2,594 results match your criteria: "Great Ormond Street Hospital for Children NHS Foundation Trust.[Affiliation]"

Background: There are challenges in providing high quality care for children and young people who are admitted to acute medical wards for mental health concerns. Although there is concern that these admissions are increasing, national data describing these patterns are scarce. We aimed to describe trends in these admissions in England over a 10-year period, and to identify factors associated with repeat admission and length of stay.

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Introduction: Given its proximity to the central nervous system, surgical site infections (SSIs) after craniotomy (SSI-CRAN) represent a serious adverse event. SSI-CRAN are associated with substantial patient morbidity and mortality. Despite the recognition of SSI in other surgical fields, there is a paucity of evidence in the neurosurgical literature devoted to skin closure, specifically in patients with brain tumors.

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Complex biological systems undergo sudden transitions in their state, which are often preceded by a critical slowing down of dynamics. This results in longer recovery times as systems approach transitions, quantified as an increase in measures such as the autocorrelation and variance. In this study, we analysed paediatric patients in intensive care for whom mechanical ventilation was discontinued through removal of the endotracheal tube (extubation).

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Objective: To present a remodeling of the electroretinogram waveform using a covariance matrix to identify regions of interest and distinction between a control and attention deficit/hyperactivity disorder (ADHD) group. Electroretinograms were recorded in n = 25 ADHD (16 male; age 11.9 ± 2.

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Radiology's role in humanising mortality.

BMJ

January 2025

Department of Radiology, Tameside and Glossop NHS Foundation Trust, Ashton-under-Lyne, UK.

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Objective: The Sedation and Weaning in Children (SANDWICH) trial of a sedation weaning and ventilator liberation bundle had a primary outcome of time to successful extubation, and showed significant but small difference. We explored the impact of the intervention on infants with bronchiolitis.

Design: Post hoc subgroup analysis of a cluster-randomized trial, 2018 to 2019 (ISRCTN16998143).

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Purpose: Proliferative vitreoretinopathy (PVR) is a complication of retinal detachment which requires multiple vitreoretinal surgical interventions and frequent use of oil endotamponade. In this study, we conducted an in-depth analysis of complications associated with the use of heavy silicone oil in the management of inferior PVR.

Methods: A retrospective cohort study of 20 eyes that underwent vitrectomy for inferior PVR with use of heavy silicone oil (Densiron 68) between March 2021 and October 2022 at Oxford Eye Hospital.

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A novel, dominant disease mechanism of distal renal tubular acidosis with specific variants in ATP6V1B1.

Nephrol Dial Transplant

January 2025

Paediatric Nephrology, UZ Leuven and Department of Cellular and Molecular Physiology, KUL, Leuven, Belgium.

Background And Hypothesis: ATP6V1B1 encodes a subunit of the vacuolar H+-ATPase and pathogenic variants are associated with autosomal recessive distal renal tubular acidosis (dRTA) with deafness. Heterozygous variants predicted to affect a specific amino acid, Arg394, have been recurrently reported in dRTA but their significance has been unclear. We hypothesised that these variants are associated with a dominant disease mechanism.

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Symptom appraisal and help-seeking before a cancer diagnosis during pregnancy: a qualitative study.

Br J Gen Pract

January 2025

University of Surrey Faculty of Health and Medical Sciences, School of Health Sciences, Guildford, United Kingdom.

Background Estimated incidence of cancer diagnosis during or shortly after pregnancy is 1 in 1,000 women. Pregnancy can impact symptom appraisal and help-seeking for symptoms subsequently diagnosed as cancer. Little is known about the pathway to cancer diagnosis in pregnancy or delays that women can encounter.

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Febrile infants often have self-limiting conditions. Differentiating them from infants with serious bacterial infections can be challenging. We aimed to understand how febrile infants are managed across London, by analysing the management steps from local clinical practical guidelines (CPGs) and comparing them to the national guideline 143 (NG143).

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X-linked hypophosphataemia (XLH) is a rare metabolic bone disorder caused by pathogenic variants in the PHEX gene, which is predominantly expressed in osteoblasts, osteocytes and odontoblasts. XLH is characterized by increased synthesis of the bone-derived phosphaturic hormone fibroblast growth factor 23 (FGF23), which results in renal phosphate wasting with consecutive hypophosphataemia, rickets, osteomalacia, disproportionate short stature, oral manifestations, pseudofractures, craniosynostosis, enthesopathies and osteoarthritis. Patients with XLH should be provided with multidisciplinary care organized by a metabolic bone expert.

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Tuberous sclerosis complex-associated kidney disease in children.

Pediatr Nephrol

January 2025

PKD Research Group, Laboratory of Ion Channel Research, Department of Cellular and Molecular Medicine, KU Leuven, Louvain, Belgium.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder which can have manifestations in the kidneys, along with other organ systems. Children with TSC may develop kidney lesions at any point during childhood, and typically these are angiomyolipomata (AML) and/or kidney cysts. Children may also have hypertension associated with TSC-associated kidney disease, and rarely reduced kidney function.

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Objectives: A conservative oxygenation strategy is recommended in adult and pediatric guidelines for the management of acute respiratory distress syndrome to reduce iatrogenic lung damage. In the recently reported Oxy-PICU trial, targeting peripheral oxygen saturations (Spo2) between 88% and 92% was associated with a shorter duration of organ support and greater survival, compared with Spo2 greater than 94%, in mechanically ventilated children following unplanned admission to PICU. We investigated whether this benefit was greater in those who had severely impaired oxygenation at randomization.

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Objective: Charles Bonnet syndrome (CBS) refers to the presence of visual hallucinations occurring secondary to visual impairment. The aim of this study was to understand the phenomenology of CBS in children and assess the emotional impact and support needs of patients and their families.

Design: Semistructured qualitative interview study.

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Background: During COVID-19 pandemic, a rapid readjustment to continued delivery of healthcare was required. Redeployment is an intentional process to mobilise human resources by reassigning a healthcare worker to a new role or new work location, to achieve sustainable delivery of patient care. We report redeployment experiences of staff from a specialist children's hospital during first and second waves of the United Kingdom COVID-19 pandemic.

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COA5 has an essential role in the early stage of mitochondrial complex IV assembly.

Life Sci Alliance

March 2025

https://ror.org/01kj2bm70 Mitochondrial Research Group, Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, UK

Pathogenic variants in cytochrome oxidase assembly factor 5 (COA5), a proposed complex IV (CIV) assembly factor, have been shown to cause clinical mitochondrial disease with two siblings affected by neonatal hypertrophic cardiomyopathy manifesting a rare, homozygous missense variant (NM_001008215.3: c.157G>C, p.

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Objective: Survivors of pediatric head and neck rhabdomyosarcoma (HNRMS) are at risk of developing endocrinopathies following local treatment, resulting from radiation damage to the pituitary gland, hypothalamus, or thyroid gland, often at a young age. Our aim was to determine the prevalence of endocrine dysfunction in long-term HNRMS survivors and compare the prevalence of anterior pituitary insufficiency (API) among different local treatment strategies: external beam radiation with photons, external beam radiation with protons, microscopically radical surgery combined with external irradiation, and macroscopic radical surgery combined with brachytherapy.

Design And Methods: Head and neck rhabdomyosarcoma survivors treated between 1993 and 2017, with ≥2 years of follow-up, without recurrent disease or secondary malignancy were eligible for this study.

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Reactive oxygen species (ROS) within the retina play a key role in maintaining function and cell survival. However, excessive ROS can lead to oxidative stress, inducing dysregulation of metabolic and inflammatory pathways. The zebrafish models choroideremia (CHM), an X-linked chorioretinal dystrophy, which predominantly affects the photoreceptors, retinal pigment epithelium (RPE), and choroid.

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Patient reported outcome measures (PROMs) capture patients' views of their health status and the use of PROMs as part of standard care of children and young people has the potential to improve communication between patients/carers and clinicians and the quality of care. Electronic systems for the collection of or access to PROMs and integrating PROMs into electronic health records facilitates their implementation in routine care and could help maximise their value. Yet little is known about the technical aspects of implementation including the electronic systems available for collection and capture and how this may influence the value of PROMs in routine care which this scoping review aims to explore.

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Vanzacaftor-tezacaftor-deutivacaftor for children aged 6-11 years with cystic fibrosis (RIDGELINE Trial VX21-121-105): an analysis from a single-arm, phase 3 trial.

Lancet Respir Med

December 2024

Population Policy and Practice Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. Electronic address:

Article Synopsis
  • Vanzacaftor-tezacaftor-deutivacaftor is a new CFTR modulator showing safety and effectiveness in phase 2 trials for adults with cystic fibrosis, leading to a study evaluating its use in children aged 6-11.
  • This phase 3 trial, called RIDGELINE, involved participants from 33 clinical sites across eight countries, focusing on children with specific CFTR variants and stable health conditions.
  • The study aimed to assess the drug's safety, tolerability, and efficacy over 24 weeks, with primary outcomes evaluated through various health metrics and participant feedback.
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Are you ready to expose yourself?

Eur J Radiol

December 2024

Great Ormond Street Hospital for Children NHS Foundation Trust, London WC1N 3JH, UK; UCL Great Ormond Street Institute of Child Health, London, UK; Great Ormond Street Hospital NIHR Biomedical Research Centre, London, UK.

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Technical Aspects of a National Service for Paediatric Pelvic Brachytherapy.

Clin Oncol (R Coll Radiol)

December 2024

Department of Oncology, University College London Hospitals NHS Foundation Trust, 250 Euston Road, London, NW1 2PG, UK. Electronic address:

Aims: Brachytherapy is advantageous for localised rhabdomyosarcomas in children compared with external beam radiotherapy, sparing close organs at risk with highly conformal dosimetry. A methodology for planning and delivering fractionated high-dose-rate paediatric pelvic brachytherapy is detailed, and the dosimetric parameters are presented. This provides a practical template for radiotherapy departments with a similar patient cohort to implement this treatment technique.

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Background: Long segmental congenital tracheal stenosis and long segmental congenital tracheobronchial stenosis are rare congenital airway anomalies with variable arborizations. This study aimed to analyze presentations and outcomes of slide tracheoplasty in long segmental congenital tracheal and tracheobronchial stenosis with variable arborizations.

Methods: Retrospective analysis included all patients who underwent slide tracheoplasty between March 1995 and February 2023 for long segmental congenital tracheal and tracheobronchial stenosis at the Great Ormond Street Hospital for Children (London, United Kingdom).

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