Tyrosine monitoring in children with early and continuously treated phenylketonuria: Results of an international practice survey

Investigations into the biochemical markers associated with executive function (EF) impairment in children with early and continuously treated phenylketonuria (ECT-PKU) remain largely phenylalanine-only focused, despite experimental data showing that a high phenylalanine:tyrosine (phe:tyr) ratio is more strongly associated with EF deficit than phe alone. A high phe:tyr ratio is hypothesized to lead to a reduction in dopamine synthesis within the brain, which in turn results in the development of EF impairment. This paper provides a snapshot of current practice in the monitoring and/or treatment of tyrosine levels in children with PKU, across 12 countries from Australasia, North America and Europe. Tyrosine monitoring in this population has increased over the last 5 years, with over 80% of clinics surveyed reporting routine monitoring of tyrosine levels in infancy alongside phe levels. Twenty-five percent of clinics surveyed reported actively treating/managing tyrosine levels (with supplemental tyrosine above that contained in PKU formulas) to ensure tyrosine levels remain within normal ranges. Anecdotally, supplemental tyrosine has been reported to ameliorate symptoms of both attention deficit hyperactivity disorder and depression in this population. EF assessment of children with ECT-PKU was likewise highly variable, with 50% of clinics surveyed reporting routine assessments of intellectual function. However when function was assessed, test instruments chosen tended towards global measures of IQ prior to school entry, rather than specific assessment of EF development. Further investigation of the role of tyrosine and its relationship with phe and EF development is needed to establish whether routine tyrosine monitoring and increased supplementation is recommended.

PESSTO: Survey description and products from the first data release by the Public ESO Spectroscopic Survey of Transient Objects

Context. The Public European Southern Observatory Spectroscopic Survey of Transient Objects (PESSTO) began as a public spectroscopic survey in April 2012. PESSTO classifies transients from publicly available sources and wide-field surveys, and selects science targets for detailed spectroscopic and photometric follow-up. PESSTO runs for nine months of the year, January - April and August - December inclusive, and typically has allocations of 10 nights per month. 

Aims. We describe the data reduction strategy and data products that are publicly available through the ESO archive as the Spectroscopic Survey data release 1 (SSDR1). 

Methods. PESSTO uses the New Technology Telescope with the instruments EFOSC2 and SOFI to provide optical and NIR spectroscopy and imaging. We target supernovae and optical transients brighter than 20.5^m for classification. Science targets are selected for follow-up based on the PESSTO science goal of extending knowledge of the extremes of the supernova population. We use standard EFOSC2 set-ups providing spectra with resolutions of 13-18 Å between 3345-9995 Å. A subset of the brighter science targets are selected for SOFI spectroscopy with the blue and red grisms (0.935-2.53 μm and resolutions 23-33 Å) and imaging with broadband JHK<inf>s</inf> filters. 

Results. This first data release (SSDR1) contains flux calibrated spectra from the first year (April 2012-2013). A total of 221 confirmed supernovae were classified, and we released calibrated optical spectra and classifications publicly within 24 h of the data being taken (via WISeREP). The data in SSDR1 replace those released spectra. They have more reliable and quantifiable flux calibrations, correction for telluric absorption, and are made available in standard ESO Phase 3 formats. We estimate the absolute accuracy of the flux calibrations for EFOSC2 across the whole survey in SSDR1 to be typically ∼15%, although a number of spectra will have less reliable absolute flux calibration because of weather and slit losses. Acquisition images for each spectrum are available which, in principle, can allow the user to refine the absolute flux calibration. The standard NIR reduction process does not produce high accuracy absolute spectrophotometry but synthetic photometry with accompanying JHK<inf>s</inf> imaging can improve this. Whenever possible, reduced SOFI images are provided to allow this. 

Conclusions. Future data releases will focus on improving the automated flux calibration of the data products. The rapid turnaround between discovery and classification and access to reliable pipeline processed data products has allowed early science papers in the first few months of the survey.

International online survey to assess current practice in equine anaesthesia.

REASONS FOR PERFORMING STUDY Multicentre Confidential Enquiries into Perioperative Equine Fatalities (CEPEF) have not been conducted since the initial CEPEF Phases 1-3, 20 years ago. OBJECTIVES To collect data on current practice in equine anaesthesia and to recruit participants for CEPEF-4. STUDY DESIGN Online questionnaire survey. METHODS An online questionnaire was prepared and the link distributed internationally to veterinarians possibly performing equine anaesthesia, using emails, posters, flyers and an editorial. The questionnaire included 52 closed, semiclosed and open questions divided into 8 subgroups: demographic data, anaesthetist, anaesthesia management (preoperative, technical equipment, monitoring, drugs, recovery), areas of improvements and risks and motivation for participation in CEPEF-4. Descriptive statistics and Chi-squared tests for comparison of categorical variables were performed. RESULTS A total of 199 questionnaires were completed by veterinarians from 14 different countries. Of the respondents, 43% worked in private hospitals, 36% in private practices and 21% in university teaching hospitals. In 40 institutions (23%) there was at least one diplomate of the European or American colleges of veterinary anaesthesia and analgesia on staff. Individual respondents reported routinely employ the following anaesthesia monitoring modalities: electrocardiography (80%), invasive arterial blood pressures (70%), pulse oximetry (60%), capnography (55%), arterial blood gases (47%), composition of inspired and expired gases (45%) and body temperature (35%). Drugs administered frequently or routinely as part of a standard protocol were: acepromazine (44%), xylazine (68%), butorphanol (59%), ketamine (96%), diazepam (83%), isoflurane (76%), dobutamine (46%), and, as a nonsteroidal anti-inflammatory drug, phenylbutazone (73%) or flunixin meglumine (66%). Recovery was routinely assisted by 40%. The main factors perceived by the respondents to affect outcome of equine anaesthesia were the preoperative health status of the animal and training of the anaesthetist. CONCLUSIONS Current practice in equine anaesthesia varies widely, and the study has highlighted important topics relevant for designing a future prospective multicentre cohort study (CEPEF-4). The Summary is available in Chinese - see Supporting information.

Medical educators: How they define themselves - Results of an international web survey.

BACKGROUND Little is known about medical educators' self-definition. AIMS The aim of this study is to survey an international community of medical educators focusing on the medical educators' self-definition. METHODS Within a comprehensive, web-based survey, an open question on the participants' views of how they would define a "medical educator" was sent to 2200 persons on the mailing list of the Association for Medical Education in Europe. The free text definitions were analysed using qualitative thematic analysis. RESULTS Of the, 200 medical educators invited to participate, 685 (31.1%) provided a definition of a "medical educator". The qualitative analysis of the free text definitions revealed that medical educators defined themselves in 13 roles, primarily as "Professional Expert", "Facilitator", "Information Provider", "Enthusiast", "Faculty Developer", "Mentor", "Undergraduate and Postgraduate Trainer", "Curriculum Developer", "Assessor and Assessment Creator", and "Researcher". CONCLUSIONS Our survey revealed that medical educators predominantly define themselves as "Professional Experts" and identified 12 further self-defined roles of a medical educator, several of which not to have been reported previously. The results can be used to further the understanding of our professional identity.

Attitudes and management of alcohol problems in general practice: Descriptive analysis based on findings of a world health organization international collaborative survey

Aims: To determine if general practitioners' (GPs) experience of education on alcohol, support in their working environment for intervening with alcohol problems, and their attitudes have an impact on the number of patients they manage with alcohol problems. Methods: 1300 GPs from nine countries were surveyed with a postal questionnaire as part of a World Health Organization (WHO) collaborative study. Results: GPs who received more education on alcohol (OR = 1.5; 95% CI, 1.3-1.7), who perceived that they were working in a supportive environment (OR = 1.6; 95% CI, 1.4-1.9), who expressed higher role security in working with alcohol problems (OR = 2.0; 95% CI, 1.5-2.5) and who reported greater therapeutic commitment to working with alcohol problems (OR = 1.4: 95% CI, 1.1-1.7) were more likely to manage patients with alcohol-related harm. Conclusion: Both education and support in the working environment need to be provided to enhance the involvement of GPs in the management of alcohol problems.

The survey for ionization in neutral gas galaxies. I. Description and initial results

We introduce the Survey for Ionization in Neutral Gas Galaxies (SINGG), a census of star formation in H I selected galaxies. The survey consists of H alpha and R-band imaging of a sample of 468 galaxies selected from the H I Parkes All Sky Survey (HIPASS). The sample spans three decades in H I mass and is free of many of the biases that affect other star-forming galaxy samples. We present the criteria for sample selection, list the entire sample, discuss our observational techniques, and describe the data reduction and calibration methods. This paper focuses on 93 SINGG targets whose observations have been fully reduced and analyzed to date. The majority of these show a single emission line galaxy (ELG). We see multiple ELGs in 13 fields, with up to four ELGs in a single field. All of the targets in this sample are detected in H alpha, indicating that dormant (non-star-forming) galaxies with M-H I greater than or similar to 3x10(7) M-circle dot are very rare. A database of the measured global properties of the ELGs is presented. The ELG sample spans 4 orders of magnitude in luminosity (H alpha and R band), and H alpha surface brightness, nearly 3 orders of magnitude in R surface brightness and nearly 2 orders of magnitude in H alpha equivalent width (EW). The surface brightness distribution of our sample is broader than that of the Sloan Digital Sky Survey (SDSS) spectroscopic sample, the EW distribution is broader than prism-selected samples, and the morphologies found include all common types of star-forming galaxies (e.g., irregular, spiral, blue compact dwarf, starbursts, merging and colliding systems, and even residual star formation in S0 and Sa spirals). Thus, SINGG presents a superior census of star formation in the local universe suitable for further studies ranging from the analysis of H II regions to determination of the local cosmic star formation rate density.

Internet use by patients with bipolar disorder:results from an international multisite survey

There is considerable international interest in online education of patients with bipolar disorder, yet little understanding of how patients use the Internet and other sources to seek information. 1171 patients with bipolar disorder diagnosis in 17 countries completed a paper-based, anonymous survey. 81% of the patients used the Internet, a percentage similar to the general public. Older age, less education, and challenges in country telecommunications infrastructure and demographics decreased the odds of using the Internet. About 78% of the Internet users looked online for information on bipolar disorder or 63% of the total sample. More years of education in relation to the country mean, and feeling very confident about managing life decreased the odds of seeking information on bipolar disorder online, while having attended support groups increased the odds. Patients who looked online for information on bipolar disorder consulted medical professionals plus a mean of 2.3 other information sources such as books, physician handouts, and others with bipolar disorder. Patients not using the Internet consulted medical professionals plus a mean of 1.6 other information sources. The percentage of patients with bipolar disorder who use the Internet is about the same as the general public. Other information sources remain important.

Online information seeking by patients with bipolar disorder:results from an international multisite survey

Background: Information seeking is an important coping mechanism for dealing with chronic illness. Despite a growing number of mental health websites, there is little understanding of how patients with bipolar disorder use the Internet to seek information. Methods: A 39 question, paper-based, anonymous survey, translated into 12 languages, was completed by 1222 patients in 17 countries as a convenience sample between March 2014 and January 2016. All patients had a diagnosis of bipolar disorder from a psychiatrist. Data were analyzed using descriptive statistics and generalized estimating equations to account for correlated data. Results: 976 (81 % of 1212 valid responses) of the patients used the Internet, and of these 750 (77 %) looked for information on bipolar disorder. When looking online for information, 89 % used a computer rather than a smartphone, and 79 % started with a general search engine. The primary reasons for searching were drug side effects (51 %), to learn anonymously (43 %), and for help coping (39 %). About 1/3 rated their search skills as expert, and 2/3 as basic or intermediate. 59 % preferred a website on mental illness and 33 % preferred Wikipedia. Only 20 % read or participated in online support groups. Most patients (62 %) searched a couple times a year. Online information seeking helped about 2/3 to cope (41 % of the entire sample). About 2/3 did not discuss Internet findings with their doctor. Conclusion: Online information seeking helps many patients to cope although alternative information sources remain important. Most patients do not discuss Internet findings with their doctor, and concern remains about the quality of online information especially related to prescription drugs. Patients may not rate search skills accurately, and may not understand limitations of online privacy. More patient education about online information searching is needed and physicians should recommend a few high quality websites.

International survey of bicycling exposure, crash involvement, behaviors, and attitudes: Preliminary results

This paper presents some results from preliminary analyses of the data of an international online survey of bicycle riders, who reported riding at least once a month. On 4 July 2015, data from 7528 participants from 17 countries was available in the survey, and were subsequently cleaned and checked for consistency. The median distance ridden ranged from 30 km/week in Israel to 150 km/week in Greece (overall median 54 km/week). City/hybrid bicycles were the most common type of bicycle ridden (44%), followed by mountain (20%) and road bikes (15%). Almost half (47%) of the respondents rode “nearly daily”. About a quarter rode daily to work or study (27%). Overall, 40% of respondents reported wearing a helmet ‘always’, varying from 2% in the Netherlands to 80% in Norway, while 25% reported ‘never’ wearing a helmet. Thus, individuals appeared to consistently either use or not use helmets. Helmet wearing rates were generally higher when riding for health/fitness than other purposes and appeared to be little affected by the type of riding location, but some divergences in these patterns were found among countries. Almost 29% of respondents reported being involved in at least one bicycle crash in the last year (ranging from 12% in Israel to 53% in Turkey). Among the most severe crashes for each respondent, about half of the crashes involved falling off a bicycle. Just under 10% of the most severe crashes for each respondent were reported to police. Among the bicycle-motor vehicle crashes, only a third were reported to police. Further analyses will address questions regarding the influence of factors such as demographic characteristics, type of bicycle ridden, and attitudes on both bi-cycle use and helmet wearing rates.

The significant effect of endometriosis on physical, mental and social wellbeing: results from an international cross-sectional survey.

STUDY QUESTION To what extent do the management of endometriosis and the symptoms that remain after treatment affect the quality of life in women with the disease? SUMMARY ANSWER Many women with endometriosis had impaired quality of life and continued to suffer from endometriosis-associated symptoms even though their endometriosis has been managed in tertiary care centres. WHAT IS KNOWN ALREADY The existing literature indicates that quality of life and work productivity is reduced in women with endometriosis. However, most studies have small sample sizes, are treatment related or examine newly diagnosed patients only. STUDY DESIGN, SIZE, DURATION A cross-sectional questionnaire-based survey among 931 women with endometriosis treated in 12 tertiary care centres in 10 countries. PARTICIPANTS/MATERIALS, SETTING, METHODS Women diagnosed with endometriosis who had at least one contact related to endometriosis-associated symptoms during 2008 with a participating centre were enrolled into the study. The study investigated the effect of endometriosis on education, work and social wellbeing, endometriosis-associated symptoms and health-related quality of life, by using questions obtained from the World Endometriosis Research Foundation (WERF) GSWH instrument (designed and validated for the WERF Global Study on Women's Health) and the Short Form 36 version 2 (SF-36v2). MAIN RESULTS AND THE ROLE OF CHANCE Of 3216 women invited to participate in the study, 1450 (45%) provided informed consent and out of these, 931 (931/3216 = 29%) returned the questionnaires. Endometriosis had affected work in 51% of the women and affected relationships in 50% of the women at some time during their life. Dysmenorrhoea was reported by 59%, dyspareunia by 56% and chronic pelvic pain by 60% of women. Quality of life was decreased in all eight dimensions of the SF-36v2 compared with norm-based scores from a general US population (all P < 0.01). Multivariate regression analysis showed that number of co-morbidities, chronic pain and dyspareunia had an independent negative effect on both the physical and mental component of the SF-36v2. LIMITATIONS, REASONS FOR CAUTION The fact that women were enrolled in tertiary care centres could lead to a possible over-representation of women with moderate-to-severe endometriosis, because the participating centres typically treat more complex and referred cases of endometriosis. The response rate was relatively low. Since there was no Institute Review Board approval to do a non-responder investigation on basic characteristics, some uncertainty remains regarding the representativeness of the investigated population. WIDER IMPLICATIONS OF THE FINDINGS This international multicentre survey represents a large group of women with endometriosis, in all phases of the disease, which increases the generalizability of the data. Women still suffer from frequent symptoms, despite tertiary care management, in particular chronic pain and dyspareunia. As a result their quality of life is significantly decreased. A patient-centred approach with extensive collaboration across disciplines, such as pain specialists, psychologists, sexologists and social workers, may be a valuable strategy to improve the long-term care of women with endometriosis. STUDY FUNDING/COMPETING INTEREST(S) The WERF EndoCost study is funded by the World Endometriosis Research Foundation (WERF) through grants received from Bayer Schering Pharma AG, Takeda Italia Farmaceutici SpA, Pfizer Ltd and the European Society of Human Reproduction and Embryology. The sponsors did not have a role in the design and conduct of the study; collection, management, analysis and interpretation of the data; and preparation, review or approval of the manuscript. L.H. is the chief executive and T.D. was a board member of WERF at the time of funding. T.D. holds the Merck-Serono Chair in Reproductive Medicine and Surgery, and the Ferring Chair in Reproductive Medicine at the Katholieke Universiteit Leuven in Belgium and has served as consultant/research collaborator for Merck-Serono, Schering-Plough, Astellas and Arresto.

Survey on Rules on Loss of Nationality in International Treaties and Case Law. CEPS Paper in Liberty and Security in Europe No. 57, 30 August 2013

This paper offers a picture of the obligations existing under international and European law in respect of the loss of nationality. It describes international instruments including obligations in this field with direct relevancy for the loss of nationality of Member States of the European Union, but also obligations regarding loss of nationality in regional non-European treaties. Attention is given to two important judicial decisions of the European Court of Justice (Janko Rottmann) and the European Court of Human Rights (Genovese v Malta) regarding nationality. Special attention is devoted to Article 15 of the Universal Declaration of Human Rights, which forbids the arbitrary deprivation of nationality. A survey is provided of possible sub-principles that can be derived from this rule. Finally, some observations are made on the burden of proof in cases of loss of nationality.

THE VLT-FLAMES TARANTULA SURVEY: THE FASTEST ROTATING O-TYPE STAR AND SHORTEST PERIOD LMC PULSAR-REMNANTS OF A SUPERNOVA DISRUPTED BINARY?

We present a spectroscopic analysis of an extremely rapidly rotating late O-type star, VFTS102, observed during a spectroscopic survey of 30 Doradus. VFTS102 has a projected rotational velocity larger than 500 km s(-1) and probably as large as 600 km s-1; as such it would appear to be the most rapidly rotating massive star currently identified. Its radial velocity differs by 40 kms(-1) from the mean for 30 Doradus, suggesting that it is a runaway. VFTS102 lies 12 pc from the X-ray pulsar PSR J0537-6910 in the tail of its X-ray diffuse emission. We suggest that these objects originated from a binary system with the rotational and radial velocities of VFTS102 resulting from mass transfer from the progenitor of PSR J0537-691 and the supernova explosion, respectively.

A green profession? A global survey of RICS members and their engagement with the sustainability agenda

Purpose – This study seeks to provide a review of the background and context to the engagement of RICS members with the sustainability agenda, and to examine the extent to which the surveying profession uses relevant information, tools and techniques to achieve the key objectives of sustainable development (or sustainability). Design/methodology/approach – The paper analyses results from a major international online survey of 4,600 RICS respondent members, supported by 31 structured telephone interviews. Findings – The results suggest that, although sustainability is highly relevant to RICS members’ work, a lack of knowledge and expertise is making it more difficult for sustainability tools and other information to be used effectively. Research limitations/implications – The survey is based on a substantial number of responses which are broadly representative of the global RICS population. A key implication is that “laggard” faculties include the disciplines of commercial property and valuation. Practical implications – The research suggests that key stakeholders must work together to provide better information, guidance and education and training to “hardwire” the sustainability agenda across RICS faculties. Originality/value – This is the first truly global survey of its kind and focuses particularly on those faculties that play a major role in property investment and finance (i.e. valuation and commercial property), comparing their position with that of other faculties in an international context.

The VIMOS-VLT Deep Survey: the evolution of type-1 AGN

Quasars and AGN play an important role in many aspects of the modern cosmology. Of particular interest is the issue of the interplay between AGN activity and formation and evolution of galaxies and structures. Studies on nearby galaxies revealed that most (and possibly all) galaxy nuclei contain a super-massive black hole (SMBH) and that between a third and half of them are showing some evidence of activity (Kormendy and Richstone, 1995). The discovery of a tight relation between black holes mass and velocity dispersion of their host galaxy suggests that the evolution of the growth of SMBH and their host galaxy are linked together. In this context, studying the evolution of AGN, through the luminosity function (LF), is fundamental to constrain the theories of galaxy and SMBH formation and evolution. Recently, many theories have been developed to describe physical processes possibly responsible of a common formation scenario for galaxies and their central black hole (Volonteri et al., 2003; Springel et al., 2005a; Vittorini et al., 2005; Hopkins et al., 2006a) and an increasing number of observations in different bands are focused on collecting larger and larger quasar samples. Many issues remain however not yet fully understood. In the context of the VVDS (VIMOS-VLT Deep Survey), we collected and studied an unbiased sample of spectroscopically selected faint type-1 AGN with a unique and straightforward selection function. Indeed, the VVDS is a large, purely magnitude limited spectroscopic survey of faint objects, free of any morphological and/or color preselection. We studied the statistical properties of this sample and its evolution up to redshift z 4. Because of the contamination of the AGN light by their host galaxies at the faint magnitudes explored by our sample, we observed that a significant fraction of AGN in our sample would be missed by the UV excess and morphological criteria usually adopted for the pre-selection of optical QSO candidates. If not properly taken into account, this failure in selecting particular sub-classes of AGN could, in principle, affect some of the conclusions drawn from samples of AGN based on these selection criteria. The absence of any pre-selection in the VVDS leads us to have a very complete sample of AGN, including also objects with unusual colors and continuum shape. The VVDS AGN sample shows in fact redder colors than those expected by comparing it, for example, with the color track derived from the SDSS composite spectrum. In particular, the faintest objects have on average redder colors than the brightest ones. This can be attributed to both a large fraction of dust-reddened objects and a significant contamination from the host galaxy. We have tested these possibilities by examining the global spectral energy distribution of each object using, in addition to the U, B, V, R and I-band magnitudes, also the UV-Galex and the IR-Spitzer bands, and fitting it with a combination of AGN and galaxy emission, allowing also for the possibility of extinction of the AGN flux. We found that for 44% of our objects the contamination from the host galaxy is not negligible and this fraction decreases to 21% if we restrict the analysis to a bright subsample (M1450 <-22.15). Our estimated integral surface density at IAB < 24.0 is 500 AGN per square degree, which represents the highest surface density of a spectroscopically confirmed sample of optically selected AGN. We derived the luminosity function in B-band for 1.0 < z < 3.6 using the 1/Vmax estimator. Our data, more than one magnitude fainter than previous optical surveys, allow us to constrain the faint part of the luminosity function up to high redshift. A comparison of our data with the 2dF sample at low redshift (1 < z < 2.1) shows that the VDDS data can not be well fitted with the pure luminosity evolution (PLE) models derived by previous optically selected samples. Qualitatively, this appears to be due to the fact that our data suggest the presence of an excess of faint objects at low redshift (1.0 < z < 1.5) with respect to these models. By combining our faint VVDS sample with the large sample of bright AGN extracted from the SDSS DR3 (Richards et al., 2006b) and testing a number of different evolutionary models, we find that the model which better represents the combined luminosity functions, over a wide range of redshift and luminosity, is a luminosity dependent density evolution (LDDE) model, similar to those derived from the major Xsurveys. Such a parameterization allows the redshift of the AGN density peak to change as a function of luminosity, thus fitting the excess of faint AGN that we find at 1.0 < z < 1.5. On the basis of this model we find, for the first time from the analysis of optically selected samples, that the peak of the AGN space density shifts significantly towards lower redshift going to lower luminosity objects. The position of this peak moves from z 2.0 for MB <-26.0 to z 0.65 for -22< MB <-20. This result, already found in a number of X-ray selected samples of AGN, is consistent with a scenario of “AGN cosmic downsizing”, in which the density of more luminous AGN, possibly associated to more massive black holes, peaks earlier in the history of the Universe (i.e. at higher redshift), than that of low luminosity ones, which reaches its maximum later (i.e. at lower redshift). This behavior has since long been claimed to be present in elliptical galaxies and it is not easy to reproduce it in the hierarchical cosmogonic scenario, where more massive Dark Matter Halos (DMH) form on average later by merging of less massive halos.