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Background Allergic bronchopulmonary aspergillosis (ABPA) is an allergic reaction to colonisation of the lungs with the fungus Aspergillus fumigatus and affects around 10% of people with cystic fibrosis. ABPA is associated with an accelerated decline in lung function. High doses of corticosteroids are the main treatment for ABPA; although the long-term benefits are not clear, their many side effects are well-documented. A group of compounds, the azoles, have activity against Aspergillus fumigatus and have been proposed as an alternative treatment for ABPA. Of this group, itraconazole is the most active. A separate antifungal compound, amphotericin B, has been employed in aerosolised form to treat invasive infection with Aspergillus fumigatus, and may have potential for the treatment of ABPA. Antifungal therapy for ABPA in cystic fibrosis needs to be evaluated. This is an update of a previously published review. Objectives The review aimed to test the hypotheses that antifungal interventions for the treatment of ABPA in cystic fibrosis: 1. improve clinical status compared to placebo or standard therapy (no placebo); 2. do not have unacceptable adverse effects. If benefit was demonstrated, we aimed to assess the optimal type, duration and dose of antifungal therapy. Search methods We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches, handsearches of relevant journals and abstract books of conference proceedings. In addition, pharmaceutical companies were approached. Date of the most recent search of the Group's Trials Register: 29 September 2016. Selection criteria Published or unpublished randomised controlled trials, where antifungal treatments have been compared to either placebo or no treatment, or where different doses of the same treatment have been used in the treatment of ABPA in people with cystic fibrosis. Data collection and analysis Four trials were identified by the searches; none of which was judged eligible for inclusion in the review. Main results No completed randomised controlled trials were included. Authors' conclusions At present, there are no randomised controlled trials to evaluate the use of antifungal therapies for the treatment of ABPA in people with cystic fibrosis, although trials in people who do not have cystic fibrosis have shown clinical and serological evidence of improvement and a reduction in the use of corticosteroids with no increase in adverse effects. Trials with clear outcome measures are needed to properly evaluate this potentially useful treatment for cystic fibrosis.

Narcolepsy is a chronic disabling neurological sleep disorder that requires lifelong treatment. We have outlined the clinical features of narcolepsy, the assessment and diagnosis process and have summarised the existing treatment options for children and adolescents with narcolepsy. In the future, the approach to management of paediatric narcolepsy should ideally be in a multidisciplinary setting, involving specialists in sleep medicine, sleep physiology, neurologists and psychologists/psychiatrists. A multidisciplinary approach will help to manage the potential impact of narcolepsy on children and adolescents who are in a stage of their life that is critical to their physical, emotional and social development and their academic attainment.

The objective was to investigate the prevalence of Pseudomonas aeruginosa (PA) in patients with complex neurodisability and current treatment practice in our centre in order to inform future guidelines. A retrospective case note review was undertaken at a tertiary children’s hospital. One hundred sixty-two patients (mean age 11.7 years) with a primary diagnosis of neuromuscular disease (NMD) or severe cerebral palsy (CP) and a respiratory sample sent for analysis during the study period were studied. Associations between PA in respiratory samples and diagnosis, long-term ventilation, presence of a gastrostomy or a tracheostomy, antibiotic choice, clinical deterioration and adverse events were analysed. Twenty-five (15%) had one or more PA isolate in respiratory samples. There was a significant association between PA in respiratory samples and tracheostomy (pConclusions: A larger prospective study may establish clearer criteria for guideline development. Techniques such as point-of-care testing to identify virulent strains of PA may improve patient outcomes and prevent the development of antibiotic resistance in the future. What is Known:•Children with complex neurodisability are at increased risk of respiratory morbidity and of infection with gram-negative organisms such as Pseudomonas aeruginosa.•There are currently no guidelines to inform treatment choices in this group of vulnerable children. What is New:•15% children in this study population had Pseudomonas aeruginosa in respiratory samples during a 12-month period, the majority of whom did not require critical care treatment. Thirteen of these children had a tracheostomy in situ and 12 did not. •In those that deteriorated clinically or developed antibiotic resistant organisms, earlier detection and targeted treatment of Pseudomonas aeruginosa may have prevented deterioration.

Non-invasive ventilation (NIV) is assisted mechanical ventilation delivered via a facemask for people with chronic conditions that affect breathing. NIV is most commonly delivered via an interface (mask) covering the nose (nasal mask) or the nose and mouth (oronasal mask). The number of children in the UK requiring NIV is currently estimated to be around 5000. Mass-produced masks are available for both the adult and paediatric markets but masks that fit well are difficult to find for children who are small or have asymmetrical facial features. A good conforming fit between the mask and the patient's face to minimise unintentional air leakage is essential to deliver the treatment effectively; most ventilators will trigger an alarm requiring action if such leakage is detected. We present an innovative use of 3D scanning and manufacturing technologies to deliver novel mask-face interfaces to optimise mask fit to the needs of individual patients. Ahead of planned user trials with paediatric patients, the project team trialled the feasibility of the process of creating and printing bespoke masks from 3D scan data and carried out testing of the masks in adult volunteers to select the strongest design concept for the paediatric trial. The evaluation of the process of designing a bespoke mask from scan data, arranging for its manufacture and carrying out user testing has been invaluable in gaining knowledge and discovering the pitfalls and timing bottlenecks in the processes. This allowed the team to iteratively refine the techniques and methods involved, informing user trials later on in the project. It has also provided indicative cost estimates for 3D printed mask prototype components which are useful in project decision making and trial planning. The value of the process extends to considerations for future implementation of the process within a clinical pathway.

Objective To assess the value of respiratory rate (RR) as a predictor of clinical deterioration in children, compared with other vital sign measurements. Design A retrospective case-control study, comparing children who deteriorated, requiring admission to critical care with children who did not deteriorate. Methods RR, heart rate (HR), and blood pressure (BP) measurements were collected from each patient for a 48-hour duration. The 95th centile was identified for each and 5% to 30% thresholds above the 95th centile were calculated. For each threshold the sensitivity, specificity, odds ratio, positive, and negative predictive value for deterioration was calculated. Results Forty cases (age range 7 weeks-15 years) and 40 control patients matched for age, gender, and hospital location were recruited. In 30/40 patients who deteriorated at least one RR ≥ 30% above the 95th centile for their age was recorded in the 48 hours before deterioration, compared with 10/40 controls, regardless of clinical diagnosis. Only 3/40 children that deteriorated had a HR > 30% greater than the 95th centile, compared with 2/40 controls. An elevated RR was the only vital sign whose odds ratios were significant at each threshold level above the 95th centile. Maximum RR occurred 16.8 hours before deterioration. Conclusion RR is a more accurate predictor of clinical deterioration in children than other vital signs. Greater weighting and importance should be placed on RR, which is often omitted in children due to difficulties with its measurement.

Non-invasive ventilation (NIV) is assisted respiratory support delivered via facemask for people with chronic respiratory failure. Commercial NIV masks are available but masks that fit well are difficult to find for children who have small or asymmetrical facial features. Compromised ventilation can have significant health and quality of life impacts for patients and their families. The overarching aim of the project was to improve comfort, fit and performance of NIV masks for children using 3D technology. The needs of patients, parents/carers and healthcare professionals from 6 UK centres was ensured through events advising on mask design and research procedures. 3D scanning technologies were compared in vitro using workflows and deviation analysis and with 3 healthy and 19 patient volunteers. Early design concepts were tested and the final prototype selected using a test mannequin and adult volunteers. Prototype masks were manufactured using biocompatible materials to IOS 13485 quality standards and evaluated with 19 patients. Feedback on fit, comfort and facial marking and oxicapnography data to demonstrate ventilator effectiveness were collected. The potential cost-effectiveness of the customised masks was assessed using an economic model. A handheld structured light 3D scanner was selected due to superior resolution, ease of use, availability and cost. The final design was a semi-bespoke hybrid approach. 11/15 (73%) evaluation participants reported comfort and fit that was as good or better than their usual mask. 5/11 (45%) were unable to wear the mask due to poorly fitting headgear despite a well-fitting mask. All physiological ventilation parameters were improved (table 1). Preliminary health economics analysis demonstrated that to achieve a 5% improvement in fit and ventilation, a custom-made mask would be cost effective at a price below £500. A series of in vitro, user-perspective, healthy volunteer and patient evaluations informed the development of custom-made masks for children using NIV.

Background Autism spectrum disorder (ASD) is often seen alongside narcolepsy in childhood; however, little is known about the potential link between the two. Our objective was to identify any similarities or differences between children with narcolepsy who also have ASD and those who do not. Methods A single-centre retrospective records review was undertaken of all children attending narcolepsy clinics as of 1st of August 2021. Data collected included: date and method of narcolepsy diagnosis, severity of narcolepsy at diagnosis, Revised Children’s Anxiety and Depressions Scale (RCADS) scores from parent and child, presence of autistic traits, date of ASD diagnosis and support received by the child’s family. Results Data was collected from 83 sets of patient records. of this sample, 75 (90.4%) had a confirmed diagnosis of narcolepsy, further analysis was conducted on this group only. A total of 21 (28.0%) children were recorded to have autistic traits, 9 (12.0%) had a confirmed diagnosis of ASD; 88% of ASD diagnoses were made before investigation for narcolepsy. Children with and without ASD had similar SOL and REMSOP results on MSLT. When collecting RCADS data, 55.6% of questionnaires from children with ASD were incomplete for both parent and child, compared to 29.6% of questionnaires from cases without, there was greater discrepancy between parent and child scores in the ASD group and higher parent-rated anxiety scores. Children with ASD were also more likely to receive enhanced school support. Discussion Descriptive analysis of this sample has shown that 40% of children with narcolepsy also have and ASD diagnosis or autistic traits. These children were more likely to be rated as anxious by their parents and went on to require enhanced support throughout school. This may suggest that ASD is could act as a clinical indicator to offer enhanced support where possible.

Background Long term ventilation (LTV) refers to mechanical support for breathing either at home or in hospital, for all or part of the day, for at least 3 months. Two national surveys carried out in 1998 and 2008, highlighted the increasing numbers of ventilator dependent children throughout the UK . Our objective was to collect current information about children receiving LTV in the UK, 10 years after the last national survey. Methods All LTV centres in the UK completed a single time point census survey on 30th September 2019 using an electronic questionnaire. Data included the child’s location, underlying diagnosis, interface and type of respiratory support, and whether disease-modifying drugs affected the decision to initiate LTV in specific groups within this target population. Results Data was collected from 25 LTV centres. The total study population was 2383 children and young people. The median age of the overall sample was x (range x-x). 40.3% female; 57.2% of those recorded were male. Diagnoses were 417 (17.5%) central nervous system, 692 (29%) musculoskeletal and 1274 (53.5%) a respiratory, of which 31.8% of the total had upper airway obstruction. Notable changes since 1998 were the decline in the use of 24-hour ventilation, negative pressure ventilation and tracheostomy as an interface, and the increase in the proportion of patients treated at home. 115 children had received a disease-modifying drug. The use of Ataluren and Myozme did not influence the decision to treat with LTV, but in 35% of the children treated with Nusinersin the clinician stated that the use of this drug had or may have influenced their decision to initiate LTV. Conclusion The number of children being treated with LTV has increased by 250% in the last 10 years with notable changes in practice over the last 20 years.

Introduction A child’s activities during the hour before bedtime forms the basis of the assessment for behavioural change interventions for children’s insomnia. The aim of this observational study was to identify patterns of behaviour around bedtime in a cohort of children with severe sleep difficulties and to explore deviations in behaviour from The Sleep Charity’s standard bedtime routine advice. Methods Data were collected by research staff using a questionnaire during a baseline face-to-face visit to the child’s home prior to sleep practitioner support as part of the Sheffield Children’s Sleeping Well study. Children were aged 2-17 years with a diagnosis of ADHD or were identified as a looked-after child. Data was input into NVivo where it was coded to identify common or recurring keywords or phrases in the responses. Results 51 parents were interviewed. Table 1 presents the coded responses to questions relating to sleep behaviours. For 32 children, bedtime routines lasted 30 mins-2 hours; 17 had no routine. Only 13 children were reported to have actively had technology removed within the hour before bedtime. 32 had no planned snack. of the 17 that did, snacks were usually cereal, toast, biscuit, warm milk although some snacks included chocolate, hot chocolate, crisps, burger and chips. Only10 children engaged in calming activities in the hour before bedtime such as reading, colouring, lego and crafts. 28 children were left to self-settle and 22 had a parent laying or sitting next to them in their bedroom in order to fall asleep. Conclusion There was marked variation from the recommended bedtime routine in children with severe behavioural sleep difficulties, accepting that in some, socially desirable responses may have been given to the research staff during the assessment. Further work to explore the important factors in helping a child to self-settle is ongoing.

Background Children and young people (CYP) with special educational needs (SEN) are more likely to experience disturbed sleep and poorer mental wellbeing. This study explored the differential impact of the COVID-19 pandemic on the sleep and mental wellbeing of CYP with and without SEN. Methods NIHR Children and Young People MedTech Co-operative, Sheffield Children’s NHS Foundation Trust, and The Sleep Charity carried out an online survey between 23 June 2020 and 17 August 2020. The 77-item survey was shared via social media platforms. Results 559 participants were included in the analyses, 15.74% of whom reported having a CYP with SEN. While sleep changes due to the pandemic were largely similar for both groups, CYP with SEN (40.91%) were more likely to get or wake up during the night than CYP without SEN (40.91% vs 27.18%). CYP with SEN were significantly more likely than children without SEN to be demotivated (61.44% vs 31.57%), sad and tearful (36.15% vs 19.35%) or anxious and stressed (43.48% vs 14.82%) during the pandemic, as well as to report that increased anxiety was more likely to contribute to poorer sleep (43.48% vs 14.82%). Conclusion While the majority of CYP in both groups reported sleep changes due to the pandemic, CYP with SEN experienced more sleep disturbance. The findings provide initial evidence to suggest that the pandemic may have had a greater impact on the sleep and mental wellbeing of CYP with SEN compared to CYP without SEN.

Achondroplasia, the most common skeletal dysplasia, is characterized by a variety of medical, functional and psychosocial challenges across the lifespan. The condition is caused by a common, recurring, gain-of-function mutation in FGFR3, the gene that encodes fibroblast growth factor receptor 3. This mutation leads to impaired endochondral ossification of the human skeleton. The clinical and radiographic hallmarks of achondroplasia make accurate diagnosis possible in most patients. However, marked variability exists in the clinical care pathways and protocols practised by clinicians who manage children and adults with this condition. A group of 55 international experts from 16 countries and 5 continents have developed consensus statements and recommendations that aim to capture the key challenges and optimal management of achondroplasia across each major life stage and sub-specialty area, using a modified Delphi process. The primary purpose of this first International Consensus Statement is to facilitate the improvement and standardization of care for children and adults with achondroplasia worldwide in order to optimize their clinical outcomes and quality of life. Achondroplasia is the most common skeletal dysplasia and is characterized by various lifelong clinical, functional and psychosocial challenges for affected individuals. This first International Consensus Statement on the care of children and adults with achondroplasia aims to facilitate the global standardization and improvement of achondroplasia clinical care.

Sleep-related Rhythmic Movement Disorder (RMD) affects around 1% of UK pre-school children. Little is known about RMD in Down syndrome (DS). We aimed to determine: (a) the prevalence of RMD in children with DS aged 1.5–8 years, (b) phenotypic and sleep quality differences between children with DS and RMD and sex- and age-matched DS controls, and (c) night-to-night variability in rhythmic movements (RMs). Parents who previously reported RMs from a DS research registry of 202 children were contacted. If clinical history suggested RMD, home videosomnography (3 nights) was used to confirm RMs and actigraphy (5 nights) was used to assess sleep quality. Phenotype was explored by demographic, strengths and difficulties, Q-CHAT-10/social communication and life events questionnaires. Eight children had confirmed RMD. Minimal and estimated maximal prevalence were 4.10% and 15.38%, respectively. Sleep efficiency was significantly lower in RMD-cases (69.1%) versus controls (85.2%), but there were no other phenotypic differences. There was considerable intra-individual night-to-night variability in RMs. In conclusion, RMD has a high prevalence in children with DS, varies from night to night and is associated with poor sleep quality but, in this small sample, no daytime phenotypic differences were found compared to controls. Children with DS should be screened for RMD, which is amenable to treatment.

BACKGROUND: The choice of antibiotic, and the use of single or combined therapy are controversial areas in the treatment of respiratory infection due to Pseudomonas aeruginosa in cystic fibrosis (CF). Advantages of combination therapy include wider range of modes of action, possible synergy and reduction of resistant organisms; advantages of monotherapy include lower cost, ease of administration and reduction of drug‐related toxicity. Current evidence does not provide a clear answer and the use of intravenous antibiotic therapy in CF requires further evaluation. This is an update of a previously published review. OBJECTIVES: To assess the effectiveness of single compared to combination intravenous anti‐pseudomonal antibiotic therapy for treating people with CF. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Most recent search of the Group's Trials Register: 07 October 2020. We also searched online trials registries on 16 November 2020. SELECTION CRITERIA: Randomised controlled trials (RCTs) comparing a single intravenous anti‐pseudomonal antibiotic with a combination of that antibiotic plus a second anti‐pseudomonal antibiotic in people with CF. DATA COLLECTION AND ANALYSIS: Two authors independently assessed trial quality and extracted data. We assessed the certainty of the data using GRADE. MAIN RESULTS: We identified 59 trials, of which we included eight trials (356 participants) comparing a single anti‐pseudomonal agent to a combination of the same antibiotic and one other. There was a wide variation in the individual antibiotics used in each trial. In total, the trials included seven comparisons of a beta‐lactam antibiotic (penicillin‐related or third generation cephalosporin) with a beta‐lactam‐aminoglycoside combination and three comparisons of an aminoglycoside with a beta‐lactam‐aminoglycoside combination. There was considerable heterogeneity amongst these trials, leading to difficulties in performing the review and interpreting the results. These results should be interpreted cautiously. Six of the included trials were published between 1977 and 1988; these were single‐centre trials with flaws in the randomisation process and small sample size. Overall, the methodological quality was poor and the certainty of the evidence ranged from low to moderate. The review did not find any differences between monotherapy and combination therapy in either the short term or in the long term for the outcomes of different lung function measures, bacteriological outcome measures, need for additional treatment, adverse effects, quality of life or symptom scores. AUTHORS' CONCLUSIONS: The results of this review are inconclusive. The review raises important methodological issues. There is a need for an RCT which needs to be well‐designed in terms of adequate randomisation allocation, blinding, power and long‐term follow‐up. Results need to be standardised to a consistent method of reporting, in order to validate the pooling of results from multiple trials.

Non-invasive ventilation (NIV) is assisted mechanical ventilation delivered via a facemask for people with chronic conditions that affect breathing. Mass-produced masks are available for both the adult and paediatric markets but masks that fit well are difficult to find for children who are small or have asymmetrical facial features. A good fit between the mask and the patient's face to minimise unintentional air leakage is essential to deliver the treatment effectively. We present an innovative use of 3D assessment and manufacturing technologies to deliver novel custom-made facemasks for children for whom a well-fitting standard mask is not available. This paper aims to describe the processes undertaken to investigate and compare currently available technologies for 3D scanning children and to explore the design of a system for creating custom-made paediatric NIV masks within the NHS. The paper therefore considers not only the quality and accuracy of the data, but also other factors such as the time and ease of process. Searches for all currently available scanning technologies were made. Photogrammetry image stitch using a smartphone and a digital camera, and two structured light scanners were selected and compared in the laboratory, in discussion with user groups, and in adult volunteers. Using the processes described, it became apparent that the optimal 3D scanning system for this purpose was the handheld structured light scanner. This option offered both superior accuracy and convenience and was more cost effective.

Children with complex neurodisability have increased risk of respiratory tract infections. Pseudomonas aeruginosa (PA) is an organism that commonly infects and colonises the airway. Treatment choices are complicated by adverse effects and development of antibiotic resistant organisms. There are no established guidelines and the treatment approach of clinicians varies. A retrospective case notes review was undertaken to look at the prevalence of PA in respiratory samples of children with neurodisability, treatment practices and outcomes. Of 226 children with a neuromuscular condition or cerebral palsy (GMFCS score 4-5), 25 had at least one PA positive sample during a 12-month period. Those using a tracheostomy were twice as likely to isolate PA; 21 (80%) had recurrent isolates, suggesting colonisation of the airway. 62 individual clinical episodes were identified. Patients most likely to deteriorate clinically were those treated with broad-spectrum antibiotics (41%) compared with PA-specific (27%) or no antibiotics (11%). All 10 patients in whom antibiotics were changed as directed by microbial results, improved clinically thereafter. No adverse effects were documented as a direct result of antibiotics. Antibiotic resistance was documented in 8/21 colonised patients with these more likely to require escalation to intensive care treatment. A guideline for management and more sophisticated methods to direct treatment choices are needed. Techniques such as the ability to identify virulent strains of PA using point-of-care testing may improve patient outcomes and prevent the development of antibiotic resistance in the future.

Sleep apnea is a sleep disorder which is common in many children and adults. It is characterised by abnormal breath pauses or shallow breathing during sleep. Traditional diagnosis of apnea requires special equipment for data collection in clinical conditions and manual analysis by clinicians which is expensive and time-consuming. This paper presents a framework for autonomous detection of sleep apnea, using peripheral blood haemoglobin oxygen saturation (SpO 2 ) data based on the fusion of multiple features and Dirichlet process mixture model. The SpO 2 signals are segmented into overlapping sub-sequences and several features are extracted from each segment. The distributions of features extracted from disorder and normal segments are modelled by two Gaussian mixture models, respectively, with the Dirichlet process as the prior. The advantage of the framework is that the number of clusters within mixture models can be learned from training data without strong assumptions, which contributes to accurate estimation of the distributions. The proposed framework is subject-independent and it is trained and tested on two publicly available databases with 10-fold cross-validation. It obtains accuracy of 84.89% on the St. Vincent's University Hospital Sleep Apnea Database and accuracy of 97.01% on the Apnea-ECG Database, outperforming state-of-the-art approaches. The results show that the proposed model is capable of representing the distributions of features independently of subjects and can accurately classify segmented signals from patients with symptoms of different severity. The results show the potential of the developed classification framework to support clinicians in their decision making.

In order for inhaled corticosteroids to be delivered adequately to the airways they require patients to take them regularly using an effective technique. Patients often have a poor inhaler technique, and this has been shown to result in sub-optimal asthma control. It is important for all clinicians prescribing inhaled medication to be experienced in the correct technique, and take time to train children so that they have mastered corrected inhaler technique. Using Teach to Goal or teach back methodology is a simple and effective way to provide this in the clinic setting. More than one training session is typically needed before children can master correct inhaler technique. Adherence to inhaled therapy has been shown to be sub-optimal in pediatric populations, with studies showing an average rate of around 50%. Subjective methods of measuring adherence have been shown to be inaccurate and overestimate rates. The advent of new technology has allowed adherence rates to be measured electronically, and it has been shown that regular feedback of these data can be effective at improving asthma control. New mobile apps and smart technology aim to engage patients and families with their asthma care. Effective use of these apps in collaboration with health care professionals has a vast potential to improve adherence rates and inhaler technique, resulting in improved asthma control.

Respiratory rate (RR) is an important vital sign used in the initial and ongoing assessment of acutely ill patients. It is also used as a predictor of serious deterioration in a patient's clinical condition. Convenient electronic devices exist for measurement of pulse, blood pressure, oxygen saturation, and temperature. Although devices which measure RR exist, none has entered everyday clinical practice. We have developed a contactless portable respiratory rate monitor (CPRM) and evaluated the agreement in respiratory rate measurements between existing methods and our new device. The CPRM uses thermal anemometry to measure breath signals during inspiration and expiration. RR data were collected from 52 healthy adult volunteers using respiratory inductance plethysmography (RIP) bands (established contact method), visual counting of chest movements (established noncontact method), and the CPRM (new method), simultaneously. Two differently shaped funnel attachments to the CPRM were evaluated for each volunteer. Data showed a good agreement between measurements from the CPRM and the gold standard RIP, with intraclass correlation coefficient (ICC): 0.836, mean difference 0.46 and 95% limits of agreement of −5.90 to 6.83. When separate air inlet funnels of the CPRM were analyzed, stronger agreement was seen with an elliptical air inlet; ICC 0.908, mean difference 0.37 with 95% limits of agreement −4.35 to 5.08. A contactless device for accurately and quickly measuring respiratory rate will be an important triage tool in the clinical assessment of patients. More testing is needed to explore the reasons for outlying measurements and to evaluate in the clinical setting.

Study objectives Obstructive sleep apnea (OSA) is common in children with Down syndrome (DS) and is associated with adverse health and cognitive outcomes. Daytime clinical assessment is poorly predictive of OSA, so regular screening with sleep studies is recommended. However, sleep studies are costly and not available to all children worldwide. We aimed to evaluate the psychometric properties and predictive value of a newly developed screening questionnaire for OSA in this population. Methods 202 children aged 6 months to 6th birthday with DS were recruited, of whom 188 completed cardio-respiratory sleep studies to generate an obstructive apnea hypopnea index (OAHI). Parents completed the 14-item Down syndrome OSA screening questionnaire. Responses were screened, a factor analysis undertaken, internal consistency calculated and receiver operator characteristic (ROC) curves drawn to generate an area under the curve (AUC) to assess criterion related validity. Results Of 188 children who completed cardiorespiratory sleep studies; parents completed the screening questionnaire for 186. Of this study population 15.4% had moderate to severe OSA defined by an OAHI of ≥5/h. Sixty-three (33.9%) participants were excluded due to "unsure" responses or where questions were not answered. Using the remaining 123 questionnaires a four-factor solution was found, with the 1st factor representing breathing related symptoms, explaining a high proportion of the variance. Internal consistency was acceptable with a Cronbach alpha of 0.87. ROC curves for the total score generated an AUC statistic of 0.497 and for the breathing subscale an AUC of 0.603 for moderate to severe OSA. Conclusion A well designed questionnaire with good psychometric properties had limited predictive value to screen for moderate to severe OSA in young children with DS. The use of a screening questionnaire is not recommended. Screening for OSA in this population requires objective sleep study measures.

ObjectiveTo evaluate the success rates of home cardiorespiratory polygraphy in children under investigation for sleep-disordered breathing and parent perspectives on equipment use at home.DesignProspective observational study.SettingSheffield, Evelina London and Southampton Children’s Hospitals.PatientsData are reported for 194 research participants with Down syndrome, aged 0.5–5.9 years across the three centres and 61 clinical patients aged 0.4–19.5 years from one centre, all of whom had home cardiorespiratory polygraphy including respiratory movements, nasal pressure flow, pulse oximetry, body position and motion.Main outcome measuresPercentage of home cardiorespiratory studies successfully acquiring ≥4 hours of artefact-free data at the first attempt. Parental report of ease of use of equipment and preparedness to repeat home diagnostics in the future.Results143/194 (74%; 95% CI 67% to 79%) of research participants and 50/61 (82%; 95% CI 71% to 90%) of clinical patients had successful home cardiorespiratory polygraphy at the first attempt. Some children required multiple attempts to achieve a successful study. Overall, this equated to 1.3 studies per research participant and 1.2 studies per clinical child. The median artefact-free sleep time for successful research studies was 515 min (range 261–673) and for clinical studies 442 min (range 291–583). 84% of research and 87% of clinical parents expressed willingness to repeat home cardiorespiratory polygraphy in the future. 67% of research parents found the equipment ‘easy or okay’ to use, while 64% of clinical parents reported it as ‘easy’ or ‘very easy’.ConclusionsHome cardiorespiratory polygraphy offers an acceptable approach to the assessment of sleep-disordered breathing in children.

BackgroundThere is uncertainty about the most appropriate ways to manage non-respiratory sleep disturbances in children with neurodisabilities (NDs).ObjectiveTo assess the clinical effectiveness and safety of NHS-relevant pharmacological and non-pharmacological interventions to manage sleep disturbance in children and young people with NDs, who have non-respiratory sleep disturbance.Data sourcesSixteen databases, including The Cochrane Central Register of Controlled Trials, EMBASE and MEDLINE, were searched up to February 2017, and grey literature searches and hand-searches were conducted.Review methodsFor pharmacological interventions, only randomised controlled trials (RCTs) were included. For non-pharmacological interventions, RCTs, non-randomised controlled studies and before-and-after studies were included. Data were extracted and quality assessed by two researchers. Meta-analysis and narrative synthesis were undertaken. Data on parents’ and children’s experiences of receiving a sleep disturbance intervention were collated into themes and reported narratively.ResultsThirty-nine studies were included. Sample sizes ranged from 5 to 244 participants. Thirteen RCTs evaluated oral melatonin. Twenty-six studies (12 RCTs and 14 before-and-after studies) evaluated non-pharmacological interventions, including comprehensive parent-directed tailored (n = 9) and non-tailored (n = 8) interventions, non-comprehensive parent-directed interventions (n = 2) and other non-pharmacological interventions (n = 7). All but one study were reported as having a high or unclear risk of bias, and studies were generally poorly reported. There was a statistically significant increase in diary-reported total sleep time (TST), which was the most commonly reported outcome for melatonin compared with placebo [pooled mean difference 29.6 minutes, 95% confidence interval (CI) 6.9 to 52.4 minutes;p = 0.01]; however, statistical heterogeneity was extremely high (97%). For the single melatonin study that was rated as having a low risk of bias, the mean increase in TST was 13.2 minutes and the lower CI included the possibility of reduced sleep time (95% CI –13.3 to 39.7 minutes). There was mixed evidence about the clinical effectiveness of the non-pharmacological interventions. Sixteen studies included interventions that investigated the feasibility, acceptability and/or parent or clinician views of sleep disturbance interventions. The majority of these studies reported the ‘family experience’ of non-pharmacological interventions.LimitationsPlanned subgroup analysis was possible in only a small number of melatonin trials.ConclusionsThere is some evidence of benefit for melatonin compared with placebo, but the degree of benefit is uncertain. There are various types of non-pharmacological interventions for managing sleep disturbance; however, clinical and methodological heterogeneity, few RCTs, a lack of standardised outcome measures and risk of bias means that it is not possible to draw conclusions with regard to their effectiveness. Future work should include the development of a core outcome, further evaluation of the clinical effectiveness and cost-effectiveness of pharmacological and non-pharmacological interventions and research exploring the prevention of, and methods for identifying, sleep disturbance. Research mapping current practices and exploring families’ understanding of sleep disturbance and their experiences of obtaining help may facilitate service provision development.Study registrationThis study is registered as PROSPERO CRD42016034067.FundingThe National Institute for Health Research Health Technology Assessment programme.

Narcolepsy is a relatively common neurological condition affecting the regulation of normal sleep/wake cycles leading to excessive daytime sleepiness (EDS). It is almost certainly under-recognised as it has a prevalence of 20–50 per 100,000 population and most cases have an onset in adolescence. Cataplexy (attacks of muscle weakness often precipitated by strong emotions) is a hallmark of this condition and represents the intrusion of REM sleep into wakefulness. Narcolepsy is caused by destruction of hypocretin producing cells due to an autoimmune process often by an infective trigger. Hypocretin is found in the hypothalamus and plays a role in stabilisation of the transition between wake and sleep states. In establishing a diagnosis a comprehensive history to exclude other causes of EDS, including poor sleep habits, is essential. Primary sleep related conditions such as sleep apnoea should be excluded. Investigations for confirmation of the diagnosis include Actigraphy, Polysomnography (PSG), Multiple Sleep Latency Testing (MSLT) and CSF analysis. The symptoms of this debilitating condition can have a huge impact on a child's life and are often vastly underestimated. The impact of EDS on cognitive function is an important factor in difficulties at school, mood, quality of life and future career opportunities. Advances in understanding the pathophysiology have led to trials of novel treatment approaches. The aim of this article is to briefly summarise the recent advances in understanding and give an overview of this important condition for those who are involved in the care of a child with this disease.

A real-time thermal imaging based, non-contact respiration rate monitoring method was developed. It measured the respiration related skin surface temperature changes under the tip of the nose. Facial tracking was required as head movements caused the face to appear in different locations in the recorded images over time. The algorithm detected the tip of the nose and then, a region just under it was selected. The pixel values in this region in successive images were processed to determine respiration rate. The segmentation method, used as part of the facial tracking, was evaluated on 55,000 thermal images recorded from 14 subjects with different extent of head movements. It separated the face from image background in all images. However, in 11.7% of the images, a section of the neck was also included, but this did not cause an error in determining respiration rate. The method was further evaluated on 15 adults, against two contact respiration rate monitoring methods that tracked thoracic and abdominal movements. The three methods gave close respiration rates in 12 subjects but in 3 subjects, where there were very large head movements, the respiration rates did not match.

Introduction Non-invasive ventilation (NIV) masks that fit well are difficult to find for children who are small or have atypical facial features. Poorly fitted masks create problems e.g. discomfort, non-adherence and facial deformity. Our project aims to design and produce masks that fit well. Children’s voices are vital, but not often heard, in respiratory research projects. Aims We constructed a patient and public involvement and engagement (PPIE) program designed to: Understand the problems children and families experience with NIV and establish their wants and needs Provide an inclusive and creative environment for non–constrained thinking Get actionable feedback and ideas for improvements from a diverse patient group Method We created a method focussed on planning, innovation and participation (the PIP model). Session activities were designed to enable parents and children of all ages and abilities to participate. Examples include: Archery target activity – a method for realising the relative importance of patient’s requirement (prioritisation). Graphic scribe recording – to reflect back to the children that they had been heard/understood and stimulate creative ideas. Use of technology – making short videos to help families understand concepts. Outcomes Our priorities and design brief changed as a result of the PPIE. The graphic scribe outputs formed part of the creative process whilst providing a unique and lasting resource. We are confident that we will produce NIV interfaces that are fit for real life purpose and that people will want to trial. Key messages For respiratory research to be truly successful, PPIE should be woven throughout a project, from concept to completion. It needs to be genuine and aligned with research aims. Time and effort spent enabling participation and creatively planning for inclusivity is rewarded by generating richer and more valuable information.

Introduction Paper sleep diaries and actigraphy are useful tools in Sleep Medicine to evaluate sleep wake patterns in the home environment over the course of 7–14 days. In our service, the demand for actigraphy far outweighs the available resources. As such the team are investigating using an electronic sleep diary, integrating with actigraphy and simplifying the whole process. The first stage of this process was to ask the stakeholders their opinions of sleep diaries and actigraphy. Methods Stakeholders were asked about sleep diaries and actigraphy considering all aspects of the process from when to request a referral and organising collection, completing a diary and wearing actigraphy, through to analysing and interpreting results. Questions asked were appropriate to the different stakeholder groups involved. Families and local hospital staff were interviewed and British Paediatric Sleep Society (BPSA) members and families undergoing actigraphy were sent appropriate brief questionnaires relevant to their part of the pathway [Service evaluation SE1347]. Results 47 stakeholders provided feedback. Interviews were conducted with 14 staff, 9 parents and 3 young people. Additional questionnaires were completed by 8 families undergoing actigraphy and 13 BPSA responders. A summary of the most common responses is shown in table 1. Most institutions were issuing sleep diaries and actigraphy to specific patient groups due to limited resources. Discussion This stakeholder engagement provides useful information to improve the use of sleep diaries and actigraphy within our paediatric clinical setting. If the process can be simplified then more sleep diary and actigraphy usage can occur at the triage stage of the sleep referral process and prevent unnecessary sleep studies, benefitting both families and limited resources. Feedback received confirms the current solution for paediatric sleep diaries and actigraphy is not ideal and there is a need to identify new approaches to increase usability, acceptability and stakeholder’s satisfaction.

Introduction Many children with sleep difficulties are prescribed melatonin. However, melatonin is costly and evidence for its efficacy is limited. The aim of this study was to evaluate a nurse-led sleep clinic to look at outcomes in terms of melatonin prescribing. Methods All new referrals attending a nurse-led sleep clinic were examined from June 2016 to March 2017. Patients/parents attending the clinic were given an individualised sleep programme to implement with their child at home, with ongoing follow-up support. Families received an average of 3 face-to-face clinic visits and 3 follow-up telephone calls from first visit to discharge. Retrospective data was gathered from clinic records [Clinical Audit approval number CA1309]. Results 69 patients aged 1–17 years (31 males) were analysed. The primary complaint was ‘problem with sleep initiation and maintenance’ (81%), ‘problem with sleep initiation’ (13%) and ‘problem with sleep maintenance’ (6%). 84% of patients had medical co-morbidities; 54% of which were neuro-disability, the commonest being ASD and/or ADHD. 40/69 patients were successfully discharged from the clinic during the evaluation period. 65% were discharged without melatonin (23% with neurodisability; 42% without neurodisability), of which 35% were weaned off melatonin and 30% avoided melatonin prescriptions. 12% felt that the sleep intervention had been successful but still required melatonin, 15% were referred to medical/psychology clinics, and 8% were non-compliant with the behavioural sleep programme and were discharged still taking melatonin. Discussion In this cohort of paediatric insomnia patients, a brief but intensive behavioural programme with support from a specialist sleep nurse was effective in resolving sleep difficulties and reducing melatonin usage. We recommend that standardised sleep support from trained practitioners should be available prior to prescribing melatonin for children with sleep difficulties. This approach is not only beneficial in effectively resolving sleep problems but is cost-effective when compared with melatonin prescribing.

Introduction Narcolepsy occurs due to an inability to regulate the sleep-wake cycle, causing disturbed night-time sleep and excessive daytime sleepiness. Children and young people with narcolepsy have increased psychiatric illness, compared to healthy controls. A recent study of 31 paediatric narcolepsy patients found 43% had psychiatric comorbidity, compared with 10% in the general population.1 The aim of this service evaluation was to determine whether Sheffield Children’s Hospital narcolepsy patients have increased psychological morbidity in order to inform service development. Methods 43 patients, aged 4–16 years, and their parents were given Revised Children’s Anxiety and Depression Scale (RCADS) questionnaires in clinic [Service Evaulation SE1473]. Software produced by the developer was used to analyse the results, using data from a cohort of children and parents separated by gender and American school grade. Scores are divided into: Separation Anxiety, General Anxiety, Panic, Social Phobia, Obsessive Compulsive Disorder and Depression. Results 34 patients and parents completed their questionnaires. A positive score was defined by a T score>65, indicating clinically significant anxiety or depression. 16 patients scored positively based on their questionnaires and 25 scored positively based on their parent’s questionnaires, giving psychological morbidity rates of 47% and 74% respectively. The category scored positively in most frequently was depression: 15 patients and 23 parents’ questionnaires scored positively here. Scoring for anxiety was lower: 13 in the parent and 2 in the patient questionnaires. Discussion This service evaluation showed raised levels of anxiety and depression in Sheffield Children’s Hospital narcolepsy children and young people, compared with national averages. Regular psychological assessment and early intervention for patients would be appropriate, given the results shown here and these results will be put forward as part of a future business case. Reference Szakacs, et al. Psychiatric comorbidity and cognitive profile in children with narcolepsy with or without association to the H1N1 influenza vaccination. Sleep 2015.

Introduction Narcolepsy is a disabling neurological sleep disorder characterised by excessive daytime sleepiness and attacks of muscle weakness precipitated by strong emotions, known as cataplexy. A previous exploratory technology workshop with children from the Sheffield Children’s Hospital narcolepsy clinic identified ‘Head and neck support in the car’ as their most important unmet need in terms of aids to daily living (32/39 participants). There is currently no suitable car seat or effective support on the market for these children. Therefore, this project aimed to design, develop and evaluate concepts for a neck stabilising aid for children with narcolepsy. Methods Detailed ‘needs capture’ through a co-design workshop with children with narcolepsy and their parents to map and discuss their travel experiences resulted in an initial specification list. A second creative workshop for idea generation using existing products and early design concepts informed further development. Results A detailed design specification list has been produced. Seven concept designs have been developed for further evaluation and selection at an upcoming ‘dragon’s den’-style workshop. Concepts will not reach prototype stage within the scope of the project so worksheets and interactive design activities will be used to capture early subjective user opinions. Discussion The use of creative, co-design methods have proven effective in capturing the voices of children and families to ensure the project is generating meaningful solutions to the core issues in this area. The project is currently ongoing with a number of possible concepts being proposed and evaluated by children and families. The preliminary concepts and supporting evaluation data will be used to apply for future funding to develop the chosen concept to prototype level and beyond.

Introduction The current gold standard sensors to measure airflow in apnoea and hypopnoea detection are the oronasal thermal airflow sensor and the nasal pressure transducer, respectively.1 Due to the contact nature of these sensors in the nasal region, they are poorly tolerated by children. In a recent audit, we found that 50% of children refused the nasal pressure sensor due to its invasiveness, and of those that accepted it, 64% removed the prongs over the course of the night. The aim of the current study was to evaluate a non-contact method to monitor respiration by developing infrared thermal imaging, whereby temperature fluctuations associated with respiration are measured and correlated with airflow. Methods 11 healthy adult volunteers participated following University Ethics. Respiratory signals were recorded over four simulated apnoea scenarios resembling normal respiration; central; obstructive and hypopnoeic pauses. Simulated apnoeas were measured via nasal pressure cannula and respiratory inductance bands [SOMNOtouchTM RESP; SOMNOmedics, Germany] versus thermal imaging techniques. Results 70% of the apnoea episodes correlated with airflow sensor readings (Example trace in figure 1). In 16% of recordings the subject’s head position did not allow correct identification of the region of interest (i.e. the nostrils). For the remaining 14% of cases there was partial agreement between the thermal measurements and nasal pressure readings. Discussion These results indicate thermal imaging may be valuable as a detection tool for sleep apnoea, particularly in the case of paediatric patients whose tolerance for contact nasal sensors is poor. Reference Berry RB, Albertario CL, Harding SM, et al. for the American Academy of Sleep Medicine. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications. Version 2.5. Darien, IL: American Academy of Sleep Medicine, 2018.

Introduction Narcolepsy is a chronic neurological condition in which the brain’s ability to regulate the sleep-wake cycle is disturbed and can result in reduced quality of life (QOL). A positive association between exercise and QOL has been found in children. The aim of this study was to assess the feasibility of implementation of an exercise intervention with a mobile app to monitor outcomes in children with narcolepsy. Methods This feasibility study received service evaluation (SE1308), and University of Sheffield ethical approval (project number: 022995). A patient and parent engagement exercise to inform study design was carried out during the ‘Sheffield Children’s Hospital Annual Narcolepsy Family Day’. An exercise intervention was designed based upon NHS recommendations and tailored to the child’s activity levels.1 Weekly goals were set and progress was reviewed weekly, using an app specifically developed by Aparito Ltd (Wrexham, UK). QOL was monitored using the Pediatric Quality of Life Inventory (PedsQL). Patients were asked for feedback at the end of the intervention, to assess its feasibility and acceptability. Results 117 children and parents took part in the engagement exercise. 12 patients took part in the feasibility study (mean age 12.9 years). The focus group highlighted the importance of motivation, timing and tailoring exercise interventions to the individual. Children said exercise should be fun to help motivation. Average physical activity increased from 229 minutes to 254 minutes. Of those who had successfully increased their exercise, baseline average PedsQL score increased from 70.6 to 77.6 (figure 1). Feedback on the app and exercise intervention were both positive. Conclusion This feasibility study has shown that a targeted exercise intervention can improve QOL in children with narcolepsy. The Aparito app, it is being developed further for children with narcolepsy. Reference Health Survey for England 2015: Physical Activity for Children

Introduction Non-invasive ventilation (NIV) masks that fit well are difficult to find for children who are small or have atypical facial features. Poorly fitted masks create problems e.g. discomfort, non-adherence and facial deformity. Our project aims to design and produce masks that fit well. Children’s voices are vital, but not often heard, in respiratory research projects. Integral to the research, we aimed to construct a patient and public involvement and engagement (PPIE) program designed to: Understand the problems children and families experience with NIV and establish their wants and needs Provide an inclusive and creative environment for non– constrained thinking Get actionable feedback and ideas for improvements from a diverse patient group Method We created a method focussed on planning, innovation and participation (the PIP model). Session activities were designed to enable parents and children of all ages and abilities to participate. Examples include: Archery target activity – a method for realising the relative importance of patient’s requirement (prioritisation). Graphic scribe recording – to reflect back to the children that they had been heard/understood and stimulate creative ideas. Use of technology – making short videos to help families understand concepts. Results Our priorities and design brief changed as a result of the PPIE. The graphic scribe outputs formed part of the creative process whilst providing a unique and lasting resource. We are confident that we will produce NIV interfaces that are fit for real life purpose that people will want to trial. Discussion For respiratory research to be truly successful, PPIE should be woven throughout a project, from concept to completion. It needs to be genuine and aligned with research aims. Time and effort spent enabling participation and creatively planning for inclusivity is rewarded by generating richer and more valuable information.

Objective/Background: children with Down syndrome (DS) commonly experience difficulties with executive function (EF). They are also vulnerable to obstructive sleep apnoea (OSA). OSA is associated with EF deficits in typically developing children. A recent study reported an association between OSA and cognitive deficits in 38 school-aged children with DS. We experimentally investigated EF behaviours in young children with DS, and their association with OSA. Participants and Methods: children with DS were recruited to take part in a larger study of OSA (N=202). Parents of 80 children (50 male) aged 36 to 71 months (M = 56.90, SD = 10.19 months) completed the Behavior Rating Inventory of Executive Function - Preschool Version (BRIEF-P). Of these 80 children, 69 were also successfully studied overnight with domiciliary cardiorespiratory polygraphy to diagnose OSA. Results: obstructive apnoea/hypopnoea index was in the normal range (0-1.49/h) for 28 children but indicated OSA (≥1.5/h) in 41 children. Consistent with previous research, we found a large effect for children experiencing particular weaknesses in working memory, planning and organising, whilst emotional control was a relative strength. OSA was associated with poorer working memory (β=.23, R2=.05, p=.025), emotional control (β=.20, R2=.04, p=.047) and shifting (β=.24, R2=.06, p=.023). Conclusions: findings suggest that known EF difficulties in DS are already evident at this young age. Children with DS already have limited cognitive reserve and can ill afford additional EF deficit associated with OSA. OSA is amendable to treatment and should be actively treated in these children to promote optimal cognitive development.

Aim Many children with sleep difficulties are prescribed melatonin, a hormone playing a key role in the timing of sleep-wake cycle, however the drug is costly and evidence for its efficacy is limited. The aim of this study was to evaluate a nurse-led sleep clinic, in which a behavioural sleep programme is delivered, to look at outcomes in terms of melatonin prescribing. Methods All new referrals attending a nurse-led Sleep Clinic were examined over a 9 month period from June 2016 to March 2017. Patients attending the clinic were given an individualised sleep programme for the parent to implement with their child at home, with ongoing support from the sleep nurse. Families received an average of 3 face to face clinic visits and 3 follow up telephone calls from first visit to discharge. Retrospective data was gathered from clinic records. Melatonin prescribing data was analysed. Results 69 patients aged 1–17 years (45% male; 55% female) were analysed. The primary complaint was ‘problem with sleep initiation and maintenance’ in 81%, ‘problem with sleep initiation’ in 13% and ‘problem with sleep maintenance’ in 6%. Of the 69 patients, 84% had medical co-morbidities. 54% of which were neuro-disability, the commonest being Autistic Spectrum Disorder and/or Attention Deficit Hyperactivity Disorder. 40/69 patients were successfully discharged from the clinic during the study period. 65% were discharged without melatonin (23% with neurodisability; 42% without neurodisability), of which 35% were weaned off melatonin and 30% avoided melatonin prescriptions. 12% felt that the sleep intervention had been successful but still required melatonin, 15% were referred to medical/psychology clinics, and 8% were non-compliant with the behavioural sleep programme and were discharged still taking melatonin. Conclusion In this cohort of patients with sleep initiation and maintenance problems despite taking melatonin, a brief but intensive behavioural programme with support from a sleep nurse was effective in resolving the sleep problems. We recommend that standardised sleep support from trained practitioners should be available prior to prescribing melatonin for children suffering sleep difficulties. This approach is not only beneficial in effectively resolving sleep problems but is cost-effective when compared with melatonin prescribing costs.

Aim A partnership involving a Children’s Trust, the City Council and a Sleep Charity evaluated a behavioural intervention to provide support to parent/carers and young people to improve sleep patterns. Methods The intervention entailed basic education about sleep, a one-to-one session with a sleep practitioner to create an individual sleep programme and telephone support to empower the parent to carry out the sleep programme at home. Results 39 children completed the intervention and evaluation, median age 8.56 years (range 1.82–15.75 years). 79.5% were male. 75% had a diagnosis of ADHD or were awaiting assessment, the remaining 25% were Looked After or Adopted Children (of whom 10% also had ADHD). Parents’ ratings of their child’s ability to self-settle to sleep improved from 1.13/10 to 6.73/10 after the intervention (MD 5.62, 95% confidence intervals 4.56–6.69, p The primary word used to describe the mood of the child on wakening before the intervention was ‘grumpy’ and after the intervention was ‘happy’. The impact of sleep deprivation on the parents’ wellbeing improved for all measures. The overall WEMWBS score improved significantly following the intervention (MD 8.84, 95% CI 5.32–12.36, p Conclusion The evaluation gave us confidence in the delivery model. ‘Regular telephone calls and support’ and ‘Learning about sleep’ were the main positive factors. Our partnership working brought together the individual strengths, drive and passion that were critical for delivery, planning, and influencing better provision for families. We have established a strategic group to support local implementation and produced a draft delivery model which we believe is replicable for other areas.

Aims To compare sleep in infants and toddlers with Down syndrome (DS) to typically developing controls, including differences in snoring and sleep ecology (sleep setting and parent behaviors).MethodsParents of 104 children with DS and 489 controls aged 6-36 months completed the Brief Infant Sleep Questionnaire. We explored group differences, controlling for demographic variables.Results Parents of children with DS reported more sleep problems (45% v 19%), snoring (19% vs 2%), room-sharing (37% vs 17%), and less night-time sleep (55 mins) and total sleep over 24 hours (38 mins). They were more likely to be present when their child fell asleep (OR 4.40). Snoring increased night waking but did not limit night-time/24-hour sleep. However, parental presence was associated with 55 minutes less night-time and 64 minutes less 24-hour sleep. After controlling for snoring and parental presence, children with DS slept less at night (38 mins) but more in the day (21 mins) with no significant difference in 24-hour sleep. ConclusionsOverall, significant differences in sleep patterns, problems, and ecology were found between children with DS and controls. Parental presence at settling, not snoring, explained most differences, including over an hour’s less 24-hour sleep. Early intervention programmes that promote self-soothing skills could prevent the burden of sleep loss in young children with DS.

AimsWe describe how patient and public involvement (PPI) was integrated into the design of an intervention for a randomised controlled trial (RCT) based within primary care. The RCT, known as the PLEASANT trial, aimed to reduce unscheduled medical contacts in children with asthma associated with start of the new school year in September with a simple postal intervention, highlighting the importance of maintaining asthma medication for helping to prevent increased asthma exacerbations.BackgroundPPI is a key feature of UK health research policy, and is often a requirement of funding from the National Institute for Health Research. There are few detailed accounts of PPI in the design and conduct of clinical trials in the PPI literature for researchers to learn from.MethodsWe held PPI consultation events to determine whether the proposed intervention for the trial was acceptable to children with asthma and their parents, and to ascertain whether enhancements should be made. Two PPI consultation events were held with children with asthma and their parents, prior to the research commencing. Detailed field notes were taken by the research team at each consultation event.FindingsAt the first consultation event, parents and children endorsed the trial’s rationale, made suggestions to the wording of the trial intervention letter, and made recommendations about to whom the letter should be sent out. At the second consultation event, parents discussed the timing of the intervention, commented on the lay summary of the Research Ethics Application, and were invited to join the trial’s steering committee, while the children selected a logo for the study. PPI has resulted in enhancements to the PLEASANT study’s intervention. A further PPI consultation event is scheduled for the end of the trial, in order for children with asthma and their parents to contribute to the trial’s dissemination strategy.

Background: Respiratory rate is a vital physiological measurement used in the immediate assessment of unwell children and adults. Convenient electronic devices exist for the measurement of pulse, blood pressure, oxygen saturation, and temperature. Although devices which measure respiratory rate exist, none have entered everyday clinical practice for acute assessment of children and adults. An accurate and practical device which has no physical contact with the patient is important to ensure readings are not affected by distress caused by the assessment method. Objective: The aim of this study was to evaluate the use of a thermal imaging method to monitor the respiratory rate in children and adults. Methods: Facial thermal images of adult volunteers and children undergoing elective polysomnography were included. Respiration was recorded for at least 2 min with the camera positioned 1 m from the subject’s face. Values obtained using the thermal imaging camera were compared with those obtained from contact methods such as the nasal thermistor, respiratory inductance plethysmography, nasal airflow, and end tidal CO2. Results: A total of 61 subjects, including 41 adults (age range 27–46 years) and 20 children (age range 0.5–18 years) were enrolled. The correlation between the respiratory rate measured using thermal imaging and the contact method was r = 0.94. Sequential refinements to the thermal imaging algorithms resulted in the ability to perform real-time measurements and an improvement of the correlation to r = 0.995. Conclusion: This exploratory study shows that thermal imaging-derived respiratory rates in children and adults correlate closely with the best performing standard method. With further refinements, this method could be implemented in both acute and chronic care in children and adults.

We read with interest the article by Leong et al on the use of polysomnography (PSG) in children,1 covering indications for PSG, along with limitations of oximetry, and clearly outlining how to undertake and interpret PSG in paediatric patients. It briefly discusses limited channel recordings (respiratory polygraphy, RP) and concludes that this ‘is not standard practice’. In many paediatric centres RP is standard practice, and routinely used for assessment of sleep-disordered breathing (SDB) in children, with the most common diagnosis being obstructive sleep apnoea (OSA). In a recent survey …