Paediatric Respiratory Reviews最新文献

Respiratory symptoms are among the most common presentations of inborn errors of immunity (IEI) and acquired immunodeficiencies in children. Pediatric pulmonologists are often the first to evaluate these patients, yet immunologic evaluations remain underutilized due to diagnostic complexity and limited familiarity with immune testing. Not all patients will have access to a timely consultation with an immunologist. This review provides a practical framework to aid pediatric pulmonologists in identifying, evaluating, and managing immune dysfunction in children with respiratory disease. It outlines clinical indicators, such as recurrent infections, bronchiectasis, failure to thrive, and syndromic features, and describes the utility and limitations of key immunologic tests. Stepwise diagnostic strategies are presented, from initial laboratory screening to functional assays and genetic testing. Common IEI with respiratory manifestations, including antibody deficiencies, combined immunodeficiencies, phagocytic disorders, and immune dysregulation syndromes, are reviewed. The article also addresses acquired immunodeficiencies, diagnostic mimics, and principles of pulmonary co-management, including prophylaxis and long-term follow-up. Early recognition and collaborative care can improve outcomes and prevent irreversible pulmonary damage in this vulnerable population.

Obstructive sleep apnoea (OSA) and obesity may co-exist in children and adolescents. Childhood obesity tends to persist into adulthood. Sustained weight loss is recommended for obesity and OSA but may be very difficult to achieve and maintain in the real world. The need to provide effective and integrated solutions for the constellation of associated pro-inflammatory and mechanical complications of obesity including obstructive sleep apnoea, metabolic syndrome, and type 2 diabetes mellitus is obvious. Sleep laboratories cannot meet the demand for diagnostic polysomnograms and under resourced paediatric obesity clinics limit themselves to treating those with severe obesity. Importantly, readily accessible resources, such as community-based dietitians and other allied health professionals, are both scarce and overwhelmed by demands for their services. The result is that many families who need assistance, especially those with socio-economic disadvantage and those with neurodiverse children, are unable to access treatments in a timely and equitable manner. In adults, glucagon-like peptide-1 receptor agonists (GLP-1 RAs) have been demonstrated to reduce body weight by up to 15 % in a 12-month period, improve glycaemic control, improve blood pressure and cardiovascular risk factors and significantly decrease the severity of obstructive sleep apnoea. As part of an integrated, multi-disciplinary approach, GLP-1 RA treatment in the medium to long term may be useful in those adolescents with severe obesity and OSA who are unable, unwilling or unsuitable for treatment with adenotonsillectomy or continuous positive airway pressure [CPAP]. Treatment benefits using GLP-1 RAs as adjunct therapy for OSA have been demonstrated in adults. The time has come to consider prioritising funded availability for adolescents with severe OSA and obesity in combination with support for behaviour change.

Omalizumab, a monoclonal antibody targeting IgE, was the first biologic therapy approved in 2003 for treating severe, allergen-driven, therapy-resistant asthma. Since then, many new biologics have been approved for use in children, targeting specific pathways, including anti-interleukin (IL)-5 (mepolizumab), IL-5 receptor (benralizumab), IL-4/IL-13 receptor (dupilumab), and thymic stromal lymphopoietin (TSLP) (tezepelumab). As the portfolio of biologics with diverse targets continues to expand, it has brought additional challenges to clinical practice. These include accurately identifying the endotype/phenotype of asthmatic inflammation and determining response criteria. Here, we summarise findings from phase 3 trials, discuss practical considerations for individual patients, and propose an algorithm for initiating biologics in children and adolescents with severe asthma.

As survival rates for premature infants improve, understanding the link between prematurity and obstructive sleep apnea (OSA) has become critical for enhancing outcomes in this high-risk population. Children born severely prematurely face a threefold increased risk of developing OSA due to anatomical and physiological factors, such as an abnormal upper airway, unstable ventilatory control, bronchopulmonary dysplasia, and increased susceptibility to respiratory infections. The multisystemic impact of prematurity also increases the likelihood of adverse OSA outcomes, such as neurodevelopmental deficits and pulmonary hypertension, generating a "Perfect Storm" for children affected by both conditions. Here, we summarize new insights into the shared pathophysiological mechanisms linking prematurity and OSA, highlighting the need to identify and characterize OSA in all premature infants. Polysomnographic assessments of sleep-disordered breathing and ventilatory responses to hypoxia/hyperoxia may enable tailored oxygen saturation targets for supplementation and weaning, based on each infant's unique physiology rather than relying on generalized protocols.

The natural closure of the ductus arteriosus is often delayed in premature newborns, especially in the most immature ones, and a significant left-to-right shunt volume raises concerns about pulmonary overcirculation, steal of systemic blood flow, and its potential contribution to neonatal respiratory morbidities such as bronchopulmonary dysplasia (BPD). Extremely low gestational age and long duration of mechanical ventilation may influence the relationship between the patent ductus arteriosus (PDA) and BPD. Decades of research has employed various pharmacologic approaches including NSAIDs and acetaminophen administered based on diverse criteria and using various timing, dose, and route combinations. Unfortunately, none of these interventions has consistently demonstrated meaningful improvements in clinical outcomes. Instead, these treatments inconsistently achieve PDA closure or restriction of flow through the ductus and are often associated with adverse effects. Thus, the lack of clear benefit from available treatments, coupled with the potential for harm, has prompted many centers to adopt conservative or expectant management of the PDA while awaiting novel strategies that could offer improved efficacy and safety. This review explores the associations between PDA and pulmonary outcomes of prematurity, reflecting on past research and outlining potential future directions.

Cerebral palsy refers to a group of disorders affecting movement and posture, caused by an impact to the developing brain. Children and young people (CYP) with cerebral palsy (CP) may have multiple associated co-morbidities, including sleep disorders. Sleep disorders, including sleep disordered breathing, are more prevalent among CYP with CP compared to typically developing children due to several contributory factors. Sleep-disordered breathing (SDB) collectively represents several different pathologies, namely obstructive sleep apnoea (OSA), central sleep apnoea (CSA), and hypoventilation. Multiple intrinsic and extrinsic factors contribute to each of these pathologies. Sleep studies (either as polysomnography or cardiorespiratory polygraphy) allow the detection of effort and apnoea, with contemporaneous measurement of oxygen saturations (SpO₂) and transcutaneous carbon dioxide (tcpCO₂). Together these enable the diagnosis of SDB, and the delineation of OSA, CSA and/or hypoventilation. The multifactorial component of SDB among CYP with CP may require intervention ranging from conservative measures (e.g. nasopharyngeal airway, soft collar), surgical options (e.g. adenotonsillectomy), or the initiation of respiratory support. Respiratory support, delivered as Continuous Positive Airway Pressure (CPAP) or Non-Invasive Ventilation (NIV), has a key role in the management of SDB in children with CP though the journey can often be turbulent with a high failure rate. Nonetheless, CYP with CP are being increasingly commenced on respiratory support, often for reasons others than SDB, for example aiding airway clearance in order to reduce the frequency of lower respiratory tract infections. Open discussions between the parents and healthcare professionals are important in setting shared goals for CYP with CP, guided by the primary aim of improving quality of life.