Bristol Royal Hospital for Children, also known as the Bristol Children's Hospital, is a paediatric hospital in Bristol and the only paediatric major trauma centre in South West England. The hospital is part of the University Hospitals Bristol and Weston NHS Foundation Trust (UHBW), which includes eight other hospitals. The hospital is located next to the Bristol Royal Infirmary in the city centre.
We sought to describe current perceptions and attitudes to management of of brain abscess (BA) or sub-/extra-dural empyema (SDE/EDE) in the United Kingdom (UK) to compare this to the 2024 European Society of Clinical Microbiology and Infectious Diseases BA guidelines. We conducted a web-based survey of infection specialists (IS) and neurosurgeons (NS) at neurosurgical centres across the UK. IS from 27/39 (69
Background:Nirsevimab, a long-acting monoclonal antibody against respiratory syncytial virus (RSV), was recently introduced in Catalonia (Spain, 2023-2024 season onwards) and Italy (2024-2025 season). The United Kingdom (UK) instead introduced maternal RSV vaccination (RSVpreF) in the 2024-2025 season. Our aim was to analyse emergency department (ED) attendances and admissions to hospital following RSVpreF and nirsevimab introduction, with hospitals in Iceland and Romania, where no intervention was introduced, used as comparators. Methods:Multi-national retrospective analysis of ED attendances and admissions for all diagnoses, respiratory diagnoses excluding bronchiolitis, and bronchiolitis from all hospitals in Catalonia (Spain), four UK hospitals (Bristol, Edinburgh, Glasgow, and Leicester), and one hospital in Italy (Rome), Romania (Bucharest), and Iceland (Reykjavík) from April 2018 to March 2025. Bronchiolitis diagnoses in the 2024-2025 season were compared to previous pre-intervention seasons (2018-2023, excluding the 2020-2021 COVID-19 year) by applying a generalised linear model in Poisson regression to obtain risk ratios (RR) and 95% confidence intervals (95% CI). Findings:In the 2024-2025 season, in Catalonia, there was a reduction in the RR for bronchiolitis ED attendances and admissions in infants of age <6 months (RR 0.45; 95% CI 0.43-0.47 and RR 0.40; 95% CI 0.37-0.43, respectively). This was not seen in Rome, where the RR for ED attendances with bronchiolitis in infants of age <6 months was 1.09 (95% CI 0.92-1.30) and the RR for admissions was 1.12 (95% CI 0.83-1.52). In the UK, for infants of age <6 months with bronchiolitis, there was a significant but modest reduction in 1 out of 4 hospitals for ED attendances (Leicester; RR 0.91, 95% CI 0.85-0.97) and in 2 out of 4 hospitals for admissions (Leicester; RR 0.80, 95% CI 0.69-0.94 and Edinburgh; RR 0.85, 95% CI 0.76-0.95). Interpretation:In Catalonia, there was a sustained reduction in bronchiolitis ED attendances and admissions for infants in the 2024-2025 season. However, no substantial reduction in bronchiolitis ED attendances or admissions was observed in Rome or the UK. These differences are likely to relate to the reduced uptake of RSV prevention products in these settings compared to Catalonia. Funding:None.
Introduction We aimed to establish the clinical characteristics of children and young people (CAYP) currently receiving deep brain stimulation (DBS) therapy for the management of movement disorders in the UK to better inform planning of future service provision. Methods Cross-sectional service evaluation of centres providing DBS for the management of movement disorders in childhood. Results A total of 139 CAYP were identified across three centres. Median age at surgery was 9.8 years (range 2.0-18.9 years), and median duration of DBS was 4.4 years (range from 1 week to 15.75 years). Modal Gross Motor Function Classification System level was V (n=66). The most common causes of movement disorder were dyskinetic cerebral palsy (69/139, 49.6%), dystonia due to mutations in the lysine methyltransferase 2B gene, aka DYT-KMT2B, (13/139, 9.4%) and dystonia due to mutations in the Torsin-1A gene, aka DYT-TOR1A, (9/139, 6.5%). A monogenetic cause of dystonia without evidence of central nervous system pathology on MRI was identified in 30 CAYP (21.6%). Clinically significant dystonia was present in all CAYP, with significant chorea in 47/139 (33.8%) and significant spasticity in only 13/139 (9.4%). No tone-reducing medications were currently used by 43/139 (30.9%) of CAYP. The remaining 96/139 CAYP were currently receiving 1-6 tone-reducing medications, most commonly gabapentin (n=58), clonidine (n=50) and a form of benzodiazepine (n=43). Despite care being provided by paediatric services, 37/139 (26.6%) of CAYP were >18 years of age. Conclusions CAYP currently receiving DBS therapy represent a heterogeneous population in terms of dystonia aetiology, functional level and additional pharmacological management. Only 102 CAYP<18 years of age are currently receiving DBS therapy in the UK, representing a small proportion of the population who could benefit from this intervention.
BACKGROUND:Massive left ventricular hypertrophy (LVH) is a risk factor for sudden cardiac death in children with hypertrophic cardiomyopathy (HCM), but little is understood about its natural history. METHODS:Patients with pediatric-onset HCM identified from 2 registries (SHaRe [Sarcomeric Human Cardiomyopathy Registry] and IPHCC [International Paediatric Hypertrophic Cardiomyopathy Consortium]) with or without massive LVH were compared. Massive LVH was defined as absolute maximal left ventricular wall thickness (MLVWT) ≥30 mm or MLVWT z score ≥+20 at <18 years of age. Data from SHaRe and IPHCC include encounters from January 1960 through March 2024 and January 1970 through March 2024, respectively. Demographic, clinical, and serial MLVWT data were collected. Composite outcomes included major ventricular arrhythmia event (sudden cardiac death, aborted sudden cardiac death, or appropriate implantable cardioverter defibrillator therapy); heart failure (HF) event (left ventricular ejection fraction <50%, New York Heart Association class III or IV, transplant, or HF-related death); major adverse cardiac event (stroke or any major ventricular arrhythmia or HF outcome aside from left ventricular ejection fraction <50%); and HCM-related mortality (sudden cardiac death or HF-related death). Time-to-event analyses were performed using Cox proportional hazards models. RESULTS:We identified 587 patients (54 female [30%]). In 186 children with massive LVH, age at diagnosis was younger (median, 9.2 years [interquartile range, 2.1-13.1 years]) versus 13.6 years (9.7-15.5 years; P<0.001) and sarcomeric genetic variants more prevalent (72% versus 61%; P=0.034), as was HCM-related mortality (unadjusted hazard ratio, 3.3 [95% CI,1.2-9.7]; P=0.026), major adverse cardiac events (hazard ratio, 2.6 [1.7-3.9]; P<0.001), major ventricular arrhythmia (hazard ratio, 3.1 [1.8-5.2]; P<0.001), and HF (hazard ratio, 1.9 [1.1-3.1]; P=0.013). These associations remained significant when adjusted for sex and age at HCM diagnosis. In 115 patients with massive LVH with serial MLVWT data (62%), MLVWT increased significantly from first to last measurements (median, 26 mm [interquartile range, 18-32 mm] versus 31 mm [26-35 mm]; P<0.001), but there was no difference between z scores (median, +22 [interquartile range, +18 to +26] versus +23 [+20 to +28]; P=0.25). The last absolute MLVWT recorded was >5 mm less than the largest recorded MLVWT in 25 patients (22%). CONCLUSIONS:In pediatric HCM, massive LVH disproportionately affects those diagnosed in early childhood with sarcomeric disease, with increased risk for adverse events. Significant MLVWT regression is seen in nearly a quarter of patients.
