bluuebunny/crossref_metadata_2025_split huggingface dataset:
Validation of the pedHALshort and HALshort in Dutch children and adults with haemophilia
Authors: Kuijlaars Isolde A. R. , van der Net Janjaap , van Vulpen Lize F. D. , Driessens Mariette H. E. , Schols Saskia E. M. , Tan Melanie , Gouw Samantha C. , Fischer Kathelijn 
Published: July 2022

AbstractIntroductionThe Haemophilia Activities List (HAL) and paediatric HAL assess self‐reported limitations in various daily activities. To reduce patient burden, shorter versions of the pedHAL (22 items) and HAL (18 items) have been developed.AimThis study aimed to determine the agreement between the pedHAL/HALfull and pedHAL/HALshort and construct validity and internal consistency of the pedHAL/HALshort in persons with haemophilia (PWH).MethodsA cross‐sectional secondary analysis of the Hemophilia in the Netherlands‐6 national survey was performed. Adult and paediatric PWH completed the original pedHAL/HALfull, from which pedHAL/HALshort were derived. Score differences between the original and short versions were calculated. Construct validity was studied by testing hypotheses regarding the relationship of the pedHAL/HALshort with the pedHAL/HALfull, Haemophilia & Exercise Project Test‐Questionnaire (HEP‐Test‐Q), Canadian Haemophilia Outcomes‐Kids’ Life Assessment Tool (CHO‐KLAT) and RAND 36‐item Health Survey (RAND‐36) (convergent/discriminant validity) as well as its ability to discriminate between subgroups (known‐group validity). Internal consistency was assessed with Cronbach's α.ResultsWe included 113 children (median 10y [range 4–17], 53% severe haemophilia) and 691 adults (median 51y [range 18–88], 35% severe). Scores of the pedHAL/HALfull and pedHAL/HALshort were similar with high correlations (>0.9). Construct validity was confirmed for the pedHAL/HALshort. The HALshort was able to discriminate between different disease severities and ages. Cronbach's α of the pedHAL/HALshort was 0.95–0.97.ConclusionThis study confirmed the agreement between the pedHAL/HALfull and the pedHAL/HALshort and the construct validity of the pedHAL/HALshort. The next step is to study construct validity of the pedHAL/HALshort when administered as short forms.
Development and spread of health literacy eLearning: A partnership across Scotland and England
Authors: Carlyle Ruth , Thain Annette , James Sally 
Published: July 2022

AbstractWith international health challenges, there are opportunities for collaboration between nations on health issues, including developing and sharing resources for teaching and learning. This article outlines collaboration across Scotland and England to develop a core resource for eLearning on health literacy. It describes the development of the resource with case studies of the implementation in Scotland and England, demonstrating the balance between shared development and tailored implementation. The eLearning was developed to increase awareness of NHS workforce and community partners, supplemented by training for NHS librarians and public health specialists to enable them to provide more tailored training on health literacy techniques.
Fear of progression in patients with mild or common type COVID‐19
Authors: Ding Shu , Dong Liang , Chen Lei , Gao Fengli 
Published: July 2022

AbstractAimTo investigate the current condition and degree of fear of disease progression and associated factors in patients with mild or common type COVID‐19.BackgroundAt the end of 2019, COVID‐19 spread from Wuhan in Hubei Province throughout China. Confirmed cases and deaths have since been reported in many countries around the world. However, fear of progression in these patients has been poorly explored.MethodsDuring February 2020, we recruited 114 patients with mild or common type COVID‐19 admitted to a Fangcang shelter hospital. We assessed patients' degree of fear using the simplified Fear of Progression Questionnaire (Chinese version). Multiple regression analysis was applied to explore potential factors.ResultsThe fear of disease progression scores of patients with mild or common COVID‐19 was at the low‐to‐moderate level. Current unemployment, disease duration of 28 days or more and not having a spouse diagnosed with COVID‐19 were factors potentially associated with fear of progression.ConclusionWith a high prevalence of fear of disease progression in patients with COVID‐19, the risk of psychological effects from the pandemic is significant and fear of progression is one of the manifestations. The need for psychological support services for patients should be included in all pandemic and disaster planning.
Association of cooking fuel with incident hypertension among adults in China: A population‐based cohort study
Authors: Peng Yue , Wang Yu , Wu Fei , Chen Yongjie 
Published: July 2022

AbstractWith an increasing prevalence of hypertension, indoor air‐pollution factors began to attract extensive attention. However, the association of cooking fuel with the incidence of hypertension was inconsistent. The aim of this study was to investigate the association of household air‐pollution caused by cooking fuel with the incidence of hypertension. Data were derived from the China Health and Nutrition Survey. Participants aged 18 years or older were eligible. A validated questionnaire was used to collect the information on the type of cooking fuel, including electricity, natural gas, coal, and wood/charcoal. Participants with a systemic blood pressure (SBP) ≥ 140 mmHg or /and a diastolic blood pressure (DBP) ≥ 90 mmHg without use of anti‐hypertensive medications, or participants with an SBP/DBP < 140/90 mmHg but having hypertensive history or currently being taking anti‐hypertensive medication were identified as hypertension. Multilevel Cox regressions were employed to examine the association of cooking fuel with incident hypertension. Compared to participants using electricity, participants using wood/charcoal had a higher incidence of hypertension (HR: 1.581; 95% CI: 1.373‐1.821; and P < .001), which was independent of sex and living areas. Furthermore, this significant association was observed only in the participants aged 18–39 years (HR: 1.443; 95% CI: 1.131‐1.840; and P = .003). Compared to participants using non‐polluting energy, participants using solid fuel were more likely to develop hypertension (HR: 1.309; 95% CI: 1.191‐1.439; and P < .001). In conclusion, household air‐pollution was associated with the incidence of hypertension among Chinese adults. Using wood/charcoal or solid fuel in youth was associated with a higher incidence of hypertension later in life.
A low‐cost storage for horticulture commodities for enhancing farmer's income: An overview on evaporative cooling
Authors: Patel Deep P. , Jain Sanjay Kumar , Lakhawat Shreedhar Singh , Wadhawan Nikita 
Published: July 2022

AbstractIndia is one of the leading countries in the production of horticultural produces. Still, the lack of scientific knowledge, infrastructure, and nonexistence of proper storage facilities forces the farmers to sell their produce in the nearby market immediately after harvest. Although refrigeration and cold storage facilities are developing now to store the horticultural produce, they are not only energy‐intensive but also require huge investment. The majority of farmers cannot afford these facilities even on a rental basis for their harvest. The metabolism of fresh horticultural commodities continues even after the harvest and the rate of deterioration rises because their maturation, senescence, and unfavorable atmospheric factors predominate, especially in tropical countries. A low‐cost, energy‐efficient, environmentally benign and made from the locally available material evaporative cooling chamber (ECC) may resolve some of these issues of on‐farm storage. It is an efficient and cost‐effective way to reduce the temperature and increase relative humidity inside the chamber. A lot of evaporative cooling structures were developed, but still, this simple technology did not make a dent in the production catchment rural areas. In this article, an attempt is made to review the basic principle, methods, factors that influence storage conditions of perishables, design consideration of different evaporative cooling systems, major limitations, challenges, and constraints of its low adoption. The recent developments for modifications and improvement in existing ECCs using the latest technological interventions with the help of Artificial Intelligence and Internet of Things and their cost analysis are also highlighted.Practical ApplicationsAdoption of a low‐cost and environmentally friendly evaporative cooling system for on‐farm storage made from locally available materials will reduce postharvest losses and improve the economic status of farmers.
Life‐cycle energy and environmental emissions of cargo ships
Authors: Zhang Yiqi , Chang Yuan , Wang Changbo , Fung Jimmy C. H. , Lau Alexis K. H. 
Published: July 2022

AbstractMaritime shipping is under increasing pressure to alleviate its environmental impact. To this end, life‐cycle footprint accounting provides a foundation for taking targeted measures in the green transition of shipping. This study used the process‐based hybrid life‐cycle inventory (LCI) modeling approach to estimate the “cradle‐to‐propeller” footprint of ships, including primary energy consumption, carbon dioxide emissions, and sulfur dioxide emissions. We used the input–output LCI model to calculate the embodied energy and emissions associated with the material and fuel use of ship manufacturing. We used a bottom‐up emission model and global marine traffic data to estimate the operational footprint of different types of ships. Based on 382 cargo ships (including bulk carriers, container ships, and general cargo ships) constructed in mainland China between 2011 and 2015, we estimated that the embodied footprint accounted for <10% of the cradle‐to‐propeller footprint under the pre‐2019 policy scenario. In terms of life‐cycle energy intensity (MJ/nm/1000 deadweight tonnage [DWT]), the large bulk carrier (>100,000 DWT) establishes the lowest value (46), followed by the small (0–100,000 DWT) bulk carrier (96), the large container ship (133), the small container ship (196), and the small general cargo (238). The bulk carrier was identified as the most energy efficient among the three ship types, and ships with larger capacities (i.e., DWT) had higher energy efficiencies than ships with lower capacities. Our study provides a comprehensive understanding of the life‐cycle footprints of cargo ships, thus enabling better evidence‐based policymaking to transition the global marine‐shipping industry to a future of greener energy.
Responsiveness to priority‐based affirmative action policy in school choice
Authors: Dur Umut , Xie Yifan 
Published: July 2022

AbstractWe consider the priority‐based affirmative action policy in school choice. We weaken the responsiveness to affirmative action policy by requiring at least one minority student to be weakly better off when their priorities are improved. We find that under both the student‐proposing deferred acceptance mechanism and the top trading cycles mechanism at least one minority student becomes weakly better off when their priorities are improved. We also strengthen the responsiveness to affirmative action policy by requiring all minority students to be weakly better off when their priorities are improved. We call this property strict responsiveness to affirmative action policy. We find that there is no nonwasteful, individually rational, mutually best, and strategy‐proof mechanism that is strictly responsive to affirmative action policy. We then find a sufficient condition for the affirmative action policy to satisfy for the student‐proposing deferred acceptance mechanism to be strictly responsive to affirmative action policy by setting restrictions on the priority improvements made by the policy.
Structure‐aware dehazing of sewer inspection images based on monocular depth cues
Authors: Xia Zixia , Guo Shuai , Sun Di , Lv Yaozhi , Li Honglie , Pan Gang 
Published: August 2022

AbstractIn sewer pipes, haze caused by the humid environment seriously impairs the quality of closed‐circuit television (CCTV) images, which leads to poor performance of subsequent pipe defects detection. Meanwhile, the complexity of sewer images, such as steep depth change and extensive textureless regions, brings great challenges to the performance or application of general dehazing algorithms. Therefore, this study estimates sewer depth maps first with the help of the water–pipewall borderlines to produce the paired dehazing dataset. Then a structure‐aware nonlocal network (SANL‐Net) is proposed with the detected borderlines and the dehazing result as two supervisory signals. SANL‐Net shows its superiority over other state‐of‐the‐art approaches with 147 in mean square error (MSE), 27.28 in peak signal to noise ratio (PSNR), 0.8963 in structural similarity index measure (SSIM), and 15.47M in parameters. Also, the outstanding performance in real image dehazing implies the accuracy of depth estimation. Experimental results indicate that SANL‐Net significantly improves the performance of defects detection tasks, such as an increase of 23.16% in mean intersection over union (mIoU) for semantic segmentation.
The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment
Authors: Fiorillo Chiara , Capodivento Giovanna , Geroldi Alessandro , Tozza Stefano , Moroni Isabella , Mohassel Payam , Cataldi Matteo , Campana Chiara , Morando Simone , Panicucci Chiara , Pedemonte Marina , Brolatti Noemi , Siliquini Sabrina , Traverso Monica , Baratto Serena , Debellis Doriana , Magri Stefania , Prada Valeria , Bellone Emilia , Salpietro Vincenzo , Donkervoort Sandra , Gable Kenneth , Gupta Sita D. , Dunn Teresa M. , Bönnemann Carsten G. , Taroni Franco , Bruno Claudio , Schenone Angelo , Mandich Paola , Nobbio Lucilla , Nolano Maria 
Published: July 2022

AbstractAimsSPTLC1‐related disorder is a late onset sensory‐autonomic neuropathy associated with perturbed sphingolipid homeostasis which can be improved by supplementation with the serine palmitoyl‐CoA transferase (SPT) substrate, l‐serine. Recently, a juvenile form of motor neuron disease has been linked to SPTLC1 variants. Variants affecting the p.S331 residue of SPTLC1 cause a distinct phenotype, whose pathogenic basis has not been established. This study aims to define the neuropathological and biochemical consequences of the SPTLC1 p.S331 variant, and test response to l‐serine in this specific genotype.MethodsWe report clinical and neurophysiological characterisation of two unrelated children carrying distinct p.S331 SPTLC1 variants. The neuropathology was investigated by analysis of sural nerve and skin innervation. To clarify the biochemical consequences of the p.S331 variant, we performed sphingolipidomic profiling of serum and skin fibroblasts. We also tested the effect of l‐serine supplementation in skin fibroblasts of patients with p.S331 mutations.ResultsIn both patients, we recognised an early onset phenotype with prevalent progressive motor neuron disease. Neuropathology showed severe damage to the sensory and autonomic systems. Sphingolipidomic analysis showed the coexistence of neurotoxic deoxy‐sphingolipids with an excess of canonical products of the SPT enzyme. l‐serine supplementation in patient fibroblasts reduced production of toxic 1‐deoxysphingolipids but further increased the overproduction of sphingolipids.ConclusionsOur findings suggest that p.S331 SPTLC1 variants lead to an overlap phenotype combining features of sensory and motor neuropathies, thus proposing a continuum in the spectrum of SPTLC1‐related disorders. l‐serine supplementation in these patients may be detrimental.
Leadless pacemakers as a new alternative for pacemaker lead‐related superior vena cava syndrome: A case report
Authors: Curnis Antonio , Milidoni Antonino , Arabia Gianmarco , Bontempi Luca , Cerini Manuel , Salghetti Francesca , Ahmed Ashraf , Ferraresi Roberto , Mitacchione Gianfranco , Mascioli Giosuè 
Published: August 2022

AbstractSuperior vena cava (SVC) syndrome is a rare disease induced by thrombosis and consequent occlusion of SVC, negatively affecting morbidity and mortality. The incidence of SVC syndrome from central venous catheters and pacemaker or defibrillator leads is increasing. Optimal treatment of pacemaker or defibrillator‐related SVC syndrome is not well defined. Lead extraction causes mechanical trauma to the vessel wall. In addition, subsequent device implantation on the contralateral side can be an added factor for venous occlusion. The use of leadless pacemakers could be an interesting option to reduce the risk of SVC restenosis after lead extraction. We report a clinical case of PM leads‐related SVC syndrome referred to our centers and treated with transvenous lead extraction, leadless pacemaker implantation and subsequent percutaneous angioplasty and stenting of the SVC and left innominate vein.
Gardenia carotenoid cleavage dioxygenase 4a is an efficient tool for biotechnological production of crocins in green and non‐green plant tissues
Authors: Zheng Xiongjie , Mi Jianing , Balakrishna Aparna , Liew Kit Xi , Ablazov Abdugaffor , Sougrat Rachid , Al‐Babili Salim 
Published: July 2022

SummaryCrocins are beneficial antioxidants and potential chemotherapeutics that give raise, together with picrocrocin, to the colour and taste of saffron, the most expensive spice, respectively. Crocins are formed from crocetin dialdehyde that is produced in Crocus sativus from zeaxanthin by the carotenoid cleavage dioxygenase 2L (CsCCD2L), while GjCCD4a from Gardenia jasminoides, another major source of crocins, converted different carotenoids, including zeaxanthin, into crocetin dialdehyde in bacterio. To establish a biotechnological platform for sustainable production of crocins, we investigated the enzymatic activity of GjCCD4a, in comparison with CsCCD2L, in citrus callus engineered by Agrobacterium‐mediated supertransformation of multi genes and in transiently transformed Nicotiana benthamiana leaves. We demonstrate that co‐expression of GjCCD4a with phytoene synthase and β‐carotene hydroxylase genes is an optimal combination for heterologous production of crocetin, crocins and picrocrocin in citrus callus. By profiling apocarotenoids and using in vitro assays, we show that GjCCD4a cleaved β‐carotene, in planta, and produced crocetin dialdehyde via C30 β‐apocarotenoid intermediate. GjCCD4a also cleaved C27 β‐apocarotenoids, providing a new route for C17‐dialdehyde biosynthesis. Callus lines overexpressing GjCCD4a contained higher number of plastoglobuli in chromoplast‐like plastids and increased contents in phytoene, C17:0 fatty acid (FA), and C18:1 cis‐9 and C22:0 FA esters. GjCCD4a showed a wider substrate specificity and higher efficiency in Nicotiana leaves, leading to the accumulation of up to 1.6 mg/g dry weight crocins. In summary, we established a system for investigating CCD enzymatic activity in planta and an efficient biotechnological platform for crocins production in green and non‐green crop tissues/organs.
Patient and caregiver perspectives on delayed childhood Gorlin syndrome diagnoses
Authors: Neale Holly , Breneiser Julie A. , Hawryluk Elena B. 
Published: August 2022

AbstractThe diagnostic trends of Gorlin syndrome (GS) in the pediatric population are not well understood. In an international survey conducted by the Gorlin Syndrome Alliance, 118 individuals who were diagnosed with GS when aged 18 years and under provided information about their diagnosis. Oral surgeons and dermatologists were the most commonly reported physicians involved in diagnosis for 48.3% and 28% of cases, respectively. For 50% of children, the diagnosis was made within a year from presenting sign(s), while 27% report over 4 years to receive GS diagnosis. Of individuals who reported >4 years between presenting signs and diagnosis, 81.3% attributed the delay to insufficient medical team knowledge and 65.6% attributed to lack of personal awareness that presenting signs were related to GS, emphasizing the need for patient and physician education of GS for prompt diagnosis.
Kynurenine metabolism is altered in mdx mice: a potential muscle to brain connection
Authors: Copeland Emily N. , Watson Colton J. F. , Whitley Kennedy C. , Baranowski Bradley J. , Kurgan Nigel , MacNeil Adam J. , MacPherson Rebecca E. K. , Fajardo Val A. , Allison David J. 
Published: August 2022

New Findings
What is the central question in this study?
Promoting muscle health with regular aerobic exercise can improve mental health through a kynurenine metabolic pathway: do conditions of muscle disease such as muscular dystrophy negatively influence this pathway?

What is the main finding and its importance?
The DBA/2J mdx model of Duchenne muscular dystrophy exhibits altered kynurenine metabolism with less kynurenic acid and peroxisome proliferator‐activated receptor‐γ coactivator 1‐α and higher levels of tumour necrosis factor α mRNA – results associated with anxiety‐like behaviour.

AbstractRegular exercise can direct muscle kynurenine (KYN) metabolism toward the neuroprotective branch of the kynurenine pathway thereby limiting the accumulation of neurotoxic metabolites in the brain and contributing to mental resilience. However, the effect of muscle disease on KYN metabolism has not yet been investigated. Previous work has highlighted anxiety‐like behaviours in approximately 25% of patients with Duchenne muscular dystrophy (DMD), possibly due to altered KYN metabolism. Here, we characterized KYN metabolism in mdx mouse models of DMD. Young (8–10 week old) DBA/2J (D2) mdx mice, but not age‐matched C57BL/10 (C57) mdx mice, had lower levels of circulating kynurenic acid (KYNA) and lower KYNA:KYN ratio compared with their respective wild‐type (WT) controls. While both C57 and D2 mdx mice displayed signs of anxiety‐like behaviour, spending more time in the corners of the arena during a novel object recognition test, this effect was more prominent in D2 mdx mice. Correlational analysis detected a significant negative association between KYNA:KYN levels and time spent in corners in D2 mice, but not C57 mice. In extensor digitorum longus muscles from D2 mdx mice, but not C57 mdx mice, we found lowered protein levels of peroxisome proliferator‐activated receptor‐γ coactivator 1‐α and kynurenine amino transferase‐1 enzyme when compared with WT. Furthermore, D2 mdx quadriceps muscles had the highest level of tumour necrosis factor α expression, which is suggestive of enhanced inflammation. Thus, our pilot work shows that KYN metabolism is altered in D2 mdx mice, with a potential contribution from altered muscle health.
NACA Sonic-Flow-Orifice Temperature Probe in High-Gas-Temperature Measurement
Authors: Blackshear P. L. 
Published: July 2022

Abstract
A brief discussion of the problem of measuring temperatures in jet engines is given. A method of measuring these temperatures by pneumatic means is described. This method involves the use of two sonic-flow orifices in series to give, through thermodynamic calculations, the total temperature of the gas entering the first orifice. An evaluation of this method is given including a discussion of the sampling error in nonhomogeneous gases, and an experimental comparison against a thermocouple and the sodium D-line reversal method is made showing favorable agreement. Results of applying this method of measuring temperature in full-scale engines are discussed.
Relative Abrasiveness of the Cast Surfaces of Various Gray-Iron Castings on Single-Point Tools of High-Speed Steel
Authors: Datsko Joseph , Boston O. W. 
Published: July 2022

Abstract
This study reveals that plain gray-iron castings made in sand molds have a very abrasive surface that results in a low tool life when machining the surface. This condition can be eliminated almost completely by pickling the castings prior to machining. Annealing improves the machinability of the casting, but it also results in a more pronounced “skin effect.” By means of an annealing and pickling treatment, the cutting speed for removing the surface was increased by 230 per cent.