A5052019557- Neonatal Respiratory Health Research
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Obstructive Sleep Apnea Research
- Respiratory Support and Mechanisms
- Asthma and respiratory diseases
- Cystic Fibrosis Research Advances
- Tracheal and airway disorders
- High Altitude and Hypoxia
- Neuroscience of respiration and sleep
- Congenital Diaphragmatic Hernia Studies
- Hemoglobinopathies and Related Disorders
- Genetic Syndromes and Imprinting
- Neurogenetic and Muscular Disorders Research
- Childhood Cancer Survivors' Quality of Life
- Cardiovascular and Diving-Related Complications
- Neurological and metabolic disorders
- Cardiac Arrest and Resuscitation
- Craniofacial Disorders and Treatments
- Dermatology and Skin Diseases
- Mitochondrial Function and Pathology
- Iron Metabolism and Disorders
- Tumors and Oncological Cases
- Esophageal and GI Pathology
- Fetal and Pediatric Neurological Disorders
- Delphi Technique in Research
Great Ormond Street Hospital for Children NHS Foundation Trust
2013-2023
Great Ormond Street Hospital
2011-2023
University College London
2011-2023
IRCCS Ospedale San Raffaele
2022
Royal Brompton Hospital
2022
NIHR Great Ormond Street Hospital Biomedical Research Centre
2022
William Harvey Hospital
1989
Nitric oxide (NO) produced within the respiratory tract is detectable in exhaled and nasal air. Its synthesis may be induced by inflammatory cytokines reduced glucocorticoids. Increased concentrations have been found asthma bronchiectasis. In this study, NO were determined 63 children with cystic fibrosis, of whom 13 on inhaled steroids (mean age 13.3 years) 50 not 12.3 years); 57 normal 12.2 also studied. was measured chemiluminescence analyser, following a relaxed vital capacity manoeuvre,...
Exercise tolerance may be reduced in patients with cystic fibrosis, but it is not always possible to predict this from standard lung function measurements. Formal exercise testing may, therefore, necessary, and the test should simple readily available. We have developed a "3-minute step test" compared 6-minute walking test. Subjects stepped up down 15-cm-high single at rate of 30 steps per minute for 3 minutes. The effect on spirometry was tested first 31 children CF (mean age, 12.0 years),...
Achondroplasia can result in respiratory difficulty early infancy. The aim of this study was to document lung growth during infancy, together with the cause any cardiorespiratory and sleep dysfunction.Seventeen prospectively ascertained infants (14 boys three girls) symptoms starting before 1 year age underwent clinical, sleep, function studies.Three distinct groups were identified. Group (n = 6) least symptomatic only had obstructive apnoea. 2 apnoea muscular aetiology and, neurologically,...
Abstract Although the prevalence of seizures in children with sickle cell disease (SCD) is 10 times that general population, there are few prospectively collected data on mechanism. With transcranial Doppler and magnetic resonance imaging (MRI) angiography, we evaluated 76 patients disease, 29 asymptomatic 47 neurological complications (seizures, stroke, transient ischemic attack, learning difficulty, headaches, or abnormal Doppler), who also underwent bolus‐tracking perfusion MRI. The six...
The Pediatric Sleep Questionnaire described by Chervin et al. (Sleep Medicine, 2000, 1, 21-32) was originally validated for children with obstructive sleep apnoea syndrome but without other disorders. aim of our study to check the applicability this questionnaire in underlying chronic medical conditions. Children aged 2-18 years who underwent a diagnostic at Great Ormond Street Hospital were recruited over 10-month period. completed their parents and cardiorespiratory polygraphy scored....
Abstract Objective To describe clinical features and disease progression of Selenoprotein N‐related myopathy in a large multicenter cohort patients. Methods Cross‐sectional data analysis 60 patients (53 families) with single‐center retrospective longitudinal 25 (21 over median period 5.3 years. Results The majority (46/60, 77%) presented before age 2 years hypotonia, poor head/neck control, developmental delay. At last assessment (median 14 years; range 2.5 to 36 years), 10/60 had minimal or...
Abstract Background Children with orphan lung diseases (defined as a prevalence of <1 in 2000) receive suboptimal care due to lack understanding pathophysiology and management. Aim: To develop low cost, multidisease, web‐based registry for children rare diseases. Methods The British Pediatric Orphan Lung Disease (BPOLD) electronic was established provide method recording monthly incidence data on nine pediatric respiratory ( www.bpold.co.uk ). An email reporting system developed which...
To test the hypothesis that children with Prader-Willi syndrome (PWS) and obstructive sleep apnoea (OSAS) have hypercapnia for higher proportion of total time (TST) than non-syndromic similar apnoea-hypopnoea index (OAHI).Cross-sectional study.Two tertiary care hospitals.Patients PWS snoring who underwent polygraphy were age, body mass (BMI) z-score OAHI.The two groups compared regarding %TST transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between diagnosis OSAS severity (OAHI <1...
The decline of respiratory function in Duchenne muscular dystrophy (DMD) is associated with sleep disordered breathing (SDB) and alteration nocturnal gas exchange, first manifesting as hypoventilation (NH). However, the correlation between pulmonary measured by spirometry (PFT) onset SDB or without NH unclear. aims this study are to identify prevalence features investigate relationship lung determined forced vital capacity (FVC) abnormalities a large pediatric DMD population.This was...
Using histamine provocation tests, bronchial responsiveness was measured in 43 children with atopic dermatitis, aged 7 to 15 years, who had attended pediatric dermatology clinics within the past 5 years. The were divided into two groups, one group of 21 whom asthma been previously diagnosed and symptoms occurred preceding year, other comprising 22 denied any such symptoms. Bronchial hyperresponsiveness (as defined by a 20% fall forced expiratory volume 1 second at provoking dose 7.8 mumol or...
OBJECTIVES : To determine whether Sommerlad palate re-repairs and Hynes pharyngoplasties cause obstructive sleep apnea/hypopnea or increased upper airway resistance. DESIGN Prospective before-and-after study. SETTING Tertiary cleft unit. PATIENTS A total of 44 patients undergoing a re-repair pharyngoplasty. Interventions Preoperative postoperative multichannel cardiorespiratory studies. MAIN OUTCOME MEASURES The main outcome measures were mean arterial oxygen saturation, desaturation index,...
We assessed seven patients with hereditary neuropathy liability to pressure palsies (HNPP) 16 electrophysiological tests and cranial MRI for CNS abnormalities. Mean latencies differed between HNPP controls the blink reflex, jaw-opening acoustic evoked potentials. abnormalities were observed in four patients. Our study suggests subclinical but functionally relevant myelin damage HNPP.
Abstract Introduction The coronavirus disease 2019 (COVID‐19) pandemic has accelerated the move towards home spirometry monitoring, including in children. aim of this study is to determine whether remote supervision by a physiologist improves technical quality and failure rate maneuvers. Method Children with cystic fibrosis who had been provided NuvoAir spirometers were randomly allocated either supervised or unsupervised following detailed training session. Home was performed every 2 weeks...
<h3>Objectives:</h3> To assess the effect of altitude and acclimatisation on cardiorespiratory function well-being in healthy children. <h3>Methods:</h3> A daily symptom diary, serial measurements spirometry, end-tidal carbon dioxide (etCO<sub>2</sub>) daytime overnight pulse oximetry (SpO<sub>2</sub>), were undertaken at sea level altitudes up to 3500 m children during a trekking holiday. SpO<sub>2</sub> was compared with that flight acute hypoxic challenge (breathing 15% oxygen) level....
We investigated the feasibility of using an online registry to provide prevalence data for multiple orphan lung diseases in Australia and New Zealand. A web-based registry, The Australasian Registry Network Orphan Lung Diseases (ARNOLD) was developed based on existing British Paediatric Disease Registry. All adult paediatric respiratory physicians who were members Thoracic Society Zealand sent regular emails between July 2009 June 2014 requesting information patients they had seen with any...
Abstract Background Multi‐center research studies that include pulmonary function as an objective outcome are increasingly important in pediatric respiratory medicine. The need for local controls rather than depending on published normative data lung remains debatable. Aim To compare childhood with no symptoms from three centers the United Kingdom and ascertain extent to which current reference equations appropriate this population. Methods Spirometry, plethysmographic volumes, specific...
Abstract Background Clinical outcomes are normally captured less frequently than data from remote technologies, leaving a disparity in volumes of these different sources. To align data, flexible polynomial regression was investigated to estimate personalised trends for continuous outcome over time. Methods Using electronic health records, models inclusive 1st up 4th order were calculated predict forced expiratory volume 1 s (FEV ) time children with cystic fibrosis. The model the lowest AIC...
Abstract Multi‐center studies provide advantages in clinical research but differences between centers can introduce bias. Three specialist pediatric respiratory laboratories standardized their methodology and examined centers. The specific aims were to (i) assess the variability of measurements on adults within (ii) exchange cross‐analyze data from children extent agreement Each laboratory used identical equipment software. Inter‐laboratory visits standardize protocols for collection...
<b>Background:</b> Increased numbers of referrals for cardiorespiratory sleep studies are being received Ear, Nose and Throat (ENT) patients (without accompanying co-morbidities); these help diagnose Obstructive Sleep Apnoea (OSA). patient have made quick diagnosis difficult. The aim was to assess if Oxygen Desaturation Index (ODI) could be used as a surrogate Apnoea-Hypopnoea (AHI) OSA, in with no comorbidities. <b>Methods:</b> laboratory database (Microsoft Access) retrieve study data...
Exercise tolerance may be reduced in patients with cystic fibrosis, but it is not always possible to predict this from standard lung function measurements. Formal exercise testing may, therefore, necessary, and the test should simple readily available. We have developed a “3-minute step test” compared 6-minute walking test. Subjects stepped up down 15-cm-high single at rate of 30 steps per minute for 3 minutes. The effect on spirometry was tested first 31 children CF (mean age, 12.0 years),...
<b>Background:</b> Non-invasive ventilation (NIV), including continuous positive airway pressure (CPAP) and bilevel support (BiPAP), has made significant impact on many conditions e.g. neuromuscular, craniofacial, obesity, metabolic diseases. As the indications for long term NIV expands, number of patients treated increases exponentially, making compliance a key issue. <b>Aim:</b> To assess objective data adherence to in children at large tertiary centre <b>Method:</b> Adherence were...