Great Ormond Street

BACKGROUND: There is limited evidence on recent trends in childhood growth trajectories in Low-/middle-income countries. We investigated how age-trajectories for height and Body Mass Index (BMI) have changed among Brazilian children born in two different time periods after 2000. METHODS: We used a population-based cohort (part of the "Cohort of 100-Million Brazilians") created by the linkage of three Brazilian administrative databases: the Cadastro Único of the Federal Government, the National System of Live Births and the National Nutritional and Food Surveillance System...

OBJECTIVE: The objective was to analyze seizure semiology in pediatric frontal lobe epilepsy patients, considering age, to localize the seizure onset zone for surgical resection in focal epilepsy. METHODS: Fifty patients were identified retrospectively, who achieved seizure freedom after frontal lobe resective surgery at Great Ormond Street Hospital. Video-electroencephalography recordings of preoperative ictal seizure semiology were analyzed, stratifying the data based on resection region (mesial or lateral frontal lobe) and age at surgery (≤4 vs >4)...

This paper outlines a 10-patient case series of chronic non-bacterial osteomyelitis (CNO) of the mandible at a tertiary paediatric hospital in the UK. Our findings highlight the homogeneous presenting signs and symptoms of an intermittently painful, swollen angle and ramus of the mandible. We present the typical laboratory investigative findings (normal inflammatory markers) and imaging appearances (sclerosis and periosteal oedema). Our paper outlines an investigation protocol, including recommendations for extraoral bone biopsies and systemic magnetic resonance imaging (MRI)...

BACKGROUND AND OBJECTIVES: Growing skull fracture (GSF) is a rare complication of pediatric head trauma. Definitive treatment is surgical repair. We have attempted to assess whether use of autologous grafts for duraplasty and cranioplasty leads to better outcomes. We have also attempted to understand how timing of surgery might affect the degree of underlying damage to cortical tissue. METHODS: This is a single-center retrospective observational study based on review from the Great Ormond Street Hospital Neurosurgery prospective surgical database...

George Kantorowicz was a senior lecturer/consultant in conservative dentistry at the Royal Dental Hospital who provided ground-breaking treatment for cleft palate children at Great Ormond Street Hospital. His father, Alfred, was a Jewish professor, dean at Bonn Dental School and a world-famous advocate of mobile dental surgeries and prevention for children's dental health, until the Nazis put him in a concentration camp. After important interventions, he became director of Istanbul Dental Institute.

To evaluate EEG monitoring during neonatal ECMO and to identify any correlations between seizure detection to abnormal neuroimaging. Eight-year, service evaluation of neonates who received at least one continuous EEG (cEEG) whilst on ECMO at Great Ormond Street Hospital. Pearson's chi-square test and multivariate logistic regression analysis were used to assess clinical and EEG variables association with seizures and neuroimaging findings. Fifty-seven neonates were studied; 57 cEEG recordings were reviewed...

This article outlines the theoretical development of an approach to simulation prebriefing that we have adopted at Great Ormond Street Children's Hospital. Many educators struggle with anxious or reticent learners during simulation. This reticence often becomes apparent first during the simulation prebrief. Previous work highlights key points to cover in a prebrief. Less work has been done on the dilemma of how to engage such learners while also attempting to maintain a pedagogically effective stance for the entire group...

AIM: To determine dental arch relationships of Saudi children born with nonsyndromic complete unilateral cleft lip and palate (UCLP). MATERIAL AND METHODS: This is a retrospective cohort study that comprised dental study models of 74 UCLP Saudi children aged 8-10 years who were recruited from 14 referral cleft centers. All participants had their cleft lip and palate repaired with no history of alveolar bone graft or any orthodontic treatment. Dental arch relationships of UCLP patients were assessed using the Great Ormond Street, London, and Oslo (GOSLON) Yardstick-a clinical tool that categorizes dental relationships of UCLP children into five discrete grades from I to V...

BACKGROUND: Methotrexate (MTX) is the gold-standard first-line disease-modifying anti-rheumatic drug for juvenile idiopathic arthritis (JIA), despite only being either effective or tolerated in half of children and young people (CYP). To facilitate stratified treatment of early JIA, novel methods in machine learning were used to i) identify clusters with distinct disease patterns following MTX initiation; ii) predict cluster membership; and iii) compare clusters to existing treatment response measures...

Spring-assisted posterior vault expansion has been adopted at the London Great Ormond Street Hospital for Children to treat raised intracranial pressure in patients affected by syndromic craniosynostosis, a congenital calvarial anomaly causing the premature fusion of skull sutures. This procedure involves elastic distractors used to dynamically reshape the skull and increase the intracranial volume (ICV). In this study, we developed and validated a patient-specific model able to predict the ICV increase and carried out a parametric study to investigate the effect of surgical parameters on that final volume...

INTRODUCTION: Tracheostomy decannulation is an important and final step in managing patients once the underlying issue requiring a tracheostomy resolves. However, no consensus exists on the optimal method to decannulate a paediatric patient. We revisit the Great Ormond Street Hospital (GOSH) tracheostomy decannulation protocol, a 5-day process involving downsizing the tracheostomy tube, capping, and observation, to evaluate its effectiveness and assess if changes to the protocol are required...

INTRODUCTION: There is growing interest in health, developmental and survival outcomes of children who are born HIV-free to women living with HIV (children born HIV-free). To date, the research agenda has been largely determined by researchers, funders and policy makers, with limited involvement of parents, who are key stakeholders. Researchers at UCL Great Ormond Street Institute of Child Health in partnership with community-based organisation 4M Network of Mentor Mothers conducted two workshops with parents in March 2022 to establish research priorities for children born HIV-free, and key considerations for methodological approaches both to research and engagement with the affected communities...

Systemic juvenile idiopathic arthritis (sJIA) is a complex, systemic inflammatory disorder driven by both innate and adaptive immunity. Improved understanding of sJIA pathophysiology has led to recent therapeutic advances including a growing evidence base for the earlier use of IL-1 or IL-6 blockade as first-line treatment. We conducted a retrospective case notes review of patients diagnosed with sJIA over a 16-year period (October 2005-October 2021) at Great Ormond Street Hospital for Children. We describe the clinical presentation, therapeutic interventions, complications, and remission rates at different timepoints over the disease course...

BACKGROUND: Newborn screening (NBS) programmes for severe combined immunodeficiency (SCID) facilitate early SCID diagnosis and promote early treatment with haematopoietic stem cell transplantation, resulting in improved clinical outcomes. Infants with congenital athymia are also identified through NBS due to severe T-cell lymphopaenia. With the expanding introduction of NBS programmes, referrals of athymic patients for treatment with thymus transplantation have recently increased at Great Ormond Street Hospital (GOSH), London, United Kingdom...

BACKGROUND: Most neonatal and infantile-onset epilepsies have presumed genetic aetiologies, and early genetic diagnoses have the potential to inform clinical management and improve outcomes. We therefore aimed to determine the feasibility, diagnostic yield, and clinical utility of rapid genome sequencing in this population. METHODS: We conducted an international, multicentre, cohort study (Gene-STEPS), which is a pilot study of the International Precision Child Health Partnership (IPCHiP)...

BACKGROUND: Rhinovirus (HRV) is a significant seasonal pathogen in children. The emergence of SARS-CoV2, and the social restrictions introduced in, disrupted viral epidemiology. Here we describe the experience of Great Ormond Street Hospital (GOSH), where HRV almost entirely disappeared from the paediatric intensive care units (PICU) during the first national lockdown and then rapidly re-emerged with a fast-increasing incidence, leading to concerns about possible nosocomial transmission in a vulnerable population...

BACKGROUND/OBJECTIVES: There is a paucity of online educational content targeting children and young people with uveitis. We evaluated the impact of a co-designed patient education video on subjective and objective understanding of childhood uveitis. SUBJECTS/METHODS: Co-designed patient education media were produced in collaboration with the Childhood Uveitis Studies steering group and the Great Ormond Street Hospital Generation R Young People's Advisory Group and narrated by children...

BACKGROUND: Sudden unexpected death in infancy (SUDI) is the most common cause of post-neonatal death in the developed world. Following an extensive investigation, the cause of ~40% of deaths remains unknown. It is hypothesized that a proportion of deaths are due to an infection that remains undetected due to limitations in routine techniques. This study aimed to apply 16S rRNA gene sequencing to post-mortem (PM) tissues collected from cases of SUDI, as well as those from the childhood equivalent (collectively known as sudden unexpected death in infancy and childhood or SUDIC), to investigate whether this molecular approach could help identify potential infection-causing bacteria to enhance the diagnosis of infection...

INTRODUCTION: The preauricular sinus is a common finding in children and may often be complicated by infection. Complete excision of the sinus is the only definitive cure. Failure to recognise the presence of a sinus, particularly when infection erupts away from the sinus, may lead to inadequate management and unnecessary surgery. AIM: We report our experience in managing infected preauricular sinuses and highlight important points in our surgical technique. METHODS: A retrospective review was performed of our electronic patient database for all paediatric patients who underwent surgical excision of preauricular sinuses by the senior author at Great Ormond Street Hospital for Children between January 2013 and October 2022...

OBJECTIVE: Characterise the diagnostic and prognostic value of muscle MRI patterns as biomarkers in a genetically heterogeneous nemaline myopathy (NM) patient cohort. METHODS: Modified Mercuri scoring of lower limb MRI in genetically characterised NM patients referred to the highly specialised service for congenital myopathies at Great Ormond Street Hospital. Findings were compared to clinical data and MRI patterns derived from collated published data. RESULTS: Twenty-seven patients with MRI were identified (8 NEB-NM, 13 ACTA1-NM, 6 TPM3-NM)...