well” (cognitively normal, N=81) or “other” (patients with any neurologic diagnosis, N=294). Data collected included: age, sex, years of formal education, Mini-Mental Status Examination score from initial visit, Center for Epidemiologic Studies Depression Scale score, Self-Rating of Memory Scale, alcohol consumption, marital status, hours per week of work, past medical history, sleep concerns, and family history of memory concerns. The two groups were compared using t-tests and χ2 tests. The same comparison was done between the same set of “worried well” patients (cognitively normal, N=81) and the subgroup of patients with a diagnosis of Alzheimer’s disease (N=146) from the “other” group. Results: Significant differences included younger age, more formal education, more frequently having previous psychiatric diagnosis and more self-reported alcohol consumption in the “worried well” group. The “worried well” and “Alzheimer’s disease” comparison had the same significant differences as the “worried well” and “other” comparison. Conclusion: We observed a pattern of differences unfold between the “worried well” patients and those with cognitive disease. No one variable was pathognomonic of a “worried well” patient. However, taking all the above into account when evaluating a patient may help clinically.
HIGHER RANDOMNESS AND GENERICITY
Authors: GREENBERG NOAM , MONIN BENOIT
Published: December 2017

We use concepts of continuous higher randomness, developed in Bienvenuet al.[‘Continuous higher randomness’, J. Math. Log. 17(1) (2017).], to investigate$\unicode[STIX]{x1D6F1}_{1}^{1}$-randomness. We discuss lowness for$\unicode[STIX]{x1D6F1}_{1}^{1}$-randomness, cupping with$\unicode[STIX]{x1D6F1}_{1}^{1}$-random sequences, and an analogue of the Hirschfeldt–Miller characterization of weak 2-randomness. We also consider analogous questions for Cohen forcing, concentrating on the class of$\unicode[STIX]{x1D6F4}_{1}^{1}$-generic reals.
Relative influence of dietary protein and energy contents on lysine requirements and voluntary feed intake of rainbow trout fry
Authors: Van Larebeke Mélusine , Dockx Guillaume , Larondelle Yvan , Rollin Xavier
Published: December 2017

The effect of dietary digestible protein (DP) and/or digestible energy (DE) levels on lysine (Lys) requirements, Lys utilisation efficiency and voluntary feed intake (VFI) were studied in rainbow trout fry when Lys was the first limiting indispensable amino acid or in excess in the diet. Two trials were conducted at 11·6°C with eighty-one experimental diets, containing 280 g DP/kg DM (low protein (LP), trial 1), 600 g DP/kg DM (high protein (HP), trial 1) or 440 g DP/kg DM (medium protein (MP), trial 2), 17 MJ DE/kg (low energy (LE)), 19·5 MJ DE/kg (medium energy (ME)) or 22 MJ DE/kg (high energy (HE)), and nine Lys levels from deeply deficient to large excess (2·3–36 g/kg DM). Each diet was given to apparent satiety to one group of fifty fry (initial body weight 0·85 g) for 24 (MP diets, trial 2) or 30 (LP and HP diets, trial 1) feeding days. Based on N gain data fitted with the broken-line model, the relative Lys requirement was significantly different with the dietary DP level, from 13·3–15·7 to 22·9–26·5 g/kg DM for LP and HP diets, respectively, but did not significantly change with the DE level for a same protein level. The Lys utilisation efficiency for protein growth above maintenance was constant across diets, suggesting no effect of either dietary DE or DP levels. In Lys excess, the VFI was markedly decreased by the DP level but not by the extra DE supply. Our results suggest that the relative Lys need is best expressed in terms of percentage of protein content for optimum fish feed formulation, at least in rainbow trout fry.
Emerson and the “Pale Scholar”
Authors: HOSSEINI REZA
Published: December 2017

A recurrent theme in Ralph Waldo Emerson’s writings is his struggles with the problem of scholarly inaction. Commentators have given much attention to “The American Scholar” but less to his remarks about the “pale scholar.” In this paper, I focus on the latter and argue that understanding the evolving nature of Emerson’s views about what counts for action could not only deepen our understanding of his philosophy and its orientation toward the conduct of life but also explain why, according to Emerson, there seems to be no reconciliation between “the theory and practice of life.”
What Should Be Done? Pragmatic Constructivist Ethics and the Responsibility to Protect
Authors: Ralph Jason
Published: December 2017

In this paper I examine what constructivist approaches to IR tell us about how states should act when confronted by atrocity crimes in the context of a politically pluralist international society. Building on the work of theorists who responded to Richard Price and Christian Reus-Smit's call to substantiate the constructivist's claim to explain “moral progress,” and to better inform normative assessments, I claim that the constructivist emphasis on historical and social contingency does not rule out ethical standpoints, suggesting instead a “pragmatic” ethic. Norms are hypotheses rather than absolute values. The task of the pragmatic constructivist is not to establish beyond doubt the normativity of a norm—the task is to test the norm for how well its “meaning-in-use” supports action that ameliorates lived social problems. Pragmatic constructivists can commit to the Responsibility to Protect (R2P) as an idea that might reconcile various communities of normative practice and ameliorate vulnerability without upsetting international order. To the extent particular practices (e.g., prevention) have proven (and continue to prove) useful in doing this, pragmatic constructivists can equate the institutionalization of those practices with normative progress. However, R2P is a “complex” norm. It recognizes that knowledge of how to respond to ongoing atrocity is context specific and cannot therefore be fixed. The task of the pragmatic constructivist here is to assess the practical judgment of those that claim to speak for the norm by weighing the consequences of acting out their prescription in the specific context of a particular crisis. I test the meanings of R2P in use during the Syria crisis from 2011 to 2012. R2P was problematic to the extent certain meanings reinforced policy ends (e.g., protection through political/criminal accountability) that were impractical and pursued at the expense of non-ideal but realizable goals (e.g., protection through peace/aid).
An overview onLeishmania(Mundinia)enriettii: biology, immunopathology, LRV and extracellular vesicles during the host–parasite interaction
Authors: Paranaiba Larissa F. , Pinheiro Lucélia J. , Macedo Diego H. , Menezes-Neto Armando , Torrecilhas Ana C. , Tafuri Wagner L. , Soares Rodrigo P.
Published: December 2017

One of theLeishmaniaspecies known to be non-infective to humans isLeishmania(Mundinia)enriettiiwhose vertebrate host is the guinea pigCavia porcellus.It is a good model for cutaneous leishmaniasis, chemotherapeutic and molecular studies. In the last years, an increased interest has emerged concerning theL. (Mundinia) subgenus after the finding ofLeishmania(M.)macropodumin Australia and with the description of other new/putative species such asL. (M.) martiniquensisand ‘L.(M.)siamensis’. This review focused on histopathology, glycoconjugates and innate immunity. The presence ofLeishmaniaRNA virus and shedding of extracellular vesicles by the parasite were also evaluated.
Reduced cardiotoxicity and increased oral efficacy of artemether polymeric nanocapsules in Plasmodium berghei-infected mice
Authors: Souza Ana Carolina Moreira , Mosqueira Vanessa Carla Furtado , Silveira Ana Paula Amariz , Antunes Lidiane Rodrigues , Richard Sylvain , Guimarães Homero Nogueira , Grabe-Guimarães Andrea
Published: December 2017

Artemether (ATM) cardiotoxicity, its short half-life and low oral bioavailability are the major limiting factors for its use to treat malaria. The purposes of this work were to study free-ATM and ATM-loaded poly-ε-caprolactone nanocapules (ATM-NC) cardiotoxicity and oral efficacy on Plasmodium berghei-infected mice. ATM-NC was obtained by interfacial polymer deposition and ATM was associated with polymeric NC oily core. For cardiotoxicity evaluation, male black C57BL6 uninfected or P. berghei-infected mice received, by oral route twice daily/4 days, vehicle (sorbitol/carboxymethylcellulose), blank-NC, free-ATM or ATM-NC at doses 40, 80 or 120 mg kg−1. Electrocardiogram (ECG) lead II signal was obtained before and after treatment. For ATM efficacy evaluation, female P. berghei-infected mice were treated the same way. ATM-NC improved antimalarial in vivo efficacy and reduced mice mortality. Free-ATM induced significantly QT and QTc intervals prolongation. ATM-NC (120 mg kg−1) given to uninfected mice reduced QT and QTc intervals prolongation 34 and 30%, respectively, compared with free-ATM. ATM-NC given to infected mice also reduced QT and QTc intervals prolongation, 28 and 27%, respectively. For the first time, the study showed a nanocarrier reducing cardiotoxicity of ATM given by oral route and it was more effective against P. berghei than free-ATM as monotherapy.
English in paradise: the Maldives
Authors: Meierkord Christiane
Published: December 2017

The spread of English has reached almost every corner of the world, and the Republic of the Maldives is no exception. Following the nation's opening to the outside world, the introduction of English as a medium of instruction at secondary and tertiary level of education, and its government's recognition of the opportunities offered through tourism, English has now firmly established itself in the country. As such, Maldives is quite similar to the countries in the Gulf region (cf. Randall & Samimi, 2010; Boyle, 2012). The nation is undergoing vast societal change, and English is part of this.
Children's comprehension of contrastive connectives
Authors: SPENADER Jennifer
Published: December 2017

Production studies show connective acquisition by age 3;0, but comprehension studies show errors until 9;0 or older. To further investigate this gap, two comprehension tasks were carried out with 78 Dutch children between the ages of 7;0 and 10;1, testing contrastive maar ‘but’ and causal want ‘because’ connectives for comparison. An existing context choice task and a task that tested children's ability to interpret pronouns dependent on the connective were used. Children did well on the context choice task for want ‘because’, but performed far below chance with maar ‘but’. In the pronoun interpretation task the youngest children performed at or near chance with both connectives, but show gradual improvement with age. Task complexity may partially explain the results but in general, they are consistent with previous experiments showing contrastive connective comprehension is acquired very late compared to their correct production. Further, the pronoun interpretation task may give a more accurate picture of understanding.
Law, Custom, and Social Norms: Civil Adjudications in Qing and Republican China
Authors: Xu Xiaoqun
Published: December 2017

This study examines how law, custom, and social norm interacted in civil justice in Qing and Republican China by looking into 152 civil cases tried in 1912, right after the founding of the Republic of China, and a body of legal interpretations from the Supreme Court during 1912-1929, and certain provisions in the Civil Code of 1929-30--the very first one in Chinese history. It shows that both law and custom were invoked by judges within their moral universe or social norm. It traces how the Supreme Court allowed local customs to be a legal ground for rulings in certain civil disputes, and which customs in civil matters in the Qing and the early Republic were, and which were not, “hardened” into the Civil Code. The interplay between law and custom, mediated by judges with their normative sense of right and wrong, constituted both continuity and change in civil justice between the Qing era and the Republican period. Ultimately, the issues addressed here speak to a larger question of how Chinese jurists, within their judicial discretions, tried to strike a difficult but necessary balance between “law-on-books” and “law-in-action,” while law on the books was undergoing important revisions.
Territorial Disputes and Their Resolution in the Recent Jurisprudence of the International Court of Justice
Authors: THIRLWAY HUGH
Published: December 2017

The workload of the International Court of Justice in recent years has increasingly featured cases of disputes classified either as ‘territorial disputes’ or as ‘boundary disputes’, or otherwise involving the Court in considerations of the law relating to acquisition or transmission of territory, or to the creation, location and effect of territorial frontiers. The present survey analyzes the contributions to international law of the Court's decisions in these recent cases. Matters examined include the significance of the terms ‘boundary dispute’ or ‘territorial dispute’; the definition of what constitutes sovereign territory; titles andeffectivitésas bases for territorial claims; decolonization and theuti possidetis juris; use of natural features or of straight lines as boundaries; and relations across a frontier once established.
The evaluation of arterial stiffness of essential hypertension and white coat hypertension in children: a case-control study
Authors: Tokgöz Semiha Terlemez , Yılmaz Dilek , Tokgöz Yavuz , Çelik Bülent , Bulut Yasin
Published: December 2017

Background

The aim of this study was to determine and compare cardiovascular risks by assessing arterial stiffness in children with essential hypertension and white coat hypertension.

Methods

Paediatric patients followed up with essential hypertension and white coat hypertension diagnoses and with no established end organ damage were involved in the study. Arterial stiffness in children included in the study was evaluated and compared by using the oscillometric device (Mobil-O-Graph) method.

Results

A total of 62 essential hypertension (34 male, 28 female), 38 white coat hypertension (21 male, 17 female), and 60 healthy controls (33 male, 27 female) were assessed in the present study. Pulse wave velocity of the essential hypertension, white coat hypertension, and control group was, respectively, as follows: 5.3±0.6 (m/s), 5.1±0.4 (m/s), 4.3±0.4 (m/s) (p<0.001); augmentation index outcomes were, respectively, determined as follows: 21.3±6.5, 19.3±6.4, 16.0±0.3 (p<0.001). Pulse wave velocity and augmentation index values of children with essential hypertension and white coat hypertension were found to be higher compared with the control group. This level was identified as correlated with the duration of hypertension in both patient groups (p<0.01).

Conclusion

Arterial stiffness in children with essential hypertension and white coat hypertension was impaired compared with healthy children. This finding has made us think that white coat hypertension is not an innocent clinical situation. This information should be taken into consideration in the follow-up and treatment approaches of the patients.
The Influence of Exposure to Natural Disasters on Depression and PTSD Symptoms among Firefighters
Authors: Pennington Michelle L. , Carpenter Thomas P. , Synett Samantha J. , Torres Victoria A. , Teague Jennifer , Morissette Sandra B. , Knight Jeffrey , Kamholz Barbara W. , Keane Terence M. , Zimering Rose T. , Gulliver Suzy B.
Published: December 2017

Introduction

Firefighters represent an important population for understanding the consequences of exposure to potentially traumatic stressors.

Hypothesis/Problem

The researchers were interested in the effects of pre-employment disaster exposure on firefighter recruits’ depression and posttraumatic stress disorder (PTSD) symptoms during the first three years of fire service and hypothesized that: (1) disaster-exposed firefighters would have greater depression and PTSD symptoms than non-exposed overall; and (2) depression and PTSD symptoms would worsen over years in fire service in exposed firefighters, but not in their unexposed counterparts.

Methods

In a baseline interview, 35 male firefighter recruits from seven US cities reported lifetime exposure to natural disaster. These disaster-exposed male firefighter recruits were matched on age, city, and education with non-exposed recruits.

Results

A generalized linear mixed model revealed a significant exposure×time interaction (ecoef =1.04; P<.001), such that depression symptoms increased with time for those with pre-employment disaster exposure only. This pattern persisted after controlling for social support from colleagues (ecoefficient=1.05; P<.001), social support from families (ecoefficient=1.04; P=.001), and on-the-job trauma exposure (coefficient=0.06; ecoefficient=1.11; P<.001). Posttraumatic stress disorder symptoms did not vary significantly between exposure groups at baseline (P=.61).

Conclusion

Depression symptoms increased with time for those with pre-employment disaster exposure only, even after controlling for social support. Posttraumatic stress disorder symptoms did not vary between exposure groups.

PenningtonML, CarpenterTP, SynettSJ, TorresVA, TeagueJ, MorissetteSB, KnightJ, KamholzBW, KeaneTM, ZimeringRT, GulliverSB. The influence of exposure to natural disasters on depression and PTSD symptoms among firefighters. Prehosp Disaster Med. 2018;33(1):102–108.
Hospital-Confirmed Acute Myocardial Infarction: Prehospital Identification Using the Medical Priority Dispatch System
Authors: Clawson Jeff J. , Gardett Isabel , Scott Greg , Fivaz Conrad , Barron Tracey , Broadbent Meghan , Olola Christopher
Published: December 2017

Introduction

Early recognition of an acute myocardial infarction (AMI) can increase the patient’s likelihood of survival. As the first point of contact for patients accessing medical care through emergency services, emergency medical dispatchers (EMDs) represent the earliest potential identification point for AMIs. The objective of the study was to determine how AMI cases were coded and prioritized at the dispatch point, and also to describe the distribution of these cases by patient age and gender.

Hypothesis/Problem

No studies currently exist that describe the EMD’s ability to correctly triage AMIs into Advanced Life Support (ALS) response tiers.

Methods

The retrospective descriptive study utilized data from three sources: emergency medical dispatch, Emergency Medical Services (EMS), and emergency departments (EDs)/hospitals. The primary outcome measure was the distributions of AMI cases, as categorized by Chief Complaint Protocol, dispatch priority code and level, and patient age and gender. The EMS and ED/hospital data came from the Utah Department of Health (UDoH), Salt Lake City, Utah. Dispatch data came from two emergency communication centers covering the entirety of Salt Lake City and Salt Lake County, Utah.

Results

Overall, 89.9% of all the AMIs (n=606) were coded in one of the three highest dispatch priority levels, all of which call for ALS response (called CHARLIE, DELTA, and ECHO in the studied system). The percentage of AMIs significantly increased for patients aged 35 years and older, and varied significantly by gender, dispatch level, and chief complaint. A total of 85.7% of all deaths occurred among patients aged 55 years and older, and 88.9% of the deaths were handled in the ALS-recommended priority levels.

Conclusion

Acute myocardial infarctions may present as a variety of clinical symptoms, and the study findings demonstrated that more than one-half were identified as having chief complaints of Chest Pain or Breathing Problems at the dispatch point, followed by Sick Person and Unconscious/Fainting. The 35-year age cutoff for assignment to higher priority levels is strongly supported. The Falls and Sick Person Protocols offer opportunities to capture atypical AMI presentations.

ClawsonJJ, GardettI, ScottG, FivazC, BarronT, BroadbentM, OlolaC. Hospital-confirmed acute myocardial infarction: prehospital identification using the Medical Priority Dispatch System. Prehosp Disaster Med. 2018;33(1):29–35.
Independent, Dependent and Interdependent Variables in Language Decay and Language Death
Authors: Dressler Wolfgang U.
Published: December 2017

This contribution gives in its first part an overview on factors of the decay and death of whole languages, focusing on dependency relations between these factors. They are organised along the following dimensions: socio-political, socio-economic, sociocultural, socio-psychological, and linguistic dimensions. The order of these dimensions partially represents a causal chain from left to right, but with many feedback relations. The second part of this article deals with early (socio-)linguistic indicators of language decay and discusses in this respect massive and asymmetric borrowing from the dominant into the recessive language, the loss of productivity of word formation patterns in the latter (illustrated from Breton), changes in name-giving (doubtful), shift of ‘foreign accent’ from the dominant to the recessive language, borrowing of morphological and syntactic patterns (inconclusive).
On some endangered Sinitic languages spoken in Northwestern China
Authors: Peyraube Alain
Published: December 2017

This paper will examine one of the most characteristic syntactic properties of languages, namely the case system for the following three Sinitic languages spoken in Northwestern China: Línxià (or Hézhōu), Tāngwāng, Gāngōu, which have been sometimes viewed as ‘mixed languages’. An answer to the following main questions will be tentatively suggested in the conclusion: do we really have case suffixes in these languages (cases are a morphological notion) or simply thematic roles expressed by postpositions (thematic roles are a semantic notion)? Do we really have a Qinghai-Gansu linguistic area (Sprachbund), as has been suggested? Can these Sinitic languages be characterized as being mixed languages?
Changes in job strain and subsequent weight gain: a longitudinal study, based on the Danish Nurse Cohort
Authors: Vesterlund Gitte Kingo , Keller Amélie Cléo , Heitmann Berit Lilienthal
Published: December 2017

Objective

Obesity as well as job strain is increasing, and job strain might contribute to weight gain. The objective of the current study was to examine associations between longitudinal alterations in the components of job strain and subsequent weight gain.

Design

The study was designed as a prospective cohort study with three questionnaire surveys enabling measurement of job-strain alterations over 6 years and subsequent measurements of weight gain after further 10 years of follow-up. ANCOVA and trend analyses were conducted. Job demands were measured as job busyness and speed, and control as amount of influence.

Setting

Employed nurses in Denmark.

Subjects

We included a sub-sample of 6188 female nurses from the Danish Nurse Cohort, which consisted of the nurses who participated in surveys in 1993, 1999 and 2009.

Results

A linear trend in weight gain was seen in nurses who were often busy in 1999 between those who were rarely v. sometimes v. often busy in 1993 (P=0·03), with the largest weight gain in individuals with sustained high busyness in both years. Loss of influence between 1993 and 1999 was associated with larger subsequent weight gain than sustained high influence (P=0·003) or sustained low influence (P=0·02). For speed, no associations were found.

Conclusions

Busyness, speed and influence differed in their relationship to subsequent weight gain. A decrease in job influence and a sustained burden of busyness were most strongly related to subsequent weight gain. Focus on job strain reduction and healthy diet is essential for public health.
Disordered eating in Sami and non-Sami Norwegian populations: the SAMINOR 2 Clinical Survey
Authors: Kvaløy Kirsti , Melhus Marita , Silviken Anne , Brustad Magritt , Sørlie Tore , Broderstad Ann Ragnhild
Published: December 2017

Objective

The present study aimed to investigate disordered eating (DE) among Sami compared with non-Sami residing in northern Norway.

Design

In a cross-sectional design, stratified by sex and ethnicity, associations were tested between DE (Eating Disturbance Scale; EDS-5) and age, education level, BMI category, anxiety and depression, physical activity and consumption of snacks.

Setting

The SAMINOR 2 Clinical Survey (2012–2014) based on the population of ten municipalities in northern Norway.

Subjects

Adults aged 40–69 years; 1811 Sami (844 male, 967 female) compared with 2578 non-Sami (1180 male, 1398 female) individuals.

Results

No overall significant ethnic difference in DE was identified, although comfort eating was reported more often by Sami individuals (P=0·01). Regardless of ethnicity and sex, symptoms of anxiety and depression were associated with DE (P<0·001). Furthermore, DE was more common at lower age and higher BMI values. Education levels were protectively associated with DE among Sami men (P=0·01). DE was associated (OR, 95% CI) with low physical activity in men in general and in non-Sami women (Sami men: 2·4, 1·4, 4·0; non-Sami men: 2·2, 1·4, 3·6; non-Sami women: 1·8, 1·2, 2·9) and so was the consumption of snacks (Sami men: 2·6, 1·3, 5·0; non-Sami men: 1·9, 1·1, 3·1; non-Sami women: 2·1, 1·3, 3·4).

Conclusions

There were no significant differences regarding overall DE comparing Sami with non-Sami, although Sami more often reported comfort eating. There were significant sex and ethnic differences related to DE and physical activity, snacking and education level.
Effect of dietary habits on the risk of metabolic syndrome: Yazd Healthy Heart Project
Authors: Sarebanhassanabadi Mohammadtaghi , Mirhosseini Seyed Jalil , Mirzaei Masoud , Namayandeh Seyedeh Mahdieh , Soltani Mohammad Hossein , Pakseresht Mohammadreza , Pedarzadeh Ali , Baramesipour Zahra , Faraji Reza , Salehi-Abargouei Amin
Published: December 2017

Objective

Metabolic syndrome (MetS) refers to a group of risk factors that increase the risk of cardiovascular mortality and morbidity. Dietary habits are among the most important risk factors for MetS. The current study aimed at assessing the effect of dietary habits on the risk of MetS in a 10-year follow-up study in central Iran.

Design

Cohort study.

Setting

Yazd, Iran.

Subjects

Participants aged 20–74 years without any history of MetS, who were originally recruited for Yazd Healthy Heart Project (YHHP) during 2005–2006, were revisited during 2015–2016. At phase I of YHHP, demographic data, anthropometric measurements, five components of MetS, biochemical tests and dietary habits were evaluated; and the same data were collected in phase II.

Results

A total of 1092 participants were eligible to be included in the present study. After follow-up, the 10-year cumulative incidence of MetS was 56·1 %. After adjustment for potential confounders, increased risk of MetS (hazard ratio; 95 % CI) was found in those who did not try to control their body weight (1·57; 1·06, 2·35), did not usually eat salad (1·91; 1·22, 3·00) and added salt to their food (1·57, 1·06, 2·33). These associations were stronger in men than in the total population after subgroup analysis, but were not present in women.

Conclusions

Dietary habits affect the risk of MetS in the Iranian population. Lifestyle interventions are needed to improve dietary habits to reduce the risk of MetS. Future studies are highly recommended to confirm our results in other populations.
Slow-wave coplanar waveguides based on inductive and capacitive loading and application to compact and harmonic suppressed power splitters
Authors: Aznar-Ballesta Francisco , Selga Jordi , Vélez Paris , Fernández-Prieto Armando , Coromina Jan , Bonache Jordi , Martín Ferran
Published: December 2017

In this paper, a slow-wave transmission line implemented in coplanar waveguide technology, based on simultaneous inductive and capacitive loading, is presented for the first time. The shunt capacitors are achieved by periodically etching transverse strips in the back substrate side, connected to the central strip through metallic vias. The series inductors are implemented by etching rectangular slots in the ground plane. The effect of these reactive elements is an enhancement of the effective shunt capacitance and series inductance of the line, leading to a significant reduction of the phase velocity (slow-wave effect). Consequently, the guided wavelength is also reduced, and these lines can be applied to the miniaturization of microwave components. Moreover, due to periodicity, these artificial lines exhibit stop bands (Bragg effect) useful for spurious or harmonic suppression. A compact harmonic suppressed power splitter, based on a slow wave 35.35 Ω impedance inverter, has been designed and fabricated in order to demonstrate the potential of the proposed approach. The length of the inverter is 48% the length of the conventional counterpart, and measured power splitting at the first (3f0) and second (5f0) harmonic frequencies is rejected more than 49 and 23 dB, respectively.
Overcrowding: Nobody's fault? When some struggle to survive waiting for everyone to take responsibility
Authors: Ballon Vincent
Published: December 2017

Visiting an overcrowded prison is a journey into the private life of each person deprived of his or her freedom, into the community of the detainees and of the staff working in such a place. Using the senses of sight, hearing, smell and touch, combined with empathy and time for observation, helps ICRC delegates to explore vulnerabilities, discover how detainees and staff cope, and grasp the intricate complexity of such a prison system. Beyond what is left of human dignity in these places of detention, when coping mechanisms become survival mechanisms, the suffering shows that overcrowding is wrong. It follows that if overcrowding is “nobody's fault”, it is the responsibility of every individual and every institution of the criminal justice system to create solutions.
Multiscale Invasive Plant Monitoring: Experiences from the Greater Everglades Restoration Area
Authors: Rodgers LeRoy , Pernas Tony , Redwine Jed , Shamblin Brooke , Bruscia Shea
Published: December 2017

Obtaining spatially explicit, cost-effective, and management-relevant data on invasive plant distributions across large natural areas presents considerable challenges. This is especially true when multiple monitoring objectives exist, because the utility of different monitoring methodologies varies with scale, logistical considerations, and information needs. The Florida Everglades is a vast wetland landscape with widespread invasive plant infestations and multiple management jurisdictions. A multi-agency team Working Group conducted a workshop in 2013 to explore opportunities to enhance the performance of a regional weed control program. Among the most important developments occurring at this meeting was the recognition that relevant management questions are scale-dependent. This led the team to define multiple monitoring objectives. Essential for conveying the success of the weed management program is quantifying large-scale patterns of change, as are quantifying fine-scale patterns informing control activities, defining mechanisms of spread, recognizing accelerating rates of spread, and detecting patterns of occupancy immediately before management intervention. The group’s deliberation resulted in the emergence of a multiscale monitoring program utilizing several distinct monitoring protocols, including systematic landscape-level reconnaissance, a sample-based spatially stratified monitoring system, detailed inventories in planned treatment areas, and a set of methods focused solely on early detection and rapid response. Here we provide an overview of the Everglades multiscale invasive plant monitoring program, highlight benefits and challenges of each program component, and discuss how this program has improved regional invasive plant management.
Ethnic differences in metabolite signatures and type 2 diabetes: a nested case–control analysis among people of South Asian, African and European origin
Authors: van Valkengoed Irene G. M. , Argmann Carmen , Ghauharali-van der Vlugt Karen , Aerts Johannes M. F. G. , Brewster Lizzy M. , Peters R. J. G. , Vaz Frédéric M. , Houtkooper Riekelt H.
Published: December 2017

Accumulation of metabolites may mark or contribute to the development of type 2 diabetes mellitus (T2D), but there is a lack of data from ethnic groups at high risk. We examined sphingolipids, acylcarnitines and amino acids, and their association with T2D in a nested case–control study among 54 South Asian Surinamese, 54 African Surinamese and 44 Dutch in the Netherlands. Plasma metabolites were determined at baseline (2001–2003), and cumulative prevalence and incidence of T2D at follow-up (2011–2012). Weighted linear and logistic regression analyses were used to study associations. The mean level of most sphingolipids was lower, and amino-acid levels higher, in the Surinamese groups than among the Dutch. Surinamese individuals had higher mono- and polyunsaturated acylcarnitines and lower plasma levels of saturated acylcarnitine species than the Dutch. Several sphingolipids and amino acids were associated with T2D. Although only the shorter acylcarnitines seemed associated with prevalent T2D, we found an association of all acylcarnitines (except C0, C18 and C18:2) with incident T2D. Further analyses suggested a potentially different association of several metabolites across ethnic groups. Extension and confirmation of these findings may improve the understanding of ethnic differences and contribute to early detection of increased individual risk.
Unravelling the specificity and mechanism of sialic acid recognition by the gut symbiont Ruminococcus gnavus
Authors: Owen C. David , Tailford Louise E. , Monaco Serena , Šuligoj Tanja , Vaux Laura , Lallement Romane , Khedri Zahra , Yu Hai , Lecointe Karine , Walshaw John , Tribolo Sandra , Horrex Marc , Bell Andrew , Chen Xi , Taylor Gary L. , Varki Ajit , Angulo Jesus , Juge Nathalie
Published: December 2017

Ruminococcus gnavus is a human gut symbiont wherein the ability to degrade mucins is mediated by an intramolecular trans-sialidase (RgNanH). RgNanH comprises a GH33 catalytic domain and a sialic acid-binding carbohydrate-binding module (CBM40). Here we used glycan arrays, STD NMR, X-ray crystallography, mutagenesis and binding assays to determine the structure and function of RgNanH_CBM40 (RgCBM40). RgCBM40 displays the canonical CBM40 β-sandwich fold and broad specificity towards sialoglycans with millimolar binding affinity towards α2,3- or α2,6-sialyllactose. RgCBM40 binds to mucus produced by goblet cells and to purified mucins, providing direct evidence for a CBM40 as a novel bacterial mucus adhesin. Bioinformatics data show that RgCBM40 canonical type domains are widespread among Firmicutes. Furthermore, binding of R. gnavus ATCC 29149 to intestinal mucus is sialic acid mediated. Together, this study reveals novel features of CBMs which may contribute to the biogeography of symbiotic bacteria in the gut.
Mutational patterns in chemotherapy resistant muscle-invasive bladder cancer
Authors: Liu David , Abbosh Philip , Keliher Daniel , Reardon Brendan , Miao Diana , Mouw Kent , Weiner-Taylor Amaro , Wankowicz Stephanie , Han Garam , Teo Min Yuen , Cipolla Catharine , Kim Jaegil , Iyer Gopa , Al-Ahmadie Hikmat , Dulaimi Essel , Chen David Y. T. , Alpaugh R. Katherine , Hoffman-Censits Jean , Garraway Levi A. , Getz Gad , Carter Scott L. , Bellmunt Joaquim , Plimack Elizabeth R. , Rosenberg Jonathan E. , Van Allen Eliezer M.
Published: December 2017

Despite continued widespread use, the genomic effects of cisplatin-based chemotherapy and implications for subsequent treatment are incompletely characterized. Here, we analyze whole exome sequencing of matched pre- and post-neoadjuvant cisplatin-based chemotherapy primary bladder tumor samples from 30 muscle-invasive bladder cancer patients. We observe no overall increase in tumor mutational burden post-chemotherapy, though a significant proportion of subclonal mutations are unique to the matched pre- or post-treatment tumor, suggesting chemotherapy-induced and/or spatial heterogeneity. We subsequently identify and validate a novel mutational signature in post-treatment tumors consistent with known characteristics of cisplatin damage and repair. We find that post-treatment tumor heterogeneity predicts worse overall survival, and further observe alterations in cell-cycle and immune checkpoint regulation genes in post-treatment tumors. These results provide insight into the clinical and genomic dynamics of tumor evolution with cisplatin-based chemotherapy, suggest mechanisms of clinical resistance, and inform development of clinically relevant biomarkers and trials of combination therapies.