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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.

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 …

ObjectiveDespite the success of behavioural sleep support interventions in the third sector, sleep support is not universally available for families in the UK. The aim of the study was to provide evidence of efficacy and to propose a delivery model for integrated sleep support for families of vulnerable children.Design and settingA sleep support intervention was carried out in Sheffield Local Authority evaluated using a preintervention and postintervention study design by Sheffield Children’s National Health Service (NHS) Trust.ParticipantsFifty-six children aged 6–16 years with significant sleep problems were recruited; 39 completed the intervention and evaluation.InterventionsBasic sleep education and an individualised programme was delivered by a sleep practitioner. Follow-on telephone support was provided to empower the parent (and/or young person) to carry out the sleep programme at home. An integrated NHS and Local Authority delivery model was designed and implemented.ResultsParents’ ratings of their child’s ability to self-settle improved from 1.1/10 to 6.4/10 (pConclusionsThe behavioural approach to sleep support for these vulnerable groups of children is highly effective. Follow-on individual support to empower parents is key to achieving success. Sleep support can be implemented in NHS and Local Authority services by integration into the existing workforce using a cross-agency model.

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.

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.

BACKGROUND—Exhaled nitric oxide levels are low in patients with cystic fibrosis (CF), despite the chronic inflammation present in the airways. This study aimed to determine whether levels of exhaled nitric oxide were reduced prior to the onset of respiratory symptoms in infants with CF. METHODS—The levels of exhaled nitric oxide were measured using a chemiluminescence analyser in five infants with CF and 11 healthy control subjects, both groups having a mean age of 48.6days. RESULTS—Mean levels of exhaled nitric oxide were significantly lower in infants with CF than in the control group (4.9 ppb v 12.1ppb; p=0.01). CONCLUSIONS—This finding may be the key to understanding the inflammatory processes in early cystic fibrosis and may lead to novel treatment approaches.