bluuebunny/crossref_metadata_2025_split huggingface dataset:
GMPPB-congenital disorders of glycosylation associate with decreased enzymatic activity of GMPPB
Authors: Liu Zhe , Wang Yan , Yang Fan , Yang Qin , Mo Xianming , Burstein Ezra , Jia Da , Cai Xiao-tang , Tu Yingfeng 
Published: May 2021

AbstractThe congenital disorders of glycosylation (CDG) are a family of metabolic diseases in which glycosylation of proteins or lipids is deficient. GDP-mannose pyrophosphorylase B (GMPPB) mutations lead to CDG, characterized by neurological and muscular defects. However, the genotype-phenotype correlation remains elusive, limiting our understanding of the underlying mechanism and development of therapeutic strategy. Here, we report a case of an individual presenting congenital muscular dystrophy with cerebellar involvement, who presents two heterozygous GMPPB mutations (V111G and G214S). The V111G mutation significantly decreases GMPPB’s enzymatic activity. By measuring enzymatic activities of 17 reported GMPPB mutants identified in patients diagnosed with GMPPB-CDG, we discover that all tested GMPPB variants exhibit significantly decreased enzymatic activity. Using a zebrafish model, we find that Gmppb is required for neuronal and muscle development, and further demonstrate that enzymatic activity of GMPPB mutants correlates with muscular and neuronal phenotypes in zebrafish. Taken together, our findings discover the importance of GMPPB enzymatic activity for the pathogenesis of GMPPB-CDG, and shed light for the development of additional indicators and therapeutic strategy.
The ENJOY Project: Usage and Factors to Support Adherence and Physical Activity Participation
Authors: Levinger Pazit , Dunn Jeremy , Panisset Maya , Haines Terry , Dow Briony , Batchelor Frances , Biddle Stuart J.H. , Duque Gustavo , Hill Keith D. 
Published: May 2021

ABSTRACT

Objectives
Long-term engagement and participation in physical activity by older adults in the general population is poor. The Exercise interveNtion outdoor proJect in the cOmmunitY for older people (the ENJOY project) is a community-based research project utilizing an outdoor Seniors Exercise Park that demonstrated increased physical activity level as well as physical and mental health benefits. This study aims to explore participants’ perceptions about barriers and facilitators to ongoing participation as well as the usage pattern of the Seniors Exercise Park.

Method
People aged ≥60 yr underwent a 12-wk structured supervised physical activity program using outdoor exercise park equipment followed by a 6-month maintenance phase. Participants completed two surveys: at the completion of the 12-wk structured supervised program and at the completion of the 6-month maintenance phase. Access to the Seniors Exercise Park was also electronically monitored during the maintenance phase.

Results
The social aspect (60%), health benefits/improvement (41.5%), and supervision (36.9%) were the most cited factors in positively supporting participation, with 60.8% continued using the Exercise Park regularly during the maintenance phase. The main barriers to continued usage for both regular users and nonusers included other commitments (71.4%), the weather (60.7%), and medical/health problems (42.8%).

Conclusions
The Seniors Exercise Park can be utilized to promote sustainable engagement in physical activity for older people. However, future implementation strategies should consider approaches to overcome barriers for participation.
Trial registration number: ACTRN12618001727235.
Ledipasvir/Sofosbuvir for 8, 12, or 24 Weeks in Hepatitis C Patients Undergoing Dialysis for End-Stage Renal Disease
Authors: Chuang Wan-Long , Hu Tsung-Hui , Buggisch Peter , Moreno Christophe , Su Wei-Wen , Biancone Luigi , Camargo Marianne , Hyland Robert , Lu Sophia , Kirby Brian J. , Dvory-Sobol Hadas , Osinusi Anu , Gaggar Anuj , Peng Cheng-Yuan , Liu Chen-Hua , Sise Meghan E. , Mangia Alessandra 
Published: May 2021

INTRODUCTION:
We evaluated 8, 12, or 24 weeks of ledipasvir/sofosbuvir in patients with hepatitis C virus and end-stage renal disease undergoing dialysis.

METHODS:
Primary efficacy end point was sustained virologic response 12 weeks after treatment. Primary safety end point was treatment discontinuation because of adverse events (AEs).

RESULTS:
Ninety-four percent (89/95) achieved sustained virologic response 12 weeks after treatment. Six patients died during treatment (n = 4) or before study completion (n = 2); no deaths were related to treatment. No patients discontinued treatment because of AEs. Thirteen percent had serious AEs; none were related to treatment.

DISCUSSION:
Treatment with ledipasvir/sofosbuvir was safe and effective in patients with end-stage renal disease undergoing dialysis.
Association Between Diarrhea Duration and Severity and Probiotic Efficacy in Children With Acute Gastroenteritis
Authors: Schnadower David , O'Connell Karen J. , VanBuren John M. , Vance Cheryl , Tarr Phillip I. , Schuh Suzanne , Hurley Katrina , Rogers Alexander J. , Poonai Naveen , Roskind Cindy G. , Bhatt Seema R. , Gouin Serge , Mahajan Prashant , Olsen Cody S. , Powell Elizabeth C. , Farion Ken , Sapien Robert E. , Chun Thomas H. , Freedman Stephen B. ,   on Behalf of the Pediatric Emergency Care Applied Research Network and Pediatric Emergency Research Canada
Published: May 2021

INTRODUCTION:
It is unclear whether the alleged efficacy of probiotics in childhood acute gastroenteritis depends on the duration and severity of symptoms before treatment.

METHODS:
Preplanned secondary analysis of 2 randomized placebo-controlled trials in children 3–48 months of age was conducted in 16 emergency departments in North America evaluating the efficacy of 2 probiotic products (Lactobacillus rhamnosus GG and a combination probiotic: L. rhamnosus and L. helveticus). Participants were categorized in severity groups according to the duration (<24, 24–<72, and ≥72 hours) and the frequency of diarrhea episodes in the 24 hours (≤3, 4–5, and ≥6) before presentation. We used regression models to assess the interaction between pretreatment diarrhea severity groups and treatment arm (probiotic or placebo) in the presence of moderate-to-severe gastroenteritis (Modified Vesikari Scale score ≥9). Secondary outcomes included diarrhea frequency and duration, unscheduled healthcare provider visits, and hospitalization.

RESULTS:
A total of 1,770 children were included, and 882 (50%) received a probiotic. The development of moderate-to-severe gastroenteritis symptoms after the initiation of treatment did not differ between groups (probiotic—18.4% [162/882] vs placebo—18.3% [162/888]; risk ratio 1.00; 95% confidence interval 0.87, 1.16; P = 0.95). There was no evidence of interaction between baseline severity and treatment (P = 0.61) for the primary or any of the secondary outcomes: diarrhea duration (P = 0.88), maximum diarrheal episodes in a 24-hour period (P = 0.87), unscheduled healthcare visits (P = 0.21), and hospitalization (P = 0.87).

DISCUSSION:
In children 3–48 months with acute gastroenteritis, the lack of effect of probiotics is not explained by the duration of symptoms or frequency of diarrheal episodes before presentation.
Effects of Screenings in Reducing Colorectal Cancer Incidence and Mortality Differ by Polygenic Risk Scores
Authors: Choi Jungyoon , Jia Guochong , Wen Wanqing , Long Jirong , Shu Xiao-Ou , Zheng Wei 
Published: May 2021

INTRODUCTION:
Colorectal cancer (CRC) screening reduces CRC incidence and mortality. However, it is unclear whether the reduction in CRC risk may differ by genetic susceptibility.

METHODS:
We evaluated this question in a cohort of 304,740 participants of European descent aged 50 years and older. Genetic susceptibility was measured using a polygenic risk score (PRS) constructed with risk variants identified in genomewide association studies. Cox models were used to estimate hazard ratios and 95% confidence intervals of CRC risk.

RESULTS:
Over a median follow-up of 7.0 years, 2,261 incident CRC cases and 528 CRC deaths were identified. CRC screening was associated with a significantly reduced CRC incidence among individuals with a high (hazard ratio, 0.80; 95% confidence interval, 0.71–0.92) and intermediate PRS (0.84, 0.71–0.98) but not among those with a low PRS (1.03, 0.86–1.25; P
interaction, 0.005). A similar but more evident difference was observed for mortality (P
interaction, 0.046), with more than 30% reduced mortality observed in the high PRS group (0.69, 0.52–0.91). Among the younger group (age 50–60 years), CRC screenings were associated with a slightly (but nonsignificantly) elevated incidence and mortality in the low PRS group but a reduced risk in the high PRS group (P
interaction, 0.043 [incidence]; 0.092 [mortality]). No significant interaction was observed in the older group (age > 60 years).

DISCUSSION:
Individuals with a higher genetic risk benefited more substantially from CRC screenings than those with a lower risk. Our findings suggest that PRS may be used to develop personalized CRC screening to maximize its effect on CRC prevention.
Cerebrospinal fluid hemoglobin levels as markers of blood contamination: relevance for α-synuclein measurement
Authors: Paciotti Silvia , Stoops Erik , François Cindy , Bellomo Giovanni , Eusebi Paolo , Vanderstichele Hugo , Chiasserini Davide , Parnetti Lucilla 
Published: May 2021

Abstract

Objectives
Cerebrospinal fluid α-synuclein (CSF α-syn) represents a possible biomarker in Parkinson’s disease (PD) diagnosis. CSF blood contamination can introduce a bias in α-syn measurement. To date, CSF samples with a red blood cells (RBC) count >50 RBC × 106/L or haemoglobin (Hb) concentration >200 μg/L are excluded from biomarker studies. However, investigations for defining reliable cut-off values are missing.

Methods
We evaluated the effect of blood contamination on CSF α-syn measurement by a systematic approach in a cohort of 42 patients with different neurological conditions who underwent lumbar puncture (LP) for diagnostic reasons. CSF samples were spiked with whole blood and serially diluted to 800, 400, 200, 100, 75, 50, 25, 5, 0 RBC × 106/L. CSF α-syn and Hb levels were measured by ELISA.

Results
In neat CSF, the average concentration of α-syn was 1,936 ± 636 ng/L. This value increased gradually in spiked CSF samples, up to 4,817 ± 1,456 ng/L (+149% α-syn variation) in samples with 800 RBC × 106/L.
We established different cut-offs for discriminating samples with α-syn level above 5, 10, and 20% variation, corresponding to a Hb (RBC) concentration of 1,569 μg/L (37 RBC × 106/L), 2,082 μg/L (62 RBC × 106/L), and 3,118 μg/L (87 RBC × 106/L), respectively.

Conclusions
Our data show the high impact of CSF blood contamination on CSF α-syn levels, highlighting the measurement of Hb concentration as mandatory when assessing CSF α-syn. The thresholds we calculated are useful to classify CSF samples for blood contamination, considering as reliable only those showing a Hb concentration <1,569 μg/L.
The UK’s shifting diasporic landscape: negotiating ethnolinguistic heterogeneity in Greek complementary schools post-2010
Authors: Karatsareas Petros 
Published: May 2021

AbstractAn estimated 65,000 Greek citizens migrated to the UK in 2010–2016, many of whom with the prospects of long-term settlement and with a view to provide better socioeconomic and educational opportunities to their children. Their arrival was felt across many sections of life both within and beyond the country’s pre-existing Greek-speaking communities. In this article, I present findings on how the arrival of Greek pupils, parents and qualified teachers diversified Greek complementary schools, which were previously run primarily by and for the Greek Cypriot community, and on the critical role language played in the process. Drawing on data from a set of semi-structured interviews with teachers and placing my investigation against the historical backdrop of migration from Greece and Cyprus to the UK, I show how teachers portrayed post-2010 migration as a much needed, albeit not always welcome, boost in ethnocultural vitality; how it helped to perpetuate the hierarchisation of standardised and non-standardised varieties of Greek and the stigmatisation of the multilingual and multidialectal repertoires of people with a Greek Cypriot background; and, how it put a strain on the ties that have historically brought the Greek and Greek Cypriot communities together in the context of the UK diaspora.
“Pride” and “profit”: a sociolinguistic profile of the Chinese communities in Britain
Authors: Curdt-Christiansen Xiao Lan , Huang Jing 
Published: May 2021

Abstract
In this study we explore the ongoing social changes and internal differences within the Chinese communities in Britain with regard to language practices, ethnic and cultural affiliations, and intra-community communications. Drawing on the notions of “pride” and “profit”, we report on how migratory experiences influence the linguistic practices of the Chinese communities. We argue that “pride” and “profit”, as manifested in language practices, are rooted in “prejudice”, a consequence of linguistic hierarchies related to broader socio-historical and socio-political systems. Data sources include ethnographic interviews with 15 stakeholders from three community pillars. The data analysis shows three major differences and changes within the communities: the multi-layered make-up of the Chinese communities whose members come from different countries and regions; the replacement of Cantonese with Putonghua as the communities’ lingua franca; and the increasing visibility of the Chinese communities in mainstream society. The findings suggest that social changes and language practices are associated with the social meanings of belonging, affiliation and reality, involving a form of symbolic struggle filled with “prejudice”. The study emphasises that “pride” and “profit” can make visible and explicit the sources of social inequality that tend to be concealed by neoliberal ideologies and discourses.
A disavowed community: the case of new Italian migrants in London
Authors: Pepe Giulia 
Published: May 2021

Abstract
This paper reports on a study of the language ideologies and practices of the post-2008 crisis Italian migrants in London. It explores how the traditional idea of a national migrant community is challenged by these practices. While the Italian post-2008 crisis migration continues a long tradition and has captured the attention of the media, it is understudied from a sociolinguistic point of view. Using a qualitative research approach, two sets of data were collected: recordings of social interactions in spontaneously organised gatherings, and interviews with 15 post-crisis migrants. The data show the multilingual practices of post-2008 migrants and their attitudes towards these practices. Participants recognise translanguaging as the main practice defining the community language. This seems to contrast with the disavowal of their national migrant community. They refuse to engage with traditional community sociocultural practices, challenge their membership of the Italian community in London and, in some cases, even deny the existence of the community by highlighting the internal diversity that characterises this group. Nevertheless, although the participants refuse to be seen as members of a national community, they describe their engagement with translanguaging as a community practice that determines their belonging to a migratory group. The paper argues that, through the practice of translanguaging, migrants negotiate and shape their migratory identities, and reflect on the concept of the community and its dynamics.
Language attitudes and language practices of the Lebanese community in the UK
Authors: Eid Zeina , Sallabank Julia 
Published: May 2021

Abstract
The number of Lebanese immigrants in the UK has dramatically increased in recent years, motivated by socio-political and economic pressures and in pursuit of academic excellence. This is one of the first studies to explore the attitudes of Lebanese parents and British-Lebanese children towards Arabic (Lebanese Arabic [LA] and Modern Standard Arabic [MSA]) in the UK. The data were collected from semi-structured interviews with 24 parents and 15 children, participant observation and field notes. Results indicate that Lebanese parents and children believe that HL maintenance can foster a sense of Lebaneseness, and help children access the highly valued socio-cultural significance of LA. MSA is regarded in high esteem, but Muslim children appreciate MSA more for its “pride” value than for instrumental “profit” purposes. This illustrates that the notions of “pride” and “profit” are not clear-cut in a linguistic market where multilingualism is undervalued and European foreign languages outweigh Arabic varieties, even MSA despite its global significance. For HL speakers, the “pride” value of the HL in family, culture and religion may outweigh both profit incentives and hegemonic language ideologies.
The intracellular fluid compartment is smaller than commonly believed when measured by whole-body bioimpedance
Authors: Hahn Robert G. , Giménez-Milà Marc 
Published: May 2021

Abstract

Objectives
To report our data on the total body water (TBW), intracellular volume (ICV), extracellular volume (ECV), and fat-free mass (FFM) from studies using whole-body bioimpedance (BIA) with the aim of contrasting them to commonly cited reference values.

Methods
Data were retrospectively retrieved from three single-center studies of adult healthy male volunteers and one study of women scheduled for abdominal hysterectomy where multifrequency BIA had been applied to obtain measurements of TBW, ICV, ECV, and FFM.

Results
Based on measurements performed in 44 males, the TBW, ICV, ECV, and FFM represented 49.1 (4.9)%, 23.32 (3.1)%, 25.8 (2.2)%, and 67.4 (7.4)% of the BW, respectively (mean, SD). In 15 females, these volumes were 40.4 (4.5)%, 18.0 (2.1)%, 22.4 (2.6)%, and 55.6 (6.1)% per kg BW, respectively. The deviation of these measurements from the reference values increased linearly with body weight and age.

Conclusions
Body fluid volumes indicated by BIA showed that TBW amounted to 80% of the reference volume, which is 60% per kg BW in adult males. The ratio between the ICV and the ECV was approximately 1:1, while this ratio is traditionally reported to be 2:1.
Time to say good-bye? Homeopathy, skeptics and thoughts on how to proceed
Authors: Pannek Jürgen , Pannek-Rademacher Susanne 
Published: May 2021

Abstract

Background
Although homeopathy is frequently used by many health professionals, there are ongoing debates concerning its effectiveness. Currently no unifying explanation how homeopathy works exists. Homeopaths are frequently challenged by skeptics, and in public opinion, the swan song for homeopathy is frequently sung.

Content
Regarding the efficacy of homeopathy, several well-designed RCTs, observational studies, case studies, and case reports, have been published, demonstrating its clinical efficacy. Regarding its mode of action, the discovery of the working principle of homeopathy would be a major advance towards a thorough scientific recognition of homeopathy. Basic research has already discovered some milestones, e.g., significant and reproducible effects of homeopathic preparations in plants.

Summary
To overcome the distrust of skeptics and public opinion, the support of basic research is indispensable. Second, homeopaths should continue to design prospective randomized clinical studies in order to create robust clinical evidence for the efficacy of homeopathy. Third, they should continue to publish their treatment outcomes, as these publications document clinical effectiveness beyond doubts about its mode of action.

Outlook
These measures will not only support homeopaths in continuing their clinical work, but may lead to a better recognition of this treatment in both the scientific world and the public.
Effects of 12 sessions of steam bath intervention on spirometry parameters among healthy adult volunteers
Authors: Pandiaraja M. , Vanitha A. , Maheshkumar K. , Manavalan N. , Venugopal V. 
Published: May 2021

Abstract

Objectives
Steam bath is extensively used around the world. However, its physiological influence is not understood in detail. Objective of the current study is to examine the effects of 12 sessions of steam bath on spirometric parameters among healthy participants.

Methods
Forty healthy adult volunteers of both sex (14 male and 26 female) aged 26.38 ± 8 years participated in the study. A steam bath session (10–15 min) was given once a week for 12 weeks. The spirometric parameters [forced expiratory volume in the 1st second (FEV1, L), forced vital capacity (FVC, L), FEV1/FVC ratio (absolute value), maximal mid-expiratory flow (MMEF, L/s) and peak expiratory flow (PEF, L/s)] were measured by Helios 401 spirometer.

Results
Compared to baseline, post-intervention spirometric parameters showed significant (p<0.05) improvement in the FVC (82.40 ± 11.24 to 96.91 ± 18.76), FEV1 (86.13 ± 14.39 to 91.17 ± 12.48), FEV1/FVC ratio (86.96 ± 3.64 to 93.75 ± 4.80), MMEF 25–75% (86.96 ± 3.64 to 94.16 ± 11.57), peak expiratory flow rate (79.82 ± 14.90 to 92.18 ± 18.96).

Conclusions
Taking steam bath improves the spirometric parameters of healthy adult participants. However, future randomized controlled trials with on a large scale have to done to validate our findings.
Discourses and practices of attention in video chat
Authors: Cserző Dorottya 
Published: May 2021

Abstract
This paper examines the use of video chat (VC) with a focus on expectations and construction of attention. It is based on micro analyses of recorded VC sessions (gathered between 2013 and 2015) and thematic analysis of 29 semi-structured interviews about VC practices (conducted in 2014 and 2015). Building on multimodal (inter)action analysis (Norris, S. (2004). Analysing multimodal interaction: a methdological framework. Routledge, Norris, S. (2016). Concepts in multimodal discourse analysis with examples from video conferencing. Yearbook of the Poznan Linguistic Meeting 2: 141–165) and key concepts from nexus analysis (Scollon, R. and Scollon, S.W. (2004). Nexus Analysis: Discourse and the emerging internet. Routledge), I examine how focused attention is constructed in VCs and how these practices are shaped by experiences with other forms of communication. I demonstrate that unlike other forms of distance communication, typical VC encounters require a full investment of attention. This can be formulated as an interactional maxim: focus your attention on the VC interaction. I discuss how other activities can be interwoven with a VC and examine the exceptional practice of lapsed VC encounters (previously open connections or always-on video). I argue that participants display an orientation towards the maxim when pursuing other courses of action, and that lapsed encounters operate under a different value system than typical focused VC encounters. Finally, I reason that VC is reserved for close relationships because of the required investment of attention.
Crystal structure of cyclopropane-1,2,3-triyltris(phenylmethanone), C24H18O3
Authors: Zeng Li , Tang Gui-Yun , Wang Yu-Cai , Liu Chun-Yan 
Published: May 2021

Abstract
C24H18O3, monoclinic, P21/n (no. 14), a = 5.376(2) Å, b = 15.787(6) Å, c = 21.103(7) Å, β = 92.356(6)°, V = 1789.5(11) Å3, Z = 4, R

gt
(F) = 0.0569, wR

ref
(F
2) = 0.1166, T = 296 K.