bluuebunny/crossref_metadata_2025_split huggingface dataset:
The complex phenotype of spinocerebellar ataxia type 48 in eight unrelated Italian families
Authors: Lieto M. , Riso V. , Galatolo D. , De Michele G. , Rossi S. , Barghigiani M. , Cocozza S. , Pontillo G. , Trovato R. , Saccà F. , Salvatore E. , Tessa A. , Filla A. , Santorelli F. M. , De Michele G. , Silvestri G. 
Published: October 2019

Background and purposeHeterozygous mutations in the STUB1 gene have recently been associated with an autosomal dominant form of spinocerebellar ataxia (SCA) associated with cerebellar cognitive‐affective syndrome (CCAS), named SCA48.MethodsMolecular screening was performed in a cohort of 235 unrelated patients with adult‐onset, autosomal dominant (17) or sporadic (218) cerebellar ataxia, negative for pathological trinucleotide expansions in the common SCAs, FRDA and FXTAS loci, by using targeted multigene panels or whole‐exome sequencing. Bioinformatics analyses, detailed neurological phenotyping and family segregation studies corroborated the pathogenicity of the novel STUB1 mutations. Clinico‐diagnostic findings were reviewed to define the phenotypic spectrum.ResultsEight heterozygous STUB1 mutations were identified, six of which were novel in 11 patients from eight index families, giving an estimated overall frequency of 3.4% (8/235) for SCA48 in our study cohort, rising to 23.5% (4/17) when considering only familial cases. All our SCA48 patients had cerebellar ataxia and dysarthria associated with cerebellar atrophy on brain magnetic resonance imaging; of note, many cases were also associated with parkinsonism, chorea and dystonia. CCAS also occurred frequently, whereas definite signs of pyramidal tract dysfunction and peripheral nervous system involvement were absent. One SCA48 patient presented with hypogonadism, associated with other autoimmune endocrine dysfunctions.ConclusionsOur results support SCA48 as a significant cause of adult‐onset SCA. Besides CCAS, our SCA48 patients often showed movement disorders and other clinical manifestations previously described in SCAR16, linked to biallelic variants in the same gene, thus suggesting a continuous clinical spectrum and significant overlap amongst recessive and dominantly inherited mutations in STUB1.
Sudden unexpected death in epilepsy. A nationwide population‐based study
Authors: Einarsdottir Anna B. , Sveinsson Olafur , Olafsson Elias 
Published: October 2019

AbstractObjectiveThe incidence of sudden unexpected death in epilepsy (SUDEP) varies between studies. We determined the incidence of SUDEP in the entire Icelandic population during a 20‐year period.MethodsAll individuals in Iceland with epilepsy who died unexpectedly from January 1, 1991 through December 31, 2010 were included. Case ascertainment was based on autopsies, reimbursement for antiepileptic drugs, death certificates, information from neurologists, and medical records. The incidence of SUDEP was calculated according to the total number of residents in Iceland during the study period and an estimated epilepsy population of Iceland.ResultsWe identified 37 individuals (26 men, 11 women) with definite SUDEP (n = 29), definite SUDEP plus (n = 4), and probable SUDEP (n = 4). Incidence of SUDEP was 0.6 per 100 000 person‐years for the general population, and higher among men. The estimated incidence of SUDEP in the epilepsy population was 1.3 per 1000 person‐years. SUDEP accounted for 0.1% of all deaths in Iceland during the study period.SignificanceSUDEP is an important cause of death in working‐age people. This study provides the incidence of SUDEP in an unselected population of an entire country. The SUDEP incidence in the epilepsy population is comparable to that of previous studies.
Attending to spatial social–ecological sensitivities to improve trade‐off analysis in natural resource management
Authors: Okamoto Daniel K. , Poe Melissa R. , Francis Tessa B. , Punt André E. , Levin Phillip S. , Shelton Andrew O. , Armitage Derek R. , Cleary Jaclyn S. , Dressell Sherri C. , Jones Russ , Kitka Harvey , Lee Lynn C. , MacCall Alec D. , McIsaac Jim A. , Reifenstuhl Steve , Silver Jennifer J. , Schmidt Jörn O. , Thornton Thomas F. , Voss Rüdiger , Woodruff John 
Published: October 2019

AbstractBalancing trade‐offs amongst social–ecological objectives is a central aim of natural resource management. However, objectives and resources often have spatial dimensions, which are usually ignored in trade‐off analyses. We examine how simultaneously integrating social–ecological benefits and their spatial complexities can improve trade‐off analysis. We use Pacific herring (Clupea pallasii, Clupeidae)—an ecologically important forage fish with social, cultural and economic value to communities and commercial fisheries—as a case study. By combining spatial management strategy evaluation with social benefits analysis, we illustrate when policies aimed at aggregate stocks versus spatially segregated substocks of fish fail to balance trade‐offs amongst social–ecological objectives. Spatial measures (e.g. area‐based closures) may achieve some objectives but produce alternative trade‐offs that are sensitive to assumptions about fish population dynamics and social complexities. Our analyses identify policies that are inefficient (e.g. yielding economic costs without producing social or ecological gains), highlight management strategies that generate trade‐offs and indicate when costs are distributed unequally for different user groups. We also point to strategies with outcomes that are robust to spatial uncertainties and reveal research priorities by identifying which performance metrics exhibit sensitivity to spatial ecological assumptions. Collectively, our analyses demonstrate how incorporating social objectives and spatial dynamics into management strategy evaluation can reveal trade‐offs and the implications of management decisions.
Historical perspective of artisanal encircling gillnet use at the Brazilian coast: Changes in fishing behaviour is mirrored by dwindling stocks
Authors: Reis‐Filho José Amorim 
Published: October 2019

AbstractManaging coastal resources sometimes requires looking into the past, as this provides a baseline against which present conditions may be gauged. The present study evaluated the circumstances that led Brazilian coastal fishers to modify their use of encircling gillnets. Other stressors (i.e. water pollution and habitat degradation) acted synergistically on the fisheries dynamic between 1950 and 2016. Declining catches of several species were observed through fishers' historical perception and through fisheries landing records. Three notable trends in net handling were associated with distinct historical periods. Specifically, pressures from modern fishery practices and gear (e.g. monofilament nets), the adoption of spiral setting arrangements and the increasing use of trammel nets appear responsible for the decline of many stocks. Mullet, seabass, jack and pilchard stocks decreased over time and were associated the changes witnessed in the encircling net operations. These changes occurred in response to overexploitation of fish stocks, to exploit the dwindling fisheries resources. The study also discusses the reasons leading fishers to accept how encircling gillnets ought to be used, their interpretation of such changes and the implications these changes have for fisheries management.
Intravenous Magnesium Sulfate to Treat Acute Headaches in the Emergency Department: A Systematic Review
Authors: Miller Andrew C. , K. Pfeffer Brandon , Lawson Michael R. , Sewell Kerry A. , King Alexandra R. , Zehtabchi Shahriar 
Published: October 2019

IntroductionNon‐traumatic headaches comprise up to 4% of all emergency department (ED) visits. Current practice is moving toward multimodal analgesia regimens that limit narcotic use.ObjectiveThe objective of this systematic review is to address the following research question: In patients with non‐traumatic headaches (Population), does administration of intravenous magnesium sulfate (Intervention) compared to placebo, corticosteroids, dopamine antagonists, ergot alkaloids, non‐steroidal anti‐inflammatory drugs (NSAIDs), triptans, or usual care result in better pain control, lower rate of recurrence at 24 hours, lower requirements for rescue analgesia, and less adverse medication effects (Outcomes)?MethodsScholarly databases and relevant bibliographies were searched, as were clinical trial registries and relevant conference proceedings to limit publication bias. Studies were not limited by date, language, or publication status. Inclusion criteria were: (1) randomized clinical trial (RCT), (2) patients age ≥18 years, (3) non‐traumatic headache, (4) patients treated in ED or an outpatient acute care treatment center, and (5) magnesium sulfate administered intravenously (IV). Eligible comparison groups included: placebo, conventional therapy, dopamine antagonist, NSAID, corticosteroid, ergot alkaloid, or triptans.ResultsOut of 4018 identified references, 7 RCTs (545 participants) that treated migraine headaches (n = 6) and benign non‐traumatic headaches (n = 1) met inclusion criteria. Pain intensity was improved with magnesium sulfate vs comparators at 60‐120 minutes, but not at earlier time points. Result for the endpoint of pain reduction by 50% were conflicting as 3 studies reported that headache was improved, unchanged, and less with magnesium sulfate. Complete pain relief was improved with magnesium sulfate in 1 study, and in the migraine with aura (MA) subgroup in another. The need for rescue analgesia at any point was improved with magnesium sulfate in 1 study, and in the MA subgroup in another. Twenty‐four‐hour headache recurrence was improved with magnesium sulfate in 1 study, but unchanged in a second. The intended meta‐analysis was not performed due to the clinical heterogeneity among studies.ConclusionWhile we cannot draw a firm conclusion on the efficacy or benefit of intravenous magnesium sulfate in the treatment of acute non‐traumatic headaches, the existing evidence indicates potential benefits in pain control beyond 1 hour, aura duration, and need for rescue analgesia.
Permian felsic magmatism in the Neoproterozoic Nagar Parkar Igneous Complex of the Malani Igneous Suite: Evidence from zircon U–Pb age
Authors: Rehman Hafiz U. , Khan Tahseenullah , Lee Hao‐Yang , Chung Sun‐Lin , Murata Mamoru , Jan M. Qasim 
Published: October 2019

AbstractWe report Permian (ca. 272 Ma ±5.4 Ma) felsic dykes that intrude into the Neoproterozoic (ca. 750 Ma) magmatic suite of the Nagar Parkar Igneous Complex (NPIC), the western extension of the Malani Igneous Suite (MIS). The NPIC consists of Neoproterozoic basement amphibolites and granites (riebeckite–aegirine gray granites and the biotite–hornblende pink granites), all of which are intruded by several generations of mafic and felsic dykes. Granitic magmatism occurred in the Late Neoproterozoic (ca. 750 Ma) due to the subduction‐, followed by the rift‐related tectonic regime during the breakup of the Rodinia supercontinent. U–Th–Pb zircon and monazite CHIME age data of 700–800 Ma from the earlier generation porphyritic felsic dykes suggest the dyke intrusion was coeval or soon after the emplacement of the host granites. Our findings of Permian age orthophyric felsic dykes provide new insights for the prevalence of active tectonics in the MIS during late Paleozoic. Textural features and geochemistry also make the orthophyric dykes distinct from the early‐formed porphyritic dykes and the host granites. Our newly obtained age data combined with geochemistry, suggest the existence of magmatism along the western margin of India (peri‐Gondwana margin) during Permian. Like elsewhere in the region, the Permian magmatism in the NPIC could be associated with the rifting of the Cimmerian micro‐continents from the Gondwana.
The efficacy of a highly occlusive formulation for dry lips
Authors: Tamura E. , Yasumori H. , Yamamoto T. 
Published: October 2019

AbstractObjectiveSince skin on the lips has a lower water content in the stratum corneum (SC) and a higher transepidermal water loss (TEWL) value than skin on the cheek, the lips are usually very dry and rough areas. Therefore, a lip balm approach to increase occlusivity of the lips is generally used to reduce water loss. Although there have been reports of an improvement effect on lip roughness by specific care agents, differences in the effects due to the occlusivity of a formulation without a specific care agent have not been reported. The purpose of this study was to clarify the improvement effect on dryness and roughness of the lips by a highly occlusive formulation consisting of a combination of common oil and wax, not by a specific care agent.MethodsTwenty Japanese female subjects with normal skin (age range 20–39 years; mean 28.1) were enrolled in this study. A 4‐week continuous use test was conducted using samples with low and high occlusivity. The degree of lip roughness and wrinkles was scored, the hollow index was assessed using ANTERA 3D images, and values of capacitance and TEWL were measured.ResultsThe hollow index was significantly correlated with both the appearance roughness score and the wrinkle score. The sample with high occlusivity showed significant improvement in the appearance roughness score, wrinkle score, the hollow index and capacitance of the SC. In a comparison between the groups of samples with different occlusive properties, a significant improvement effect in rough appearance was seen in subjects using the highly occlusive formulation.ConclusionThe hollow index assessed by ANTERA 3D imaging allowed the objective evaluation of the improvement in appearance. In the case of continuous use of the highly occlusive formulation, an improvement of capacitance in the SC, which is most correlated with lip roughness, was obtained after 2 and 4 weeks. These results demonstrate for the first time that a highly occlusive formulation is effective in improving the roughness of the lips, and this effect is due to the difference in the occlusive property not by a specific care agent.
Effect of immersion in distilled water or phosphate‐buffered saline on the solubility, volumetric change and presence of voids within new calcium silicate‐based root canal sealers
Authors: Torres F. F. E. , Zordan‐Bronzel C. L. , Guerreiro‐Tanomaru J. M. , Chávez‐Andrade G. M. , Pinto J. C. , Tanomaru‐Filho M. 
Published: October 2019

AbstractAimTo assess the effect of immersion in distilled water or phosphate‐buffered saline (PBS) on the solubility, volumetric change and presence of voids of calcium silicate‐based root canal sealers (TotalFill BC, Sealer Plus BC and Bio‐C), in comparison with the gold standard epoxy resin‐based sealer (AH Plus).MethodologyAll properties were evaluated after immersion in distilled water or PBS. Solubility was determined by the percentage of mass loss, whereas volumetric change and presence of voids were evaluated by micro‐computed tomography, after 7 days of immersion. The volumetric change and percentage of voids between the baseline (after setting) and the experimental period were calculated. Statistical analysis was performed using one‐way anova and Tukey's or Student's t‐tests (α = 0.05).ResultsThe calcium silicate‐based sealers had significantly greater solubility and volumetric loss than AH Plus, after immersion in distilled water or PBS (P < 0.05). Bio‐C had the greatest solubility (P < 0.05), followed by TotalFill BC and Sealer Plus BC, which were similar (P > 0.05). Regarding the volumetric change, AH Plus had a volume increase, with similar values in distilled water and PBS (P > 0.05). TotalFill BC, Sealer Plus BC and Bio‐C had a similar volumetric change (P > 0.05). The calcium silicate‐based materials had the greatest solubility and volume loss after immersion in distilled water (P < 0.05). There was no difference in the percentage of voids amongst the sealers, before and after immersion in distilled water or PBS (P > 0.05).ConclusionsTotalFill BC, Sealer Plus BC and Bio‐C had significantly greater solubility and volumetric loss than AH Plus. Although storage in PBS significantly reduced the solubility and volumetric change of calcium silicate‐based sealers, their solubility remained above that recommend by ISO 6876. All the sealers evaluated had low and similar voids, even after immersion in distilled water or PBS.
Highly concentrated aqueous SiC slurry containing fine SiC powder: A new approach for ultra‐dense green body
Authors: Vu Viet Hung , Gu Jian , Kim Jun Seop , Lee Sea Hoon , Lee Hee Soo 
Published: October 2019

AbstractSiC slurry with ultra‐high concentration up to 70 vol% was prepared using oxidized fine and coarse SiC powder mixture, and dense SiC green body with a relative density of 76% was fabricated by drying the slurry at ambient condition. Three approaches were performed to prepare highly concentrated SiC slurry; preparation of SiC powder having good dispersion behavior, optimization of the oxidation condition, and optimization of bi‐modal particle size distribution. An aqueous slurry with the solid loading up to 62 vol% could be prepared using fine (150 nm) SiC powder prepared by the mechanical alloying of Si and carbon. The surface property of the fine and coarse (10 μm) SiC powders was optimized using an oxidation treatment. The maximum solid loading of the fine SiC slurry prepared using oxidized powder was 66 vol%. By optimizing the mixing ratio of the oxidized fine and coarse SiC powder to 75:25, the solid loading of the SiC slurry could increase up to 70 vol%. The relative densities of the green bodies after drying 66, 68, and 70 vol% slurries were 69, 75.7, and 76.1%, respectively, which values were higher than those (58%) prepared by cold isostatic pressing under 200 MPa.
The effects of cast industry waste of scale instead of cement on the mechanical and microstructural properties
Authors: Mutuk Tuğba , Demirbaş Arife Kübra , Çevik Sinem , Mesci Oktay Başak 
Published: October 2019

AbstractThe scale is a waste coming from the metal casting industry. It is an iron oxide layer formed as a result of oxidation after annealing on steel surfaces. Casting wastes, which have been left uncontrolled for many years, have caused considerable environmental damage. In the present paper, the mechanical properties and microstructural characterization of cement mortars, prepared by using scale coming from Turkey steel manufacturer were experimentally investigated. The steel scale was used as a partial replacement of cement. Recycling of solid waste scale is considered as an environmental‐friendly alternative to solve the problem of disposing of wastes. Cement mortar admixtures were prepared by variable percentages of scale from 2% to 10%. The effect of scale on the strength of cement mortar was analyzed. The compressive strength values of all the specimens were recorded for 3, 7, and 28 days and the results showed that when the amount of scale replacement was increased, the strength increased slightly. However, cement mortar containing 4% wt. additive has shown the highest strength value at 3 and 28 days. As a result an optimum additive amount of scale in the cement mortar is found as 4% wt. Microstructural observation of the samples using SEM showed that scale particles were well embedded in the c matrix.
Do randomised controlled trials relevant to pharmacy meet best practice standards for quality conduct and reporting? A systematic review
Authors: Ritchie Alison , Seubert Liza , Clifford Rhonda , Perry Danae , Bond Christine 
Published: October 2019

Abstract

Objectives
Evidence-based pharmacy practice requires a dependable evidence base. Randomised controlled trials (RCTs) are the gold standard of high-quality primary research, and tools exist to assist researchers in conducting and reporting high-quality RCTs. This review aimed to explore whether RCTs relevant to pharmacy are conducted and reported in line with Cochrane risk of bias and CONSORT standards, respectively.

Methods
A MEDLINE search identified potential papers. After screening of titles, abstracts and full texts, the 50 most recent papers were reviewed and assessment of bias according to Cochrane domains and compliance with CONSORT checklist items was recorded. Each domain of the Cochrane tool and CONSORT checklist item and each article were given a percentage score, reported as median and interquartile range (IQR). Correlation between quality of conduct, quality of reporting, continent of origin, and journal impact factor was conducted using the R2 statistic. The median domain score for risk of bias by paper according to the Cochrane risk of bias tool was 53.0% (IQR 38.5–68.5), while the median compliance score by paper for the CONSORT checklist was 64.0% (IQR 36.0–94.0%).

key findings
The median Cochrane domain and median CONSORT item completion scores, respectively, were 50.0% (IQR 33.3–66.7%) and 59.5% (IQR 52.0–70.3%). The highest risk of bias was associated with allocation concealment and blinding, and the least well-reported items were randomisation details, sequence generation and allocation concealment. A positive relationship between conduct and reporting of RCTs was found (R2 = 0.75), while no correlation was found between quality of conduct or quality of reporting and journal impact factor, correlation coefficients (R2 = 0.06 and R2 = 0.05, respectively).

Summary
This review identified that issues related to randomisation and blinding are often inadequately conducted or not comprehensively reported by researchers conducting pharmacy relevant RCTs, providing useful information for education and future research.
Promotion of appropriate knowledge and attitude towards medicines among schoolchildren in Jordan: the role of teachers
Authors: Sharaydih Rawia , Abuloha Sumaya , Wazaify Mayyada 
Published: October 2019

Abstract

Objectives
The current study aimed to examine the knowledge, attitude and practice of schoolteachers in Jordan towards medication. Furthermore, it assessed teachers' views regarding the importance of school education in the promotion of appropriate and safe use of medicines.

Methods
All Facebook groups of teachers who were members of the Jordan Teachers Syndicate and/or teachers employed by Jordan Ministry of Education were invited to participate. A cross-sectional online survey using a validated pre-piloted anonymous Arabic questionnaire was created using Google documents. The questionnaire was automatically hosted via a unique URL. Researchers downloaded real-time questionnaire responses. Data were coded and entered into SPSS database for windows version 22 for statistical analysis.

Key findings
A total of 902 teachers filled in the questionnaire. The mean knowledge score achieved in multiple-choice questions was 2.43 ± 0.51 out of 4.00. The top three factors affecting all of teachers’ knowledge questions were as follows: age, the presence of chronic disease, and the school grade that they taught. Teachers showed variation in their attitude towards education about medicines in children. The majority (80.7%) of teachers were unfamiliar with education about medication and had never taught schoolchildren about any issues related to proper use of medications.

Conclusions
The study highlighted that teachers’ knowledge about medicines was not satisfactory enough for them to become active health educators. At the same time, they were willing to educate children about proper use of medicines, and such education should be started at the first grade (i.e. 6 years old).