Delayed sleep wake phase disorder in adolescents: an updated review.

PURPOSE OF REVIEW: This review examines the most common circadian rhythm disorder in adolescents, delayed sleep phase disorder. It explores the etiology, prevalence, clinical features, diagnostic tools and criteria, and treatment options to identify sleep disorders early in the course. This is important to help improve youths in terms of education and quality of life. RECENT FINDINGS: Recent studies indicate that delayed sleep wake phase disorder has a range of prevalence between 1% and 16%. It is often associated with neurodevelopmental disorders (i.e. attention deficit hyperactivity disorder and autism spectrum disorder) as well as psychopathology (i.e. substance use, anxiety, and depression). It can present with a myriad of symptoms, such as insomnia, restless sleep, and poor daytime cognitive function, often seen in pediatric practice. Important diagnostic measures incorporate history-taking, sleep logs, actigraphy (i.e. Apple watches) and measurement of dim light melatonin onset. Treatments include improved sleep hygiene, chronotherapy, exogenous melatonin administration, and bright light therapy. SUMMARY: There are many environmental and genetic factors that can predispose an individual to circadian rhythm disorders. Delayed sleep phase disorder has detrimental effects on overall health, cognition, and behavior. It is important to screen for this disorder in routine pediatric clinic visits. The goal of early intervention is to prevent health and behavioral complications and treat adolescents using a multimodal approach, especially those with affective/neurodevelopmental conditions, who are prone to having delayed sleep wake phase disorder.

Something to consider: rapid palatal expansion for treatment of obstructive sleep apnea in pediatric patients.

PURPOSE OF REVIEW: This review examines the potential of rapid palatal expansion (RPE) as a treatment for pediatric obstructive sleep apnea (OSA). The focus is on recent findings related to its efficacy, safety, patient selection, timing, appliance options, cost considerations, and long-term outcomes. RECENT FINDINGS: Recent studies indicate that RPE can lead to significant improvements in pediatric OSA, with a 70% reduction in the Apnea Hypopnea Index (AHI) and increased oxygen saturation levels. It has been particularly effective in children with small or absent tonsils and has been found to reduce adenoid and tonsil size. Long-term follow-up studies suggest the need for ongoing monitoring, as some patients may experience relapse over time. SUMMARY: RPE shows promise as an additional treatment for pediatric obstructive sleep apnea. It offers improvements in respiratory function and reduced symptoms in certain patients. However, long-term efficacy and safety require further investigation. Comparative studies and patient-reported outcomes are necessary to optimize treatment approaches. Collaboration between orthodontists, sleep specialists, and ear-nose-throat (ENT) specialists may be essential for optimal outcomes in pediatric OSA patients treated with RPE.

Obstructive lung disease is common in children with syndromic and congenital scoliosis: a preliminary study.

BACKGROUND: It is well known that restrictive lung disease (RLD) is associated with scoliosis. This study identifies that obstructive lung disease (OLD) is associated with syndromic scoliosis and congenital scoliosis. METHODS: We searched a local database for patients with scoliosis who underwent a pulmonary function testing (PFT) from 2004 to 2009. All patients with congenital scoliosis or syndromic thoracolumbar scoliosis with a Cobb angle of ≥40 degrees and acceptable and repeatable PFT testing were included in the study. OLD was defined as an forced expiratory volume in the first second/forced vital capacity ratio below 95% confidence interval. Bronchoscopy videos and computed tomography scans or magnetic resonance images were reviewed to identify anatomic causes of lower airway disease. RESULTS: A total of 18 patients met the criteria for inclusion. The median age at diagnosis was 11.3 years. The median primary Cobb angle was 60 degrees. The prevalence of OLD was 33% and RLD was 57%. The 6 children with OLD underwent preoperative bronchoscopy and chest computed tomography or magnetic resonance imaging to identify anatomic causes of lower airway obstruction. The 4 children with OLD and right-sided major thoracic curves had compression of the right mainstem bronchus between the spine (posterior) and the right pulmonary artery (anterior). The 2 children with OLD and left-sided major thoracic curves had compression of the left mainstem bronchus between the spine (posterior) and the descending aorta (anterior) or the left atrium (anterior). CONCLUSIONS: In our study, the prevalence of OLD in children with congenital scoliosis or syndromic scoliosis was 33%, which was elevated when compared with the population prevalence of 2% to 5%. Mainstem airway compression from spine rotation was discovered to be the potential mechanism of disease. LEVEL OF EVIDENCE: Level IV, prognostic study investigating the effect of a patient characteristic on the outcome of disease.

Sleep-related respiratory abnormalities and arousal pattern in achondroplasia during early infancy.

OBJECTIVE: To assess sleep-disordered breathing (SDB), sleep architecture, and arousal pattern in infants with achondroplasia and to evaluate the relationship between foramen magnum size and the severity of SDB. STUDY DESIGN: A retrospective review of polysomnographic recordings and medical records was performed in infants with achondroplasia and in aged-matched control subjects. All studies were re-scored with the emphasis on respiratory events, sleep state, and arousals. In addition, the neuroimaging study of the brain (magnetic resonance imaging) was reviewed to evaluate foramen magnum diameters and to assess their relationship to SDB. RESULTS: Twenty-four infants met the criteria for entry into analysis, 12 infants with achondroplasia (A) and 12 control infants (C). There was no significant difference in age or sex. Infants with achondroplasia had a significant increase in total respiratory disturbance index (RDI; A, 13.9 +/- 10.8 versus C, 2.0 +/- 0.9; P < .05). However, there was no significant difference in percentages of active sleep, quiet sleep, or sleep efficiency. Analysis of arousals demonstrated that infants with achondroplasia had a significant decrease in both spontaneous arousal index (A, 10.5 +/- 3.5/hr versus C, 18.6 +/- 2.7; P < .0001) and respiratory arousals (A, 10.3% +/- 6.3% versus C, 27.5 +/- 9.5%; P < .0001). Evaluation of foramen magnum dimensions demonstrated smaller foramen magnum size, but there were no significant correlations between anteroposterior or transverse diameters and RDI. CONCLUSION: Infants with achondroplasia have significant SDB during early infancy. SDB in infants with achondroplasia is not associated with alteration in sleep architecture, possibly because of attenuation of the arousal response. We speculate that the concomitant increased apneic events and decreased arousal response will lead to vulnerability in these infants and may underlie the pathophysiologic mechanism of sudden unexpected death in this population.

A review of the effects of sleep during the first year of life on cognitive, psychomotor, and temperament development.

During the first year of life, infants spend most of their time in the sleeping state. Assessment of sleep during infancy presents an opportunity to study the impact of sleep on the maturation of the central nervous system (CNS), overall functioning, and future cognitive, psychomotor, and temperament development. To assess what is currently known regarding sleep during infancy and its effects on cognitive, psychomotor, and temperament development, we assessed the relevant literature published over the last several decades. To provide a foundation for a more in-depth understanding of this literature, we preface this with an overview of brain maturation, sleep development, and various assessment tools of both sleep and development during this unique period. At present, we do not have sufficient data to conclude that a causal relationship exists between infant sleep and cognitive, psychomotor, and temperament development. Caution should be used in predicting outcomes, as the timing and subjectivity of evaluations may obviate accurate assessment. Collectively, studies assess a wide array of sleep measures, and findings from one developmental period cannot be generalized readily to other developmental periods. Future studies should follow patients longitudinally. Additionally, refinements of existing assessment tools would be useful. In view of the relatively high reported pediatric prevalence of cognitive and behavioral deficits that carry significant long-term costs to individuals and society, early screening of sleep-related issues may be a useful tool to guide targeted prevention and early intervention.

The impact of adenotonsillectomy on pediatric asthma.

OBJECTIVE: To determine asthma outcomes in children undergoing adenotonsillectomy (T&A) for treatment of sleep-disordered breathing (SDB). HYPOTHESIS: Asthmatic children will demonstrate improvement in asthma control after T&A compared to asthmatic children not undergoing surgical treatment. STUDY DESIGN: Prospective cohort. PATIENT-SUBJECT SELECTION: 80 children with diagnosed asthma, aged 4-11, undergoing T&A and 62 controls matched to the T&A subjects by age, sex, and asthma severity classification. METHODOLOGY: Parents and children completed the Childhood Asthma Control Test (C-ACT) and the Pediatric Sleep Questionnaire (PSQ). Parents were queried regarding the number of asthma exacerbations, the frequency of the use of systemic steroids, the number of emergency room visits and the number of hospitalizations in the prior 6 months. The identical questionnaires and interviews were completed 6 months after entry. RESULTS: The adjusted mean (95% CI) C-ACT score was 21.86 (20.94-22.68) at entry and 25.15 (24.55-25.71) at follow-up for the T&A group compared with 22.42 (21.46-23.28) and 23.59 (22.77-24.33) for the control group. There was a significant group by time interaction (P < 0.001). Simple effects analysis showed that group means did not differ at entry (P = 1.00) but did differ at follow-up (P = 0.006). Baseline PSQ was a significant predictor of improvement in C-ACT scores. Statistical modeling did not demonstrate significant group by time interactions for any of the asthma clinical outcomes, although these outcomes were very infrequent in both groups. CONCLUSION: Treatment of SDB improves asthma outcomes as measured by the C-ACT.

Prevention of tracheostomy-related pressure ulcers in children.

BACKGROUND AND OBJECTIVES: Pressure ulcers are commonly acquired in pediatric institutions, and they are a key indicator of the standard and effectiveness of care. We recognized a high rate of tracheostomy-related pressure ulcers (TRPUs) in our ventilator unit and instituted a quality improvement program to develop and test potential interventions for TRPU prevention, condensed them into a clinical bundle, and then implemented the bundle into our standard practice. METHODS: The intervention model used a rapid-cycle, Plan-Do-Study-Act (PDSA), framework for improvement research. All tracheostomy-dependent patients admitted to our 18-bed ventilator unit from July 2008 through December 2010 were included. TRPU stage and description, number of days each TRPU persisted, and bundle compliance were recorded in real time. All TRPUs were staged by a wound-care expert within 24 hours. The interventions incorporated into the TRPU-prevention bundle included frequent skin and device assessments, moisture-reducing device interface, and pressure-free device interface. RESULTS: There was a significant decrease in the rate of patients who developed a TRPU from 8.1% during the preintervention period, to 2.6% during bundle development, to 0.3% after bundle implementation. There was a marked difference between standard and extended tracheostomy tubes in TRPU occurrence (3.4% vs 0%, P = .007) and days affected by a TRPU (5.2% vs 0.1%, P < .0001). CONCLUSIONS: Education and ongoing assessment of skin integrity and the use of devices that minimize pressure at the tracheostomy-skin interface effectively reduce TRPU even among a population of children at high risk. These interventions can be integrated into daily workflow and result in sustained effect.

Improving follow-up in hospitalised children.

OBJECTIVE: To improve the clinic follow-up rate of paediatric inpatients in a tertiary care hospital. PATIENTS AND METHODS: Inpatients who received pulmonary consultations from July 2007 to June 2008 at Cincinnati Children's Hospital Medical Center were eligible for this quality-improvement project. Multiple interventions were introduced to improve follow-up in our subspecialty clinic. A χ(2) test for association was used to assess the dependence between the clinic follow-up rate and the type of care coordination intervention. We hypothesised that generalisable care coordination interventions would result in improvements. RESULTS: Two hundred and eleven patients were included. Two interventions were independently associated with improvements in the hospital follow-up rate. When a reminder to follow-up in the pulmonary clinic was inserted into the hospital discharge summary (partial intervention), the clinic follow-up rate improved from 27% to 55%. When the follow-up appointments were made for the families, with the appointments' noted in the discharge summaries, and automated appointment reminder phone calls were provided (full intervention), the follow-up rate improved further from 55% to 80%. The full intervention, when compared with no intervention, improved the clinic follow-up rate from 27% to 80%. CONCLUSION: Establishing clinic appointments for patients and providing appointment reminders increases the hospital follow-up rate for hospitalised children in outpatient clinics.

Improving evidence-based care in cystic fibrosis through quality improvement.

OBJECTIVE: To increase clinician adherence to prescribing guidelines for pulmonary medications in children with cystic fibrosis (CF). DESIGN: Quality improvement project with multiple time series design. SETTING: The CF center at a tertiary care pediatric hospital in the United States. PATIENTS: Children with CF who were eligible to receive oral azithromycin, nebulized dornase alfa, or inhaled tobramycin sulfate based on prescribing guidelines for CF lung disease. INTERVENTION: Evidence-based prescribing guidelines were designed by a local committee to reflect consensus recommendations from the CF Foundation. Clinicians and families were educated about guidelines. Adherence to prescribing guidelines was tracked using a local CF database and record reviews. Weekly meetings were used to highlight adherence failures and promote clinician accountability. MAIN OUTCOME MEASURE: The rate of clinician adherence to prescribing guidelines. RESULTS: One hundred seventy patients with CF were included. At the start of the project, the rate of clinician adherence to prescribing guidelines was 62%. After 3 months of the project, the rate of clinician adherence to prescribing guidelines was 87% (odds ratio = 4.6; 95% confidence interval, 3.0-7.0). The improvements in adherence to prescribing guidelines were sustained for 21 months of follow-up. CONCLUSIONS: Educating clinicians about prescribing guidelines, sharing guidelines with families, and monitoring clinician adherence improve prescribing adherence to evidence-based recommendations.

Telemetric recording of intrapleural pressure.

BACKGROUND: Monitoring of intrapleural pressure (IPP) is used for evaluation of lung function in a number of pathophysiological conditions. We describe a telemetric method of non-invasive monitoring of the IPP in conscious animals intermittently or continuously for a prolonged period of time. MATERIALS AND METHODS: After IACUC approval, six mongrel dogs were used for the study. After sedation, each dog was intubated and anesthetized using 0.5% Isoflurane. A telemetric implant model TL11M2-D70-PCT from Data Science International was secured subcutaneously. The pressure sensor tip of the catheter from the implant was inserted into the pleural space, and the catheter was secured with sutures. The IPP signals were recorded at a sampling rate of 100 points/second for 30 to 60 min daily for 4 days. From these recordings, the total mean negative IPP (mmHg), and the total mean negative IPP for a standard time of 30 min were calculated. In addition, the actual inspiratory and expiratory pressures were also measured from stable recording of the IPP waveforms. RESULTS: In six dogs, the total mean +/- SD negative IPP was -10.8 +/- 10.6 mmHg. After normalizing with respect to acquisition time it was -13.2 +/- 11.2 mmHg/min. The actual inspiratory pressure was -19.7 +/- 15.3, and the expiratory pressure was -11.0 +/- 12.9. CONCLUSIONS: Our study demonstrates that telemetric monitoring of IPP can be performed reliably and non-invasively in conscious experimental animals. The values for IPP in our study are compatible with the results of other investigators who used different methods of IPP measurement. Further work may show this method to be helpful in understanding the pathophysiology of various breathing disorders.