Volume assured pressure support mode use for non-invasive ventilation in pediatrics.

There has been increasing interest in utilizing volume assured pressure support (VAPS) modes of ventilation for children, which historically had only been favored in adult populations. In addition to patients with obesity hypoventilation syndrome, newer pediatric populations for which it has recently been prescribed include congenital central hypoventilation syndrome and children with neuromuscular disease such as Duchenne muscular dystrophy and spinal muscular atrophy. Given its expanding use in pediatrics, greater familiarity with VAPS is essential for pediatric pulmonologists and sleep physicians.

Understanding caregiver experiences with disease-modifying therapies for spinal muscular atrophy: a qualitative study.

Objective: Spinal muscular atrophy (SMA) is a neuromuscular disorder that manifests with motor deterioration and respiratory complications. The paradigm of care is shifting as disease-modifying therapies including nusinersen, onasemnogene abeparvovec and risdiplam alter the disease trajectory of SMA. The objective of this study was to explore caregivers' experiences with disease-modifying therapies for SMA.

The association between sleep quality and obstructive sleep apnea with health-related quality of life in children with obesity.

Study Objectives: Obstructive sleep apnea (OSA) and poor sleep quality are highly prevalent in children with obesity, but their individual associations with health-related quality of life (HRQOL) are unknown in this population. The primary objective was to describe the independent association of OSA and sleep quality with HRQOL in children with obesity.

Methods: This was a cross-sectional study of children with obesity at 2 tertiary care centers.

Polysomnography in hospitalized children: Characteristics and clinical practice at a single tertiary care center.

Background: Polysomnography (PSG) is the gold standard for the diagnosis of pediatric sleep-disordered breathing (SDB). However, the literature characterizing the indications for inpatient PSGs and the impact on clinical decision-making is limited.

Objective: To determine the indications, results, and outcomes for children undergoing inpatient PSGs at our institution.

Long-term mechanical ventilation and transitions in care: A narrative review.

Objectives: Individuals dependent on long-term mechanical ventilation (LTMV) for their day-to-day living are a heterogenous population who go through several transitions over their lifetime. This paper describes three transitions: 1) institution/hospital to community/home, 2) pediatric to adult care, and 3) active treatment to end-of-life for ventilator-assisted individuals (VAIs).

Methods: A narrative review based on literature and the author's collective practical and research experience.

Scoliosis in Spinal Muscular Atrophy Type 1 in the Nusinersen Era.

Background And Objectives: The introduction of spinal muscular dystrophy (SMA)-modifying therapies, such as antisense oligonucleotide therapy, has changed the natural history of SMA. Most reports on treatment outcomes have focused on motor scores and respiratory function. The objective of this study is to document the development and progression of scoliosis in patients with SMA1 treated with nusinersen.

Respiratory characteristics in children with spinal muscular atrophy type 1 receiving nusinersen.

Background: Spinal muscular atrophy type 1 (SMA1) is a neuromuscular disorder with a natural history of chronic respiratory failure and death during infancy without ventilation. Recently, disease-modifying therapies such as nusinersen have improved disease trajectory. However, objective data on the trajectory of polysomnography outcomes, the relationship between motor scores and respiratory parameters, respiratory technology dependence and healthcare utilization in children with SMA1 remain to be elucidated.

Unexplained Significant Central Sleep Apnea in Infants: Clinical Presentation and Outcomes.

Objective: Unexplained significant central sleep apnea in term infants presents as central apneas with associated oxygen desaturations requiring respiratory support and monitoring for prolonged periods. However, there is a paucity of literature describing idiopathic central sleep apnea (ICSA) in term or near-term infants. Our aim was to describe the clinical manifestations, polysomnography data, interventions, and trajectory of ICSA in infants.

Risk factors for respiratory adverse events after adenoidectomy and tonsillectomy in children with down syndrome: a retrospective cohort study.

Unlabelled: Obstructive sleep apnea syndrome is a major cause of morbidity in the Down syndrome population and is commonly treated with adenoidectomy and/or tonsillectomy (AT). However, these children are at increased risk for perioperative respiratory adverse events (PRAEs). The objective of this study was to examine risk factors for major PRAEs requiring intervention in children with Down syndrome undergoing AT and to describe their postoperative monitoring environment.

Pediatric tracheostomy tube decannulation with or without polysomnography: A PRO-CON debate.

Determining the timing for decannulation in children with a tracheostomy is a complex process, as the appropriate timing varies based on the initial indication for the tracheostomy tube as well as individual patient characteristics. The original condition for which a tracheostomy was created may improve over time with decannulation being a very important long-term goal for many families and multidisciplinary teams. However, decannulation is an inherently risky procedure associated with morbidity and mortality.

Positional device therapy for the treatment of positional obstructive sleep apnea in children: a pilot study.

Background: There is a critical gap in identifying effective interventions for children with obstructive sleep apnea (OSA) who do not tolerate continuous positive airway pressure therapy. Positional OSA (POSA) is a common clinical phenotype whereby OSA occurs predominantly while sleeping in supine position. POSA may be amenable to treatment with a positional device, a belt worn around the chest with cushions on the back to prevent supine positioning, but no data exists in children.

Polysomnography parameters as predictors of respiratory adverse events following adenotonsillectomy in children.

Study Objectives: The first-line treatment of obstructive sleep apnea syndrome in children is adenotonsillectomy, but this may result in perioperative respiratory adverse events (PRAEs). The primary aim of this study is to examine whether the McGill oximetry score (MOS) and other polysomnography parameters can predict major PRAEs following adenotonsillectomy. We secondarily evaluated the MOS interrater reliability and correlation with other polysomnography parameters.

Narcolepsy Treatment: Voices of Adolescents.

Introduction: Narcolepsy, characterized by excessive daytime sleepiness, is a debilitating lifelong sleep disorder for which there is no cure. Current pharmacological and nonpharmacological treatments directed toward symptom management may be suboptimal. This qualitative study explores the perspective of adolescents on therapeutic interventions for narcolepsy.