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.
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 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.
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.
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