bluuebunny/crossref_metadata_2025_split huggingface dataset:
Risks associated with oral deferiprone in the treatment of infratentorial superficial siderosis
Authors: Sammaraiee Y. , Banerjee G. , Farmer S. , Hylton B. , Cowley P. , Eleftheriou P. , Porter J. , Werring D. J. 
Published: October 2019

Abstract

Objective
Deferiprone is an iron chelator that has recently been used to treat patients with infratentorial superficial siderosis (iSS). It is considered to have a generally favourable safety profile but concerns have been raised due to the risk of agranulocytosis. We aimed to evaluate the safety and tolerability of oral deferiprone as a treatment for patients with iSS.

Methods
We present a case series of 10 consecutive patients presenting with classical iSS treated with deferiprone.

Results
Ten patients were followed up for a mean period of 2.3 years (range 0.5–5.5 years). Four patients (40%) were withdrawn from treatment because of treatment-related side effects. The reasons for treatment discontinuation were neutropenic sepsis (n = 3) and fatigue (n = 1). In 2 out of the 3 cases of neutropenic sepsis, patients initially developed neutropenia without sepsis. The mean time to neutropenic sepsis following deferiprone was 1.2 years (range 0.3–2.5) with mean neutrophil count of 0.4 (range 0.3–0.5). Six patients (60%) reported no change in neurological function while on treatment, and four patients (40%) reported that their condition deteriorated.

Conclusions
Deferiprone was poorly tolerated, with 40% of patients withdrawing from treatment, most commonly due to neutropenic sepsis, after an average of 2 years on treatment. This study increases the number of reported cases of agranulocytosis in patients with iSS treated with deferiprone. Clinicians treating iSS patients with deferiprone should be aware that this drug has a potentially life-threatening side effect of neutropenic sepsis, and should ensure that appropriate haematological monitoring is in place.
Marfan syndrome in adolescence: adolescents’ perspectives on (physical) functioning, disability, contextual factors and support needs
Authors: Warnink-Kavelaars Jessica , Beelen Anita , Goedhart Tine M. H. J. , de Koning Lisanne E. , Nollet Frans , Alsem Mattijs W. , Menke Leonie A. , Engelbert Raoul H. H. 
Published: October 2019

Abstract
Although essential for providing optimal adolescent patient support, knowledge of the impact of Marfan syndrome in adolescence is limited. To explore adolescents’ perceived impact of Marfan syndrome on (physical) functioning (activities, participation), disability (limitations, restrictions), contextual factors and support needs, we interviewed 19 adolescents with Marfan syndrome. Audio-recordings were transcribed, coded and analysed using thematic analysis. Identified themes were “difficulties in keeping up with peers” and “being and feeling different from peers”. Furthermore, an adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY) model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and its contextual factors. Adolescents perceived problems in keeping up with peers in school, sports, leisure and friendships/relationships, and they could not meet work requirements. Moreover, participants perceived to differ from peers due to their appearance and disability. Contextual factors: coping with Marfan syndrome, self-esteem/image, knowledge about Marfan syndrome, support from family/friends/teachers, ability to express needs and peer-group acceptation acted individually as barrier or facilitator for identified themes.
Conclusion: Adolescents with Marfan syndrome perceived limitations and restrictions in (physical) functioning. They perceived problems in keeping up with peers and perceived to differ from peers due to their appearance and disability. This warrants awareness and tailored physical, psychosocial, educational and environmental support programmes to improve (physical) functioning and empowerment of adolescents with Marfan syndrome.What is known:• Marfan syndrome is a hereditary connective tissue disorder.• Marfan syndrome affects multiple systems.What is new:• Adolescents with Marfan syndrome perceive (1) problems in keeping up with peers in school, sports, leisure, friendships/relationships and work (2) to differ from peers due to their appearance and disability.• An adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and contextual factors.
A high-quality cucumber genome assembly enhances computational comparative genomics
Authors: Osipowski Paweł , Pawełkowicz Magdalena , Wojcieszek Michał , Skarzyńska Agnieszka , Przybecki Zbigniew , Pląder Wojciech 
Published: October 2019

AbstractGenetic variation is expressed by the presence of polymorphisms in compared genomes of individuals that can be transferred to next generations. The aim of this work was to reveal genome dynamics by predicting polymorphisms among the genomes of three individuals of the highly inbred B10 cucumber (Cucumis sativusL.) line. In this study, bioinformatic comparative genomics was used to uncover cucumber genome dynamics (also called real-time evolution). We obtained a new genome draft assembly from long single molecule real-time (SMRT) sequencing reads and used short paired-end read data from three individuals to analyse the polymorphisms. Using this approach, we uncovered differentiation aspects in the genomes of the inbred B10 line. The newly assembled genome sequence (B10v3) has the highest contiguity and quality characteristics among the currently available cucumber genome draft sequences. Standard and newly designed approaches were used to predict single nucleotide and structural variants that were unique among the three individual genomes. Some of the variant predictions spanned protein-coding genes and their promoters, and some were in the neighbourhood of annotated interspersed repetitive elements, indicating that the highly inbred homozygous plants remained genetically dynamic. This is the first bioinformatic comparative genomics study of a single highly inbred plant line. For this project, we developed a polymorphism prediction method with optimized precision parameters, which allowed the effective detection of small nucleotide variants (SNVs). This methodology could significantly improve bioinformatic pipelines for comparative genomics and thus has great practical potential in genomic metadata handling.
Pertuzumab plus trastuzumab and chemotherapy for Japanese patients with HER2-positive metastatic gastric or gastroesophageal junction cancer: a subgroup analysis of the JACOB trial
Authors: Shitara Kohei , Hara Hiroki , Yoshikawa Takaki , Fujitani Kazumasa , Nishina Tomohiro , Hosokawa Ayumu , Asakawa Takashi , Kawakami Satoe , Muro Kei 
Published: October 2019

Abstract

Background
The phase III JACOB trial (NCT01774786) compared the efficacy and safety of pertuzumab and trastuzumab plus chemotherapy with placebo and trastuzumab plus chemotherapy in patients with previously untreated human epidermal growth factor receptor 2 (HER2)-positive metastatic gastric or gastroesophageal junction cancer. We conducted a subgroup analysis in Japanese patients.

Methods
Patients were randomized 1:1 to pertuzumab 840 mg or placebo, plus trastuzumab (loading dose, 8 mg/kg; maintenance dose, 6 mg/kg) and chemotherapy (cisplatin 80 mg/m2, and capecitabine 1000 mg/m2 twice daily for 28 doses or 5-fluorouracil 800 mg/m2 every 24 h for 120 h), every 3 weeks. Continuation of chemotherapy after 6 cycles was at the discretion of the patient and the treating physician.

Results
A total of 40 Japanese patients were included in each arm. Median overall survival was 22.0 months (95% confidence interval [CI] 13.8–not evaluable) and 15.6 months (95% CI 9.7–19.2) in the pertuzumab and placebo arms, respectively (hazard ratio [HR] 0.64 [95% CI 0.37–1.10]). Median progression-free survival was 12.4 months (95% CI 6.1–14.1) in the pertuzumab arm and 6.3 months (95% CI 4.3–8.1) in the placebo arm (HR 0.50 [95% CI 0.30–0.82]). Grade ≥ 3 adverse events and serious adverse events were more frequent in the pertuzumab arm than the placebo arm.

Conclusions
Results from this subgroup analysis of the JACOB trial suggest similar efficacy of pertuzumab in Japanese patients and patients in the overall population, encouraging continued investigation of new agents for gastric cancer in Japanese patients.
Anxiety Disorders in Adults with Autism Spectrum Disorder: A Population-Based Study
Authors: Nimmo-Smith Victoria , Heuvelman Hein , Dalman Christina , Lundberg Michael , Idring Selma , Carpenter Peter , Magnusson Cecilia , Rai Dheeraj 
Published: October 2019

Abstract
Anxiety is common in children with ASD; however, the burden of specific anxiety disorders for adults with ASD is under-researched. Using the Stockholm Youth Cohort, we compared anxiety disorder diagnoses among autistic adults (n = 4049), with or without intellectual disability, and population controls (n = 217,645). We conducted additional sibling analyses. Anxiety disorders were diagnosed in 20.1% of adults with ASD compared with 8.7% of controls (RR = 2.62 [95% CI 2.47–2.79]), with greatest risk for autistic people without intellectual disability. Rates of almost all individual anxiety disorders were raised, notably obsessive–compulsive disorder and phobic anxiety disorders. Anxiety disorders were more common in full siblings and half-siblings of people with ASD. The implications of this are explored.
Health-related quality-of-life among patients with premature ovarian insufficiency: a systematic review and meta-analysis
Authors: Li X. T. , Li P. Y. , Liu Y. , Yang H. S. , He L. Y. , Fang Y. G. , Liu J. , Liu B. Y. , Chaplin J. E. 
Published: October 2019

AbstractPurposeTo systematically review studies investigating health-related quality-of-life (HrQoL) in patients with premature ovarian insufficiency (POI), to examine questionnaires used and to conduct a meta-analysis of control studies with normal ovarian function.MethodsData sources: PubMed, Embase, Web of science, CNKI, and CQVIP, searched from inception until June 2018. The search strategy was a combination of medical (e.g. POI), subjective (e.g. well-being) and methodological (e.g. questionnaires) keywords. PRISMA guidelines were used to assess outcome data quality/validity by one reviewer, verified by a second reviewer. Risk of bias within studies was evaluated. A meta-analysis compared HrQoL in patients and non-patients. Due to measurement differences in the studies, the effect size was calculated as standard mean difference.ResultsWe identified 6869 HrQoL studies. Nineteen geographically diverse studies met inclusion criteria, dated from 2006, using 23 questionnaires. The meta-analysis included six studies with 645 POI participants (age 33.3 ± 5.47) and 492 normal-ovarian control subjects (age 32.87 ± 5.61). Medium effect sizes were found for lower overall HrQoL (pooled SMD = − 0.73, 95% CI − 0.94, − 0.51;I2 = 54%) and physical function (pooled SMD = − 0.54, 95% CI − 0.69, − 0.39;I2 = 55%). Heterogeneity was investigated. Effect sizes varied for sexual function depending on the measure (SMD = − 0.27 to − 0.74), overall HrQoL (SF-36) had the largest effect size (− 0.93) in one study. The effect sizes for psychological and social HrQoL were small.ConclusionPOI is associated with low-to-medium effect size on HrQoL compared to normal ovarian controls. The greatest effects are found in general HrQoL and most sexual function areas. Condition-specific questionnaires and RCTs are recommended for further investigation.
Narrative philosophy of religion: apologetic and pluralistic orientations
Authors: Burley Mikel 
Published: October 2019

AbstractRecent decades have witnessed a growing interest in narrative both in certain areas of philosophy and in the study of religion. The philosophy of religion has not itself been at the forefront of this narrative turn, but exceptions exist—most notably Eleonore Stump’s work on biblical stories and the problem of suffering. Characterizing Stump’s approach as an apologetic orientation, this article contrasts it with pluralistic orientations that, rather than seeking to defend religious faith, are concerned with doing conceptual justice to the range of possible human perspectives, both religious and nonreligious. By discussing various examples, the article makes a case for narrative philosophy of religion, especially in its pluralistic form.
Comparison of three common whole blood platelet function tests for in vitro P2Y12 induced platelet inhibition
Authors: Dias Joao D. , Pottgiesser Torben , Hartmann Jan , Duerschmied Daniel , Bode Christoph , Achneck Hardean E. 
Published: October 2019

AbstractIn the context of interventional cardiology, platelet function testing may identify patients treated with P2Y12-inhibitors at an increased risk of mortality, thrombosis and bleeding. Several whole blood point-of-care platelet function analyzers are available; however, inter-device differences have not been examined systematically. To compare three platelet function tests under standardized in vitro conditions. Healthy volunteer (n = 10) blood samples were spiked with increasing concentrations of ticagrelor (0–7500 ng/mL) and/or ASA (0–3280 ng/mL), measured on three platelet function analyzers (TEG®6s, Multiplate®, and VerifyNow®) and respective Effective Concentration (EC) levels EC10, EC50 and EC90 were calculated. Repeatability was assessed in a separate group of pooled blood samples (n = 10) spiked with ticagrelor at EC10, EC50 and EC90. ASA had no impact on ADP-activated channels for all three devices. TEG®6s was able to distinguish (p ≤ 0.05) between all ticagrelor EC zones; VerifyNow®and Multiplate®were able to distinguish between three and two zones, respectively. Multiplate®showed the largest window between EC10 and EC90 (19–9153 ng/mL), followed by TEG®6s (144–2589 ng/mL), and VerifyNow®(191–1100 ng/mL). Drug effect models distribution of disagreements were identified for TEG®6s (5.0%), VerifyNow®(8.3%), and Multiplate®(13.3%). TEG®6s showed the smallest average coefficient of variation between EC conditions (5.1%), followed by Multiplate®(14.1%), and VerifyNow®(17.7%). Linear models could be generated between TEG®6s and Multiplate®, but not VerifyNow®. Significant differences were found between whole blood point-of-care platelet function analyzers and the clinical impact of these differences needs to be further investigated.
Vaporization of Ni, Al and Cr in Ni-Base Alloys and Its Influence on Surface Defect Formation During Manufacturing of Single-Crystal Components
Authors: Dong Z. H. , Sergeev D. , Kobertz D. , D’Souza N. , Feng S. , Müller M. , Dong H. B. 
Published: October 2019

Abstract
Vaporization and its associated surface defect formation have become one of the most important challenges in manufacturing single-crystal components. During the kinetic-influenced casting and solution heat treatment of Ni-base superalloys, elements undergo processes of vaporization and deposition causing unpredictable defects. To quantitatively examine the vaporization phenomenon, partial vapor pressures of Ni, Al and Cr in Ni-base alloys were measured in the γ phase over the temperature range of 1473 K to 1650 K using Knudsen effusion mass spectrometry. Experimental results showed that the partial pressure of Al is about two orders of magnitude lower than that of Ni and five times lower than that of Cr, revealing that the vaporization of Al is almost negligible compared with those of Ni and Cr at solution heat treatment temperatures. Variation of partial pressures during homogenization of the as-cast Ni-base alloys was measured in long-term isothermal experiments at 1573 K. It was found that Cr vapor pressure decreases by a factor of two in the first 20 hours whereas the Ni and Al remain nearly constant.
Development of Pore-Free Ti-Si-C MAX/Al-Si Composite Materials Manufactured by Squeeze Casting Infiltration
Authors: Dmitruk Anna , Naplocha Krzysztof , Żak Andrzej , Strojny-Nędza Agata , Dieringa Hajo , Kainer Karl Ulrich 
Published: October 2019

Abstract
Open-porous MAX phase skeletons from Ti3SiC2 were manufactured by Microwave-Assisted Self-propagating High-temperature Synthesis (MASHS) and subsequently subjected to squeeze casting infiltration with an Al-Si lightweight casting alloy (EN AC-44200). This alloy was chosen due to its high flowability, corrosion resistance and good machinability. The manufactured composites, together with a reference sample of the original alloy, underwent testing of thermal properties, including thermal conductivity and diffusivity, specific heat and thermal expansion in the temperature range 50-500 °C, which corresponds to the expected working temperatures of the material. The fabricated AlSi/Ti3SiC2 composites have significantly increased thermal stability, with coefficients of thermal expansion (approximately 10-11 × 10−6 °C−1) half that of the original alloy. As regards mechanical properties, the instrumental Young’s modulus and Vickers hardness of the composite materials are 170.8 and 8.5 GPa, respectively. Moreover, the microstructure and phase composition, structural defects and potential impacts between constituents of the manufactured composites were characterized using SEM, TEM and STEM microscopy and EDS and XRD analysis.
Growth responses of seedlings produced by parent seeds from specific altitudes
Authors: Iakovoglou Valasia , Takos Ioannis , Pantazi Georgia , Pipsou Aikaterini , Neofotistou Maria 
Published: October 2019

AbstractRestoration activities in semi-arid Mediterranean areas like Greece face many obstacles, such as summer droughts that are becoming more intense with climate change, that pose limitations to transplanting success. Seedlings for restoration must be of high quality; a vigorous root system is critical to enable seedlings to tolerate adverse conditions. Here we investigated the effects of altitude and source parent for seeds on the growth of subsequent seedlings to determine the best seed sources for obtaining highest-quality, most-tolerant seedlings for restoration efforts. Seeds ofQuercus cocciferaL. were collected on an altitudinal gradient of 50 m (200, 250 and 300 m a.s.l.) and from specific parents at each altitude. Subsequent seedlings were grown for 3 months in a greenhouse with controlled irrigation. The results indicated a strong altitudinal and parental seed effect on seedling characteristics. As altitude decreased, biomass of the seedlings increased, and they developed more vigorous roots and more photosynthetic leaf tissue. Thus, altitude and parent are critical factors to consider when producing seed-derived seedling. By collecting seeds from a specific altitude and parent, more vigorous and stress-tolerant seedlings can be obtained to enhance transplanting success.
Study of Uranium in Drinking Water around the Sohna Fault Line in Haryana
Authors: Chahal Anju , Kumar Suneel , Panghal Amanjeet , Kumar Ajay , Singh Joga , Singh Parminder , Bajwa B.S. 
Published: October 2019

ABSTRACT
Quality of drinking water is associated directly with the health of mass population. Therefore, study on radiation level in groundwater has been taken up around the Sohna fault line, Haryana state, India. Uranium concentration has been measured in drinking water samples collected from sources such as hand pump, tube well from different depths around the Sohna fault line, using LED Fluorimetry Technique. Uranium concentration in study area varies from 0.10 μg/L to 223.16 μg/L with an average value of 22.09 μg/L. The average value of uranium concentration is within the safe limit recommended by World Health Organization (WHO, 2011) and Atomic Energy Regulatory Board. The annual effective dose has also been measured in all the water samples and is found to be below the prescribed dose limit of 100 μSvy–1 recommended by WHO (2011). Risk assessment of uranium in water is also calculated using life time cancer risk, life time average daily dose and hazard quotient. The high uranium concentration observed in certain areas is due to interaction of ground water with the soil formation of this region and the local sub-surface geology of the region.
Biosorption of lanthanum and samarium by chemically modified free Bacillus subtilis cells
Authors: Giese Ellen C. , Jordão Caio S. 
Published: October 2019

Abstract
Previous studies showed that Bacillus subtilis possessed high physiological activity in industrial waste treatment as a biosorbent for recovery metals, and in this study, the sorption capacity for both La3+ and Sm3+ was demonstrated. The effects of lanthanide concentration and contact time were tested to acid and alkali pre-treated cells. Very high levels of removal, reaching up to 99% were obtained for both lanthanide ions. Langmuir isotherm model was applied to describe the adsorption isotherm and indicated a better correlation with experimental data R2 = 0.84 for La3+ using sodium hydroxide pre-treated free cells and R2 = 0.73 for Sm3+ to acid pre-treated B. subtilis cells as biosorbents. Results of this study indicated that chemically modified B. subtilis cells are a very good candidate for the removal of light rare-earth elements from aquatic environments. The process is feasible, reliable, and eco-friendly.
Shear force estimation in rough boundaries using SVR method
Authors: Sheikh Khozani Zohreh , Hosseinjanzadeh Hossein , Wan Mohtar Wan Hanna Melini 
Published: October 2019

AbstractThe accuracy of support vector regression (SVR) procedure in modeling the percentage of shear force carried by walls in a rectangular channel with rough boundaries was investigated. The SVR model is extended, and the more appropriate kernel function and input combination are studied. Finally, the SVR model with an exponential kernel function and three influence parameters was selected as the best SVR model with the lowest error. The output of this more appropriate SVR model is presented as a program. Then, this most appropriate SVR model is compared with three equations presented by other researchers for rough and smooth channels. The SVR model with the highest accuracy and lowest statistical values (RMSE of 0.565) performed the best compared with the other equations.