bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effect of nusinersen after three years of treatment in 61 young children with SMA type 1 or 2: a French real-life observational study
Authors: AUDIC frédérique , Dubois Sonia M , Durigneux Julien , Barnerias Christine , Isapof Arnaud , Nougues Marie-Christine , Davion Jean-Baptiste , Richelme Christian , Vuillerot Carole , Legoff Laure , Sabouraud Pascal , Cances Claude , Laugel Vincent , Ropars Juliette , Espil-Taris Caroline , Trommsdorff Valérie , Pervillé Anne , Banda Marta Gomez Garcia de la , Testard Hervé , Chouchane Mondher , Walther-Louvier Ulrike , Schweizer Cyril , Halbert Cécile , Badri Myriam , Pauly Vanessa , Quijano-Roy Susana , Chabrol Brigitte , Desguerre Isabelle 
Published: August 2022

Abstract
Background
Spinal muscular atrophy (SMA) is a rare genetic neuromuscular disorder due to an autosomal recessive mutation in the survival motor neuron 1 gene (SMN1) causing degeneration of the anterior horn cells of the spinal cord and resulting in muscle atrophy. The aim of this paper is to report a 36-month follow-up of children with SMA treated with nusinersen before the age of 3 years. Change in motor function, nutritional and ventilatory support and orthopedics outcomes were evaluated at baseline and 36 months after intrathecal nusinersen and correlated with SMA type and SMN2 copy number.
Results
We report 88% of the patients who have gained new motor skills during the 3-years period, reaching standing without help and walking with help for 12/37 and 10/37 patients harboring 3 SMN2 copies respectively. No patients with 2 copies SMN2 can stand alone or walk. Patients bearing 3 copies of SMN2 are also more likely to be preserved from respiratory, nutritional and orthopedic complications than patients with 2 SMN2 copies.
Conclusion
Eventhough SMA patients improve with nusinersen, a lower number of SMN2 gene copies remains a predictor a of poorer evolution under treatment.
Characterization of Metagenome Assembled Genomes from the International Space Station
Authors: Singh Nitin K. , Wood Jason M. , Patane Jose , Moura Livia Maria Silva , Lombardino Jonathan , Setubal João Carlos , Venkateswaran Kasthuri 
Published: August 2022

Abstract
Background
Several investigations on the microbial diversity and functional properties of the International Space Station (ISS) environment were carried out to understand the influence of spaceflight conditions on the microbial population. However, metagenome-assembled genomes (MAGs) of ISS samples are yet to be generated and subjected to various genomic analyses, including phylogenetic affiliation, predicted functional pathways, antimicrobial resistance, and virulence characteristics.
Results
In total, 46 MAGs were assembled from 21 ISS environmental metagenomes, in which metaSPAdes yielded 20 MAGs and metaWRAP generated 26 MAGs. Among 46 MAGs retrieved, 18 bacterial species were identified, including one novel genus/species combination (Kalamiella piersonii) and one novel bacterial species (Methylobacterium ajmalii). In addition, four bins exhibited fungal genomes; this is the first-time fungal genomes were assembled from ISS metagenomes. Variations in the antimicrobial resistant (AMR) and virulence genes of the selected 20 MAGs were characterized to predict the ecology and evolution of biosafety level (BSL) 2 microorganisms in space. Since microbial virulence increases in microgravity, AMR gene sequences of MAGs were compared with genomes of respective ISS isolates and corresponding type strains. Among these 20 MAGs characterized, AMR genes were more prevalent in the Enterobacter bugandensis MAG, which has been predominantly isolated from clinical samples. MAGs were further used to analyze if genes involved in AMR and biofilm formation of viable microbes in ISS have variation due to generational evolution in microgravity and radiation pressure.
Conclusions
Comparative analyses of MAGs and whole genome sequences of related ISS isolates and their type strains were characterized to understand the variation related to the microbial evolution under microgravity. The Pantoea/Kalamiella strains have the maximum single nucleotide polymorphisms found within the ISS strains examined. This may suggest that Pantoea/Kalamiella strains are much more subjective to microgravity changes. The reconstructed genomes will enable researchers to study the evolution of genomes under microgravity and low dose irradiation compared to the evolution of microbes here on Earth.
Unintentional injury deaths among children under five in Hunan Province, China, 2015-2020
Authors: Zhou Xu , Xie Zhiqun , He Jian , Lin Hong , Xiao Juan , Wang Hua , Fang Junqun 
Published: August 2022

Abstract
Objective
In China, unintentional injuries are one of the leading causes of preventable death among children under five. This study aimed to describe the epidemiological characteristics of unintentional injury death rates in children under five and to provide evidence for future preventive strategies.
Methods
We analyzed 2015–2020 data from the Under-Five Mortality Surveillance Network of Hunan Province to determine the unintentional injury death rate (the number of unintentional injury deaths per 1,000 live births, the unit is ‰) for children under five by year, residence address, sex, and age group. The compositions of unintentional injury types and epidemiological characteristics were calculated by proportion.
Results
From 2015–2020, the overall unintentional injury death rate was 1.770‰, increased by 11.54%, from 1.776‰ in 2015 to 1.981‰ in 2020, showing an upward trend (P = 0.01). Death rates were higher for children in rural than urban areas (1.883‰ vs. 1.575‰) and higher for males than females (2.055‰ vs. 1.449‰) (P < 0.05). The main fatal injury types for under-five children were unintentional drowning (39.05%), suffocation (30.32%), traffic injury (15.82%), fall (8.27%), and poisoning (2.06%). Among children aged 0–11 months, suffocation was the leading cause of unintentional injury death (82.91%), and among children aged 12–59 months, drowning was the leading cause of unintentional injury death (54.17%). Death rates of unintentional drowning, suffocation, and traffic injury were higher in rural than urban areas (P < 0.05). Death rates of unintentional drowning, suffocation, and fall were higher for males than females (P < 0.05). From 2015–2020, death rates of unintentional suffocation, fall, and poisoning showed increasing trends (P < 0.05). The maximum number of unintentional injury deaths was in February, the maximum number of unintentional drowning deaths was in July, and the maximum number of unintentional suffocation deaths was in January. Most unintentional traffic injury deaths occurred on the way to the hospital (41.58%) and in the hospital (34.08%). 49.79% of unintentional injury deaths occurred at home. 69.01% of children did not receive treatment after unintentional injury until they died, and 60.98% of unintentional injury deaths did not receive treatment because it was too late to get to the hospital.
Conclusion
In this study, the unintentional injury death rate among children under five showed an increasing trend, and we have described its epidemiological characteristics. We should focus on priority target groups and injury types to reduce the unintentional injury death rate. We suggest that preventing unintentional injuries should be priorities, and we should develop safe environments and improve residents' safety literacy and first-aid abilities.
Genetic diversity in early maturity Chinese and European elite soybeans: A comparative analysis
Authors: Yao Xindong , Xu Jiang-yuan , Liu Zhang-xiong , Pachner Martin , Molin Eva Maria , Rittler Leopold , Hahn Volker , Leiser Willmar , Gu Yong-zhe , Lu Yu-qing , Qiu Li-juan , Vollmann Johann 
Published: August 2022

Abstract
Soybean (Glycine max [L.] Merr.) is a legume crop important for global food security due to supplying high-quality vegetable protein and oil. China is the origin and rich center of soybean genetic diversity represented by numerous landraces and other genetic resources. During the 19th and 20th centuries, world-wide dissemination of plant introductions laid the foundation of modern soybean production. Due to selection for regional adaptation, present-day elite soybean cultivars do have a comparatively narrow genetic base. However, as genetic variation is the major component of future breeding progress, diversity of early maturity Chinese and European elite soybeans was comparatively analyzed using a high-throughput functional SNP array and an SSR marker set. Results revealed a clear differentiation between Chinese and European elite cultivars, and the level of genetic diversity was similar between the two populations. Both in Chinese and European cultivars, unique SSR alleles were found which indicates that selection for specific adaptation can preserve genetic variation. The European population was markedly structured by maturity groups which was less clear in the Chinese population. Structure analysis indicated that European cultivars are based on two major ancestral lines, whereas Chinese elite soybean cultivars trace back to more ancestral lines pointing to the rich natural soybean diversity of China. The results confirm that long-term selection under divergent environmental and agronomic conditions can produce specific diversity which could potentially be utilized for future enhancing both Chinese and European soybean breeding.
Prioritization of pig farm biosecurity for control of Salmonella and hepatitis E virus infections; results of a European Expert Opinion Elicitation
Authors: Galipó Erika , Zoche-Golob Veit , Sassu Elena Lucia , Prigge Christopher , Sjölund Marie , Tobias Tijs , Rzeżutka Artur , Smith Richard Piers , Burow Elke 
Published: August 2022

Abstract

Background: In the literature, there is absent or weak evidence on the effectiveness of biosecurity measures to the control ofSalmonellaspp. and hepatitis E virus (HEV) on pig farms. Therefore, the present study aimed to collect, weigh, and compare opinions from experts on the relevance of several biosecurity measures. An online questionnaire was submitted to selected experts, from multiple European countries, knowledgeable on either HEV orSalmonella, in either indoor or outdoor pig farming systems (settings). The experts ranked the relevance of eight biosecurity categories with regards to effectiveness in reducing the two pathogens separately, by assigning a score from a total of 80, and within each biosecurity category they scored the relevance of specific biosecurity measures (scale 1–5). Agreement among experts was analysed across pathogens and acrosssettings. Results: After filtering for completeness and expertise, 46 responses were analysed, with 52% of the experts identified as researchers/scientists, whereas the remaining 48% consisted of non-researchers, veterinary practitioners and advisors, governmental staff, and consultant/industrial experts. The experts self-declared their level of knowledge but neither Multidimensional Scaling nor k-means cluster analyses produced evidence of an association between expertise and the biosecurity answers, and so all experts’ responses were analysed together without weighting or adaptation. Overall, the top-ranked biosecurity categories werepig mixing;cleaning and disinfection;feed, water and bedding; andpurchase of pigs or semen, while the lowest ranked categories weretransport,equipment,animalsandhumans.Cleaning and disinfectionwas ranked highest for both pathogens in the indoor setting, whereaspig mixingwas highest for outdoorsettings. Several (94/222, 42.3%) measures across all foursettingswere considered highly relevant. Measures with high disagreement between the respondents were uncommon (21/222, 9.6%), but more frequent for HEV compared toSalmonella. Conclusions: The implementation of measures from multiple biosecurity categories was considered important to controlSalmonellaand HEV on farms, and pig mixing activities, as well as cleaning and disinfection practices, were perceived as consistently more important than others. Similarities and differences in the prioritised biosecurity measures were identified between indoor and outdoor systems and pathogens. The study identified the need for further research especially for control of HEV and for biosecurity in outdoor farming.
How frail is frail in oncology studies? A scoping review
Authors: Fletcher James A , Logan Benignus , Reid Natasha , Gordon Emily H , Ladwa Rahul , Hubbard Ruth E 
Published: August 2022

Abstract
Aims:The frailty index (FI) is one way in which frailty can be quantified. While it is measured as a continuous variable, various cut-off points have been used to categorise older adults as frail or non-frail, and these have largely been validated in the acute care or community settings for older adults without cancer. This study aimed to explore which FI categories have been applied to older adults with cancer and to determine why these categories were selected by study authors.Methods:This scoping review searched Medline, EMBASE, and Cochrane databases for studies which measured and categorised an FI in adults with cancer. Of the 1165 screened, 40 were eligible for inclusion. Data including oncological setting, FI categories, and the references or rationale for categorisation were extract and analysed.Results:The FI score used to categorise participants as frail ranged from 0.06 to 0.35, with 0.35 being the most frequently used, followed by 0.25 and 0.20. The rationale for FI categories was provided in most studies but was not always relevant. Three of the included studies using an FI > 0.35 to define frailty were frequently referenced as the rationale for subsequent studies, however the original rationale for this categorisation was unclear. Few studies sought to determine or validate optimum FI categorises in this population.Conclusion:There is significant variability in how studies have categorised the FI in older adults with cancer. An FI ≥ 0.35 to categorise frailty was used most frequently, however an FI in this range has often represented at least moderate to severe frailty in other studies. These findings contrast with a scoping review of highly cited studies categorising FI in older adults without cancer, where an FI ≥ 0.25 was most common. Maintaining the FI as a continuous variable is likely to be beneficial until further validation studies determine optimum FI categories in this population. Disparities in how the FI has been categorised, and indeed how older adults have been labelled as ‘frail’, limits our ability to synthesise results and to understand the impact of frailty in cancer care.
Supplemental Clostridium butyricumon modulates lipid metabolism of IUGR suckling piglets through reshaping the composition of gut microbiota and bile acid profile
Authors: Zhang Xin , Yun Yang , Lai Zheng , Ji Shuli , Yu Ge , Xie Zechen , Zhang Hao , Zhong Xiang , Wang Tian , Zhang Lili 
Published: August 2022

Abstract
Background
Intrauterine growth retardation (IUGR) can cause lipid disorders in infants and have long-term adverse effects on their growth and development. Clostridium butyricumon, emerging as a probiotic, has been reported to attenuate dysfunctions in lipid metabolism with effect. Therefore, the objective of this study was to investigate the effects of C. butyricum supplementation on hepatic lipid disorders in IUGR suckling piglets and to explore its underlying mechanism.
Methods
sixteen IUGR and eight normal birth weight (NBW) neonatal male piglets were divided into three groups. Besides drinking milk, these neonates were orally gavaged with 10 mL sterile saline (NBW-CON and IUGR-CON groups) or bacterial fluid containing 2 × 108 CFU/kg C. butyricum (IUGR-CB group) once in the morning for 3 to 24 d.
Results
Piglets with IUGR showed retarded growth performance, impaired hepatic morphology, disordered lipid metabolism, increased abundance of opportunistic pathogens and altered bile acid (BA) profile of ileum and liver. C. butyricum supplementation reshaped the gut microbiota of IUGR piglets and was characterized by a decreased abundance of opportunistic pathogens in the ileum. Among these opportunistic pathogens, Streptococcus and Enterococcus are also bile salt hydrolase (BSH)-producing microbes, and the decrease of them increased the content of conjugated BAs, which could be transported to liver and functioned as signaling molecules to activate liver X receptor α (LXRα) and farnesoid X receptor (FXR). Their activation effectively accelerated the synthesis and oxidation of fatty acids and down-regulated the total cholesterol level by decreasing the synthesis and promoting the efflux of cholesterol. As a result, the final body weight of the IUGR piglets and the morphological structure of their livers improved.
Conclusions
These results indicate that supplemental 2 × 108 CFU/kg C. butyricum could decrease the abundance of Streptococcus and Enterococcus, which were considered BSH-producing microbes. Their decrease altered the BA profile of the ileum and liver and, as a result, activated the expression of hepatic LXRα and FXR. The activation of these two signalling molecules could effectively normalize lipid metabolism and improve the growth performance of IUGR suckling piglets.
Peroxisome proliferator-activated receptor-α activation by aspirin facilitates contextual fear extinction and modulates intrinsic excitability of dentate gyrus neurons
Authors: Zhang Di , Xiang Guo , Liu Xia , Wang Jiangong , Lu Shunshun , Yu Meng , Zhang Yuhan , Sun Bin , Huang Bin , Lu Xin-Yun , Li Xingang 
Published: August 2022

Abstract
Post-traumatic stress disorder (PTSD) is characterized by the incapability to extinguish learned fear. The persistent expression of fear and the impairment in fear extinction are often caused by the loss of contextual modulation of fear memories. The dentate gyrus (DG) of the hippocampus encodes contextual information associated with fear, and its activity is required for contextual fear acquisition and extinction. However, the molecular mechanisms underlying the DG-modulation on contextual fear are not well understood. Here we report that Peroxisome Proliferator-Activated Receptor-α (PPARα) in the DG is critical for maintaining the intrinsic excitability of DG granule neurons and is required for the extinction of contextual fear. Moreover, activation of PPARα by aspirin exerted a bi-phase modulation on DG granule neurons excitability and facilitated contextual fear extinction. Furthermore, using RNA-Seq transcriptome, we further identified Npsr1 as the downstream molecule mediating effects of PPARα on modulating DG function. Our findings revealed the direct evidence linking PPARα activation with DG neuronal excitability and contextual fear extinction and provide the biological basis of aspirin to assist extinction-based exposure therapies for PTSD.
Low field slice-selective ZTE imaging ofultra-short T2 tissues based on spin-locking
Authors: Morata Jose Borreguero , Conde Fernando Galve , Guisado Jose Miguel Algarín , Baviera Jose María Benlloch , Otamendi Joseba Alonso 
Published: August 2022

Abstract
Magnetic Resonance Imaging (MRI) of hard biological tissues is very challenging due to small proton abundance and to ultra-short T2 decay times, especially at low magnetic fields where sample magnetization is weak. While several pulse sequences, such as Ultra-short Echo Time(UTE), Zero Echo Time (ZTE) and SWeep Imaging with Fourier Transformation (SWIFT), have been developed to cope with ultra-short lived MR signals, only the latter two hold promise of imaging tissues with sub-millisecond T2 times at low fields. All these sequences are intrinsically volumetric, thus 3D, because standard slice selection using a long soft radio-frequency pulse is incompatible with ultra-short lived signals. The exception is UTE, where double half pulses can perform slice selection,although at the cost of doubling the acquisition time. Here we demonstrate that spin-locking is a very versatile and robust method for slice selection for ultra-short lived signals, and present three ways of joining this pulse sequence to ZTE imaging of the selected slice. With these tools,we demonstrate slice-selected 2D ex vivo imaging of the hardest tissues in the body at low field (260mT) within clinically acceptable times.
Effects of Atropine and Tropicamide on Ocular Biological Parameters in Children: A Prospective Observational Study
Authors: Tao Yulin , Tan Jian , Huang Jing , Cheng Xiaokang , Xie Ping , Liu Xiansheng , Zhou Qiong , Ouyang Jun 
Published: August 2022

Abstract
Background
The effectiveness of cycloplegia in delaying the progression of myopia and its application in refractive examination in children have been extensively studied, but there are still few studies on the effects of atropine/tropicamide on ocular biological parameters. Therefore, the purpose of this study is to explore the effects of atropine/tropicamide on children's ocular biological parameters in different age groups, and the differences between them.
Methods
This was a prospective observational study in which all school children were examined for diopter and ocular biological parameters in the outpatient clinic, and 1% atropine and tropicamide were applied for the treatment. After examination, we enrolled the patients grouped by age (age from 2 to 12 years treated by atropine, 55 cases; age from 2 to 10 years treated by tropicamide, 70 cases; age from 14 to 17 years treated by tropicamide, 70 cases). The ocular biological parameters of each patient before and after cycloplegia were measured and the difference value and its absolute value were calculated for statistical analysis using independent-samples t-test.
Results
There was no significant difference in the difference value of ocular biological parameters and the absolute value of the difference in the same age groups before and after cycloplegia (P > 0.05). There were significant statistical differences in the corresponding values of AL, K1 and ACD among different age groups (P < 0.05). Before cycloplegia, there were significant differences in AL, K, K1, K2 and ACD in different age groups (P < 0.05). However, the differences in AL, K, K1, K2 and ACD among different age groups disappeared after cycloplegia (P > 0.05).
Conclusions
This study demonstrated that atropine/tropicamide have different effects on cycloplegia in children of different ages. The effects of atropine/tropicamide on ocular biological parameters should be fully taken into account when evaluating the refractive state before refractive surgery or calculating the degree of intraocular lens before cataract surgery.
Explicitly Modeling Relationships between Domain-Specific and Domain-Invariant Interests for Cross-Domain Recommendation
Authors: Zang Tianzi , Zhu Yanmin , Zhang Ruohan , Zhu Jing , Tang Feilong 
Published: August 2022

Abstract

This paper focuses on cross-domain recommendation (CDR) without auxiliary information. Existing works on CDR all ignore the bi-directional transformation relationships between users' domain-invariant interests and domain-specific interests. Moreover, they only rely on the sparse interactions as supervised signals for model training, which can not guarantee the generated representations are effective. In response to the limitation of these existing works, we propose a model named MRCDR which explicitly models relationships between domain-specific and domain-invariant interests for cross-domain recommendation. We project the domain-specific representations of users to a common space generating their domain-invariant representations. To remedy the problem of insufficient supervised signals, we propose two strategies that generate extra self-supervision signals to enhance model training. The aligned strategy tries to make the two domain-invariant representations an overlapped user to be consistent. The cycle strategy tries to make the reversely projected representation of the domain-invariant representation of a non-overlapped user to be consistent with its original domain-specific representation. We conduct extensive experiments on real-world datasets and the results show the effectiveness of our proposed model against the state-of-the-art methods.