Pulmonary hypertension and chronic hypoventilation in ROHHAD syndrome treated with average-volume assured pressure support.

INTRODUCTION: Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with challenging-to-treat hypoventilation. CASE PRESENTATION: An 11-year-old morbidly obese Chinese female presented with a putative diagnosis of ROHHAD associated with a left psoas ganglioneuroma. Initial polysomnography showed severe obstructive sleep apnea and hypoventilation. She was not adherent to prescribed non-invasive positive pressure ventilation (NIPPV). Echocardiography demonstrated evidence of pulmonary hypertension, likely secondary to chronic hypoventilation. With behavioral modification and trial of average volume-assured pressure support (AVAPS), adherence improved with eventual improvement of her pulmonary hypertension. CONCLUSION: AVAPS may improve ventilation and NIPPV adherence in central hypoventilation disorders such as ROHHAD, reducing risk of morbidity and mortality

Kleine-Levin Syndrome.

Kleine-Levin syndrome (KLS) is a rare disorder characterized by discrete episodes of hypersomnia associated with cognitive and behavioural abnormalities, as well as normal alertness and function between episodes. The prevalence of KLS may be underestimated as it is often misdiagnosed and managed as another sleep disorder, neurological disorder or psychiatric condition. KLS is more typically seen in adolescence than at other ages, and is more common in males than in females. There are currently neither standard biomarkers nor specific imaging study findings, making the diagnosis of KLS a challenge. Furthermore, there are no consistently effective therapies. The prognosis, however, is felt to be overall favorable, as episodes become progressively milder and less frequent before resolving entirely in most patients

Pediatric pulmonary year in review 2021: Sleep medicine.

Pediatric pulmonology publishes original research, review articles, and case reports on a wide variety of pediatric respiratory disorders. In this article, we summarized the past year\u27s publications in sleep medicine and reviewed selected literature from other journals in this field. We focused on original research articles exploring aspects of sleep-disordered breathing in patients with underlying conditions such as cystic fibrosis, asthma, and sickle cell disease. We also explored sleep-disordered breathing risk factors, monitoring, diagnosis, and treatment; and included recent recommendations for drug-induced sleep endoscopy and ways to monitor and improve PAP adherence remotely

Bronchoscopic and nonbronchoscopic methods of airway culturing in tracheostomized children.

INTRODUCTION: Distal airway secretions can be sampled by bronchoscopic bronchoalveolar lavage (B-BAL), blind protected BAL (BP-BAL), and tracheal aspiration (TA). We quantitatively compared the cultures of distal airway secretions from BP-BAL, B-BAL, and TA specimens, and assessed the efficacy of the three above methods in diagnosing bronchitis in tracheostomized children. METHODS: Twenty children with tracheostomies underwent BP-BAL, B-BAL, and TA. Samples were sent for quantitative bacterial cultures. The diagnosis of bronchitis was made based on a validated visual grading system as well as on positive quantitative cultures from the BAL fluid. Diagnostic agreement between cultures obtained by the three methods and the visual grading scores was determined by kappa statistics. RESULTS: The diagnosis of bronchitis by visual grading score had substantial agreement with BP-BAL, moderate agreement with B-BAL, and fair agreement with TA results. BP-BAL specimens had significantly lower pathogenic colonies (P \u3c .05) than either B-BAL or TA specimens. CONCLUSIONS: BP-BAL allows for more accurate sampling of lower airway secretions in tracheostomized children and is more accurate in the diagnosis of bronchitis in this group

A social media intervention for the families of young Black men with obstructive sleep apnoea.

OBJECTIVE: To address positive airway pressure (PAP) adherence in adolescents diagnosed with obstructive sleep apnoea (OSA) by pilot testing a novel, online, facilitated, peer-support and health education programme for families. DESIGN SETTING AND METHODS: Families participated in separate Facebook peer-groups (adolescent [n=6] and parent [n=6]) for four weeks, followed by face-to-face interviews. Participants received OSA and PAP educational videos and posts, engaged with questions and polls, and viewed de-identified postings of peer PAP use data. RESULTS: Adolescent participants were young Black males aged 13-17 years (n=6) with obesity (n=5), severe sleep apnoea (100%) and 4-15 months of prior PAP use. Parent participants were mothers (n=4) and fathers (n=2). Four of six young males increased their mean PAP use during the intervention period. Overall, parents were more engaged with the Facebook group page than adolescents, but interviews revealed the online group/peer-support and education provided was highly regarded and appreciated by families. Parents were particularly appreciative of being involved in care and diagnosis in this way. CONCLUSION: Results of this pilot trial provide important data regarding intervention design, content, and delivery approaches to be considered in the development of future interventions aiming to engage families and improve adolescent PAP adherence

Positive airway pressure adherence in pediatric obstructive sleep apnea: A systematic scoping review.

Positive airway pressure (PAP) therapy is a commonly prescribed treatment for pediatric obstructive sleep apnea (OSA). Negative health consequences associated with untreated OSA make understanding the utilization of PAP therapy imperative. The aim of this review was to describe PAP use in children and adolescents with OSA, explore factors that influence use, and describe published scientific or clinical approaches to improve use. Among 20 studies, average PAP adherence was 56.9% (range, 24-87%). PAP use averaged 4.0 h (SD = 3.1) to 5.2 h (SD = 3.4) per night. Cautious consideration of summary estimates of PAP use is necessary as studies were heterogeneous and adherence definitions widely varied across studies. Age, sex, and developmental delay were the only factors associated with PAP use in more than one study. The majority of approaches to improve use were program evaluations rather than scientifically tested interventions. This review identified critical gaps in the existing literature and sets forth a research agenda for the future

The role of polysomnography in tracheostomy decannulation of children with bronchopulmonary dysplasia

BACKGROUND: Patients with bronchopulmonary dysplasia (BPD) may require tracheostomy for long-term mechanical ventilation. Polysomnography (PSG) may predict successful decannulation in children, however it is unclear how this success compares with children without a PSG. To better evaluate this role, we compared decannulation outcomes between tracheostomy-dependent children with BPD who underwent PSG before decannulation to those who did not. METHODS: This is a retrospective cohort study between 1 January 2007 and 1 June 2017 of tracheostomy-dependent children with BPD who were clinically considered for decannulation. Patient demographics, PSG results, and medical comorbidities were abstracted from medical records and compared between groups. Decannulation outcomes were compared between children with BPD who underwent PSG before decannulation and those who did not. RESULTS: One hundred twenty-five patients with BPD were considered for tracheostomy decannulation. Forty-six (37%) had a pre-decannulation PSG while 79 (63%) did not. Nineteen (41%) patients did not undergo decannulation within 6 months of the PSG. One (3%) patient with pre-decannulation PSG failed decannulation. Four (5%) patients without pre-decannulation PSG failed decannulation. Nineteen patients with PSG and no decannulation had significantly higher obstructive apnea-hypopnea index (OAHI) (13.62 vs 2.68 events per hour, P = 0.004), higher end-tidal CO CONCLUSIONS: While successful decannulation can be achieved without a PSG in some patients, PSG is a valuable tool to identify BPD patients undergoing clinical evaluation for decannulation who would benefit from treatment of OSA before decannulation

Age and weight considerations for the use of continuous positive airway pressure therapy in pediatric populations: an American Academy of Sleep Medicine position statement.

UNLABELLED: This position statement provides guidance for age and weight considerations for using continuous positive airway pressure therapy in pediatric populations. The American Academy of Sleep Medicine commissioned a task force of experts in pediatric sleep medicine to review the medical literature and develop a position statement based on a thorough review of these studies and their clinical expertise. The American Academy of Sleep Medicine Board of Directors approved the final position statement. It is the position of the American Academy of Sleep Medicine that continuous positive airway pressure can be safe and effective for the treatment of obstructive sleep apnea for pediatric patients, even in children of younger ages and lower weights, when managed by a clinician with expertise in evaluating and treating pediatric obstructive sleep apnea. The clinician must make the ultimate judgment regarding any specific care in light of the individual circumstances presented by the patient, accessible treatment options, patient/parental preference, and resources. CITATION: Amos L, Afolabi-Brown O, Gault D, et al. Age and weight considerations for the use of continuous positive airway pressure therapy in pediatric populations: an American Academy of Sleep Medicine position statement