bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinicogenetic lessons from 370 patients with autosomal recessive limb‐girdle muscular dystrophy
Authors: Winckler Pablo B. , da Silva André M.S. , Coimbra‐Neto Antônio R. , Carvalho Elmano , Cavalcanti Eduardo B.U. , Sobreira Cláudia F.R. , Marrone Carlo D. , Machado‐Costa Marcela C. , Carvalho Alzira A.S. , Feio Raimunda H.F. , Rodrigues Cleonísio L. , Gonçalves Marcus V.M. , Tenório Renata B. , Mendonça Rodrigo H. , Cotta Ana , Paim Júlia F.O. , Costa e Silva Cynthia , de Aquino Cruz Camila , Bená Marjory I. , Betancur Daniel F.A. , El Husny Antonette S. , de Souza Isabel C.N. , Duarte Regina C.B. , Reed Umbertina C. , Chaves Márcia L.F. , Zanoteli Edmar , França Marcondes C. , Saute Jonas A. 
Published: July 2019

AbstractLimb‐girdle muscular dystrophies (LGMD) are a group of genetically heterogeneous disorders characterized by predominantly proximal muscle weakness. We aimed to characterize epidemiological, clinical and molecular data of patients with autosomal recessive LGMD2/LGMD‐R in Brazil. A multicenter historical cohort study was performed at 13 centers, in which index cases and their affected relatives' data from consecutive families with genetic or pathological diagnosis of LGMD2/LGMD‐R were reviewed from July 2017 to August 2018. Survival curves to major handicap for LGMD2A/LGMD‐R1‐calpain3‐related, LGMD2B/LGMD‐R2‐dysferlin‐related and sarcoglycanopathies were built and progressions according to sex and genotype were estimated. In 370 patients (305 families) with LGMD2/LGMD‐R, most frequent subtypes were LGMD2A/LGMD‐R1‐calpain3‐related and LGMD2B/LGMD‐R2‐dysferlin‐related, each representing around 30% of families. Sarcoglycanopathies were the most frequent childhood‐onset subtype, representing 21% of families. Five percent of families had LGMD2G/LGMD‐R7‐telethonin‐related, an ultra‐rare subtype worldwide. Females with LGMD2B/LGMD‐R2‐dysferlin‐related had less severe progression to handicap than males and LGMD2A/LGMD‐R1‐calpain3‐related patients with truncating variants had earlier disease onset and more severe progression to handicap than patients without truncating variants. We have provided paramount epidemiological data of LGMD2/LGMD‐R in Brazil that might help on differential diagnosis, better patient care and guiding future collaborative clinical trials and natural history studies in the field.
Climate change amplifies plant invasion hotspots in Nepal
Authors: Shrestha Uttam Babu , Shrestha Bharat Babu 
Published: July 2019

AbstractAimClimate change has increased the risk of biological invasions, particularly by increasing the climatically suitable regions for invasive alien species. The distribution of many native and invasive species has been predicted to change under future climate. We performed species distribution modelling of invasive alien plants (IAPs) to identify hotspots under current and future climate scenarios in Nepal, a country ranked among the most vulnerable countries to biological invasions and climate change in the world.LocationNepal.MethodsWe predicted climatically suitable niches of 24 out of the total 26 reported IAPs in Nepal under current and future climate (2050 for RCP 6.0) using an ensemble of species distribution models. We also conducted hotspot analysis to highlight the geographic hotspots for IAPs in different climatic zones, land cover, ecoregions, physiography and federal states.ResultsUnder future climate, climatically suitable regions for 75% of IAPs will expand in contrast to a contraction of the climatically suitable regions for the remaining 25% of the IAPs. A high proportion of the modelled suitable niches of IAPs occurred on agricultural lands followed by forests. In aggregation, both extent and intensity (invasion hotspots) of the climatically suitable regions for IAPs will increase in Nepal under future climate scenarios. The invasion hotspots will expand towards the high‐elevation mountainous regions. In these regions, land use is rapidly transforming due to the development of infrastructure and expansion of tourism and trade.Main conclusionsNegative impacts on livelihood, biodiversity and ecosystem services, as well as economic loss caused by IAPs in the future, may be amplified if preventive and control measures are not immediately initiated. Therefore, the management of IAPs in Nepal should account for the vulnerability of climate change‐induced biological invasions into new areas, primarily in the mountains.
Value of three‐dimensional speckle‐tracking imaging in detecting left ventricular systolic function in patients with dilated cardiomyopathy
Authors: He Jionghong , Yang Long 
Published: July 2019

AbstractObjectiveTo explore the value of three‐dimensional speckle‐tracking imaging (3DSTI) in detecting left ventricular systolic function in patients with dilated cardiomyopathy (DCM).MethodsTotally 31 DCM patients were enrolled in this study. Left ventricular end‐systolic volume (LVESV), left ventricular end‐diastolic volume (LVEDV), and left ventricular ejection fraction (LVEF) were measured using the 3DSTI, two‐dimensional echocardiography (2DE), and magnetic resonance imaging (MRI). Left ventricular end‐diastolic mass (EDmass) and left ventricular end‐diastolic mass index (LVEDmass I) were also detected by 3DSTI and MRI. The differences in these measurements were analyzed and compared.ResultsThe values of LVESV, LVEDV, and LVEF showed significantly positive correlations among 2DE group, 3DSTI group, and MRI group. The LVEF value showed significant difference among these three groups [(33.3 ± 11.1)%, (30.3 ± 10.6)%, and (26.2 ± 10.7)%; P = 0.04], whereas LVEDV and LVESV values were not significantly different (P &gt; 0.05; respectively). Inter‐group comparison showed the mean of LVEF was significantly lower in MRI group than in 2DE group (P = 0.031), whereas there was no significant difference between 2DE group and 3DSTI group and between 3DSTI group and MRI group (P &gt; 0.05; respectively). The EDmass and EDmassI detected by 3DSTI and MRI were (143.2 ± 40.2) g vs (190.0 ± 58.3) g and (83.2 ± 21.1) g/m2 vs (110.1 ± 29.7) g/m2 (P < 0.001; respectively).ConclusionsThe LVEF value detected by 3DSTI is closer to that detected by MRI in DCM patients.
Greater than the sum of the parts: how the species composition in different forest strata influence ecosystem function
Authors: Luo Ya‐Huang , Cadotte Marc W. , Burgess Kevin S. , Liu Jie , Tan Shao‐Lin , Zou Jia‐Yun , Xu Kun , Li De‐Zhu , Gao Lian‐Ming 
Published: July 2019

AbstractThe mechanisms underpinning forest biodiversity‐ecosystem function relationships remain unresolved. Yet, in heterogeneous forests, ecosystem function of different strata could be associated with traits or evolutionary relationships differently. Here, we integrate phylogenies and traits to evaluate the effects of elevational diversity on above‐ground biomass across forest strata and spatial scales. Community‐weighted means of height and leaf phosphorous concentration and functional diversity in specific leaf area exhibited positive correlations with tree biomass, suggesting that both positive selection effects and complementarity occur. However, high shrub biomass is associated with greater dissimilarity in seed mass and multidimensional trait space, while species richness or phylogenetic diversity is the most important predictor for herbaceous biomass, indicating that species complementarity is especially important for understory function. The strength of diversity‐biomass relationships increases at larger spatial scales. We conclude that strata‐ and scale‐ dependent assessments of community structure and function are needed to fully understand how biodiversity influences ecosystem function.
High air humidity is sufficient for successful egg incubation and early post‐embryonic development in the marbled crayfish (Procambarus virginalis)
Authors: Guo Wei , Kubec Jan , Veselý Lukáš , Hossain Md Shakhawate , Buřič Miloš , McClain Ray , Kouba Antonín 
Published: July 2019

Abstract

Severe weather events, such as long‐term droughts, are challenging for many freshwater species. To survive drought, freshwater crayfish tend to inhabit shelters or burrows where they can remain in contact with water or high humidity environments. However, it is not known whether embryogenesis or post‐embryonic development can occur without free standing water.

To address this question, three experiments were conducted using artificial burrows with high air humidity and using marbled crayfish (Procambarus virginalis Lyko, 2017) as a model species. Marbled crayfish are capable of parthenogenetic reproduction, burrow extensively and are able to travel long distances over land. In the first experiment, ovigerous females were transferred to simulated burrows without free water, but with high air humidity. A control group of females were kept in burrows with free water. Successful hatching was achieved in both groups.

In the second experiment, ovigerous females were transferred to simulated burrows with no free water but high air humidity and post‐embryonic development were observed. Following successful hatching, offspring moulted to the second developmental stage (stage 2 juveniles). Stage 2 juveniles remained viable without free water for 20 days, but further development was not observed. However, when some of these stage 2 juveniles were placed back into fully aquatic conditions (experiment 3), they moulted to stage 3 within 4 to 8 days.

These results demonstrated the ability of marbled crayfish to undergo terminal phases of embryogenesis, including hatching, as well as early post‐embryonic development under high air humidity conditions only. Post‐embryonic development was suspended in the absence of free water, and successfully resumed when re‐immersed.

This similar ability to tolerate drought‐like conditions during post‐embryonic development may also occur in other crayfish species, especially primary burrowers. This unprecedented life history trait may be crucial for inhabiting ecosystems with rapidly changing water regimes. In drying climates, it may confer advantages on some crayfish species (including some invasive species) over others.
Factors of post‐stroke dementia: A nationwide cohort study in Taiwan
Authors: Li Chien‐Hsun , Chang Yu‐Han , Chou Mei‐Chuan , Chen Chun‐Hung , Ho Bo‐Lin , Hsieh Sheng‐Wen , Yang Yuan‐Han 
Published: July 2019

AimWe investigated the long‐term risk of dementia for up to 10 years in patients with stroke and broadened the correlates.MethodsWe carried out a case–control study using the Taiwan National Health Insurance Research database in 2000 with a sampled population of 1 million. The study cohort comprised 8236 patients with stroke and no dementia history. We carried out a 1:1 case–control matched analysis on estimated propensity scores. Cox proportional hazards regressions were carried out to estimate the risk of dementia during the 5‐ and 10‐year follow‐up periods. The risk factors were also investigated.ResultsThe stroke cohort was significantly at more risk of dementia during the 5‐ and 10‐year follow‐up periods, with adjusted hazard ratios 1.87 and 1.53, respectively. The patients with ischemic stroke, transient ischemic attack and intracerebral hemorrhage had a significantly higher risk of dementia after 5 and 10 years, with adjusted hazard ratios of 1.81 and 1.49, 1.92 and 1.61, and 2.14 and 1.61, respectively. The significant risk factors of dementia were age ≥60 years, resident in southern and eastern regions, having low insurance range, and antiplatelet use.ConclusionsStroke and the subtypes, including ischemic stroke, transient ischemic attack and intracerebral hemorrhage, increase the long‐term risk of dementia. The incidence of post‐stroke dementia increases yearly, but the relative risk decreases gradually. Older adults, residents in southern and eastern regions, having low insurance range and antiplatelet use were prominent risk factors of post‐stroke dementia in Taiwan. Careful management of stroke and risk factors of post‐stroke dementia with long‐term follow up of cognition should be reinforced. Geriatr Gerontol Int 2019; 19: 815–822.
An analysis of the homeworker network in Pakistan: a global justice network (GJN) perspective
Authors: ZULFIQAR GHAZAL MIR 
Published: July 2019

AbstractUsing a global justice network (GJN) approach, in this article I examine the localization of a transnational network for homeworker rights. Based on my field research undertaken in Pakistan between January 2015 and December 2017, I compare different organizing approaches to establish how a politics of vulnerability may be transformed into a politics of voice and mobility. I found that, from the vantage point of the homeworker, the process of organizing rather than the results achieved is what really matters. In the case of Pakistan, union‐style organizing by the Home‐worker Federation, which is mindful of gender and class hierarchies, enhances the homeworkers’ voice, agency and mobility, while also building translocal labour solidarities. Conversely, an NGO‐led national network, with its top–down approach, perpetuates the very hierarchies it was mission bound to dismantle, thus forcing the women to stay spatially imprisoned. Without arguing that one institutional form is superior to the other, I demonstrate that for a GJN to articulate diverse local and global struggles it must be mindful of the hierarchies and boundaries that isolate and silence marginalized workers. It must also genuinely include the grassroots in the production and transference of knowledge.
Resource‐based view of city quality: Scales development and validation
Authors: Carvalho João M. S. , Costa Ricardo V. , Marnoto Sandra , Vieira José Carvalho , Sousa Célio A. A. 
Published: July 2019

AbstractAs the urban world population grows steadily, cities have become the main habitat for human beings. Against this backdrop, city quality or the level of development of the city's habitat that ensures the satisfaction of objective and subjective human needs become a matter of growing interest and concern for academics, policy makers, and citizens. Building on a resource‐based view of city quality, the aim of this paper is twofold. First, it proposes and validates scales for six city sub‐habitats: political, economic, social, natural, artificial, and technological. Second, it tests a model and the underlying hypothesis about the ranking of those sub‐habitats and of the perceived controversy regarding decision making upon them. For those purposes, a survey of 768 city inhabitants was conducted in Portugal to measure city quality and their sub‐habitats. Both the predicted ranking of importance of the sub‐habitats and the perceived ranking of controversy were empirically validated. The results constitute a novel and important contribution to understand city quality and its sub‐habitats, whose conceptual power relies on hierarchized factors linked to citizens’ happiness and to the level of controversy of the solutions.
Incidental splenic findings in pancreatosplenectomy specimens resected for primary pancreatic lesions
Authors: Patel Nisha , Evans Andrew G , Rothberg Paul G , Gonzalez Raul S 
Published: July 2019

AimsLittle has been written on the frequency and nature of incidental splenic lesions diagnosed on histopathological examination of pancreatosplenectomy specimens.Methods and resultsFor 191 such specimens, incidental histological findings after haematopathologist re‐review were tabulated. Cases suspicious for lymphoid malignancy underwent molecular analysis for immunoglobulin heavy and kappa light chain rearrangement. Follow‐up was obtained on selected cases. In five cases (3%), the spleen was sampled but not mentioned in the original microscopic report; all were normal on re‐review. Otherwise, most (171 of 186, 92%) were initially diagnosed as normal, with 160 (94%) remaining so on re‐review. Findings on re‐review not initially described (n = 11, 6%) included four cases with splenic morphology suspicious for possible leukaemia/lymphoma involvement. Additional findings included abscess formation, foamy macrophages, necrotising granulomas and simple cysts. Fifteen spleens were initially diagnosed as abnormal; the histopathological process was confirmed in all, including non‐necrotising granulomas, cysts, Gamna–Gandy bodies, foamy macrophages, involvement by pancreatic neoplasm and involvement by known chronic lymphocytic leukaemia (CLL). Molecular analysis was performed on the five cases of known/suspected lymphoma and two were positive for monoclonal gene rearrangement, including the known CLL and a previously undiagnosed case with similar immunophenotype.ConclusionsIncidental splenic findings are not uncommon in pancreatosplenectomy specimens. While most are of limited clinical significance, low‐grade lymphoproliferative disorders may go undetected if the spleen is overlooked. We recommend careful observation of splenic findings in these specimens, with a low threshold for haematopathological consultation when in doubt.
A critical appraisal of studies on cyclic fatigue resistance of engine‐driven endodontic instruments
Authors: Hülsmann M. , Donnermeyer D. , Schäfer E. 
Published: July 2019

AbstractThe endodontic literature contains a plethora of studies on static and dynamic cyclic fatigue resistance tests performed on a large array of rotary or reciprocating nickel–titanium endodontic instruments. It was the aim of this review to summarize the currently available evidence to point out the different outcomes from static versus dynamic tests and to assess whether cyclic fatigue tests provide useful data and information for clinical practice. An electronic literature research in the database PubMed was performed using appropriate search terms, and the titles and abstract were screened for relevance. Language was restricted to English. The review reveals marked differences between the results obtained in static and dynamic tests, and also, the results for the same instruments assessed either under static or dynamic experimental conditions are widely inconsistent. Between the lowest and the highest value for one and the same pathfinding instrument was a factor of about 123 when cyclic fatigue was assessed either under static or dynamic experimental conditions. Moreover, standard deviations of up to 30% have been reported. Environmental temperature has a 500% impact on the lifetime of instruments. In conclusion, fatigue resistance tests conducted under room temperature should be regarded as having little meaning and the scientific and clinical benefits of fatigue resistance tests are very limited. These data should be provided by the manufacturer of the instruments.
Experimental measurement of the bullet trajectory after perforation of a chambered window
Authors: Sielicki Piotr W. , Pludra Adrian , Przybylski Michał 
Published: July 2019

AbstractThe assessment of the failure and final response for glass structures loaded by high‐speed events such as blast or projectile loading are challenging tasks for researchers. For many studies, knowledge about the strain rate effect in brittle materials and well described finite element simulations are not enough to reach a correct outcome. For that reason, only experimental assessments can give credible results. Moreover, the impacting glass fragments or projectiles often change the angle of the trajectory after perforation of a target. In this study, the authors present the change in the 3000 J military bullet trajectory after total penetration of a two‐chambered civil engineering facade window. A series of actual tests were performed for different angles of the soda‐lime float glass specimen that were subjected to bullet impact. The results obtained chambered windows given an understanding of the behavior of the separate glass surfaces under such complex scenarios. Furthermore, the presented tests support the designers and military shooters by the assessment methodology of projectile bullet trajectory change as a function of the incident angle.
Structure and enhanced antimicrobial activity of mechanically activated nano TiO2
Authors: Pavlović Vera P. , Vujančević Jelena D. , Mašković Pavle , Ćirković Jovana , Papan Jelena M. , Kosanović Darko , Dramićanin Miroslav D. , Petrović Predrag B. , Vlahović Branislav , Pavlović Vladimir B. 
Published: July 2019

AbstractTitanium dioxide is a photocatalyst, known not only for its ability to oxidize organic contaminants, but also for its antimicrobial properties. In this article, significant enhancement of the antimicrobial activity of TiO2 (up to 32 times) was demonstrated after its activation by ball milling. The antimicrobial activity was analyzed for one fungal and 13 bacterial ATCC strains using the microdilution method and recording the minimum inhibitory concentration (MIC) values. In order to further investigate the correlation between the mechanical activation of TiO2 and its antimicrobial activity, the structure, morphology and phase composition of the material were studied by means of Electron Microscopy, X‐ray diffraction and nitrogen adsorption‐desorption measurements. UV‐Vis diffuse reflectance spectra were recorded and the Kubelka‐Munk function was applied to convert reflectance into the equivalent band gap energy (Eg) and, consequently, to investigate changes in the Eg value. X‐ray photoelectron spectroscopy was used to analyze the influence of mechanical activation on the Ti 2p and O 1s spectra. The presented results are expected to enable the development of more sustainable and effective advanced TiO2‐based materials with antimicrobial properties that could be used in numerous green technology applications.
Thermal properties of polymer‐derived ceramic reinforced with boron nitride nanotubes
Authors: Jia Yujun , Ajayi Tosin D. , Morales Justin , Chowdhury Md Atiqur Rahman , Sauti Godfrey , Chu Sang‐Hyon , Park Cheol , Xu Chengying 
Published: July 2019

AbstractWe report the thermal properties of boron nitride nanotube (BNNT) reinforced ceramic composites using the polymer derived ceramic (PDC) processing route. The nano‐composites had a BNNT loading of up to 35.4 vol.%. TGA results showed that nano‐composites have good thermal stability up to 900°C in air. BNNTs in nano‐composites survived in an oxidizing environment up to 900°C, revealing that nano‐composites can be used for high temperature applications. Thermal conductivity of PDC reinforced with 35.4 vol.% BNNT was measured as 4.123 W/(m·K) at room temperature, which is a 2100 % increase compared to that of pristine PDC. The thermal conductivity value increases with the increase of BNNT content. A thermal conductivity percolation phenomenon appeared when the BNNT content increased to 36 ± 5 vol.%. The results of this study showed that BNNTs could effectively improve the thermal conductivity of PDC materials. BNNT reinforced PDC could be used as thermal structural materials in a harsh environment at temperatures up to 900°C.
Fabrication and microstructural characterizations of lasing grade Nd:Y2O3 ceramics
Authors: Yin Danlei , Wang Jun , Liu Peng , Zhu Haiyong , Yao Bingqing , Dong Zhili , Tang Dingyuan 
Published: July 2019

AbstractTransparent 0.6 at% Nd:Y2O3 ceramics were fabricated by vacuum sintering at 1550°C and hot isostatic pressing (HIP) at 1540°C. The ceramics sintered at such temperatures had good homogeneity with dense microstructures, without any residual pores and secondary phases. The in‐line transmittance reached 81.6% at 1000 nm and remained 81.1% at 650 nm. Continuous wave (CW) laser operation of an uncoated ceramic slab was evaluated. A maximum output power of 3.6 W with slope efficiency of 45.2% at 1.08 μm was obtained.