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ObjectiveChildhood burns represent a burden on health services, yet the full extent of the problem is difficult to quantify. We estimated the annual UK incidence from primary care (PC), emergency attendances (EA), hospital admissions (HA) and deaths.MethodsThe population was children (0–15 years), across England, Wales, Scotland and Northern Ireland (NI), with medically attended burns 2013–2015. Routinely collected data sources included PC attendances from Clinical Practice Research Datalink 2013–2015), EAs from Paediatric Emergency Research in the United Kingdom and Ireland (PERUKI, 2014) and National Health Services Wales Informatics Services, HAs from Hospital Episode Statistics, National Services Scotland and Social Services and Public Safety (2014), and mortality from the Office for National Statistics, National Records of Scotland and NI Statistics and Research Agency 2013–2015. The population denominators were based on Office for National Statistics mid-year population estimates.ResultsThe annual PC burns attendance was 16.1/10 000 persons at risk (95% CI 15.6 to 16.6); EAs were 35.1/10 000 persons at risk (95% CI 34.7 to 35.5) in England and 28.9 (95% CI 27.5 to 30.3) in Wales. HAs ranged from 6.0/10 000 person at risk (95% CI 5.9 to 6.2) in England to 3.1 in Wales and Scotland (95% CI 2.7 to 3.8 and 2.7 to 3.5, respectively) and 2.8 (95% CI 2.4 to 3.4) in NI. In England, Wales and Scotland, 75% of HAs were aged <5 years. Mortality was low with 0.1/1 000 000 persons at risk (95% CI 0.06 to 0.2).ConclusionsWith an estimated 19 574 PC attendances, 37 703 EAs (England and Wales only), 6639 HAs and 1–6 childhood deaths annually, there is an urgent need to improve UK childhood burns prevention.

AimsThis study of children seen in the paediatric emergency department set out to determine how useful the sepsis screening tool was in alerting clinicians to unwell potentially septic children, and to establish if the initial BPEWS (beside paediatric early warning score) was predictive of admission.MethodsA retrospective study of the electronic patient records for patients who had triggered on the sepsis screening tool over a 4 month period. Information gathered included initial BPEWS with breakdown for each parameter, and the outcome – discharged or admission (admission included children observed in the clinical decision unit or use of the nursing outreach service).Of those who required admission, further information was gathered as to final diagnosis, whether antibiotics were given (IV or oral) and the microbiology results.ResultsIn the study period 719 patients triggered on the sepsis screening tool of which 713 were analysed. 172 of these were admitted (24%), and of these only 1 had an invasive bacteraemia.Respiratory infections were a common cause of illness with 19% (33) having upper respiratory tract infection and 17% (29) having lower. 16% (27) of those admitted were ultimately diagnosed with viral induced wheeze. 63 (37%) were positive for one or more viruses on respiratory or stool samples.Correlation analysis of the BPEWS showed that the higher the initial BPEWS, the more likely that children would be admitted. There was a correlation of 95% between these variables and chi squared analysis of the relationship showed a significant p value of <0.01.ConclusionWhilst large numbers of children triggered on the sepsis screening tool, 76% were not admitted and thus raises questions as to the sensitivity of the tool. Increasing BPEWS was found to be predictive in identifying children who require admission. This suggests that perhaps the tool requires some adaptations, such as using high BPEWS as a significant trigger, to allow it to be more sensitive and applicable for this population and workforce.

Objectives & BackgroundSevere sepsis in the paediatric population is a rare, but serious condition that requires prompt recognition and management in the Emergency Department. Whereas severe sepsis in the adult population is being continuously monitored using standardized quality measures, data on performance in the paediatric population is sparse in our institution. We hypothesized that performance can be measured using available data sources. The aim of this study was to measure baseline performance using the RCEM adult-sepsis audit standards in the paediatric population with severe sepsis.Audit result: Paediatric severe sepsisAudit standardRCEM adult standardAudit result paediatrics (n= 19)Missing data (n) Vital signs recorded fully within 15 mins100%47%0Capillary glucose recorded in ED (anytime) 100%84%1Intravensous fluid bolus given within 1 hour75%42%1Serum lactate measured in ED (anytime)100%89%1Antibiotics within 1 hour of arrival50%42%1Blood cultures obtained prior to antibioticsStandard (%) not defined by RCEM37%0Urine dip obtained in EDNot part of RCEM standard21%1Assessment by doctors within 30 minsNot part of RCEM standard84%1MethodsThis is a single-centre descriptive study from a university hospital with 30,000 A&E-paediatric attendances per year. Data were collected from January 2012 through April 2015. Inclusion criteria were children (<16y) admitted through the A&E with direct transfer to paediatric ITU with evidence of sepsis in the A&E. Cases were identified by ICD diagnosis of sepsis upon discharge from PICU. We did not include transfers from other hospitals.Baseline data was collected from the electronic patient system: Age, gender, pre-morbid conditions, as well as in-hospital mortality and length of stay (LOS). The RCEM standards for adult sepsis, as well as adapted measures, were extracted from the scanned hand written ED charts: Timing of bedside assessment by doctor, blood cultures, antibiotics, and fluids. Measurements of lactate and blood glucose. Monitoring of vital signs upon arrival, and documentation of urine dip and presumed focus of infection.ResultsWe identified 19 cases of paediatric sepsis admitted through the A&E. 13 of 19 cases (68%) were male, median age was 70 days and 8 of 19 (42%) were born at term with no significant past medical history. Median LOS in hospital was 5 days, and all patients (100%) survived to discharge. Please see table for audit results.ConclusionAvailable data sources can be used to measure baseline performance in the paediatric population with severe sepsis. We did not fulfil any of the RCEM adult-sepsis audit standards. Timeliness of vital signs and administration of first fluid bolus seem to be amongst the biggest challenges in our institution. Further research is needed to investigate if continuous monitoring on these measures will improve performance.

Acute severe asthma (ASA) is a leading cause of hospital attendance in children. Standard first-line therapy consists of high-dose inhaled bronchodilators plus oral corticosteroids. Treatment for children who fail to respond to first-line therapy is problematic: the use of intravenous agents is inconsistent, and side effects are frequent. High-flow humidified oxygen (HiFlo) is widely used in respiratory conditions and is increasingly being used in ASA, but with little evidence for its effectiveness. A well-designed, adequately powered randomized controlled trial (RCT) of HiFlo therapy in ASA is urgently needed, and feasibility data are required to plan such an RCT. In this study, we describe the protocol for a feasibility study designed to fill this knowledge gap. This study aims to establish whether a full RCT of early HiFlo therapy in children with ASA can be conducted successfully and safely, to establish whether recruitment using deferred consent is practicable, and to define appropriate outcome measures and sample sizes for a definitive RCT. The underlying hypothesis is that early HiFlo therapy in ASA will reduce the need for more invasive treatments, allow faster recovery and discharge from hospital, and in both these ways reduce distress to children and their families. We conducted a feasibility RCT with deferred consent to assess the use of early HiFlo therapy in children aged 2 to 11 years with acute severe wheeze not responding to burst therapy (ie, high-dose inhaled salbutamol with or without ipratropium). Children with a Preschool Respiratory Assessment Measure score ≥5 after burst therapy were randomized to commence HiFlo therapy or follow standard care. The candidate primary outcomes assessed were treatment failure requiring escalation and time to meet hospital discharge criteria. Patient and parent experiences were also assessed using questionnaires and telephone interviews. The trial was opened to recruitment in February 2020 but was paused for 15 months owing to the COVID-19 pandemic. The trial was reopened at the lead site in July 2021 and opened at the other 3 sites from August to December 2022. Recruitment was completed in June 2023. This feasibility RCT of early HiFlo therapy in children with ASA recruited to the target despite major disturbances owing to the COVID-19 pandemic. The data are currently being analyzed and will be published separately. International Standard Randomised Controlled Trial Number Registry ISRCTN78297040; https://www.isrctn.com/ISRCTN78297040. DERR1-10.2196/54081.

Objectives & BackgroundMajor trauma in children, while being relatively uncommon, is still a principal cause of preventable death with severe haemorrhage being one of the main responsible reasons. ‘Major haemorrhage protocols’ (known in our institution as a ‘Code Red’) have been widely introduced in order to make large amounts of blood products available quickly. Most protocols, including the 'Code Red' involve adaptations for paediatrics. The ratio of blood products is 1:1 packed red blood cells (PRBCs) to Fresh Frozen Plasma (FFP) mirroring the adult literature that has evidence of improved outcomes.The objective was to investigate the incidence, demographics, physiology and outcomes of the children triggering a ‘Code Red’ protocol.MethodsAll ‘code red’ activations for children (all those ≤18 years) from 1st Jan 2010 to 31st Dec 2015 were identified then resuscitation room records and computer databases were scrutinised.Results1104 children presented during this period as 'trauma call' pre-alerts with only 36 children triggered the ‘Code Red’ protocol (3%). 31 actually received blood with 5 receiving products from prehospital teams.The age distribution was unsurprisingly skewed towards older age groups (median 17 years, mean 15.4 years) and predominantly male 89%. Only 3 cases were under 12.28 (78%) had penetrating trauma of which 4 were gun shot wounds.4 arrived in traumatic cardiac arrest (100% mortality) and were given the most blood products. Of the other patients, only 25% were hypotensive and only 47% were tachycardic.Low haemoglobin, deranged clotting or both were seen in 27 cases, with worsening coagulation in 13 patients post transfusion.5 children (18%) of patients receiving more than one unit of PRBCs, had a 1:1* ratio of PRBC:FFP. *(permitted to be a difference of 1 unit only)25 cases were taken direct to theatre with an overall mortality of 25%, with 2 deaths in the emergency department, 4 in theatre and 3 later in critical care.ConclusionThe most common paediatric case to trigger a major haemorrhage protocol at our intitution (72%) is a teenage male with penetrating trauma. 'Code Red' calls for smaller children or those sustaining blunt trauma are extremely rare.The data supports the caution that vital signs are poor indicators of hypovolaemia and, despite the protocols, it is still difficult to ensure recommended ratios of blood to plasma are administered.

ObjectivesTo determine the need for recovery (NFR) among emergency physicians and to identify demographic and occupational characteristics associated with higher NFR scores.DesignCross-sectional electronic survey.SettingEmergency departments (EDs) (n=112) in the UK and Ireland.ParticipantsEmergency physicians, defined as any registered physician working principally within the ED, responding between June and July 2019.Main outcome measureNFR Scale, an 11-item self-administered questionnaire that assesses how work demands affect intershift recovery.ResultsThe median NFR Score for all 4247 eligible, consented participants with a valid NFR Score was 70.0 (95% CI: 65.5 to 74.5), with an IQR of 45.5–90.0. A linear regression model indicated statistically significant associations between gender, health conditions, type of ED, clinical grade, access to annual and study leave, and time spent working out-of-hours. Groups including male physicians, consultants, general practitioners (GPs) within the ED, those working in paediatric EDs and those with no long-term health condition or disability had a lower NFR Score. After adjusting for these characteristics, the NFR Score increased by 3.7 (95% CI: 0.3 to 7.1) and 6.43 (95% CI: 2.0 to 10.8) for those with difficulty accessing annual and study leave, respectively. Increased percentage of out-of-hours work increased NFR Score almost linearly: 26%–50% out-of-hours work=5.7 (95% CI: 3.1 to 8.4); 51%–75% out-of-hours work=10.3 (95% CI: 7.6 to 13.0); 76%–100% out-of-hours work=14.5 (95% CI: 11.0 to 17.9).ConclusionHigher NFR scores were observed among emergency physicians than reported in any other profession or population to date. While out-of-hours working is unavoidable, the linear relationship observed suggests that any reduction may result in NFR improvement. Evidence-based strategies to improve well-being such as proportional out-of-hours working and improved access to annual and study leave should be carefully considered and implemented where feasible.

Perinatal mental health is a major concern among women of childbearing age. Women from a black and minority ethnic background are widely believed to have particular needs that are often not given the attention they deserve. NHS Croydon launched a perinatal mental health project to develop a closer and better partnership between the Primary Care Trust (PCT), Croydon Council and black and minority ethnic (BME) voluntary organisations through an action learning approach. Experience was shared to improve engagement and use of health services by mothers from BME communities in Croydon who had encountered mental health problems during pregnancy or following childbirth. By exploring and identifying such issues and problems, the action learning set endeavoured to find solutions for a joined-up approach to achieve identifiable benefits. Some problems were encountered, such as a lack of communication between health professionals and BME community groups. The learning outcomes were to raise awareness and to recognise the cultural differences with mothers of BME background experiencing perinatal mental health problems. The learning from the project will be disseminated to a wider audience to promote best practice.

BackgroundSerious bacterial infections in young infants with bronchiolitis are rare. Febrile infants <1 month old with bronchiolitis often receive a lumbar puncture (LP), despite limited data for this practice and lack of clinical practice guidelines for this population. The primary objective was to investigate practice patterns in performance of LPs in the ED management of febrile infants aged ≤30 days with bronchiolitis.MethodsA cross-sectional survey of two national paediatric emergency research networks (PediatricEmergency Research Canada (PERC) and the PediatricEmergency Research UK/Ireland (PERUKI)) was conducted January to November 2017 using a modified Dillman technique. The survey was preceded by a clinical vignette describing a well appearing, 21-day-old infant with low-grade fever, respiratory findings typical of bronchiolitis and no perinatal serious bacterial infection (SBI) risk features.ResultsThe response rate from PERC was 169/250 (68%) and 172/201 (86%) from PERUKI. Nine physicians in training were excluded, leaving 332 eligible participants. Although most physicians believe that neonates with bronchiolitis rarely have meningitis (PERC 141/161 (87.6%); PERUKI 154/171 (90%)) and feel comfortable diagnosing bronchiolitis in this group (PERC 136/161 (84.5%); PERUKI 143/171 (83.6%)), there was significant variation in the proportion who would be likely/very likely to perform an LP (PERC 100/161 (62.1%); PERUKI 15/171 (8.8%)) (p<0.0001). Practice in Canada, <10 years in practice and lack of comfort with diagnosing bronchiolitis represent multivariable predictors of LP; OR 23.7 (95% CI 11.7 to 47.9), 2.3 (95% CI 1.2 to 4.2) and 2.5 (95% CI 1.1 to 5.0), respectively. Rapid knowledge of respiratory syncytial virus positivity would decrease LP probability from 35.4% to 20.2%.ConclusionEstimated probability of performing LPs and other interventions in otherwise healthy febrile neonates with bronchiolitis is highly variable between emergency physicians in Canada and the UK/Ireland. Network, <10 years in ED practice and comfort level with diagnosing bronchiolitis in newborns constitute independent predictors of the likelihood of LP performance.

Huntington’s disease arises from a CAG expansion in the huntingtin gene beyond a critical threshold. Current therapeutics primarily aim to reduce toxicity by lowering levels of mutant HTT mRNA and protein. Genetic data support a role for somatic instability in HTT ’s CAG repeat as a driver of age of motor dysfunction onset, but currently, the relationship between instability and HTT lowering remains unexplored. Here, we investigate various HTT-lowering modalities to establish the relationship between HTT lowering and instability in Huntington’s disease knock-in mice. We find that repressing transcription of mutant Htt reduces instability, using genetic and pharmacological approaches. Remarkably, zinc finger proteins that target CAG repeats, but lack a repressive domain, protect from somatic instability despite not reducing HTT mRNA or protein levels. These results suggest that DNA-targeted HTT-lowering treatments may have advantages compared to other HTT-lowering approaches, and that steric blockage of CAG repeats may reduce instability while sparing HTT expression. Somatic instability plays a role in Huntington’s disease. Here, the authors show that lowering HTT levels by transcriptional repression suppresses somatic instability. This can also be done without affecting HTT levels by direct CAG repeat binding.

Huntington’s disease results from expansion of a glutamine-coding CAG tract in the huntingtin (HTT) gene, producing an aberrantly functioning form of HTT. Both wildtype and disease-state HTT form a hetero-dimer with HAP40 of unknown functional relevance. We demonstrate in vivo and in cell models that HTT and HAP40 cellular abundance are coupled. Integrating data from a 2.6 Å cryo-electron microscopy structure, cross-linking mass spectrometry, small-angle X-ray scattering, and modeling, we provide a near-atomic-level view of HTT, its molecular interaction surfaces and compacted domain architecture, orchestrated by HAP40. Native mass spectrometry reveals a remarkably stable hetero-dimer, potentially explaining the cellular inter-dependence of HTT and HAP40. The exon 1 region of HTT is dynamic but shows greater conformational variety in the polyglutamine expanded mutant than wildtype exon 1. Our data provide a foundation for future functional and drug discovery studies targeting Huntington’s disease and illuminate the structural consequences of HTT polyglutamine expansion.Harding et al. present a biophysical and structural characterization of the complex between huntingtin (HTT) and HAP40 proteins. They show that the abundance of HAP40 is coupled with that of HTT and that there is greater conformational variety in the exon 1 of the mutant HTT than WT, important for the future drug discovery studies targeting Huntington’s disease.