bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hunter disease eClinic:interactive, computer-assisted, problem-based approach to independent learning about a rare genetic disease
Authors: Al-Jasmi Fatma , Moldovan Laura , Clarke Joe TR 
Published: October 2010

Abstract

Background
Computer-based teaching (CBT) is a well-known educational device, but it has never been applied systematically to the teaching of a complex, rare, genetic disease, such as Hunter disease (MPS II).

Aim
To develop interactive teaching software functioning as a virtual clinic for the management of MPS II.

Implementation and Results
The Hunter disease eClinic, a self-training, user-friendly educational software program, available at the Lysosomal Storage Research Group (http://www.lysosomalstorageresearch.ca), was developed using the Adobe Flash multimedia platform. It was designed to function both to provide a realistic, interactive virtual clinic and instantaneous access to supporting literature on Hunter disease. The Hunter disease eClinic consists of an eBook and an eClinic. The eClinic is the interactive virtual clinic component of the software. Within an environment resembling a real clinic, the trainee is instructed to perform a medical history, to examine the patient, and to order appropriate investigation. The program provides clinical data derived from the management of actual patients with Hunter disease. The eBook provides instantaneous, electronic access to a vast collection of reference information to provide detailed background clinical and basic science, including relevant biochemistry, physiology, and genetics. In the eClinic, the trainee is presented with quizzes designed to provide immediate feedback on both trainee effectiveness and efficiency. User feedback on the merits of the program was collected at several seminars and formal clinical rounds at several medical centres, primarily in Canada. In addition, online usage statistics were documented for a 2-year period. Feedback was consistently positive and confirmed the practical benefit of the program. The online English-language version is accessed daily by users from all over the world; a Japanese translation of the program is also available.

Conclusions
The Hunter disease eClinic employs a CBT model providing the trainee with realistic clinical problems, coupled with comprehensive basic and clinical reference information by instantaneous access to an electronic textbook, the eBook. The program was rated highly by attendees at national and international presentations. It provides a potential model for use as an educational approach to other rare genetic diseases.
FasL and FADD delivery by a glioma-specific and cell cycle-dependent HSV-1 amplicon virus enhanced apoptosis in primary human brain tumors
Authors: Ho Ivy A , Ng Wai H , Lam Paula Y 
Published: October 2010

Abstract

Background
Glioblastoma multiforme is the most malignant cancer of the brain and is notoriously difficult to treat due to the highly proliferative and infiltrative nature of the cells. Herein, we explored the combination treatment of pre-established human glioma xenograft using multiple therapeutic genes whereby the gene expression is regulated by both cell-type and cell cycle-dependent transcriptional regulatory mechanism conferred by recombinant HSV-1 amplicon vectors.

Results
We demonstrated for the first time that Ki67-positive proliferating primary human glioma cells cultured from biopsy samples were effectively induced into cell death by the dual-specific function of the pG8-FasL amplicon vectors. These vectors were relatively stable and exhibited minimal cytotoxicity in vivo. Intracranial implantation of pre-transduced glioma cells resulted in better survival outcome when compared with viral vectors inoculated one week post-implantation of tumor cells, indicating that therapeutic efficacy is dependent on the viral spread and mode of viral vectors administration. We further showed that pG8-FasL amplicon vectors are functional in the presence of commonly used treatment regimens for human brain cancer. In fact, the combined therapies of pG8-FasL and pG8-FADD in the presence of temozolomide significantly improved the survival of mice bearing intracranial high-grade gliomas.

Conclusion
Taken together, our results showed that the glioma-specific and cell cycle-dependent HSV-1 amplicon vector is potentially useful as an adjuvant therapy to complement the current gene therapy strategy for gliomas.
Effects of propranolol in combination with radiation on apoptosis and survival of gastric cancer cells in vitro
Authors: Liao Xinhua , Che Xiangming , Zhao Wei , Zhang Danjie , Long Houlong , Chaudhary Prakash , LI Haijun 
Published: October 2010

Abstract

Background
The National Comprehensive Cancer Network (NCCN) guidelines recommend radiotherapy as a standard treatment for patients with a high risk of recurrence in gastric cancer. Because gastric cancer demonstrates limited sensitivity to radiotherapy, a radiosensitizer might therefore be useful to enhance the radiosensitivity of patients with advanced gastric carcinoma. In this study, we evaluated if propranolol, a β-adrenoceptor (β-AR) antagonist, could enhance radiosensitivity and explored its precise molecular mechanism in gastric cancer cells.

Methods
Human gastric adenocarcinoma cell lines (SGC-7901 and BGC-823) were treated with or without propranolol and exposed to radiation. Cell viability and clonogenic survival assays were performed, and cell apoptosis was evaluated with flow cytometry. In addition, the expression of nuclear factor κB (NF-κB), vascular endothelial growth factor (VEGF), cyclooxygenase 2 (COX-2), and epidermal growth factor receptor (EGFR) were detected by western blot and real-time reverse transcription polymerase chain reaction (PCR).

Results
Propranolol combined with radiation decreased cell viability and clonogenic survivability. Furthermore, it also induced apoptosis in both cell lines tested, as determined by Annexin V staining. In addition, treatment with propranolol decreased the level of NF-κB and, subsequently, down-regulated VEGF, COX-2, and EGFR expression.

Conclusions
Taken together, these results suggested that propranolol enhanced the sensitivity of gastric cancer cells to radiation through the inhibition of β-ARs and the downstream NF-κB-VEGF/EGFR/COX-2 pathway.
Information propagation within the Genetic Network of Saccharomyces cerevisiae
Authors: Chowdhury Sharif , Lloyd-Price Jason , Smolander Olli-Pekka , Baici Wayne CV , Hughes Timothy R , Yli-Harja Olli , Chua Gordon , Ribeiro Andre S 
Published: October 2010

Abstract

Background
A gene network's capacity to process information, so as to bind past events to future actions, depends on its structure and logic. From previous and new microarray measurements in Saccharomyces cerevisiae following gene deletions and overexpressions, we identify a core gene regulatory network (GRN) of functional interactions between 328 genes and the transfer functions of each gene. Inferred connections are verified by gene enrichment.

Results
We find that this core network has a generalized clustering coefficient that is much higher than chance. The inferred Boolean transfer functions have a mean p-bias of 0.41, and thus similar amounts of activation and repression interactions. However, the distribution of p-biases differs significantly from what is expected by chance that, along with the high mean connectivity, is found to cause the core GRN of S. cerevisiae's to have an overall sensitivity similar to critical Boolean networks. In agreement, we find that the amount of information propagated between nodes in finite time series is much higher in the inferred core GRN of S. cerevisiae than what is expected by chance.

Conclusions
We suggest that S. cerevisiae is likely to have evolved a core GRN with enhanced information propagation among its genes.
Collagen-induced arthritis in common marmosets: a new nonhuman primate model for chronic arthritis
Authors: Vierboom Michel PM , Breedveld Elia , Kondova Ivanela , 't Hart Bert A 
Published: October 2010

Abstract
Introduction
There is an ever-increasing need for animal models to evaluate efficacy and safety of new therapeutics in the field of rheumatoid arthritis (RA). Particularly for the early preclinical evaluation of human-specific biologicals targeting the progressive phase of the disease, there is a need for relevant animal models. In response to this requirement we set out to develop a model of collagen-induced arthritis (CIA) in a small-sized nonhuman primate species (300 to 400 g at adult age); that is, the common marmoset (Callithrix jacchus).

Methods
Twenty-two animals divided into three experiments were immunized with collagen type II (CII) of either bovine or chicken origin with different immunization strategies. The animals were analyzed for clinical manifestation of arthritis, hematology and clinical chemistry, immunological responses against CII and histopathological features of the arthritis.

Results
Clinically manifest arthritis was observed in almost 100% (21 out of 22) of the animals. Fifty percent of the animals developed semi-acute CIA while the other 50% displayed a more chronic disease. Both cellular (CD3/CD4 and CD3/CD8) and humoral responses (IgM and IgG) against CII were involved in the development of the disease. Besides mild histopathological changes in bone and cartilage, severe inflammation in extraarticular tissues like periosteum and subcutaneous tissues was observed.

Conclusions
This new model in marmosets more closely resembles chronic RA with respect to the chronic disease course and pathomorphological presentation than the more acute monophasic and destructive CIA model in macaques. This model can therefore fill a niche in preclinical testing of new human specific therapeutics.
Politics, Populism, and Professionalism: Reflections on the Role of the Academic Historian in the Production of Public History
Authors: Dresser Madge 
Published: October 2010

Abstract
This article explores some of the challenges and opportunities facing academic historians involved in large British public history projects and examines how government priorities and the particular ways in which public funds are deployed can affect the critical intellectual content of such projects. To this end it first broadly outlines the context in which British public history has recently developed and then focuses on my own experiences as leader of a British public history project on 1001 years of ethnic minorities in Bristol, England, which was sponsored by the “England's Past for Everyone” initiative.
Just Say “I Don't”: Lack of Concordance Between Teen Report and Biological Measures of Drug Use
Authors: Delaney-Black Virginia , Chiodo Lisa M. , Hannigan John H. , Greenwald Mark K. , Janisse James , Patterson Grace , Huestis Marilyn A. , Ager Joel , Sokol Robert J. 
Published: October 2010

BACKGROUND:
Prevalence estimates of illicit drug use by teens are typically generated from confidential or anonymous self-report. While data comparing teen self-report with biological measures are limited, adult studies identify varying degrees of under-reporting.

METHODS:
Hair analyses for cocaine, opiates and marijuana were compared to confidential teen self- and parent-reported teen drug use in a longitudinal cohort of &gt;400 high-risk urban teens and parents.

RESULTS:
Both teens and parents substantially underreported recent teen cocaine and opiate use. However, compared with parents, teens were more likely to deny biomarker-verified cocaine use. Teen specimens (hair) were 52 times more likely to identify cocaine use compared with self-report. Parent hair analyses for cocaine and opiate use were 6.5 times and 5.5 times, respectively, more likely to indicate drug use than were parental self-report. The lack of concordance between self-report and bioassay occurred despite participant's knowledge that a “certificate of confidentiality” protected both teen and adult participants, and that the biological specimens would be tested for drugs.

CONCLUSIONS:
These findings confirm prior reports of adult under-reporting of their own drug use while extending our understanding of teen's self-admitted drug use. The lack of concordance between teen self- or parent-reported teen drug use and biomarkers confirm our concerns that both teen- and parent-reported teen drug use is limited, at least for youth in high-risk urban settings. Methods of ascertainment other than self- or parent-report must be considered when health care providers, researchers and public health agencies attempt to estimate teen drug-use prevalence.
Variation in Methods of Predicting Adult Height for Children With Idiopathic Short Stature
Authors: Topor Lisa Swartz , Feldman Henry A. , Bauchner Howard , Cohen Laurie E. 
Published: October 2010

OBJECTIVE:Recombinant human growth hormone (GH) is approved for treatment of children with idiopathic short stature, and endocrinologists often depend on algorithms to predict adult height. Because algorithm performance often is included in treatment decisions, we sought to evaluate agreement among height prediction formulas.METHODS:We identified 3 commonly used algorithms for height prediction, the Bayley-Pinneau, Roche-Wainer-Thissen, and Khamis-Roche methods. We constructed simulated samples of children with typical distributions of ages, heights, weights, bone ages, and parental heights seen in patients with idiopathic short stature, and we applied the algorithms to the simulated sample to determine whether predicted adult height was &lt;160 cm for boys or &lt;150 cm for girls (&lt;1.2nd height percentiles for adults).RESULTS:We found substantial disagreement among algorithms in the proportions of simulated cases with predicted adult heights of &lt;1.2nd percentile, a cutoff value that may influence GH treatment decisions. With the Bayley-Pinneau formula, 43% of boys and 81% of girls had predicted adult heights below this threshold; with the Khamis-Roche method, only 3% of boys and 0.2% of girls had predicted heights of &lt;1.2nd percentile. Roche-Wainer-Thissen predictions were between those values. Overall agreement of the methods was poor (κ = 0.21) for boys and negative for girls.CONCLUSIONS:Wide variation exists among formulas used to predict adult heights. Because these algorithms may be used in decisions regarding whether to initiate GH treatment and assessment of the efficacy of GH in research trials, it is important for parents, pediatricians, and investigators to recognize the considerable variation involved in height predictions.
The Effects of Gestational Age and Birth Weight on False-Positive Newborn-Screening Rates
Authors: Slaughter Jonathan L. , Meinzen-Derr Jareen , Rose Susan R. , Leslie Nancy D. , Chandrasekar Ram , Linard Sharon M. , Akinbi Henry T. 
Published: October 2010

OBJECTIVE:Newborn-screening false-positive rates (FPRs) are disproportionately increased in preterm infants. The objective of this study was to determine variation in newborn screening FPRs according to birth weight and gestational age. Our secondary objective was to examine the effect of postnatal age on FPRs in preterm infants.METHODS:The Ohio State Newborn Screening Program Database was analyzed to determine the overall and birth weight–specific FPRs for 18 analytes. Data were stratified into birth weight categories (&lt;1000 g, 1000–1499 g, 1500–2499 g, 2500–3999 g, and &gt;4000 g). In addition, to examine the effect of postnatal age on FPRs, we examined the 2 analytes with the highest FPRs, thyrotropin with back-up thyroxine and 17-hydroxyprogesterone, in infants whose gestational age was &lt;32 weeks, determined on the basis of postnatal age at screening.RESULTS:Data from 448 766 neonates were reviewed. Infants with very low birth weight (VLBW) comprised 1.9% of the study cohort, but accounted for 18% of false-positive results. For 14 of 18 analytes studied, FPRs increased with decreasing birth weight/gestational age and were significantly increased in infants with VLBW compared with infants who weighed 2500 to 3999 g (P &lt; .001). Thyrotropin/back-up thyroxine and 17-hydroxyprogesterone accounted for 62% of total false-positive results in VLBW infants. When blood specimens were collected at a postnatal age of ≥48 hours in infants born at &lt;32 weeks, a 44% relative reduction in 17-hydroxyprogesterone false-positive results was detected.CONCLUSIONS:False-positive newborn-screening rates are disproportionately increased in VLBW infants. FPRs may be reduced by delaying screening of &lt;32 weeks' gestation, preterm infants until 24 to 48 hours' postnatal age.
Trends in the Prevalence of Autism on the Basis of Special Education Data
Authors: Maenner Matthew J. , Durkin Maureen S. 
Published: October 2010

OBJECTIVE:The goal was to determine whether the increasing prevalence of autism, on the basis of educational data, in Wisconsin between 2002 and 2008 was uniform in all school districts or was greatest in districts with lower baseline (2002) prevalence.METHODS:Special education counts were obtained for all Wisconsin elementary school districts from 2002 through 2008. The annual prevalence of children being served under the autism category was calculated for each district, districts were grouped into 8 categories (octiles) according to their baseline prevalence, and prevalence trends were plotted according to octile.RESULTS:The overall prevalence of use of the autism category in Wisconsin elementary schools increased from 4.9 to 9.0 cases per 1000 children between 2002 and 2008. The magnitude of this increase was not uniform across districts and was inversely associated with baseline prevalence. Prevalence in the lowest octile increased from 0.5 cases per 1000 students in 2002 to 7.0 cases per 1000 students in 2008 (P &lt; .0001), whereas no significant trend was seen for the octile with the highest baseline prevalence (range: 11.2–12.3 cases per 1000 students; P = .11). The highest-octile/lowest-octile prevalence ratio decreased from 24.6 (95% confidence interval: 16.2–37.3) in 2002 to 1.8 (95% confidence interval: 1.6–2.1) in 2008.CONCLUSIONS:The prevalence of use of the autism special education category in Wisconsin seems to be leveling off in the school districts with the highest prevalence rates, at ∼12 cases per 1000 students, whereas the gap in prevalence between districts overall has narrowed.
Economic Outcomes in Young Adulthood for Extremely Low Birth Weight Survivors
Authors: Goddeeris John H. , Saigal Saroj , Boyle Michael H. , Paneth Nigel , Streiner David L. , Stoskopf Barbara 
Published: October 2010

OBJECTIVE:
The goal was to compare educational attainment and labor market outcomes in young adulthood (21–26 years of age) for a Canadian, population-based cohort of 149 extremely low birth weight (ELBW) (&lt;1000 g) survivors and a normal birth weight (NBW) cohort of 133 young adults from the same geographic area who were matched to the ELBW cohort in childhood.

METHODS:
We estimated the effects of ELBW status, according to gender, on continuous outcomes through least-squares regression and those on binary outcomes through logistic regression. We controlled for family background and considered neurosensory impairment and IQ as mediating variables.

RESULTS:
Controlling for family background, ELBW male subjects were less likely to complete high school or to attend a university than were their NBW counterparts, and their educational attainment was reduced by &gt;1 year. Among subjects who were working, weekly earnings were ∼27% lower. ELBW female effects on education were not significant, but ELBW female subjects were less likely than NBWsubjects to be employed or in school and they also seemed to experience lower earnings.

CONCLUSION:
Our findings suggested that ELBW survivors are somewhat less productive as adults, on average, than are subjects born NBW and that effects are not confined to subjects with severe neurosensory impairments. In accord with other studies, however, we found that productivity deficits for most ELBW subjects were not large.
Characterisation of den sites of American mink Neovison vison in central Spain
Authors: García Pablo , Arévalo Valentín , Lizana Miguel 
Published: October 2010

AbstractThe American mink Neovison vison is a widely distributed species in Spain, for which important basic ecological data are lacking. We studied mink den site selection in a location in central Spain during the winter of 2008/09 using snow‐tracking and direct observation. Dens were located more frequently in tree roots (51.9%; N = 79) inside emergent vegetation (15.2%) and on human‐created enbankments (15.2%). Human buildings and burrows were scarcely used (< 10%). The logistic regressions we carried out supported that minks used secondary river beds for shelter more often than their availability in the environment should predict. These secondary areas are characterised by their low width and the frequent presence of small streams (of < 1m width). In rough terms, this pattern could be a way of avoiding potential interactions with other animals sharing the same habitat, and also for thermoregulation while resting. On the basis of the wide habitat niche, ecosystem management as a measure of mink control appears to be ineffective.
Ecological aspects of transient killer whales Orcinus orca as predators in southeastern Alaska
Authors: Dahlheim Marilyn E. , White Paula A. 
Published: October 2010

AbstractIn this study we present empirical data on predator numbers, movements and area usage, and predation obtained from tracking transient killer whales Orcinus orca throughout the inland waters of southeastern Alaska, USA. During 1991‐2007, we documented 155 transient killer whales via photo‐identification methodology within the large study area (27,808 km2). Transient killer whales were distributed throughout southeastern Alaska and were present during all seasons, although not all individuals were seen every year. Resighting data suggested that within southeastern Alaska, maternal groups may partition area usage of their environment. By following whales for 1,467 km, we calculated a mean travel speed of 7.2 km/hour with mean daily movements of 134 km ± 88 km/24 hours and ranging within 59‐240 km/24 hours. Photographic matches demonstrated that most of the transient killer whales (86%) identified in southeastern Alaska also utilized British Columbia and Washington State waters. In contrast, photographic matches between whales in southeastern Alaska and whales seen off of California, USA, were rare, suggesting that different transient killer whale stocks occupy these two regions. Transient killer whales preyed upon Dall's porpoise Phocoenoides dalli, Pacific white‐sided dolphins Lagenoryhncus obliquidens, harbor porpoise Phocoena phocoena, minke whales Balaenoptera acutorostrata, Steller sea lions Eumetopias jubatus, harbor seals Phoca vitulina and seabirds. Potential prey species that were available, but not targeted, included humpback whales Megaptera novaeangliae, elephant seals Mirounga angustirostris and sea otters Enhydra lutris. Prey‐handling techniques varied depending on the prey being targeted with no evidence of prey specialization. During 114 encounters totaling 332.5 hours of direct observations of transient killer whales, we documented 36 predation events for a calculated kill rate of 0.62 prey items/24‐hour period/whale. The data we present in this article provide a foundation of transient killer whale ecology aimed at improving our ability to understand the impact of transient killer whale predation on southeastern Alaska prey populations.