19 research outputs found
Hitomi (ASTRO-H) X-ray Astronomy Satellite
The Hitomi (ASTRO-H) mission is the sixth Japanese x-ray astronomy satellite developed by a large international collaboration, including Japan, USA, Canada, and Europe. The mission aimed to provide the highest energy resolution ever achieved at E > 2 keV, using a microcalorimeter instrument, and to cover a wide energy range spanning four decades in energy from soft x-rays to gamma rays. After a successful launch on February 17, 2016, the spacecraft lost its function on March 26, 2016, but the commissioning phase for about a month provided valuable information on the onboard instruments and the spacecraft system, including astrophysical results obtained from first light observations. The paper describes the Hitomi (ASTRO-H) mission, its capabilities, the initial operation, and the instruments/spacecraft performances confirmed during the commissioning operations for about a month
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Longitudinal Assessment of Resident Performance Using Entrustable Professional Activities
Question What is the progression of performance for entrustable professional activities (EPAs) throughout pediatric residency training and at graduation? Findings This multisite cohort study of 1987 pediatric residents found that developmental growth curves can be established for EPAs. When generated to reflect the results in this study, at least 90% of trainees achieved the level of unsupervised practice at the end of residency for only 8 of the 17 EPAs studied. Meaning This study suggests that gaps exist between observed practice readiness and standards needed to produce physicians able to meet the health needs of the patient populations they serve based on the general pediatrics EPAs.
Importance Entrustable professional activities (EPAs) are an emerging workplace-based, patient-oriented assessment approach with limited empirical evidence. Objective To measure the development of pediatric trainees' clinical skills over time using EPA-based assessment data. Design, Setting, and Participants Prospective cohort study of categorical pediatric residents over 3 academic years (2015-2016, 2016-2017, and 2017-2018) assessed on 17 American Board of Pediatrics EPAs. Residents in training at 23 pediatric residency programs in the Association of Pediatric Program Directors Longitudinal Educational Assessment Research Network were included. Assessment was conducted by clinical competency committee members, who made summative assessment decisions regarding levels of supervision required for each resident and each EPA. Data were collected from May 2016 to November 2018 and analyzed from November to December 2018. Interventions Longitudinal, prospective assessment using EPAs. Main Outcomes and Measures Trajectories of supervision levels by EPA during residency training and how often graduating residents were deemed ready for unsupervised practice in each EPA. Results Across the 5 data collection cycles, 1987 residents from all 3 postgraduate years in 23 residency programs were assigned 25503 supervision level reports for the 17 general pediatrics EPAs. The 4 EPAs that required the most supervision across training were EPA 14 (quality improvement) on the 5-level scale (estimated mean level at graduation, 3.7; 95% CI, 3.6-3.7) and EPAs 8 (transition to adult care; mean, 7.0; 95% CI, 7.0-7.1), 9 (behavioral and mental health; mean, 6.6; 95% CI, 6.5-6.6), and 10 (resuscitate and stabilize; mean, 6.9; 95% CI, 6.8-7.0) on the expanded 5-level scale. At the time of graduation (36 months), the percentage of trainees who were rated at a supervision level corresponding to "unsupervised practice" varied by EPA from 53% to 98%. If performance standards were set to align with 90% of trainees achieving the level of unsupervised practice, this standard would be met for only 8 of the 17 EPAs (although 89% met this standard for EPA 17, performing the common procedures of the general pediatrician). Conclusions and Relevance This study presents initial evidence for empirically derived practice readiness and sets the stage for identifying curricular gaps that contribute to discrepancy between observed practice readiness and standards needed to produce physicians able to meet the health needs of the patient populations they serve. Future work should compare these findings with postgraduation outcomes data as a means of seeking validity evidence.
This cohort study measures the development of pediatric resident clinical skills using assessments based on entrustable professional activities
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73. ETIOLOGY OF UNABLE TO ASSESS ENTRUSTABLE PROFESSIONAL ACTIVITIES IN A NATIONAL STUDY
Expanded phenotypic spectrum of neurodevelopmental and neurodegenerative disorder Bryant-Li-Bhoj syndrome with 38 additional individuals.
Bryant-Li-Bhoj syndrome (BLBS), which became OMIM-classified in 2022 (OMIM: 619720, 619721), is caused by germline variants in the two genes that encode histone H3.3 (H3-3A/H3F3A and H3-3B/H3F3B) [1-4]. This syndrome is characterized by developmental delay/intellectual disability, craniofacial anomalies, hyper/hypotonia, and abnormal neuroimaging [1, 5]. BLBS was initially categorized as a progressive neurodegenerative syndrome caused by de novo heterozygous variants in either H3-3A or H3-3B [1-4]. Here, we analyze the data of the 58 previously published individuals along 38 unpublished, unrelated individuals. In this larger cohort of 96 people, we identify causative missense, synonymous, and stop-loss variants. We also expand upon the phenotypic characterization by elaborating on the neurodevelopmental component of BLBS. Notably, phenotypic heterogeneity was present even amongst individuals harboring the same variant. To explore the complex phenotypic variation in this expanded cohort, the relationships between syndromic phenotypes with three variables of interest were interrogated: sex, gene containing the causative variant, and variant location in the H3.3 protein. While specific genotype-phenotype correlations have not been conclusively delineated, the results presented here suggest that the location of the variants within the H3.3 protein and the affected gene (H3-3A or H3-3B) contribute more to the severity of distinct phenotypes than sex. Since these variables do not account for all BLBS phenotypic variability, these findings suggest that additional factors may play a role in modifying the phenotypes of affected individuals. Histones are poised at the interface of genetics and epigenetics, highlighting the potential role for gene-environment interactions and the importance of future research
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A randomized clinical trial of high-dosage coenzyme Q10 in early Parkinson disease: no evidence of benefit.
ImportanceCoenzyme Q10 (CoQ10), an antioxidant that supports mitochondrial function, has been shown in preclinical Parkinson disease (PD) models to reduce the loss of dopamine neurons, and was safe and well tolerated in early-phase human studies. A previous phase II study suggested possible clinical benefit.ObjectiveTo examine whether CoQ10 could slow disease progression in early PD.Design, setting, and participantsA phase III randomized, placebo-controlled, double-blind clinical trial at 67 North American sites consisting of participants 30 years of age or older who received a diagnosis of PD within 5 years and who had the following inclusion criteria: the presence of a rest tremor, bradykinesia, and rigidity; a modified Hoehn and Yahr stage of 2.5 or less; and no anticipated need for dopaminergic therapy within 3 months. Exclusion criteria included the use of any PD medication within 60 days, the use of any symptomatic PD medication for more than 90 days, atypical or drug-induced parkinsonism, a Unified Parkinson's Disease Rating Scale (UPDRS) rest tremor score of 3 or greater for any limb, a Mini-Mental State Examination score of 25 or less, a history of stroke, the use of certain supplements, and substantial recent exposure to CoQ10. Of 696 participants screened, 78 were found to be ineligible, and 18 declined participation.InterventionsThe remaining 600 participants were randomly assigned to receive placebo, 1200 mg/d of CoQ10, or 2400 mg/d of CoQ10; all participants received 1200 IU/d of vitamin E.Main outcomes and measuresParticipants were observed for 16 months or until a disability requiring dopaminergic treatment. The prospectively defined primary outcome measure was the change in total UPDRS score (Parts I-III) from baseline to final visit. The study was powered to detect a 3-point difference between an active treatment and placebo.ResultsThe baseline characteristics of the participants were well balanced, the mean age was 62.5 years, 66% of participants were male, and the mean baseline total UPDRS score was 22.7. A total of 267 participants required treatment (94 received placebo, 87 received 1200 mg/d of CoQ10, and 86 received 2400 mg/d of CoQ10), and 65 participants (29 who received placebo, 19 who received 1200 mg/d of CoQ10, and 17 who received 2400 mg/d of CoQ10) withdrew prematurely. Treatments were well tolerated with no safety concerns. The study was terminated after a prespecified futility criterion was reached. At study termination, both active treatment groups showed slight adverse trends relative to placebo. Adjusted mean changes (worsening) in total UPDRS scores from baseline to final visit were 6.9 points (placebo), 7.5 points (1200 mg/d of CoQ10; P = .49 relative to placebo), and 8.0 points (2400 mg/d of CoQ10; P = .21 relative to placebo).Conclusions and relevanceCoenzyme Q10 was safe and well tolerated in this population, but showed no evidence of clinical benefit.Trial registrationclinicaltrials.gov Identifier: NCT00740714