bluuebunny/crossref_metadata_2025_split huggingface dataset:
Osteopathia striata with cranial sclerosis owing to WTX gene defect
Authors: Perdu Bram , Freitas Fenna de , Frints Suzanne GM , Schouten Meyke , Schrander-Stumpel Connie , Barbosa Mafalda , Pinto-Basto Jorge , Reis-Lima Margarida , Vernejoul Marie-Christine de , Becker Kristin , Freckmann Marie-Louise , Keymolen Kathlijn , Haan Eric , Savarirayan Ravi , Koenig Rainer , Zabel Bernhard , Vanhoenacker Filip M , Hul Wim Van 
Published: July 2009

Abstract
Osteopathia striata with cranial sclerosis (OSCS) is an X-linked dominant condition marked by linear striations mainly affecting the metaphyseal region of the long bones and pelvis in combination with cranial sclerosis. Recently, the disease-causing gene was identified as the WTX gene (FAM123B), an inhibitor of WNT signaling. A correlation was suggested between the position of the mutation and male lethality. We performed genotype and phenotype studies using 18 patients from eight families with possible WTX gene defects and expanded the clinical spectrum of the affected females. All investigated families diagnosed with OSCS had WTX gene defects. One family had a WTX gene deletion; three of four point mutations were novel. The earlier reported WTX c.1072C&gt;T was detected in four sporadic patients and appears to be a hotspot for mutations. Based on the nature of the mutation present in a surviving male patient, our data do not support the hypothesis raised by Jenkins et al. (2009) regarding a genotype-phenotype correlation for male lethality. The finding of a gene involved in WNT signaling as the cause of this sclerosing bone phenotype is not unexpected, but further functional studies are needed to explain the specific features. The WTX gene is mutated in different types of cancer, and it remains to be explained why osteopathia striata patients appear not to have an increased risk of cancer. Copyright © 2010 American Society for Bone and Mineral Research
Restoration of regenerative osteoblastogenesis in aged mice: Modulation of TNF
Authors: Wahl Elizabeth C , Aronson James , Liu Lichu , Fowlkes John L , Thrailkill Kathryn M , Bunn Robert C , Skinner Robert A , Miller Mike J , Cockrell Gael E , Clark Lindsey M , Ou Yang , Isales Carlos M , Badger Thomas M , Ronis Martin J , Sims John , Lumpkin Charles K 
Published: July 2009

Abstract
Skeletal changes accompanying aging are associated with both increased risk of fractures and impaired fracture healing, which, in turn, is due to compromised bone regeneration potential. These changes are associated with increased serum levels of selected proinflammatory cytokines, e.g., tumor necrosis factor α (TNF-α). We have used a unique model of bone regeneration to demonstrate (1) that aged-related deficits in direct bone formation can be restored to young mice by treatment with TNF blockers and (2) that the cyclin-dependent kinase inhibitor p21 is a candidate for mediation of the osteoinhibitory effects of TNF. It has been hypothesized recently that TNF antagonists may represent novel anabolic agents, and we believe that the data presented here represent a successful test of this hypothesis. © 2010 American Society for Bone and Mineral Research
Randomized trial of intensive bisphosphonate treatment versus symptomatic management in paget's disease of bone
Authors: Langston Anne L , Campbell Marion K , Fraser William D , MacLennan Graeme S , Selby Peter L , Ralston Stuart H 
Published: July 2009

Abstract
Bisphosphonates are widely regarded as the treatment of choice for Paget's disease of bone (PDB) because of their potent inhibitory effects on bone turnover, but the effects of bisphosphonate therapy on symptoms and complications of PDB have been little studied. Here we report the results of a randomized trial that compared the effects of symptomatic treatment with intensive bisphosphonate therapy in a cohort of 1324 patients with PDB who were followed up for a median of 3 years (range 2 to 5 years). The symptomatic treatment group was treated only if they had pagetic bone pain, for which they were first given analgesics or anti-inflammatory drugs, followed by bisphosphonates if they did not respond. The intensive group received repeat courses of bisphosphonates irrespective of symptoms with the aim of reducing and maintaining serum alkaline phosphatase (ALP) levels within the normal range. The endpoints were fracture, orthopedic surgery, quality of life, bone pain, and hearing thresholds. Serum ALP levels were significantly lower in the intensive treatment group than in with the symptomatic treatment group within 4 months of commencing treatment and remained lower throughout the study (p &lt; .001). There was no difference between the groups in quality of life (as assessed by the SF36 questionnaire), in overall bodily pain, or in pagetic bone pain. Hearing thresholds, as assessed by audiometry did not change significantly and did not differ between the treatment groups. Clinical fractures occurred in 46 of 661 patients (7.0%) in the intensive treatment group compared with 49 of 663 patients (7.4%) in the symptomatic treatment group, and orthopedic surgery was required in 50 of 661 patients (7.3%) in the intensive treatment group and in 55 of 663 patients (8.3%) in the symptomatic treatment group. These differences were not significant. Subgroup analyses of patients with elevated ALP levels at baseline and those who did or did not receive bisphosphonates during the study yielded similar results to those in the study group as a whole. We conclude that striving to maintain normal ALP levels with intensive bisphosphonate therapy confers no clinical advantage over symptom-driven management in patients with established PDB. Neither management strategy had a significant beneficial impact on pain or quality of life (Clinical trial registration number ISRCTN12989577). © 2010 American Society for Bone and Mineral Research
Deficiency of annexins A5 and A6 induces complex changes in the transcriptome of growth plate cartilage but does not inhibit the induction of mineralization
Authors: Belluoccio Daniele , Grskovic Ivan , Niehoff Anja , Schlötzer-Schrehardt Ursula , Rosenbaum Sabrina , Etich Julia , Frie Christian , Pausch Friedericke , Moss Stephen E , Pöschl Ernst , Bateman John F , Brachvogel Bent 
Published: July 2009

Abstract
Initiation of mineralization during endochondral ossification is a multistep process and has been assumed to correlate with specific interactions of annexins A5 and A6 and collagens. However, skeletal development appears to be normal in mice deficient for either A5 or A6, and the highly conserved structures led to the assumption that A5 and A6 may fulfill redundant functions. We have now generated mice deficient of both proteins. These mice were viable and fertile and showed no obvious abnormalities. Assessment of skeletal elements using histologic, ultrastructural, and peripheral quantitative computed tomographic methods revealed that mineralization and development of the skeleton were not significantly affected in mutant mice. Otherwise, global gene expression analysis showed subtle changes at the transcriptome level of genes involved in cell growth and intermediate metabolism. These results indicate that annexins A5 and A6 may not represent the essential annexins that promote mineralization in vivo. © 2010 American Society for Bone and Mineral Research
A randomized, controlled dose-ranging study of risedronate in children with moderate and severe osteogenesis imperfecta
Authors: Bishop Nick , Harrison Rachel , Ahmed Faisal , Shaw Nick , Eastell Richard , Campbell Mike , Knowles Elizabeth , Hill Claire , Hall Christine , Chapman Steve , Sprigg Alan , Rigby Alan 
Published: July 2009

Abstract
Moderate to severe osteogenesis imperfecta is associated with multiple fractures in childhood. There are no published data regarding the effects of third-generation bisphosphonates in these children. This randomized study investigated which of three different doses of risedronate was most effective in reducing fracture incidence. We randomly assigned 53 children with moderate to severe osteogenesis imperfecta to receive 0.2, 1, or 2 mg/kg per week of risedronate. We assessed safety, fracture incidence, and bone measurement outcomes at 3, 6, 12, 18, and 24 months. At 24 months, 69% of children assigned 0.2 mg/kg per week had had new fractures compared with 44% receiving 1 mg/kg per week and 75% receiving 2 mg/kg per week. Poisson regression with age and prior fracture as covariates showed that there was no difference in incident nonvertebral fracture between groups. Fracture rate diminished in each group during the trial compared with previous the 2 years (p = .005). Lumbar spine bone mineral density increased significantly (p = .009) only in the 2 mg/kg per week group. Long bone bowing deformities reduced more in children receiving 1 or 2 mg/kg per week of risedronate [odds ratio (OR) 0.67, 95% confidence interval (CI) 0.48–0.93 per unit increase in risedronate dose, p = .015]. There were no serious adverse events. Bone mass increased and bowing deformities reduced with increasing risedronate dose. Children suffered fewer fractures irrespective of risedronate dose. The most appropriate dose of risedronate for children with moderate to severe osteogenesis imperfecta in this study was 2 mg/kg per week. © 2010 American Society for Bone and Mineral Research
Cancer treatment dosing regimens of zoledronic acid result in near-complete suppression of mandible intracortical bone remodeling in beagle dogs
Authors: Allen Matthew R , Kubek Daniel J , Burr David B 
Published: July 2009

AbstractBisphosphonate doses used in cancer treatment are substantially higher than those used for osteoporosis. Little is known about the effects of these high doses on tissue-level remodeling suppression. The aim of this study was to assess the effects of cancer dosing regimens of zoledronic acid on tissue-level bone remodeling at different skeletal sites. Skeletally mature female beagle dogs were treated with monthly intravenous infusions of vehicle (VEH, saline) or zoledronic acid (ZOL, 0.067 mg/kg); an additional group of animals was treated daily with oral alendronate (ALN, 0.2 mg/kg/day). Doses of ZOL and ALN were, on a milligram per kilogram basis, consistent with those used for cancer and osteoporosis, respectively. Following either 3 or 6 months of treatment, animals were euthanized, and mandible, rib, and tibia were processed for dynamic bone histology. There was no evidence of oral lesions or bone matrix necrosis in the mandibles of any animals. After 3 months, the rate of intracortical bone remodeling in the mandible was significantly suppressed with ZOL (−95%) compared with VEH; by 6 months, ZOL had produced nearly complete suppression (−99%) compared with VEH. ZOL also significantly suppressed remodeling in the rib cortex at both 3 (−83%) and 6 (−85%) months compared with VEH; tibia cortex bone formation rate was nonsignificantly lower with ZOL treatment (−68% to −75%). Remodeling suppression in ZOL-treated animals was significantly greater than in ALN-treated animals at both the mandible and the rib; ALN and VEH were not different for any of the assessed parameters at any of the sites. Compared across skeletal sites, the absolute level of remodeling suppression with ZOL treatment was significantly greater at sites with higher remodeling, whereas the percent reduction was similar among the sites. These results document nearly complete intracortical remodeling suppression resulting from monthly intravenous zoledronic acid dosing, with changes being most dramatic at the mandible. Copyright © 2010 American Society for Bone and Mineral Research
Fat and failure: Marcel Duchamp's military imagination
Authors: Lyons Kieran 
Published: July 2009

Marcel Duchamp's preoccupation with the French army can be seen in a note he included in the Box of 1914. The note is known as loignement, and with it he declares his opposition to military conscription and then speculates that the army might telephonically reconnect the limbs
and organs of its soldiers that have been left scattered across the battlefield. Duchamp wrote this note before field telephones were issued to French troops but his grotesque proposition can be seen in light of his experience as an army corporal.
In 1912, before making these caustic suggestions
Duchamp had taken a motor journey and written an account of it where he describes the aggressive invasion of territory by an alien force. This invasion travelled from France's liminal frontiers to its metropolitan centre. His text is known as the Jura-Paris Road and again Duchamp reveals
his military concerns. He formulates territory that proceeds from topographical amplitude to the delimitation of a straight line. In spite of its sophisticated morphology, it is threatened by alien forces aimed at testing boundaries. Duchamp published his text as a facsimile of his handwritten
original, which displays the amendments and uncertainties of a man of military age attempting to evade the imperatives of military service.
The essay continues with an analysis of Duchamp's influences in writing this text and suggests a link with the philosopher Edmund Husserl in the context
of the profoundly anti-German atmosphere in the French military build-up to war between 1912 and 1914. The argument closes with a survey of a military thematic in Duchamp's work after he left France for America in 1915.
HALO: An experimental station project
Authors: Courdy Keiko , Parnel Jacques 
Published: July 2009

The planet is experiencing a deep metamorphosis. The pressure that is being generated nowadays on the living can be felt in a human lifetime. The extreme speed of climate change and its impact on our ecosystem can no longer be ignored. When looking at the phenomenon of ecological resilience,
we notice that after a disturbance, a shock on an ecosystem, like the burning of a forest, the resilient system rebuilds itself but not exactly as it was before, surprisingly new species grow. In social systems, humans have the capacity to anticipate, adapt and invent new patterns of behaviours.
In
a world where everything is uncertain, moving, shifting, we are making an artistic proposal as an open dynamic system. We are developing two versions of the same HALO project to be presented on the sea and in public places.
The Many Challenges of Pension Accounting
Authors: Beechy Thomas H. 
Published: July 2009

ABSTRACTAccounting for defined benefit pension plans has long been a major issue in accounting. Standard‐setters are grappling with revisions to pension accounting standards, and much change has already occurred in the United Kingdom. This paper identifies and discusses most of the major issues that standard‐setters must confront in developing new approaches to financial reporting for pensions. Key issues concern how to report the impact of changes in assumptions, how to recognize pension costs on the balance sheet and income statement, and how to reconcile the differences between accountants' and actuaries' approaches to pensions. Current standards assume that accounting estimates are independent of actuarial assumptions, and yet require a direct comparison of the accounting liability with the pension plan assets, when in fact they are incompatible measures based on differing assumptions and differing methodologies. As well, accounting has been complicit in managers' wishes to hide the volatility inherent in a pension plan investment strategy that focuses on higher‐risk equities to fund estimated monetary liabilities that have been discounted at low‐risk interest rates. Drawing on studies and research done largely in Europe, this paper attempts to consolidate some of the current thinking on the topic and to propose some preferred approaches to dealing with the problems of pension accounting.
The Development of a Conceptual Framework for the Design, Delivery, and Assessment of a Typical Management Accounting Syllabus
Authors: Wessels Philippus L. , Roos Shelley‐Anne 
Published: July 2009

ABSTRACTThis article identifies common issues relating to management accounting education in order to determine whether using a competency‐based approach would assist educators in the design, delivery, and assessment of syllabi at educational institutions. A conceptual framework is developed and discussed with regard to the critical success factors methodology to design syllabi that assist educators in attaining the main outcomes in the delivery and assessment of the curriculum. This framework is applied to a typical management accounting curriculum to demonstrate how this approach will enable educators to design, deliver, and assess their syllabi in line with the critical outcomes required. In following this approach, lecturers would constantly have to focus on the knowledge and issues that are relevant and critical for students to understand and apply in order to achieve the aim of the syllabi.
Smith & Jones LLP: Charting a Path to the Future*
Authors: Sheehan Norman T. 
Published: July 2009

ABSTRACTThis case requires accounting students to shift their focus from learning about the practice of auditing to learning about the business of auditing. It describes the strategy, vision, and mission of a medium‐sized, Canadian audit firm and the external and internal challenges the firm faces as it looks to improve its profitability. Students should reflect on which behaviors Smith & Jones would like to encourage and what the partners can do to encourage its employees to exhibit these behaviors.
Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement from the AHA and AAP
Authors: Mahle William T. , Newburger Jane W. , Matherne G. Paul , Smith Frank C. , Hoke Tracey R. , Koppel Robert , Gidding Samuel S. , Beekman Robert H. , Grosse Scott D. ,   on behalf of the American Heart Association Congenital Heart Defects Committee of the Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Interdisciplinary Council on Quality of Care and Outcomes Research; and the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery, and Committee on Fetus and Newborn
Published: July 2009

BACKGROUND: The purpose of this statement is to address the state of evidence on the routine use of pulse oximetry in newborns to detect critical congenital heart disease (CCHD).METHODS AND RESULTS: A writing group appointed by the American Heart Association and the American Academy of Pediatrics reviewed the available literature addressing current detection methods for CCHD, burden of missed and/or delayed diagnosis of CCHD, rationale of oximetry screening, and clinical studies of oximetry in otherwise asymptomatic newborns. MEDLINE database searches from 1966 to 2008 were done for English-language papers using the following search terms: congenital heart disease, pulse oximetry, physical examination, murmur, echocardiography, fetal echocardiography, and newborn screening. The reference lists of identified papers were also searched. Published abstracts from major pediatric scientific meetings in 2006 to 2008 were also reviewed. The American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. In an analysis of pooled studies of oximetry assessment performed after 24 hours of life, the estimated sensitivity for detecting CCHD was 69.6%, and the positive predictive value was 47.0%; however, sensitivity varied dramatically among studies from 0% to 100%. False-positive screens that required further evaluation occurred in only 0.035% of infants screened after 24 hours.CONCLUSIONS: Currently, CCHD is not detected in some newborns until after their hospital discharge, which results in significant morbidity and occasional mortality. Furthermore, routine pulse oximetry performed on asymptomatic newborns after 24 hours of life, but before hospital discharge, may detect CCHD. Routine pulse oximetry performed after 24 hours in hospitals that have on-site pediatric cardiovascular services incurs very low cost and risk of harm. Future studies in larger populations and across a broad range of newborn delivery systems are needed to determine whether this practice should become standard of care in the routine assessment of the neonate.
EFfects of Diesel and Interactions with Copper and Other Metals in an Estuarine Sediment Microbial Community
Authors: Hedrick David B. , Peacock Aaron D. , Tita Guglielmo , Fleeger John W. , Carman Kevin R. , White David C. 
Published: July 2009

Abstract
Estuarine sediment microcosms were treated with combinations of diesel, copper (at two levels), and a mixture of heavy metals (mercury, cadmium, lead, and chromium; at two levels) mimicking the contaminant loadings found in harbor sediments. The effects on the microbial community were monitored by polar lipid fatty acid analysis. Diesel addition increased microbial biomass, caused shifts in some fatty acid structural groups, and decreased starvation biomarkers. Incorporation of diesel hydrocarbons into lipids was expressed as an increase in the proportion of odd-carbon-number fatty acids. No treatment with the metals mixture (mercury, cadmium, lead, and chromium) alone significantly changed any parameter derived from the polar lipid fatty acids, but the increase in microbial biomass from diesel addition was higher with the metals mixture, possibly because of indirect effects caused by reductions in grazing resulting from metal-induced toxicity to bacteriovorous nematodes. Copper also modified the effects of diesel addition, preventing biomass increase but not diesel degradation, suggesting that some of the energy gained from diesel oxidation was expended combating copper toxicity. In the present study, observations indicate that metals in general, and copper in particular, can modify the response of sedimentary microorganisms to petroleum-hydrocarbon contaminants.
Factors affecting the degradation of pharmaceuticals in agricultural soils
Authors: Monteiro Sara C. , Boxall Alistair B. A. 
Published: July 2009

Abstract
Pharmaceuticals may be released to the soil environment through the application of biosolids to land. To understand those factors affecting the persistence of pharmaceuticals in the soil environment, the present study was performed to assess the effects of soil type, the presence of biosolids, and the impact of chemical mixture interactions on the degradation of three pharmaceuticals: naproxen, carbamazepine, and fluoxetine. Single-compound studies showed that naproxen degraded in a range of soils with half-lives ranging from 3.1 to 6.9 d and in biosolids with a half-life of 10.2 d. No relationships were observed between degradation rate and soil physicochemical properties and soil bioactivity. For naproxen, addition of biosolids to soils reduced the degradation rate observed in the soil-only studies, with half-lives in the soil-biosolid systems ranging from 3.9 to 15.1 d. Carbamazepine and fluoxetine were found to be persistent in soils, biosolids, and soil-biosolid mixtures. When degradation was assessed using a mixture of the three study compounds and the sulfonamide antibiotic sulfamethazine, the degradation behavior of fluoxetine and carbamazepine was similar to that observed in the single compound studies (i.e., no degradation). However, the degradation rate of naproxen in soils, biosolids, and soil-biosolid systems spiked with the mixture was significantly slower than in the single-compound studies. As degradation studies for risk assessment purposes are performed using single substances in soil-only studies, it is possible that current risk assessment procedures will underestimate environmental impacts. Further work is therefore warranted on a larger range of substances, soils, biosolid types, and chemical mixtures to better understand the fate of pharmaceuticals in terrestrial systems.