Epidemiology of Multiple Congenital Anomalies in Europe: A EUROCAT Population-Based Registry Study

Background: This study describes the prevalence, associated anomalies, and demographic characteristics of cases of multiple congenital anomalies (MCA) in 19 population-based European registries (EUROCAT) covering 959,446 births in 2004 and 2010. Methods: EUROCAT implemented a computer algorithm for classification of congenital anomaly cases followed by manual review of potential MCA cases by geneticists. MCA cases are defined as cases with two or more major anomalies of different organ systems, excluding sequences, chromosomal and monogenic syndromes. Results: The combination of an epidemiological and clinical approach for classification of cases has improved the quality and accuracy of the MCA data. Total prevalence of MCA cases was 15.8 per 10,000 births. Fetal deaths and termination of pregnancy were significantly more frequent in MCA cases compared with isolated cases (p<0.001) and MCA cases were more frequently prenatally diagnosed (p<0.001). Live born infants with MCA were more often born preterm (p<0.01) and with birth weight<2500 grams (p<0.01). Respiratory and ear, face, and neck anomalies were the most likely to occur with other anomalies (34% and 32%) and congenital heart defects and limb anomalies were the least likely to occur with other anomalies (13%) (p<0.01). However, due to their high prevalence, congenital heart defects were present in half of all MCA cases. Among males with MCA, the frequency of genital anomalies was significantly greater than the frequency of genital anomalies among females with MCA (p<0.001). Conclusion: Although rare, MCA cases are an important public health issue, because of their severity. The EUROCAT database of MCA cases will allow future investigation on the epidemiology of these conditions and related clinical and diagnostic problems

Velocity-space sensitivity of the time-of-flight neutron spectrometer at JET

The velocity-space sensitivities of fast-ion diagnostics are often described by so-called weight functions. Recently, we formulated weight functions showing the velocity-space sensitivity of the often dominant beam-target part of neutron energy spectra. These weight functions for neutron emission spectrometry (NES) are independent of the particular NES diagnostic. Here we apply these NES weight functions to the time-of-flight spectrometer TOFOR at JET. By taking the instrumental response function of TOFOR into account, we calculate time-of-flight NES weight functions that enable us to directly determine the velocity-space sensitivity of a given part of a measured time-of-flight spectrum from TOFOR

Relationship of edge localized mode burst times with divertor flux loop signal phase in JET

A phase relationship is identified between sequential edge localized modes (ELMs) occurrence times in a set of H-mode tokamak plasmas to the voltage measured in full flux azimuthal loops in the divertor region. We focus on plasmas in the Joint European Torus where a steady H-mode is sustained over several seconds, during which ELMs are observed in the Be II emission at the divertor. The ELMs analysed arise from intrinsic ELMing, in that there is no deliberate intent to control the ELMing process by external means. We use ELM timings derived from the Be II signal to perform direct time domain analysis of the full flux loop VLD2 and VLD3 signals, which provide a high cadence global measurement proportional to the voltage induced by changes in poloidal magnetic flux. Specifically, we examine how the time interval between pairs of successive ELMs is linked to the time-evolving phase of the full flux loop signals. Each ELM produces a clear early pulse in the full flux loop signals, whose peak time is used to condition our analysis. The arrival time of the following ELM, relative to this pulse, is found to fall into one of two categories: (i) prompt ELMs, which are directly paced by the initial response seen in the flux loop signals; and (ii) all other ELMs, which occur after the initial response of the full flux loop signals has decayed in amplitude. The times at which ELMs in category (ii) occur, relative to the first ELM of the pair, are clustered at times when the instantaneous phase of the full flux loop signal is close to its value at the time of the first ELM

Finishing the euchromatic sequence of the human genome

The sequence of the human genome encodes the genetic instructions for human physiology, as well as rich information about human evolution. In 2001, the International Human Genome Sequencing Consortium reported a draft sequence of the euchromatic portion of the human genome. Since then, the international collaboration has worked to convert this draft into a genome sequence with high accuracy and nearly complete coverage. Here, we report the result of this finishing process. The current genome sequence (Build 35) contains 2.85 billion nucleotides interrupted by only 341 gaps. It covers ∼99% of the euchromatic genome and is accurate to an error rate of ∼1 event per 100,000 bases. Many of the remaining euchromatic gaps are associated with segmental duplications and will require focused work with new methods. The near-complete sequence, the first for a vertebrate, greatly improves the precision of biological analyses of the human genome including studies of gene number, birth and death. Notably, the human enome seems to encode only 20,000-25,000 protein-coding genes. The genome sequence reported here should serve as a firm foundation for biomedical research in the decades ahead

Effect of angiotensin-converting enzyme inhibitor and angiotensin receptor blocker initiation on organ support-free days in patients hospitalized with COVID-19

IMPORTANCE Overactivation of the renin-angiotensin system (RAS) may contribute to poor clinical outcomes in patients with COVID-19. Objective To determine whether angiotensin-converting enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB) initiation improves outcomes in patients hospitalized for COVID-19. DESIGN, SETTING, AND PARTICIPANTS In an ongoing, adaptive platform randomized clinical trial, 721 critically ill and 58 non–critically ill hospitalized adults were randomized to receive an RAS inhibitor or control between March 16, 2021, and February 25, 2022, at 69 sites in 7 countries (final follow-up on June 1, 2022). INTERVENTIONS Patients were randomized to receive open-label initiation of an ACE inhibitor (n = 257), ARB (n = 248), ARB in combination with DMX-200 (a chemokine receptor-2 inhibitor; n = 10), or no RAS inhibitor (control; n = 264) for up to 10 days. MAIN OUTCOMES AND MEASURES The primary outcome was organ support–free days, a composite of hospital survival and days alive without cardiovascular or respiratory organ support through 21 days. The primary analysis was a bayesian cumulative logistic model. Odds ratios (ORs) greater than 1 represent improved outcomes. RESULTS On February 25, 2022, enrollment was discontinued due to safety concerns. Among 679 critically ill patients with available primary outcome data, the median age was 56 years and 239 participants (35.2%) were women. Median (IQR) organ support–free days among critically ill patients was 10 (–1 to 16) in the ACE inhibitor group (n = 231), 8 (–1 to 17) in the ARB group (n = 217), and 12 (0 to 17) in the control group (n = 231) (median adjusted odds ratios of 0.77 [95% bayesian credible interval, 0.58-1.06] for improvement for ACE inhibitor and 0.76 [95% credible interval, 0.56-1.05] for ARB compared with control). The posterior probabilities that ACE inhibitors and ARBs worsened organ support–free days compared with control were 94.9% and 95.4%, respectively. Hospital survival occurred in 166 of 231 critically ill participants (71.9%) in the ACE inhibitor group, 152 of 217 (70.0%) in the ARB group, and 182 of 231 (78.8%) in the control group (posterior probabilities that ACE inhibitor and ARB worsened hospital survival compared with control were 95.3% and 98.1%, respectively). CONCLUSIONS AND RELEVANCE In this trial, among critically ill adults with COVID-19, initiation of an ACE inhibitor or ARB did not improve, and likely worsened, clinical outcomes. TRIAL REGISTRATION ClinicalTrials.gov Identifier: NCT0273570

“The Best and Worst Decisions I’ve Made”

Given the amount of time I have driven around, or stood around thinking about this topic, I can now definitively say that it is one of the meanest assignments Professor Hixon has ever given out. The fact that he gave me several weeks to ruminate on the topic only makes it more distressing, and the loss of sleep has been severe. If the time frame were more strict, such as what is the worst or best decision you made yesterday, or last week, this would not be nearly as stressful, but to force me to think of every stupid thing I have done in the past eight years is a little harsh. For that I say, “thanks Doug,” because all of us should do this more often, but not all the time. My discomfort obviously hinges on having to account for all of the bone-headed things I have done, and then having to compare that with a rather meager list of great, foresighted wisdom. So let me try to explain the process I followed as I thought this stuff through. First, there are three basic components to making ranches work, and the public fails to see all of them at the same time. Simply put, ranching is a blend and balancing between ecological goals and realities, economics, and cultural demands. I tried to make the assessment of best and worst of each, and it led me to a lot of thinking. I won’t belabor the point, but as you already know, some of the really great decisions made in one area turned out to be disasters in another, and when you add time to the mix, this question becomes a massive tar baby. I only relate this because the mental exercise has been fantastic, and I encourage people to do this on their own – you will get more out of the process than you will out of this discussion, I assure you

Management at the Edge of Opportunity

The Rangelands archives are made available by the Society for Range Management and the University of Arizona Libraries. Contact [email protected] for further information.Migrated from OJS platform March 202

Solitude, attachment and self-reflection in adolescence : an exploration of relationships

Attachment has long been viewed as the fundamental mechanism underlying the way in which one relates to others. However little attention has been paid to the impact of attachment on spending time alone with the self. This would seem of particular importance to the adolescent population where spending time alone increases and is seen as part of the developmental process of individuation. It has been proposed that this increase in solitude coincides with advancing cognitive skills with which to make constructive-reflection. To explore the possible relationship between solitude (choosing to spend time alone), attachment, self-reflection and the ability to cope with emotional distress. Non-experimental, cross-sectional between subjects design. Using self-report measures, differences in level of preference for solitude were explored in relation to attachment styles, reflection, rumination and emotional self-efficacy.EThOS - Electronic Theses Online ServiceGBUnited Kingdo

Sage-Grouse and Energy Development

Conservation efforts surrounding Sage-grouse began at the turn of the century, with generally broad-based efforts to determine the threats and challenges to the species. Since that time, Sage-grouse have become a commonly litigated species, ultimately landing on the candidate species list for endangerment by the U.S. Fish and Wildlife Service. Simply listing the species may not have the intended benefits for the bird, and may cause considerable economic harm, particularly to energy development and ranching. Using community based approaches and sound science, conservation on the ground may be enhanced and maintained over long periods of time. This requires commitment from all parties involved, and will generally require a solutions-based approach to both conservation and development of natural resources. Comprehensive evaluation of realities associated with long-term conservation is the cornerstone to effective communication and problem-solving on the ground. Application of effective conservation strategies is a responsibility of all affected parties. Bob Budd, Executive Director, Wyoming Wildlife and Natural Resource Trust, Hathaway Building, 1st Floor, 2300 Capitol Avenue, Cheyenne, WY, 82002, [email protected] Bob Budd is the founding Executive Director for the Wyoming Wildlife and Natural Resource Trust, a program that funds and assists conservation projects in Wyoming. He is Chairman of the Wyoming Governor’s Sage- Grouse Implementation Team, an effort that developed a partner-based strategy for Sage-grouse conservation in Wyoming and the west. Most recently, he helped the states of Utah and Nevada design similar strategies for conservation, and served as a member of the Conservation Objectives Team (COT) for the Fish and Wildlife Service. Budd is a native Wyomingite, and served as president of the international Society for Range Management, and the Wyoming Chapter of the Wildlife Society. He holds an MS degree in Range Management, and BS degrees in Animal Science and Agricultural Business, all from the University of Wyoming. He is a published author, and received the Kurt Bucholz Conservation Award from the Wyoming Stock Growers Ag Land Trust