Bone mineral density in patients with inherited bone marrow failure syndromes.

BackgroundPatients with inherited bone marrow failure syndromes (IBMFS) may have several risk factors for low bone mineral density (BMD). We aimed to evaluate the prevalence of low BMD in IBMFS and determine the associated risk factors.MethodsPatients with IBMFS with at least one dual-energy X-ray absorptiometry (DXA) scan were evaluated. Diagnosis of each IBMFS, Fanconi anemia (FA), dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome was confirmed by syndrome-specific tests. Data were gathered on age, height, and clinical history. DXA scans were completed at the lumbar spine, femoral neck, and forearm. BMD was adjusted for height (HAZ) in children (age ≤20 years). Low BMD was defined as a BMD Z-score and HAZ ≤-2 in adults and children, respectively, in addition to patients currently on bisphosphonate therapy.ResultsNine of thirty-five adults (26%) and eleven of forty children (27%) had low BMD. Adults with FA had significantly lower BMD Z-scores than those with other diagnoses; however, HAZ did not vary significantly in children by diagnosis. Risk factors included hypogonadism, iron overload, and glucocorticoid use.ConclusionsAdults and children with IBMFS have high prevalence of low BMD. Prompt recognition of risk factors and management are essential to optimize bone health

Connecting Communities: Third Generation Community Network Projects

This article discuss the evolution of the community network movement and provides practical advice about how Extension educators can work with local leaders and community residents to initiate projects that increase diffusion and adoption of information technologies in their communities. Experience in Pennsylvania shows that the community development processes used to develop third generation community network projects increases the diffusion and adoption of information technologies and builds human and organizational capacity useful for addressing a wide variety of community issues. Readers are introduced to Connecting Rural Communities, a guide to enhancing adoption of technology tools and infrastructure in rural communities

Sequential effects of propofol on functional brain activation induced by auditory language processing: an event‐related functional magnetic resonance imaging study

Background. We have investigated the effect of propofol on language processing using event‐related functional magnetic resonance imaging (MRI). Methods. Twelve healthy male volunteers underwent MRI scanning at a magnetic field strength of 3 Tesla while performing an auditory language processing task. Functional images were acquired from the perisylvian cortical regions that are associated with auditory and language processing. The experiment consisted of three blocks: awake state (block 1), induction of anaesthesia with 3 mg kg-1 propofol (block 2), and maintenance of anaesthesia with 3 mg kg-1 h-1 propofol (block 3). During each block normal sentences and pseudo‐word sentences were presented in random order. The subjects were instructed to press a button to indicate whether a sentence was made up of pseudo‐words or not. All subjects stopped responding during block two. The data collected before and after the subjects stopped responding during this block were analyzed separately. In addition, propofol plasma concentrations were measured and the effect‐site concentrations of propofol were calculated. Results. During wakefulness, language processing induced brain activation in a widely distributed temporofrontal network. Immediately after unresponsiveness, activation disappeared in frontal areas but persisted in both temporal lobes (block 2 second half, propofol effect‐site concentration: 1.51 µg ml-1). No activation differences related to the task were observed during block 3 (propofol effect‐site concentration: 4.35 µg ml-1). Conclusion. Our findings suggest sequential effects of propofol on auditory language processing networks. Brain activation firstly declines in the frontal lobe before it disappears in the temporal lobe. Br J Anaesth 2004; 92: 641-5

Renewable beta-elemene based cyclic carbonates for the preparation of oligo(hydroxyurethane)s

Conversion of β-elemene into new β-elemene dicarbonates through epoxidation and halide salt-catalyzed CO2 cycloaddition reactions is reported. Step-growth polyaddition of this dicarbonate to five different, commercial diamines was investigated under neat conditions at 150 °C yielding non-isocyanate-based low molecular weight oligo(hydroxyurethane)s with 1.3≤Mn≤6.3 kDa and 1.3≤Ð≤2.1, and with glass transition temperatures ranging from −59 to 84 °C. The preparation of one selected polyhydroxyurethane material, obtained in the presence of Jeffamine® D-2010 was scaled-up to 43 g. The latter, when combined in a formulation using Irgacure® 2100 and Laromer® LR 9000 allowed the preparation of coatings that were analyzed with several techniques showing the potential of these biobased oligourethanes towards the preparation of commercially relevant materials

IMMUNE RESPONSES IN VITRO : VI. GENETIC CONTROL OF THE IN VIVO-IN VITRO DISCREPANCIES IN 19S ANTIBODY SYNTHESIS

The finding that the relationship of the in vitro and in vivo responses of different strains of mice is under genetic control indicates that at least two mechanisms must operate under in vivo conditions to control 19S antibody synthesis. One is involved in the termination of 19S antibody synthesis; the other has a regulatory role on the magnitude of the response. In light of these findings, various concepts based on other genetically controlled immune responses and on the limiting dilution technique should be reassessed. Furthermore, the suppressive in vivo mechanism may be an important type of control in the resistance or susceptibility to the establishment or maintainance of neoplasms

Comment on ``Protective measurements of the wave function of a single squeezed harmonic-oscillator state''

Alter and Yamamoto [Phys. Rev. A 53, R2911 (1996)] claimed to consider ``protective measurements'' [Phys. Lett. A 178, 38 (1993)] which we have recently introduced. We show that the measurements discussed by Alter and Yamamoto ``are not'' the protective measurements we proposed. Therefore, their results are irrelevant to the nature of protective measurements.Comment: 2 pages LaTe

The brain generates its own sentence melody: A Gestalt phenomenon in speech perception

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Health information portals: How can we improve the user\u27s search experience?

Health information portals (HIP) are now common place. Governments and other health agencies are using HIPs extensively to reduce costs and distribute information more effectively. Generally, HIPs are not very technically sophisticated specifically in terms of options for improving searching. There are many ways in which searching and retrieving relevant information can be improved. This paper presents an exploratory study which investigated five HIPs. Each HIP offered a range of features and functionality to assist with searching. Our research explored the features and functionality of each HIP. Through usability evaluations we compared the response of users to each HIP and identified users’ preferences for improved searching. We found that HIPs with improved search functionality and other features that assisted searching were better received by the users. Users regarded these portals as easier to understand, easier to use, required fewer steps in retrieving information and were more likely to say they would return. Comments from users are provided to illustrate further the importance of providing effective functionality. The paper concludes with recommendations for Health Information Portal builders on what is needed to improve the user search experience