Performance on the iSTEP and 10 m-ISWT in boys with haemophilia.

Introduction: Boys with haemophilia (BwH) have improved health outcomes. Measures of physical function in haemophilia are not challenging or sensitive enough to reflect physical limitations or guide rehabilitation. To identify meaningful tests, we aimed to: evaluate the performance of BwH on two physical performance measures: iSTEP and 10 m-ISWT; identify factors which predict performance and compare BwH to their unaffected peers.

Early Release Science of the exoplanet WASP-39b with JWST NIRISS.

The Saturn-mass exoplanet WASP-39b has been the subject of extensive efforts to determine its atmospheric properties using transmission spectroscopy. However, these efforts have been hampered by modelling degeneracies between composition and cloud properties that are caused by limited data quality. Here we present the transmission spectrum of WASP-39b obtained using the Single-Object Slitless Spectroscopy (SOSS) mode of the Near Infrared Imager and Slitless Spectrograph (NIRISS) instrument on the JWST.

Early Release Science of the exoplanet WASP-39b with JWST NIRSpec G395H.

Measuring the abundances of carbon and oxygen in exoplanet atmospheres is considered a crucial avenue for unlocking the formation and evolution of exoplanetary systems. Access to the chemical inventory of an exoplanet requires high-precision observations, often inferred from individual molecular detections with low-resolution space-based and high-resolution ground-based facilities. Here we report the medium-resolution (R ≈ 600) transmission spectrum of an exoplanet atmosphere between 3 and 5 μm covering several absorption features for the Saturn-mass exoplanet WASP-39b (ref.

Early Release Science of the exoplanet WASP-39b with JWST NIRSpec PRISM.

Transmission spectroscopy of exoplanets has revealed signatures of water vapour, aerosols and alkali metals in a few dozen exoplanet atmospheres. However, these previous inferences with the Hubble and Spitzer Space Telescopes were hindered by the observations' relatively narrow wavelength range and spectral resolving power, which precluded the unambiguous identification of other chemical species-in particular the primary carbon-bearing molecules. Here we report a broad-wavelength 0.

Clinical status, biochemical profile and management of a single cohort of patients with arginase deficiency.

Arginase deficiency is a rare autosomal recessive urea cycle disorder (UCD) caused by mutations in the gene encoding arginase that catalyses the hydrolysis of arginine to ornithine and urea. Patients have hyperargininaemia and progressive neurological impairment but generally suffer fewer metabolic decompensations compared to other UCDs. The objective is to describe the clinical features, biochemical profile, neuroradiological findings and experience of managing children with arginase deficiency.

Radical resection of lumbosacral lipomas in children: the Great Ormond Street Hospital experience.

Purpose: In 2009, Pang described a radical resection technique for congenital lumbosacral lipomas, with lower long-term symptomatic re-tethering rates compared with partial resections and conservative management, and low surgical morbidity. We adopted this technique in 2011, and aim to describe our first results.

Methods: In this monocentric retrospective audit, we included dorsal, transitional, chaotic, and caudal-type lumbosacral lipomas.

The effect of lockdown during SARS-CoV-2 pandemic on maxillofacial injuries in a level I trauma centre: a comparative study.

Introduction: The SARS-CoV-2 pandemic forced many governments to impose nation-wide lockdowns. Government legislation forced limited travel on the population with restrictions on the normal way of life to limit spread of the SARS-CoV-2 virus. The aim of this study is to explore the effects of lockdown on the presentation of maxillofacial trauma in a level I trauma centre.

Population genetic structure and predominance of cyclical parthenogenesis in the bird cherry-oat aphid in England.

Genetic diversity is the determinant for pest species' success and vector competence. Understanding the ecological and evolutionary processes that determine the genetic diversity is fundamental to help identify the spatial scale at which pest populations are best managed. In the present study, we present the first comprehensive analysis of the genetic diversity and evolution of , a major pest of cereals and a main vector of the barley yellow dwarf virus (BYDV), in England.

Acute Decompensated Heart Failure Secondary to Exogenous Triiodothyronine Use in a Young Non-athlete Weightlifter.

Heart failure (HF) is a common cardiovascular disorder and is associated with increased morbidity and mortality. HF is usually detected in the elderly population, in particular, in patients with coronary artery disease, valvular disease, myocarditis, and hypertension. Acute decompensated HF in previously healthy young patients should raise suspicion for other rare etiologies.

Age-related gait standards for healthy children and young people: the GOS-ICH paediatric gait centiles.

Objective: To develop paediatric gait standards in healthy children and young people.

Methods: This observational study aims to address the lack of population standards for gait measurements in children. Analysing gait in children affected by neurological or musculoskeletal conditions is an important component of paediatric assessment but is often confounded by developmental changes.

Immunotherapy-responsive childhood neurodegeneration with systemic and central nervous system inflammation.

Subacute neuroregression in association with raised neopterin and overexpression of interferon stimulated genes (ISGs) could indicate a type 1 interferonopathy. Here we describe a novel immunotherapy-responsive, clinico-immunological and imaging phenotype with evidence of innate immune activation. Three children (patient 1: 22-month-old boy; patient 2: 5-year-old girl; patient 3: 4-year-old girl) presented with asymmetric bilateral mixed dystonia and spasticity, regression in language (expressive more than receptive) and bulbar symptoms with no evidence of seizures.

Investigation of Polymer-Plasticizer Blends as SH-SAW Sensor Coatings for Detection of Benzene in Water with High Sensitivity and Long-Term Stability.

We report the first-ever direct detection of benzene in water at concentrations below 100 ppb (parts per billion) using acoustic wave (specifically, shear-horizontal surface acoustic wave, SH-SAW) sensors with plasticized polymer coatings. Two polymers and two plasticizers were studied as materials for sensor coatings. For each polymer-plasticizer combination, the influence of the mixing ratio of the blend on the sensitivity to benzene was measured and compared to commercially available polymers that were used for BTEX (benzene, toluene, ethylbenzene, and xylene) detection in previous work.

Long-term phenological trends, species accumulation rates, aphid traits and climate: five decades of change in migrating aphids.

Aphids represent a significant challenge to food production. The Rothamsted Insect Survey (RIS) runs a network of 12·2-m suction-traps throughout the year to collect migrating aphids. In 2014, the RIS celebrated its 50th anniversary.

Median life span of a cohort of National Institute for Health and Care Excellence clinical guidelines was about 60 months.

Objectives: To describe the length of time National Institute for Health and Care Excellence (NICE) clinical guidelines have remained valid.

Study Design And Setting: The present study is a survival analysis of a cohort of published NICE clinical guidelines. The National Health Service in England and Wales uses NICE clinical practice guidelines as a reference for treatment and care of individuals.

Parametric four-wave mixing using a single cw laser.

Four-wave mixing can be used to generate coherent output beams, with frequencies difficult to acquire in commercial lasers. Here, a single narrow external cavity diode laser locked to the two photon 5s-5d transition in rubidium is combined with a tapered amplifier system to produce a high power cw beam at 778 nm and used to generate coherent light at 420 nm through parametric four-wave mixing. This process is analyzed in terms of the intensity and frequency of the incoming beam as well as the atomic density of the sample.

Tracing the genetic roots of the indigenous White Park Cattle.

The White Park Cattle (WPC) is an indigenous ancient breed from the British Isles which has a long-standing history in heroic sagas and documents. The WPC has retained many primitive traits, especially in their grazing behaviour and preferences. Altogether, the aura of this breed has led to much speculation surrounding its origin.