Catalogue of BRITE-Constellation targets I. Fields 1 to 14 (November 2013 - April 2016)

The BRIght Target Explorer (BRITE) mission collects photometric time series in two passbands aiming to investigate stellar structure and evolution. Since their launches in the years 2013 and 2014, the constellation of five BRITE nano-satellites has observed a total of more than 700 individual bright stars in 64 fields. Some targets have been observed multiple times. Thus, the total time base of the data sets acquired for those stars can be as long as nine years. Our aim is to provide a complete description of ready-to-use BRITE data, to show the scientific potential of the BRITE-Constellation data by identifying the most interesting targets, and to demonstrate and encourage how scientists can use these data in their research. We apply a decorrelation process to the automatically reduced BRITE-Constellation data to correct for instrumental effects. We perform a statistical analysis of the light curves obtained for the 300 stars observed in the first 14 fields during the first ~2.5 years of the mission. We also perform cross-identification with the International Variable Star Index. We present the data obtained by the BRITE-Constellation mission in the first 14 fields it observed from November 2013 to April 2016. We also describe the properties of the data for these fields and the 300 stars observed in them. Using these data, we detected variability in 64% of the presented sample of stars. Sixty-four stars or 21.3% of the sample have not yet been identified as variable in the literature and their data have not been analysed in detail. They can therefore provide valuable scientific material for further research. All data are made publicly available through the BRITE Public Data Archive and the Canadian Astronomy Data Centre.Comment: accepted by Astronomy & Astrophysics, 13 pages main text, 22 pages of appendi

The employment distribution and the creation of financial dependence

A fall in national income has varied consequences for the working population: some carry on working as normal, others become unemployed. Those excluded from work lose their main income source and must usually rely on public welfare, entering a financial dependence created endogenously as the economy adjusts. The current paper examines this induced financial dependence and its implications within a Post Keynesian model. A skewed employment distribution forces higher transfer payments than would occur if employment was distributed more evenly. The additional expenditures help to sustain profitability, so it is in the collective interest of employers and profit recipients to concentrate unemployment in a subset of the working population

The broad phenotypic spectrum of PPP2R1A-related neurodevelopmental disorders correlates with the degree of biochemical dysfunction

Purpose: Neurodevelopmental disorders (NDD) caused by protein phosphatase 2A (PP2A) dysfunction have mainly been associated with de novo variants in PPP2R5D and PPP2CA, and more rarely in PPP2R1A. Here, we aimed to better understand the latter by characterizing 30 individuals with de novo and often recurrent variants in this PP2A scaffolding Aα subunit. Methods: Most cases were identified through routine clinical diagnostics. Variants were biochemically characterized for phosphatase activity and interaction with other PP2A subunits. Results: We describe 30 individuals with 16 different variants in PPP2R1A, 21 of whom had variants not previously reported. The severity of developmental delay ranged from mild learning problems to severe intellectual disability (ID) with or without epilepsy. Common features were language delay, hypotonia, and hypermobile joints. Macrocephaly was only seen in individuals without B55α subunit-binding deficit, and these patients had less severe ID and no seizures. Biochemically more disruptive variants with impaired B55α but increased striatin binding were associated with profound ID, epilepsy, corpus callosum hypoplasia, and sometimes microcephaly. Conclusion: We significantly expand the phenotypic spectrum of PPP2R1A-related NDD, revealing a broader clinical presentation of the patients and that the functional consequences of the variants are more diverse than previously reported

MED27 Variants Cause Developmental Delay, Dystonia, and Cerebellar Hypoplasia

The Mediator multiprotein complex functions as a regulator of RNA polymerase II-catalyzed gene transcription. In this study, exome sequencing detected biallelic putative disease-causing variants in MED27, encoding Mediator complex subunit 27, in 16 patients from 11 families with a novel neurodevelopmental syndrome. Patient phenotypes are highly homogeneous, including global developmental delay, intellectual disability, axial hypotonia with distal spasticity, dystonic movements, and cerebellar hypoplasia. Seizures and cataracts were noted in severely affected individuals. Identification of multiple patients with biallelic MED27 variants supports the critical role of MED27 in normal human neural development, particularly for the cerebellum. ANN NEUROL 2021Peer reviewe

Global patient outcomes after elective surgery: prospective cohort study in 27 low-, middle- and high-income countries.

BACKGROUND: As global initiatives increase patient access to surgical treatments, there remains a need to understand the adverse effects of surgery and define appropriate levels of perioperative care. METHODS: We designed a prospective international 7-day cohort study of outcomes following elective adult inpatient surgery in 27 countries. The primary outcome was in-hospital complications. Secondary outcomes were death following a complication (failure to rescue) and death in hospital. Process measures were admission to critical care immediately after surgery or to treat a complication and duration of hospital stay. A single definition of critical care was used for all countries. RESULTS: A total of 474 hospitals in 19 high-, 7 middle- and 1 low-income country were included in the primary analysis. Data included 44 814 patients with a median hospital stay of 4 (range 2-7) days. A total of 7508 patients (16.8%) developed one or more postoperative complication and 207 died (0.5%). The overall mortality among patients who developed complications was 2.8%. Mortality following complications ranged from 2.4% for pulmonary embolism to 43.9% for cardiac arrest. A total of 4360 (9.7%) patients were admitted to a critical care unit as routine immediately after surgery, of whom 2198 (50.4%) developed a complication, with 105 (2.4%) deaths. A total of 1233 patients (16.4%) were admitted to a critical care unit to treat complications, with 119 (9.7%) deaths. Despite lower baseline risk, outcomes were similar in low- and middle-income compared with high-income countries. CONCLUSIONS: Poor patient outcomes are common after inpatient surgery. Global initiatives to increase access to surgical treatments should also address the need for safe perioperative care. STUDY REGISTRATION: ISRCTN5181700