1,073 research outputs found
Orthopedic management of the extremities in patients with Morquio A syndrome.
BackgroundMusculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities.PurposeThe objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome.MethodsA group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached.Results and conclusionsThis present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes
Nonclassical statistics of intracavity coupled waveguides: the quantum optical dimer
A model is proposed where two nonlinear waveguides are contained
in a cavity suited for second-harmonic generation. The evanescent wave coupling
between the waveguides is considered as weak, and the interplay between this
coupling and the nonlinear interaction within the waveguides gives rise to
quantum violations of the classical limit. These violations are particularly
strong when two instabilities are competing, where twin-beam behavior is found
as almost complete noise suppression in the difference of the fundamental
intensities. Moreover, close to bistable transitions perfect twin-beam
correlations are seen in the sum of the fundamental intensities, and also the
self-pulsing instability as well as the transition from symmetric to asymmetric
states display nonclassical twin-beam correlations of both fundamental and
second-harmonic intensities. The results are based on the full quantum Langevin
equations derived from the Hamiltonian and including cavity damping effects.
The intensity correlations of the output fields are calculated
semi-analytically using a linearized version of the Langevin equations derived
through the positive-P representation. Confirmation of the analytical results
are obtained by numerical simulations of the nonlinear Langevin equations
derived using the truncated Wigner representation.Comment: 15 pages, 8 figures, submitted to Phys. Rev.
Characterisation of an Advanced Nickel Based Superalloy Post Cold Work by Swaging
Cylindrical bars of the advanced nickel based superalloy RR1000 were subjected to swaging to induce approximately 30% cold work. Grain size analysis demonstrated a distinct modification to the microstructure whilst electron back scattered diffraction (EBSD) measurements confirmed the evolution of a relatively strong texture parallel with the longitudinal bar axis. Intragranular strain damage was identified. The effects of the swaging on bulk mechanical properties are illustrated across a range of test temperatures
Epidemiology of developmental dysplasia of the hip within the UK: refining the risk factors.
The epidemiology and risk factors for developmental dysplasia of the hip (DDH) are still being refined. We investigated the local epidemiology of DDH in order to define incidence, identify risk factors, and refine our policy on selective ultrasound screening.This article is freely available via Open Access. Click on the Additional Link above to access the full-text via the publisher's site
Traps of multi-level governance. Lessons from the implementation of the Water Framework Directive in Italy
During recent decades, different patterns of multi-level governance (MLG) have spread across Europe as a consequence of Europeanisation of public policies, which have increasingly adopted decentralized and participatory procedures conceived as a tool of more effective and accountable policy-making. It appears, however, that the implementation of operational designs based on MLG may be rather problematic and it does not necessarily bring to the expected performance improvements. Referring to the case of the EU Water Framework Directive (2000/60/EC), which conceives the creation of new multi-level institutional settings as a key tool for enacting a new holistic approach to water management and protection, this article explores the difficulties that the implementation of such settings has brought in Italy, despite some favorable pre-conditions existing in the country. Evidence is provided that along with institutional and agency variables, the implementation effectiveness of MLG arrangements promoted by the EU can be challenged by their inherent characteristics
Puck protocol use case at obsea. Puck is the newest ogc standard for instrument identification and communication
Peer Reviewe
Quantum properties of transverse pattern formation in second-harmonic generation
We investigate the spatial quantum noise properties of the one dimensional
transverse pattern formation instability in intra-cavity second-harmonic
generation. The Q representation of a quasi-probability distribution is
implemented in terms of nonlinear stochastic Langevin equations. We study these
equations through extensive numerical simulations and analytically in the
linearized limit. Our study, made below and above the threshold of pattern
formation, is guided by a microscopic scheme of photon interaction underlying
pattern formation in second-harmonic generation. Close to the threshold for
pattern formation, beams with opposite direction of the off-axis critical wave
numbers are shown to be highly correlated. This is observed for the fundamental
field, for the second harmonic field and also for the cross-correlation between
the two fields. Nonlinear correlations involving the homogeneous transverse
wave number, which are not identified in a linearized analysis, are also
described. The intensity differences between opposite points of the far fields
are shown to exhibit sub-Poissonian statistics, revealing the quantum nature of
the correlations. We observe twin beam correlations in both the fundamental and
second-harmonic fields, and also nonclassical correlations between them.Comment: 18 pages, 17 figures, submitted to Phys. Rev.
Determinants of antibody persistence across doses and continents after single-dose rVSV-ZEBOV vaccination for Ebola virus disease: an observational cohort study.
BACKGROUND: The recombinant vesicular stomatitis virus (rVSV) vaccine expressing the Zaire Ebola virus (ZEBOV) glycoprotein is efficacious in the weeks following single-dose injection, but duration of immunity is unknown. We aimed to assess antibody persistence at 1 and 2 years in volunteers who received single-dose rVSV-ZEBOV in three previous trials. METHODS: In this observational cohort study, we prospectively followed-up participants from the African and European phase 1 rVSV-ZEBOV trials, who were vaccinated once in 2014-15 with 300â000 (low dose) or 10-50 million (high dose) plaque-forming units (pfu) of rVSV-ZEBOV vaccine to assess ZEBOV glycoprotein (IgG) antibody persistence. The primary outcome was ZEBOV glycoprotein-specific IgG geometric mean concentrations (GMCs) measured yearly by ELISA compared with 1 month (ie, 28 days) after immunisation. We report GMCs up to 2 years (Geneva, Switzerland, including neutralising antibodies up to 6 months) and 1 year (LambarĂ©nĂ©, Gabon; Kilifi, Kenya) after vaccination and factors associated with higher antibody persistence beyond 6 months, according to multivariable analyses. Trials and the observational study were registered at ClinicalTrials.gov (Geneva: NCT02287480 and NCT02933931; Kilifi: NCT02296983) and the Pan-African Clinical Trials Registry (LambarĂ©nĂ© PACTR201411000919191). FINDINGS: Of 217 vaccinees from the original studies (102 from the Geneva study, 75 from the LambarĂ©nĂ© study, and 40 from the Kilifi study), 197 returned and provided samples at 1 year (95 from the Geneva study, 63 from the LambarĂ©nĂ©, and 39 from the Kilifi study) and 90 at 2 years (all from the Geneva study). In the Geneva group, 44 (100%) of 44 participants who had been given a high dose (ie, 10-50 million pfu) of vaccine and who were seropositive at day 28 remained seropositive at 2 years, whereas 33 (89%) of 37 who had been given the low dose (ie, 300â000 pfu) remained seropositive for 2 years (p=0·042). In participants who had received a high dose, ZEBOV glycoprotein IgG GMCs decreased significantly between their peak (at 1-3 months) and month 6 after vaccination in Geneva (p0·05). Neutralising antibodies seem to be less durable, with seropositivity dropping from 64-71% at 28 days to 27-31% at 6 months in participants from the Geneva study. INTERPRETATION: Antibody responses to single-dose rVSV-ZEBOV vaccination are sustained across dose ranges and settings, a key criterion in countries where booster vaccinations would be impractical. FUNDING: The Wellcome Trust and Innovative Medicines Initiative 2 Joint Undertaking
Systems of education governance and cultures of justice in Ireland, Scotland and Pakistan
This chapter compares the issue of cultures of justice in the systems of education governance in three education systems: Ireland, Scotland and Pakistan. The focus for the comparison are the current policies which shape the regulation of education. These policies were reviewed to identify key issues relating to social justice and equality, decision-making and accountability. From the analysis of each system, three central issues were identified: firstly, the improvement of a state education system; secondly, the degree of decentralisation and centralisation in governance structures and thirdly, the expectations placed on school leaders. The chapter concludes by discussing the tensions between the drive for system improvement and opportunities for school leaders to build strategies to address issues of inequality in schools
Application of Bayesian statistics to estimate nitrous oxide emission factors of the nitrogen fertilisers in UK grasslands
Trapezoidal integration by linear interpolation of data points is by far the most commonly used method of
cumulative flux calculations of nitrous oxide (N2O) in studies that use flux chambers; however, this method is
incapable of providing accurate uncertainty estimates. A Bayesian approach was used to calculate N2O emission
factors (EFs) and their associated uncertainties from flux chamber measurements made after the application of
nitrogen fertilisers, in the form of ammonium nitrate (AN), urea (Ur) and urea treated with AgrotainÂź urease
inhibitor (UI) at four grassland sites in the UK. The comparison between the cumulative fluxes estimated using
the Bayesian and linear interpolation methods were broadly similar (R2=0.79); however, the Bayesian method
was capable of providing realistic uncertainties when a limited number of data points is available. The study
reports mean EF values (and 95% confidence intervals) of 0.60 ± 0.63, 0.29 ± 0.22 and 0.26 ± 0.17% of
applied N emitted as N2O for the AN, Ur and UI treatments, respectively. There was no significant difference
between N2O emissions from the Ur and UI treatments. In the case of the automatic chamber data collected at
one site in this study, the data did not fit the log-normal model, implying that more complex models may be
needed, particularly for measurement data with high temporal resolutioninfo:eu-repo/semantics/publishedVersio
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