Friedreich's ataxia-associated childhood hypertrophic cardiomyopathy: a national cohort study

OBJECTIVE: Hypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich's ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM. DESIGN AND SETTING: Retrospective, longitudinal cohort study of children with FA-HCM from the UK. PATIENTS: 78 children (<18 years) with FA-HCM diagnosed over four decades. INTERVENTION: Anonymised retrospective demographic and clinical data were collected from baseline evaluation and follow-up. MAIN OUTCOME MEASURES: The primary study end-point was all-cause mortality (sudden cardiac death, atrial arrhythmia-related death, heart failure-related death, non-cardiac death) or cardiac transplantation. RESULTS: The mean age at diagnosis of FA-HCM was 10.9 (±3.1) years. Diagnosis was within 1 year of cardiac referral in 34 (65.0%) patients, but preceded the diagnosis of FA in 4 (5.3%). At baseline, 65 (90.3%) had concentric left ventricular hypertrophy and 6 (12.5%) had systolic impairment. Over a median follow-up of 5.1 years (IQR 2.4-7.3), 8 (10.5%) had documented supraventricular arrhythmias and 8 (10.5%) died (atrial arrhythmia-related n=2; heart failure-related n=1; non-cardiac n=2; or unknown cause n=3), but there were no sudden cardiac deaths. Freedom from death or transplantation at 10 years was 80.8% (95% CI 62.5 to 90.8). CONCLUSIONS: This is the largest cohort of childhood FA-HCM reported to date and describes a high prevalence of atrial arrhythmias and impaired systolic function in childhood, suggesting early progression to end-stage disease. Overall mortality is similar to that reported in non-syndromic childhood HCM, but no patients died suddenly

Surface and Temporal Biosignatures

Recent discoveries of potentially habitable exoplanets have ignited the prospect of spectroscopic investigations of exoplanet surfaces and atmospheres for signs of life. This chapter provides an overview of potential surface and temporal exoplanet biosignatures, reviewing Earth analogues and proposed applications based on observations and models. The vegetation red-edge (VRE) remains the most well-studied surface biosignature. Extensions of the VRE, spectral "edges" produced in part by photosynthetic or nonphotosynthetic pigments, may likewise present potential evidence of life. Polarization signatures have the capacity to discriminate between biotic and abiotic "edge" features in the face of false positives from band-gap generating material. Temporal biosignatures -- modulations in measurable quantities such as gas abundances (e.g., CO2), surface features, or emission of light (e.g., fluorescence, bioluminescence) that can be directly linked to the actions of a biosphere -- are in general less well studied than surface or gaseous biosignatures. However, remote observations of Earth's biosphere nonetheless provide proofs of concept for these techniques and are reviewed here. Surface and temporal biosignatures provide complementary information to gaseous biosignatures, and while likely more challenging to observe, would contribute information inaccessible from study of the time-averaged atmospheric composition alone.Comment: 26 pages, 9 figures, review to appear in Handbook of Exoplanets. Fixed figure conversion error

The hydrodynamic efficiency of laser-target acceleration

The acceleration of a thin foil using a laser pulse is studied. It is shown that the acceleration efficiency eta H is heavily dependent on the behaviour of the corona ejected by the foil: there is no universal relation eta H( Delta M/M0),M0 and Delta M being initial foil mass and ablated mass, respectively. Known results on the coronal flow are used to check the theory against experimental data available in the literature; effects due to both a non-planar corona, and the time-dependence of the laser irradiance, are considered. The agreement with experiments is substantially better than that for previous analyses. Acceleration of thin spherical shells is also discussed

SMART-1 Impact Ground-based campaign

Based on predictions of impact magnitude and cloud ejecta dynamics, we organized a SMART-1 ground-based observation campaign to perform coordinated measurements of the impact. Results from the coordinated multi-site campaign will be discussed

Multicolour photometry for exoplanet candidate validation

Context. The TESS and PLATO missions are expected to find vast numbers of new transiting planet candidates. However, only a fraction of these candidates will be legitimate planets, and the candidate validation will require a significant amount of follow-up resources. Radial velocity follow-up can be carried out only for the most promising candidates around bright, slowly rotating, stars. Thus, before devoting RV resources to candidates, they need to be vetted using cheaper methods, and, in the cases for which an RV confirmation is not feasible, the candidate's true nature needs to be determined based on these alternative methods alone. Aims. We study the applicability of multicolour transit photometry in the validation of transiting planet candidates when the candidate signal arises from a real astrophysical source. We seek to answer how securely can we estimate the true uncontaminated star-planet radius ratio when the light curve may contain contamination from unresolved light sources inside the photometry aperture when combining multicolour transit observations with a physics-based contamination model. Methods. The study is based on simulations and ground-based transit observations. The analyses are carried out with a contamination model integrated into the PyTransit v2 transit modelling package, and the observations are carried out with the MuSCAT2 multicolour imager installed in the 1.5 m TCS in the Teide Observatory. Results. We show that multicolour transit photometry can be used to estimate the amount of flux contamination and the true radius ratio. Combining the true radius ratio with an estimate for the stellar radius yields the true absolute radius of the transiting object, which is a valuable quantity in statistical candidate validation, and enough in itself to validate a candidate whose radius falls below the theoretical lower limit for a brown dwarf.Comment: Accepted to A&