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 χ(2)\chi^{(2)} waveguides: the quantum optical dimer

A model is proposed where two $\chi^{(2)}$ 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

Detection of sub-shot-noise spatial correlation in high-gain parametric down-conversion

Using a 1GW-1ps pump laser pulse in high gain parametric down-conversion allows us to detect sub-shot-noise spatial quantum correlation with up to one hundred photoelectrons per mode, by means of a high efficiency CCD. The statistics is performed in single-shot over independent spatial replica of the system. The paper highlights the evidence of quantum correlation between symmetrical signal and idler spatial areas in the far field, in the high gain regime. In accordance with the predictions of numerical calculations the observed transition from the quantum to the classical regime is interpreted as a consequence of the narrowing of the down-converted beams in the very high gain regime.Comment: 4,2 pages, 4 figure

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

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.

Puck protocol use case at obsea. Puck is the newest ogc standard for instrument identification and communication

Peer Reviewe

Ultraearly thrombolysis by an anesthesiologist in a mobile stroke unit: A prospective, controlled intervention study

Background Acute stroke treatment in mobile stroke units (MSU) is feasible and reduces time-to-treatment, but the optimal staffing model is unknown. We wanted to explore if integrating thrombolysis of acute ischemic stroke (AIS) in an anesthesiologist-based emergency medical services (EMS) reduces time-to-treatment and is safe. Methods A nonrandomized, prospective, controlled intervention study. Inclusion criteria: age ≥18 years, nonpregnant, stroke symptoms with onset ≤4 h. The MSU staffing is inspired by the Norwegian Helicopter Emergency Medical Services crew with an anesthesiologist, a paramedic-nurse and a paramedic. Controls were included by conventional ambulances in the same catchment area. Primary outcome was onset-to-treatment time. Secondary outcomes were alarm-to-treatment time, thrombolytic rate and functional outcome. Safety outcomes were symptomatic intracranial hemorrhage and mortality. Results We included 440 patients. MSU median (IQR) onset-to-treatment time was 101 (71–155) minutes versus 118 (90–176) minutes in controls, p = 0.007. MSU median (IQR) alarm-to-treatment time was 53 (44–65) minutes versus 74 (63–95) minutes in controls, p < 0.001. Golden hour treatment was achieved in 15.2% of the MSU patients versus 3.7% in the controls, p = 0.005. The thrombolytic rate was higher in the MSU (81% vs 59%, p = 0.001). MSU patients were more often discharged home (adjusted OR [95% CI]: 2.36 [1.11–5.03]). There were no other significant differences in outcomes. Conclusions Integrating thrombolysis of AIS in the anesthesiologist-based EMS reduces time-to-treatment without negatively affecting outcomes. An MSU based on the EMS enables prehospital assessment of acute stroke in addition to other medical and traumatic emergencies and may facilitate future implementation.publishedVersio

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

Effects of osteopontin inhibition on radiosensitivity of MDA-MB-231 breast cancer cells

<p>Abstract</p> <p>Background</p> <p>Osteopontin (OPN) is a secreted glycophosphoprotein that is overexpressed in various tumors, and high levels of OPN have been associated with poor prognosis of cancer patients. In patients with head and neck cancer, high OPN plasma levels have been associated with poor prognosis following radiotherapy. Since little is known about the relationship between OPN expression and radiosensitivity, we investigated the cellular and radiation induced effects of OPN siRNA in human MDA-MB-231 breast cancer cells.</p> <p>Methods</p> <p>MDA-MB-231 cells were transfected with OPN-specific siRNAs and irradiated after 24 h. To verify the OPN knockdown, we measured the OPN mRNA and protein levels using qRT-PCR and Western blot analysis. Furthermore, the functional effects of OPN siRNAs were studied by assays to assess clonogenic survival, migration and induction of apoptosis.</p> <p>Results</p> <p>Treatment of MDA-MB-231 cells with OPN siRNAs resulted in an 80% decrease in the OPN mRNA level and in a decrease in extracellular OPN protein level. Transfection reduced clonogenic survival to 42% (p = 0.008), decreased the migration rate to 60% (p = 0.15) and increased apoptosis from 0.3% to 1.7% (p = 0.04). Combination of OPN siRNA and irradiation at 2 Gy resulted in a further reduction of clonogenic survival to 27% (p < 0.001), decreased the migration rate to 40% (p = 0.03) and increased apoptosis to 4% (p < 0.005). Furthermore, OPN knockdown caused a weak radiosensitization with an enhancement factor of 1.5 at 6 Gy (p = 0.09) and a dose modifying factor (DMF₁₀) of 1.1.</p> <p>Conclusion</p> <p>Our results suggest that an OPN knockdown improves radiobiological effects in MDA-MB-231 cells. Therefore, OPN seems to be an attractive target to improve the effectiveness of radiotherapy.</p