Updates in Pediatric Sickle Cell Lung Disease.

Sickle cell lung disease presents a challenging care paradigm involving acute and chronic lower airway disease, sleep-disordered breathing, pulmonary vascular disease, and modification by environmental factors. Understanding the presentation, pathophysiology, and diagnostic approaches is essential for accurate identification and management. While significant progress has been made, there remains a need for research to develop effective treatments and interventions to decrease disease burden in these children.

The impact of obstructive sleep apnea in children with sickle cell disease and asthma.

Introduction: Asthma and obstructive sleep apnea (OSA) are chronic diseases that disproportionately affect children with sickle cell disease (SCD). The literature describes the negative impact that both conditions have on children with SCD separately; however, the effect of OSA on asthmatic children with OSA is less specific. We hypothesized that the presence of OSA in children with SCD and asthma is associated with specific hematologic markers, worse clinical outcomes, and greater healthcare utilization.

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.

Continuous positive airway pressure care for pediatric obstructive sleep apnea: A long-term quality improvement initiative.

Successful treatment of pediatric obstructive sleep apnea syndrome (OSAS) with continuous positive airway pressure (CPAP) is challenging due to behavioral, technical, medical, and systems factors. We undertook a quality improvement (QI) initiative involving physicians, nurses, psychologists, and respiratory therapists to improve CPAP outpatient care and processes. We aimed to: (1) increase the proportion of patients with a follow-up visit within 4 months of initiation of CPAP, (2) reduce the median time to first follow-up visit to under 4 months, and (3) increase the proportion of patients obtaining a post-initiation polysomnogram within 1 year to >50%.

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's 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.

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.

Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma.

Objective: Asthma is a recognized comorbidity in children with sickle cell disease (SCD). It increases the risk of acute chest syndrome (ACS), vaso-occlusive episodes, and early mortality. We aim to determine whether evaluation and management of children with SCD and asthma by a pulmonologist reduce rate of asthma exacerbation and ACS.

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.

Updates on management of pediatric obstructive sleep apnea.

Obstructive sleep apnea (OSA) affects about 1%-5% of the pediatric population. The consequences of untreated OSA in children include neurocognitive deficits, behavioral problems, poor school performance as well as systemic and pulmonary hypertension. The treatment options for pediatric OSA are numerous with a variety of surgical and non-surgical interventions.

Pediatric pulmonology year in review 2019: Sleep medicine.

Pediatric Pulmonology publishes original research, review articles as well as case reports on a wide variety of pediatric respiratory disorders. In this article, we summarize the past year's publications in sleep medicine and we review selected literature from other journals within this field. Articles highlighted are topics on risk factors of sleep-disordered breathing, diagnosis, and treatment of obstructive sleep apnea as well as the utility of polysomnography in various complex conditions.

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.

Pediatric polysomnography-A review of indications, technical aspects, and interpretation.

Polysomnography is an elaborate diagnostic test composed of numerous data-collecting sensors working concomitantly to aid in the evaluation of varied sleep disorders in all age groups. Polysomnography is the study of choice for the assessment of pediatric sleep-disordered breathing, including obstructive sleep apnea syndrome, central apnea, and hypoventilation disorders, and is used to help determine treatment efficacy. Beyond the purview of snoring and breathing pauses, polysomnography can elucidate the etiology of hypersomnolence, when associated with a multiple sleep latency test, and abnormal movements or events, whether nocturnal seizure or complex parasomnia, when a thorough patient history cannot provide clear answers.

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.

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.