ATS core curriculum 2023. Pediatric pulmonary medicine: Respiratory disorders in infants.

The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a summary of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2023 American Thoracic Society International Conference. The respiratory disorders of infancy discussed in this year's review include: the care of the patient with bronchopulmonary dysplasia in the neonatal intensive care unit, clinical phenotypes and comorbidities; diffuse lung disease; pulmonary hypertension; central and obstructive sleep apnea. The care of infants with respiratory disorders often poses significant challenges to the general pediatric pulmonologist, sleep clinician, and neonatologist. This review aims to highlight the most clinically relevant aspects of the evaluation, management, and outcomes of infants with these key respiratory disorders, while emphasizing the importance of multidisciplinary care. Furthermore, this document summarizes essential aspects of genetic testing, novel imaging and treatment modalities, and includes multiple resources for clinical practice.

ATS Core Curriculum 2022. Pediatric Pulmonary Medicine: Updates in pediatric neuromuscular disease.

The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a concise review of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2022 American Thoracic Society International Conference. Neuromuscular diseases (NMD) comprise a variety of conditions that commonly affect the respiratory system and cause significant morbidity including dysphagia, chronic respiratory failure, and sleep disordered breathing. Respiratory failure is the most common cause of mortality in this population. Substantial progress has been made in diagnosis, monitoring and treatment for NMD over the last decade. Pulmonary function testing (PFT) is utilized to objectively measure respiratory pump function and PFT milestones are utilized in NMD-specific pulmonary care guidelines. New disease modifying therapies are approved for the treatment of patients with Duchenne muscular dystrophy and spinal muscular atrophy (SMA), including the first ever approved systemic gene therapy, in the case of SMA. Despite extraordinary progress in the medical management of NMD, little is known regarding the respiratory implications and long-term outcomes for patients in the era of advanced therapeutics and precision medicine. The combination of technological and biomedical advancements has increased the complexity of the medical decision-making process for patients and families, thus emphasizing the importance of balancing respect for autonomy with the other foundational principles of medical ethics. This review features an overview of PFT, noninvasive ventilation strategies, novel and developing therapies, as well as the ethical considerations specific to the management of patients with pediatric NMD.

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. 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.

Positive Airway Pressure Therapy for Pediatric Obstructive Sleep Apnea.

Pediatric obstructive sleep apnea syndrome (OSAS) is a disorder of breathing during sleep, characterized by intermittent or prolonged upper airway obstruction that can disrupt normal ventilation and/or sleep patterns. It can affect an estimated 2-4% of children worldwide. Untreated OSAS can have far reaching consequences on a child's health, including low mood and concentration as well as metabolic derangements and pulmonary vascular disease. Most children are treated with surgical intervention (e.g., first-line therapy, adenotonsillectomy); however, for those for whom surgery is not indicated or desired, or for those with postoperative residual OSAS, positive airway pressure (PAP) therapy is often employed. PAP therapy can be used to relieve upper airway obstruction as well as aid in ventilation. PAP therapy is effective in treatment of OSAS in children and adults, although with pediatric patients, additional considerations and limitations exist. Active management and care for various considerations important to pediatric patients with OSAS can allow PAP to be an effective and safe therapy in this population.

The Use of Auto-Titrating Continuous Positive Airway Pressure (AutoCPAP) for Obstructive Sleep Apnea Syndrome in Children with Obesity.

BACKGROUND: Positive airway pressure can be an effective and safe therapy for children with obstructive sleep apnea syndrome (OSAS). Few studies have assessed the safety and efficacy of autoCPAP in pediatric patients with obesity. METHODS: This was a retrospective chart review of children with obesity (Body Mass Index (BMI) > 99th percentile), ages 2-18, diagnosed with OSAS (Obstructive Apnea-Hypopnea Index (OAHI) > 1/h) and used autoCPAP with 30-day adherence. Exclusion criteria included patients with complex comorbidities. Adherence was defined as autoCPAP use ≥4 h/night for at least 21/30 days. Baseline PSG OAHI was compared to the AHI from the 30-day autoCPAP compliance report. We also compared autoCPAP 30-day 95th percentile pressures with the pressures from PAP titration. RESULTS: The study included 19 children, ranging 5-15 years old. The median BMI was 99.6th percentile and average adherence was 25/30 nights with mean of 7.3 h/night. The median OAHI was 12.3/h on baseline PSG and the 30-day autoCPAP download AHI decreased to 1.7/h. No adverse outcomes were identified. The average difference between 95th percentile autoCPAP pressure and PAP titration pressure was 0.89 cmH20. CONCLUSION: Our study suggests autoCPAP is effective and safe for the treatment of OSAS in pediatric patients with obesity. Using autoCPAP may reduce delays in treatment. Additional research is needed to verify the long-term effectiveness of autoCPAP in this population.

ATS Core Curriculum 2021. Pediatric Pulmonary Medicine: Pulmonary Infections.

The following is a concise review of the Pediatric Pulmonary Medicine Core reviewing pediatric pulmonary infections, diagnostic assays, and imaging techniques presented at the 2021 American Thoracic Society Core Curriculum. Molecular methods have revolutionized microbiology. We highlight the need to collect appropriate samples for detection of specific pathogens or for panels and understand the limitations of the assays. Considerable progress has been made in imaging modalities for detecting pediatric pulmonary infections. Specifically, lung ultrasound and lung magnetic resonance imaging are promising radiation-free diagnostic tools, with results comparable with their radiation-exposing counterparts, for the evaluation and management of pulmonary infections. Clinicians caring for children with pulmonary disease should ensure that patients at risk for nontuberculous mycobacteria disease are identified and receive appropriate nontuberculous mycobacteria screening, monitoring, and treatment. Children with coronavirus disease (COVID-19) typically present with mild symptoms, but some may develop severe disease. Treatment is mainly supportive care, and most patients make a full recovery. Anticipatory guidance and appropriate counseling from pediatricians on social distancing and diagnostic testing remain vital to curbing the pandemic. The pediatric immunocompromised patient is at risk for invasive and opportunistic pulmonary infections. Prompt recognition of predisposing risk factors, combined with knowledge of clinical characteristics of microbial pathogens, can assist in the diagnosis and treatment of specific bacterial, viral, or fungal diseases.

Allergic Bronchopulmonary Aspergillosis.

Allergic bronchopulmonary aspergillosis (ABPA), a progressive fungal allergic lung disease, is a common complication of asthma or cystic fibrosis. Although ABPA has been recognized since the 1950s, recent research has underscored the importance of Th2 immune deviation and granulocyte activation in its pathogenesis. There is also strong evidence of widespread under-diagnosis due to the complexity and lack of standardization of diagnostic criteria. Treatment has long focused on downregulation of the inflammatory response with prolonged courses of oral glucocorticosteroids, but more recently concerns with steroid toxicity and availability of new treatment modalities has led to trials of oral azoles, inhaled amphotericin, pulse intravenous steroids, and subcutaneously-injected anti-IgE monoclonal antibody omalizumab, all of which show evidence of efficacy and reduced toxicity.