bluuebunny/crossref_metadata_2025_split huggingface dataset:
Burden of illness of Pompe disease in patients only receiving supportive care
Authors: Kanters Tim A. , Hagemans Marloes L. C. , van der Beek Nadine A. M. E. , Rutten Frans F. H. , van der Ploeg Ans T. , Hakkaart Leona 
Published: April 2011

AbstractBackgroundPompe disease is an orphan disease for which enzyme replacement therapy (ERT) recently became available. This study aims to estimate all relevant aspects of burden of illness—societal costs, use of home care and informal care, productivity losses, and losses in health‐related quality of life (HRQoL)—for adult Pompe patients only receiving supportive care.MethodsWe collected data on all relevant aspects of burden of illness via a questionnaire. We applied a societal perspective in calculating costs. The EQ‐5D was used to estimate HRQoL.ResultsEighty adult patients (87% of the total Dutch adult Pompe population) completed a questionnaire. Disease severity ranged from mild to severe. Total annual costs were estimated at €22,475 (range €0–169,539) per adult Pompe patient. Patients on average received 8 h of home care and 19 h of informal care per week. Eighty‐five percent of the patients received informal care from one or more caregivers; 40% had stopped working due to their disease; another 20% had reduced their working hours. HRQoL for Pompe patients who only received supportive care was estimated at 0.72, 17% lower than the Dutch population at large.ConclusionsAdult Pompe disease is associated with a considerable burden of illness at both the societal and patient levels. The disease leads to substantial costs and dependency on medical devices, home care, and informal care, and has a high impact on the patient's social network. In addition, patients are limited in their ability to work and have significantly reduced HRQoL.
Newborn screening: how are we travelling, and where should we be going?
Authors: Wilcken Bridget 
Published: April 2011

AbstractIn general, newborn screening is now a highly successful enterprise. The introduction of tandem mass spectrometry in the mid‐1990s changed the pace of screening, raising its profile and increasing its relevance to a wider range of health professionals. The clinical effectiveness is not in doubt for some conditions, but is lacking for others. Evaluation has major difficulties for the rarer disorders and has been sadly neglected. Partly because clinical effectiveness has not been enthusiastically addressed, but also because of undue caution on the part of regulators, who often seem to ignore available evidence, there are huge differences in the adoption of screening programmes in different jurisdictions. New treatments, especially mutation‐specific treatments, and technological advances in diagnostic testing are being rapidly developed, and this will further change the face of newborn screening and probably magnify these differences. The challenges will be considerable, especially with the increasing availability of DNA testing at modest cost. It is likely that there will be pressure to change the aims of newborn screening to encompass “personalised medicine”. We must all prepare in a thoughtful way for these future challenges.
Heteronormativity, school climates, and perceived safety for gender nonconforming peers
Authors: Toomey Russell B. , McGuire Jenifer K. , Russell Stephen T. 
Published: April 2011

ABSTRACTStudents’ perceptions of their school climates are associated with psychosocial and academic adjustment. The present study examined the role of school strategies to promote safety in predicting students’ perceptions of safety for gender nonconforming peers among 1415 students in 28 high schools. Using multilevel modeling techniques, we examined student‐ and school‐level effects on students’ perceptions of safety for gender nonconforming peers. We found that older students, bisexual youth, Latino youth, and youth who experienced school violence perceived their gender nonconforming male peers to be less safe. Similarly, we found that older students and students who experienced school violence and harassment due to gender nonconformity perceived their gender nonconforming female peers to be less safe. At the school‐level, we found that when schools included lesbian, gay, bisexual, transgender, and queer (LGBTQ) issues in the curriculum and had a Gay‐Straight Alliance, students perceived their schools as safer for gender nonconforming male peers.
Brief report: Emotional intelligence, victimisation and bullying in adolescents
Authors: Lomas Justine , Stough Con , Hansen Karen , Downey Luke A. 
Published: April 2011

ABSTRACTIn order to better understand bullying behaviours we examined for the first time the relationship between emotional intelligence (EI) of adolescents, bullying behaviours and peer victimisation. The sample consisted of 68 adolescents from a secondary college. Participants completed a self‐report questionnaire which assessed their EI, how frequently they engaged in bullying behaviours and how often they were the target of peer victimisation. Results of the study indicated that the EI dimensions of Emotions Direct Cognition and Emotional Management and Control, significantly predicted the propensity of adolescents to be subjected to peer victimisation. The EI dimension of Understanding the Emotions of Others was found to be negatively related with bullying behaviours. It was concluded that anti‐bullying programs in schools could be improved by addressing deficits in EI in adolescents who bully others as well as those who are at a greater risk of being subjected to peer victimisation.
How Exporters’ and Intermediaries’ Resources Influence Export Performance
Authors: Theingi  , Purchase Sharon 
Published: April 2011

Purpose The purpose of the paper is to investigate the influence of firm resources and export intermediary resources on export performance using the resource-based view (RBV). Methodology Data was collected from 320 small and medium sized exporters from Thailand and the unit of analysis was an export venture. Cluster analysis and discriminant analysis were used to test whether the availability of firm resources and intermediary resources vary between different SME clusters. Finding Results show that the high performing cluster used their intermediary resources and their own internal marketing capabilities to a greater extent than lower performing clusters. Practical implications The findings are particularly important for resource-constrained SMEs, as they need to focus on developing marketing capabilities and choosing intermediaries with a high level of experience and knowledge for their particular export market. Originality value This research confirms the applicability of RBV in export literature extending current RBV theory. The findings in the paper indicate that the availability of firm resources as well as intermediary resources are important in determining export performance in an export-driven Thai economy. Research highlights The impact of firm resources and intermediaries resources on export performance was investigated. High performing firms used more intermediaries resources and own marketing capabilities. It is important to choose the intermediary with high level of knowledge and experience. Both firm resources and intermediary resources are important in determining export performance.
Post-Russian Eurasia and the Proto-Eurasian Usage of the Runet in Kazakhstan: A Plea for a Cyberlinguistic Turn in Area Studies
Authors: Uffelmann Dirk 
Published: April 2011

Drawing on the theoretical discussion of common features of cultures in the post-Soviet space, this paper proposes to refocus on the linguistic dimension and to investigate post-Russian Eurasia. Is not the role of the Russian language coming under serious challenge in the post-Soviet context, where independent states are downgrading the status of Russian in administration and education and where ethnic Russians are ‘remigrating’ from former Soviet republics to the Russian Federation? There is, however, one medium in which Russian is gaining new significance as a language of inter-regional communication: the Internet. Albeit to a lesser degree than English and Chinese, Russian serves as a means of communication between Russian-speaking communities all over the world. What is more, the Russian Internet (Runet) offers access to elaborated resources of contemporary culture (video and music downloads etc.). The paper discusses the role the Russian-based Runet plays for Eurasian webcommunities outside the Russian Federation, mostly relying on Kazakh material, and asks whether post-colonial anxieties about Russian cultural imperialism through the Runet are justified or not and what the Kazakh, possibly post-colonial strategies of coping with this situation are. Essential to this essay is the notion of cyberimperialism, which combines aspects of media studies with post-colonial studies. The interdisciplinary approach to Internet studies is completed by a linguistic focus on the performativity of language usage online for creating situational language identities. The essay rounds off by offering an analysis of Nursultan Nazarbaev's ambiguous inclusive-exclusive logic of argumentation and confronting it with Russian Eurasianism.
Early testosterone modulated sex differences in behavioral outcome following neonatal hypoxia ischemia in rats
Authors: Hill C.A. , Threlkeld S.W. , Fitch R.H. 
Published: April 2011

AbstractHypoxia ischemia (HI; reduced blood oxygenation and/or flow to the brain) represents one of the most common injuries for both term and preterm/very low birth weight (VLBW) infants. These children experience elevated incidence of cognitive and/or sensory processing disabilities, including language based learning disabilities. Clinical data also indicate more substantial long‐term deficits for HI injured male babies as compared to HI injured females. Previously, we reported significant deficits in rapid auditory processing and spatial learning in male rats with postnatal day 1 (P1), P7, or P10 HI injury. We also showed sex differences in HI injured animals, with more severe deficits in males as compared to females. Given these findings, combined with extant clinical data, the current study sought to assess a putative role for perinatal testosterone in modulating behavioral outcome following early hypoxic‐ischemic injury in rats. Male, female, and testosterone‐propionate (TP) treated females were subjected to P7 HI or sham surgery, and subsequently (P30+) underwent a battery of auditory testing and water maze assessment. Results confirm previous reports of sex differences following HI, and add new findings of significantly worse performance in TP‐treated HI females compared to vehicle treated HI females. Post mortem anatomic analyses showed consistent effects, with significant brain weight decreases seen in HI male and TP‐treated HI females but not female HI or sham groups. Further neuromorphometric analysis of brain structures showed that HI male animals exhibited increased pathology relative to HI females as reflected in ventricular enlargement. Findings suggest that neonatal testosterone may act to enhance the deleterious consequences of early HI brain injury, as measured by both neuropathology and behavior.
Neuroprotective effects of the beta‐catenin stabilization in an oxygen‐ and glucose‐deprived human neural progenitor cell culture system
Authors: Skardelly Marco , Gaber Khaled , Schwarz Johannes , Milosevic Javorina 
Published: April 2011

Abstractβ‐Catenin stabilization achieved either via GSK‐3β specific inhibition or involving canonical Wnt signalling pathway, contributes to neuroprotection in an oxygen–glucose deprivation (4 h OGD) in vitro hypoxia model performed on human cortical neural progenitor cells previously differentiated into neurons and glia. Neuroprotection mechanisms include both acquiring tolerance to injury throughout preconditioning (72 h prior to OGD) or being pro‐survival during 24 h reoxygenation after the insult. Four hours of OGD induced apoptotic cell death elevation to 73 ± 1% vs. 12% measured in control and the LDH level, indicative of necrotic cell injury, elevation by 67 ± 7% (set to 100%). A significant reduction in apoptosis occurred at 24 h reoxygenation with indirubin supplement which was 49 ± 6% at 2.5 μM BIO while LDH level was only 47 ± 5% of OGD. Kenpaullone was efficient in reducing both cell deaths at 5 μM (apoptosis 38 ± 1% and necrosis 33 ± 3% less than in OGD). Wnt agonist reduced apoptosis to 45 ± 3% at 0.01 μM, while LDH value was decreased to a level of 53 ± 5% of control. Our findings suggest that GSK‐3beta inhibitors/β‐catenin stabilizers may ultimately be useful drugs in neuroprotection and neuroregeneration therapies in vivo.
Reproductive outcome after letrozole versus laparoscopic ovarian drilling for clomiphene‐resistant polycystic ovary syndrome
Authors: Abdellah Mohamad S. 
Published: April 2011

AbstractObjectiveTo compare the clinical outcomes of letrozole and laparoscopic ovarian drilling (LOD) in patients with clomiphene‐citrate‐resistant polycystic ovary syndrome (PCOS).MethodsIn the present prospective randomized trial, 140 women with clomiphene‐citrate‐resistant PCOS were randomly allocated to receive 5 mg letrozole from day 3 to day 7 of menses for 6 consecutive cycles, or to undergo LOD. When a leading follicle of at least 18 mm was present, ovulation was triggered with human chorionic gonadotropin (hCG). The 6‐month rates of ovulation, pregnancy, abortion, and live births were evaluated.ResultsThe groups were similar with regard to baseline clinical characteristics and hormonal profiles. The ovulation rate was significantly higher in the letrozole group than in the LOD group (59.0% versus 47.5%). On the days of the hCG injection, women in the letrozole group had a significantly thicker endometrium than those in the LOD group (P < 0.0001). Women receiving letrozole had a higher pregnancy rate (35.7% versus 28.6%) and a lower rate of spontaneous abortion (8.0% versus 20.0%, respectively), but these differences were not statistically significant.ConclusionLetrozole seems to be a suitable second‐line ovulation‐inducing alternative to LOD in women with PCOS who do not conceive with clomiphene citrate.
Genital tuberculosis among infertile women and fertility outcome after antitubercular therapy
Authors: Kulshrestha Vidushi , Kriplani Alka , Agarwal Nutan , Singh Urvashi B. , Rana Tanu 
Published: April 2011

AbstractObjectiveTo compare modalities for diagnosing genital tuberculosis (GTB) and to assess fertility outcome after antitubercular therapy (ATT).MethodsInfertile women underwent endometrial aspiration (EA) and peritoneal washing (PW) for histopathologic examination, PCR, and acid‐fast bacilli (AFB) smear and culture of Mycobacterium tuberculosis; laparoscopy and hysteroscopy were also performed. Women with a positive laboratory test and/or laparoscopic finding classified as definitive/probable received ATT for 6 months.ResultsOf 196 women recruited, 187 underwent laparoscopy. Genital tuberculosis was diagnosed in 118 (60.2%). In 41.3%, EA PCR was positive; PW PCR was positive in 7.6%. The remaining laboratory tests were positive in a small number. Laparoscopy indicated definitive GTB in 9.1% and probable GTB in 37.4%. Among the 118 women treated for GTB, 22.9% conceived without in vitro fertilization; of these women, 74.1% had a positive EA PCR and 59.3% had a positive laparoscopy finding. A quarter of the women received ATT solely on the basis of the PCR result and 31.0% of these women conceived.ConclusionNo single test can detect all instances of GTB. A combination of tests is needed to increase the detection rate. Treatment given solely on the basis of a positive PCR result can result in conception.
A comparison of barbed and smooth sutures for ovine cesarean delivery
Authors: Greenberg James A. , Walden Scott , Hammer Carolyn M. , Grazul‐Bilska Anna T. , Vonnahme Kimberly A. 
Published: April 2011

AbstractObjectiveTo determine the adequacy of barbed and smooth sutures for closing the uterus and fascia in pregnant ewes.MethodsNine ewes that underwent cesarean delivery were randomized to each receive 2 different suture materials for both the uterus and the fascia. The sutures used were: barbed poliglecaprone 25, smooth poliglecaprone 25, braided polyglactin 910, and smooth chromic sutures on the uterus; and barbed polydioxanone (PDO), barbed poliglecaprone 25, braided polyglactin 910, and smooth chromic sutures on the fascia.ResultsIn 4 of the ewes, the fascia suture line failed prematurely, leading to dehiscence prior to planned euthanasia and necropsy. The remaining 5 ewes were euthanized on days 2, 7, or 28. All suture materials (smooth and barbed) were adequate for uterine closures. Barbed PDO, smooth poliglecaprone 25, and braided polyglactin 910 were adequate for fascia closures, whereas both barbed poliglecaprone 25 and smooth chromic resulted in premature suture line ruptures and fascia dehiscence.ConclusionIn a small pilot study, absorbable knotless barbed suture was adequate and equivalent to absorbable knotted smooth suture for closing the uterus following ovine cesarean delivery. On the rectus fascia, however, sutures—whether barbed or smooth—with lower tensile strength resulted in fascial dehiscence.
Maternal and neonatal survival and mortality in the Upper West Region of Ghana
Authors: Issah Kofi , Nang‐Beifubah Alexis , Opoku Chris F. 
Published: April 2011

AbstractObjectiveTo describe the health facility‐based factors affecting maternal/neonatal health and the outcomes of maternal death notifications and audits in the Upper West Region of Ghana.MethodsMaternal death notifications and audits were conducted at 6 hospitals and in communities in the Upper West Region of Ghana in 2009. Furthermore, a kangaroo mother care strategy was implemented at 5 health facilities in the region to improve neonatal survival. The results of these implementations were recorded between July and December 2009.ResultsForty‐seven maternal deaths were reported and audited, with 46.5% occurring within 24 hours after admission. Twenty‐three deaths were linked to delays in receiving care and non‐adherence to treatment protocols. Of 155 midwives expected to provide skilled care, 129 (83.2%) were between 46 and 59 years of age. The kangaroo mother care strategy resulted in 622 (89.5%) of 695 targeted infants surviving. At the end of 2009, only 30% of the recommendations of audit committees had been implemented.ConclusionMaternal death notifications and audits are useful tools for improving quality of care and outcomes. With almost half of maternal deaths occurring within 24 hours of admission, emergency care in the Upper West Region of Ghana must be improved.
Efficiency under quantile regression: What is the relationship with risk in the EU banking industry?
Authors: Koutsomanoli‐Filippaki Anastasia I. , Mamatzakis Emmanuel C. 
Published: April 2011

AbstractThis study estimates cost efficiency under a quantile regression framework. Our purpose is to investigate whether cost efficiency differs across quantiles of the conditional distribution. Efficiency scores are derived using the distribution‐free approach. Results show that for higher conditional distributions, efficiency scores are lower. In a second stage analysis, we examine the relationship between efficiency and risk, measured as distance to default. Cross section regressions show that the higher the risk, the lower the level of efficiency. The magnitude and the significance of the coefficient of the distance to default increases for conditional distributions associated with lower levels of efficiency.
Effect of sea-level lowering on ELA depression during the LGM
Authors: Hanson Brian , Hooke Roger LeB. 
Published: April 2011

AbstractDecreases in equilibrium-line altitudes (ELAs) varied geographically during the last glacial maximum (LGM), with a mid-range value of ~ 900 m commonly deduced from altitude ratio and accumulation–area ratio calculations. Sea level, however, was 120 m lower during the LGM, so the ELA lowering relative to sea level would only be 780 m for a 900-m absolute lowering. With a lapse rate of 0.006°C m−1, this implies a 4.7°C lowering of global temperature. It has been argued that this correction for sea-level change is unnecessary, but the logic on which this is based requires adiabatic compression to apply over much longer time scales than is typically invoked. We find that the correction is necessary. In addition, geometric changes in the atmosphere during the LGM, pointed out by Osmaston (2006), could lead to 0.4°C decrease in the average temperature of the troposphere. Additionally, orographic effects could significantly change the snow distribution on mountain masses near sea level.
Sensitive grain-size records of Holocene East Asian summer monsoon in sediments of northern South China Sea slope
Authors: Huang Jie , Li Anchun , Wan Shiming 
Published: April 2011

AbstractChanges in paleoenvironments over the last 17,500 yr have been documented by a high-resolution clay mineralogy and grain–size records of Core KNG5 from the northern slope of the South China Sea. Our results indicate that clay minerals are mainly from the Pearl River from 17,500 to 12,500 cal yr BP, and the South China Sea modern current system began to form since 12,500 cal yr BP, as a result, Taiwan turns to be the major contributor of clay minerals after 12,500 cal yr BP. Two grain-size populations with high variability through time were identified in the 13–28 μm and 1–2.2 μm grain-size intervals. The 1–2.2 μm grain-size population are mainly controlled by provenance supply and current transport. The 13–28 μm grain-size fraction could be controlled mainly by the sea-level change. The 1–2.2 μm grain-size population record demonstrates that East Asian Summer Monsoon intensity generally follows changes in insolation and that the response is similar for a large area of China and other northern low-latitude records, implying the globality of the monsoon evolution since Holocene. The anomalous environmental conditions in the northern South China Sea may imply intensified ENSO activity during the late Holocene.
Nutritional and socio-economic determinants of cognitive function and educational achievement of Aboriginal schoolchildren in rural Malaysia
Authors: Al-Mekhlafi Hesham M. , Mahdy Mohammed A. , Sallam Atiya A. , Ariffin W. A. , Al-Mekhlafi Abdulsalam M. , Amran Adel A. , Surin Johari 
Published: April 2011

A community-based cross-sectional study was carried out among Aboriginal schoolchildren aged 7–12 years living in remote areas in Pos Betau, Pahang, Malaysia to investigate the potential determinants influencing the cognitive function and educational achievement of these children. Cognitive function was measured by intelligence quotient (IQ), while examination scores of selected school subjects were used in assessing educational achievement. Blood samples were collected to assess serum Fe status. All children were screened for soil-transmitted helminthes. Demographic and socio-economic data were collected using pre-tested questionnaires. Almost two-thirds (67·6 %) of the subjects had poor IQ and most of them (72·6 %) had insufficient educational achievement. Output of the stepwise multiple regression model showed that poor IQ was significantly associated with low household income which contributed the most to the regression variance (r2 0·059; P = 0·020). Low maternal education was also identified as a significant predictor of low IQ scores (r2 0·042; P = 0·043). With educational achievement, Fe-deficiency anaemia (IDA) was the only variable to show significant association (r2 0·025; P = 0·015). In conclusion, the cognitive function and educational achievement of Aboriginal schoolchildren are poor and influenced by household income, maternal education and IDA. Thus, effective and integrated measures to improve the nutritional and socio-economic status of rural children would have a pronounced positive effect on their education.