The revised FLACC observational pain tool: improved reliability and validity for pain assessment in children with cognitive impairment

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/72254/1/j.1460-9592.2005.01773.x.pd

A Case of Noncompaction of the Ventricular Myocardium Combined with Situs Ambiguous with Polysplenia

A 33-year-old man was admitted to our hospital with chest pain and exertional dyspnea. Two-dimensional echocardiography showed prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Thoracoabdominal CT and cardiac magnetic resonance imaging (CMR) revealed situs ambiguous with polysplenia and noncompaction of the left ventricular myocardium. CMR also demonstrated delayed enhancement of the trabeculations located at the apical portion of the left ventricle. The coronary angiogram was normal. This is the first case of noncompaction of the ventricular myocardium associated with situs ambiguous with polysplenia

Agalsidase beta treatment is associated with improved quality of life in patients with Fabry disease: Findings from the Fabry Registry

Purpose: To evaluate the effect of agalsidase beta on longitudinal health-related quality of life in patients with Fabry disease. Methods: the SF-36 (R) Health Survey was used to measure health-related quality of life in Fabry Registry patients. Seventy-one men and 59 women who were treated with agalsidase beta (median dose: 1.0 mg/kg/2 weeks) and who had baseline and at least 2 yearly posttreatment health-related quality of life measurements were included in these analyses. A repeated measures model was used to analyze change in score from baseline. Results: Men improved in the physical component summary and in all eight scales of the SF-36 after 1 and 2 years and in the mental component summary after 1 year of agalsidase beta treatment (P < 0.05). Women improved in the mental component summary and in six of the eight scales after 1 and/or 2 years of treatment. Patients whose baseline SF-36 scores were below the median showed the greatest improvements. These responses were comparable with or greater than the published effects of various treatments for multiple sclerosis, rheumatoid arthritis, central neuropathic pain, and Gaucher disease. Conclusion: Long-term treatment with agalsidase beta resulted in substantial improvements in health-related quality of life in both men and women; the effect was more pronounced in men. Genet Med 2010:12(11):703 712.Genzyme CorporationGenzymeNatl Univ Hosp, Dept Endocrinol, DK-2100 Copenhagen, DenmarkSan Bassano Hosp, Dept Neurol, Bassano Del Grappa, ItalyUniv Padua, Dept Neurosci, Padua, ItalyUniv Wurzburg, Dept Med, Wurzburg, GermanyColumbia Univ, Dept Pediat, Div Clin Genet, Coll Phys & Surg, New York, NY 10027 USACincinnati Childrens Hosp, Div Human Genet, Cincinnati, OH USAUniversidade Federal de São Paulo, Inatos Metab CREIM, São Paulo, BrazilMassachusetts Gen Hosp, Dept Neurol, Boston, MA 02114 USAGenzyme Corp, Dept Biomed Data Sci & Informat, Cambridge, MA USAUniversidade Federal de São Paulo, Inatos Metab CREIM, São Paulo, BrazilWeb of Scienc

Challenges in Survey Research

While being an important and often used research method, survey research has been less often discussed on a methodological level in empirical software engineering than other types of research. This chapter compiles a set of important and challenging issues in survey research based on experiences with several large-scale international surveys. The chapter covers theory building, sampling, invitation and follow-up, statistical as well as qualitative analysis of survey data and the usage of psychometrics in software engineering surveys.Comment: Accepted version of chapter in the upcoming book on Contemporary Empirical Methods in Software Engineering. Update includes revision of typos and additional figures. Last update includes fixing two small issues and typo

How Much Does Effortful Thinking Underlie Observers’ Reactions to Victimization?

From blaming to helping innocent victims, just-world research has revealed that observers react to victimization in a variety of ways. Recent research suggests that such responses to victimization require effortful thought, whereas other research has shown that people can react to these situations intuitively. Along with manipulating just-world threat, across seven experiments, we manipulated or measured participants’ level of mental processing before assessing judgments of victim derogation, blame, willingness to help, and ultimate justice reasoning. The effect of just-world threat on these responses held constant over a range of manipulations/measures, suggesting that the processes involved in maintaining a belief in a just world are not restricted to the rational, deliberative level of mental processing but also occur intuitively

Receptor-Mediated Endocytosis of α-Galactosidase A in Human Podocytes in Fabry Disease

Injury to the glomerular podocyte is a key mechanism in human glomerular disease and podocyte repair is an important therapeutic target. In Fabry disease, podocyte injury is caused by the intracellular accumulation of globotriaosylceramide. This study identifies in the human podocyte three endocytic receptors, mannose 6-phosphate/insulin-like growth II receptor, megalin, and sortilin and demonstrates their drug delivery capabilities for enzyme replacement therapy. Sortilin, a novel α-galactosidase A binding protein, reveals a predominant intracellular expression but also surface expression in the podocyte. The present study provides the rationale for the renal effect of treatment with α-galactosidase A and identifies potential pathways for future non-carbohydrate based drug delivery to the kidney podocyte and other potential affected organs

Near-infrared scattered light properties of the HR 4796 A dust ring. A measured scattering phase function from 13.6° to 166.6°

$\textit{Context}$. HR 4796 A is surrounded by a debris disc, observed in scattered light as an inclined ring with a high surface brightness. Past observations have raised several questions. First, a strong brightness asymmetry detected in polarised reflected light has recently challenged our understanding of scattering by the dust particles in this system. Secondly, the morphology of the ring strongly suggests the presence of planets, although no planets have been detected to date. $\textit{Aims}$. We aim here at measuring with high accuracy the morphology and photometry of the ring in scattered light, in order to derive the phase function of the dust and constrain its near-infrared spectral properties. We also want to constrain the presence of planets and set improved constraints on the origin of the observed ring morphology. $\textit{Methods}$. We obtained high-angular resolution coronagraphic images of the circumstellar environment around HR 4796 A with VLT/SPHERE during the commissioning of the instrument in May 2014 and during guaranteed-time observations in February 2015. The observations reveal for the first time the entire ring of dust, including the semi-minor axis that was previously hidden either behind the coronagraphic spot or in the speckle noise. $\textit{Results}$. We determine empirically the scattering phase function of the dust in the H band from 13.6° to 166.6°. It shows a prominent peak of forward scattering, never detected before, for scattering angles below 30°. We analyse the reflectance spectra of the disc from the 0.95 μm to 1.6 μm, confirming the red colour of the dust, and derive detection limits on the presence of planetary mass objects. $\textit{Conclusions}$. We confirm which side of the disc is inclined towards the Earth. The analysis of the phase function, especially below 45°, suggests that the dust population is dominated by particles much larger than the observation wavelength, of about 20 μm. Compact Mie grains of this size are incompatible with the spectral energy distribution of the disc, however the observed rise in scattering efficiency beyond 50° points towards aggregates which could reconcile both observables. We do not detect companions orbiting the star, but our high-contrast observations provide the most stringent constraints yet on the presence of planets responsible for the morphology of the dust.J.M. acknowledges financial support from the ESO fellowship programme. A.M.L. acknowledges the support from the ANR blanche GIPSE (ANR-14-CE33-0018) and the Labex OSUG. L.M. acknowledges support by STFC and ESO through graduate studentships and by the European Union through ERC grant number 279973. We would like to thank ESO staff and technical operators at the Paranal Observatory. We thank M. Meyer and D. Rouan for their valueable suggestions and comments during the review by the SPHERE internal board. We thank P. Delorme and E. Lagadec (SPHERE Data Center) for their work during the data reduction process. We thank V. Faramaz for the discussion on the eccentricity of the disc. SPHERE is an instrument designed and built by a consortium consisting of IPAG (Grenoble, France), MPIA (Heidelberg, Germany), LAM (Marseille, France), LESIA (Paris, France), Laboratoire Lagrange (Nice, France), INAF – Osservatorio di Padova (Italy), Observatoire de Genève (Switzerland), ETH Zurich (Switzerland), NOVA (Netherlands), ONERA (France) and ASTRON (Netherlands) in collaboration with ESO. SPHERE was funded by ESO, with additional contributions from CNRS (France), MPIA (Germany), INAF (Italy), FINES (Switzerland) and NOVA (Netherlands). SPHERE also received funding from the European Commission Sixth and Seventh Framework Programmes as part of the Optical Infrared Coordination Network for Astronomy (OPTICON) under grant number RII3-Ct-2004-001566 for FP6 (2004–2008), grant number 226604 for FP7 (2009–2012) and grant number 312430 for FP7 (2013–2016)