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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.

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.

Objective Children with Down syndrome are at high risk of obstructive sleep apnoea (OSA) and screening is recommended. Diagnosis of OSA should be confirmed with multichannel sleep studies. We aimed to determine whether home pulse oximetry (HPO) discriminates children at high risk of OSA, who need further diagnostic multichannel sleep studies.Design Cross-sectional prospective study in a training sample recruited through three UK centres. Validation sample used single-centre retrospective analysis of clinical data.Patients Children with Down syndrome aged 0.5–6 years.Intervention Diagnostic multichannel sleep study and HPO.Main outcome measures Sensitivity and specificity of HPO to predict moderate-to-severe OSA.Results 161/202 children with Down syndrome met quality criteria for inclusion and 25 had OSA. In this training sample, the best HPO parameter predictors of OSA were the delta 12 s index >0.555 (sensitivity 92%, specificity 65%) and 3% oxyhaemoglobin (SpO2) desaturation index (3% ODI)>6.15 dips/hour (sensitivity 92%, specificity 63%). Combining variables (delta 12 s index, 3% ODI, mean and minimum SpO2) achieved sensitivity of 96% but reduced specificity to 52%. All predictors retained or improved sensitivity in a clinical validation sample of 50 children with variable loss of specificity, best overall was the delta 12 s index, a measure of baseline SpO2 variability (sensitivity 92%; specificity 63%). Conclusions HPO screening could halve the number of children with Down syndrome needing multichannel sleep studies and reduce the burden on children, families and health services alike. This approach offers a practical universal screening approach for OSA in Down syndrome that is accessible to the non-specialist paediatrician.

Objective: To investigate the validity and reliability of computerised acoustic analysis in the detection of abnormal respiratory noises in infants. Methods: Blinded, prospective comparison of acoustic analysis with stethoscope examination. Validity and reliability of acoustic analysis were assessed by calculating the degree of observer agreement using the κ statistic with 95% confidence intervals (CI). Results: 102 infants under 18 months were recruited. Convergent validity for agreement between stethoscope examination and acoustic analysis was poor for wheeze (κ = 0.07 (95% CI, −0.13 to 0.26)) and rattles (κ = 0.11 (−0.05 to 0.27)) and fair for crackles (κ = 0.36 (0.18 to 0.54)). Both the stethoscope and acoustic analysis distinguished well between sounds (discriminant validity). Agreement between observers for the presence of wheeze was poor for both stethoscope examination and acoustic analysis. Agreement for rattles was moderate for the stethoscope but poor for acoustic analysis. Agreement for crackles was moderate using both techniques. Within-observer reliability for all sounds using acoustic analysis was moderate to good. Conclusions: The stethoscope is unreliable for assessing respiratory sounds in infants. This has important implications for its use as a diagnostic tool for lung disorders in infants, and confirms that it cannot be used as a gold standard. Because of the unreliability of the stethoscope, the validity of acoustic analysis could not be demonstrated, although it could discriminate between sounds well and showed good within-observer reliability. For acoustic analysis, targeted training and the development of computerised pattern recognition systems may improve reliability so that it can be used in clinical practice.

Adherence to inhaled steroids is suboptimal in many children with asthma and can lead to poor disease control. Many previous studies in paediatric populations have used subjective and inaccurate adherence measurements, reducing their validity. Adherence studies now often use objective electronic monitoring, which can give us an accurate indication of the extent of non-adherence in children with asthma. A review of the studies using electronic adherence monitoring shows that half of them report mean adherence rates of 50% or below, and the majority report rates below 75%. Reasons for non-adherence are both intentional and non-intentional, incorporating illness perceptions, medication beliefs and practical adherence barriers. Interventions to improve adherence in the paediatric population have had limited success, with the most effective containing both educational and behavioural aspects.

Background Respiratory rate (RR) is an important vital sign used in the initial and ongoing assessment of unwell children. It is used in Paediatric Early Warning Scores to assess a child’s clinical status and as a predictor of serious deterioration. Convenient electronic devices exist for the measurement of pulse, blood pressure, oxygen saturation and temperature that provide accurate and prompt measures of vital signs. Devices for monitoring RR have entered the commercial market but there is no device currently available that gives an accurate and rapid assessment of RR in clinical practice. Aim We have developed a contactless portable respiratory rate monitor (CPRM) and aimed to measure agreement with existing methods of RR measurement. Method Respiratory rate data were collected from 30 children undergoing polysomnography sleep studies at a tertiary children’s hospital. Measurements from respiratory inductance plethysmography (RIP) bands (established contact method), visual counting of chest movements (established non-contact method) and the CPRM (developed device) were all obtained simultaneously and compared. Two to three data sets were collected for each child. A total of 61 recordings were obtained from children ranging from 8 months to 15 years. Results Data showed substantial agreement between measurements from the CPRM and the gold standard RIP (Interclass correlation coefficient 0.762: 95% CI 0.633–0.850). When two patients with significantly dysfunctional breathing were removed from the analysis, the CPRM showed a much higher correlation with the gold standard RIP method (Interclass correlation coefficient 0.981: 95% CI 0.968- 0.989) with 95% limits of agreement –2.49–0.77 breaths/min (Figure 1). Conclusion A portable contactless device developed in this study can accurately and quickly measure respiratory rate. Such a device will be an important tool in the initial assessment of unwell children. There is currently no such device on the market. More testing is needed to explore outlying measurements and to evaluate in different clinical settings. Further development and modification of the device and software are ongoing.

Computerised breath sounds analysis was used to assess the response of infants with wheeze and ruttles to the drug ipratropium bromide. A mean decrease in breath sounds intensity occurred in infants with ruttles after five minutes (20.5 dB), but not until 20 minutes in those with wheeze (8.1 dB). This differential response may be related to different underlying pathophysiology.