Travel for families of children with complex medical needs can offer significant benefits for family wellbeing and quality of life, yet it is often perceived as complex and high risk. As the population of technology-dependent children increases, the need to support safe and feasible travel has become more prominent. Families face multiple barriers, including the need to maintain continuity of care, transport essential equipment and medications, navigate variable regulations, and coordinate extensive documentation across healthcare and travel systems. These demands, alongside financial costs and administrative burden, frequently contribute to increased anxiety and risk aversion. Experiential accounts from parents demonstrate that travel for this cohort of children is safe, achievable, and rewarding with comprehensive and anticipatory planning across key domains, including destination selection, accommodation suitability, equipment and oxygen provision, medication and nutrition management, transport arrangements, and emergency preparedness. Healthcare professionals play a crucial role in supporting families through pre-travel assessment, documentation, and risk mitigation strategies. With appropriate preparation and multidisciplinary collaboration, travel can be undertaken safely, enabling families to access important psychosocial benefits while minimising potential clinical risks.
Electronic cigarettes (e-cigarettes) have become a leading form of nicotine use among children and adolescents worldwide. The World Health Organization (WHO) estimates that approximately 15 million young teenagers currently use these products. Contrary to early claims regarding reduced harm, e‑cigarettes expose users to a range of toxic substances, including carcinogens and, increasingly, synthetic nicotine compounds and other psychoactive agents. Epidemiological studies associate adolescent e‑cigarette use with respiratory morbidity, adverse mental health outcomes, and a substantially increased likelihood of subsequent initiation of combustible cigarette smoking. Dual use of e‑cigarettes and conventional cigarettes, reported among more than one third of nicotine‑using high school students in the United States, is associated with greater health risks than use of either product alone. The rise in adolescent vaping has been influenced by social media promotion backed by tobacco companies, flavored products, low-cost disposable devices, and peer norms, alongside persistent misperceptions of harm. Global regulatory responses remain inconsistent, with limited adoption of comprehensive bans and continued challenges from illicit markets. The International Society of Pediatric Respiratory Diseases (INSPiRED) calls for a global ban of e-cigarettes sales, advertising bans, increased taxation, and development of cessation services for youth.
Pediatric airway endoscopy, including nasopharyngolaryngoscopy (NPL), microlaryngobronchoscopy (MLB), and flexible bronchoscopy, plays an essential role diagnosing and managing airway disorders in children. Each technique offers unique distinct advantages, and when paired with imaging modalities such as X-rays, fluoroscopy, computed tomography (CT), and magnetic resonance imaging (MRI), clinicians gain a more comprehensive and accurate assessment of the airway. This review explores the complementary role of endoscopy and imaging in pediatric airway evaluation, emphasizing how their combined use improves diagnostic accuracy, guides treatment planning, and facilitates multidisciplinary care. While endoscopy remains the gold standard for visualizing airway pathology, integrating imaging enhances understanding of both structural and functional abnormalities, ultimately improving clinical outcomes in this vulnerable patient population.
Tracheobronchomalacia (TBM) remains a challenging condition in pediatric respiratory medicine. It arises from diverse etiologies involving intrinsic tracheal collapse and extrinsic airway compression. Although diagnostic criteria have progressed over time, flexible bronchoscopy remains the gold standard, as it enables direct assessment of dynamic airway changes, with a >50% reduction in airway lumen during forced expiration considered indicative of TBM. TBM represents one of the principal consequences of bronchopulmonary dysplasia (BPD) and plays a decisive role in the clinical course of affected children. The pathophysiological connection between BPD and TBM stems from multiple factors: prolonged mechanical ventilation, chronic inflammation, and altered development of airway cartilage in premature infants. Pharmacological treatment plays a key role in managing large airway disease (LAD) in infants with severe BPD by optimizing airway stability and reducing respiratory distress. Chest physiotherapy can enhance mucociliary clearance and support respiratory mechanics in patients with TBM, while in more severe cases surgical or bronchoscopic interventions, such as airway stenting, are often required. TBM in the context of BPD adds substantial clinical complexity, necessitating a comprehensive understanding of disease mechanisms and individualized therapeutic strategies. Evidence supporting available interventions is heterogeneous; non-invasive ventilation strategies hold the strongest support from randomized controlled trials, whereas bronchoscopic and surgical procedures rely largely on observational studies, case series, and expert opinion. This variability underscores the need for further research, particularly controlled trials comparing different management strategies where ethically and practically feasible.
Bronchopulmonary dysplasia is a frequent complication of preterm birth and is often associated with the development of pulmonary hypertension (BPD-PH). BPD-PH is a multifactorial disease with variable hemodynamic phenotypes typically characterized by underdeveloped pulmonary vascular networks with altered microvascular function, carrying significant morbidity and mortality in infancy.Advances in neonatal care have improved the survival of preterm infants. Amongst survivors, there is a trend toward resolution of BPD-PH with adequate respiratory support and somatic growth, highlighting the importance of early recognition and intervention to optimize vascular growth potential. However, as this population ages the idea of “resolution” has been brought into question. There is increasing awareness of subclinical or recurrent pulmonary hypertension as survivors of prematurity from childhood through early adulthood suggesting that the vasculature remains at risk throughout life.The natural history of pulmonary vasculature is one of development, growth and aging. Each window presents unique opportunities and potential insults that impact overall BPD-PHseverity and progression. Here we review care of the BPD-PH patient across the lifespan highlighting windows of opportunity to reduce lifetime exposure to pulmonary hypertension.
Severe paediatric asthma imposes a substantial health and financial burden. Biologics-monoclonal antibodies targeting specific asthma inflammatory pathways, are transforming severe asthma management. Currently approved biologics for paediatric use (≥6 or ≥12 years of age) include anti-immunoglobulin E (omalizumab), anti-interleukin (IL)-5 (mepolizumab), ultra-long acting anti-IL5 (depemokimab), anti-IL-5 receptor (benralizumab), anti-IL4/IL-13 (dupilumab), and anti-thymic stromal lymphopoietin (TSLP) (tezepelumab). While clinical trials have shown promising results, real-world data on long-term efficacy and safety in children and adolescents remain limited. Moreover, guidelines for initiating, monitoring biologics and assessing treatment response varies across countries lacking consensus. This is the second in a series of narrative reviews on biologics in severe paediatric asthma. The first review summarised key clinical trials findings and offered practical guidance for initiating therapy. In this review, we focus on strategies for monitoring treatment response, the role of asthma biomarkers and the potential of emerging therapies. We also highlight current research gaps and future directions to optimise biologics use in children and adolescents with severe asthma.
Bronchopulmonary dysplasia (BPD) is characterized by impaired lung development and persistent respiratory morbidity. BPD comprises three primary phenotypes: parenchymal, airway, and pulmonary vascular disease. Parenchymal disease involves disrupted alveolar growth, emphysema, hyperinflation, inflammation, and fibrosis, leading to impaired gas exchange. Airway disease, including airway malacia and subglottic stenosis, causes airflow obstruction and increased work of breathing. Pulmonary vascular disease involves abnormal vascular remodeling and increased pulmonary vascular resistance, which contributes to the development of pulmonary hypertension. Imaging plays a central role in evaluating these phenotypes which may occur independently or overlap with other phenotypes. Advanced MRI techniques enable non-invasive assessment of lung structure, ventilation, perfusion, airway shape and dynamics, vascular remodeling, and ventricular morphology and function. Imaging-based phenotyping can enhance disease monitoring, risk stratification, and help to inform personalized management strategies in infants with BPD.
Recurrent wheezing in preschool aged children is a common reason for consultation to the pediatric respirologist. However, the definition of preschool asthma, the various phenotypes and associated investigations required to define these phenotypes, the goals of therapy, and considerations for therapeutics, including biologics, are all areas needing further development. Let's begin with an illustrative case.
The long-term consequences of early adverse exposures are well known but is becoming clear that early means not just in pregnancy but also in at least the two preceding generations. Furthermore, there are new existential threats to long term respiratory health, some of which have been presented at the INSPiRED meeting in Bologna and are reviewed in this manuscript. One such is premature delivery, with ever smaller babies surviving with new respiratory and systemic complications, but there is also an appreciation that even those who do not develop bronchopulmonary dysplasia may have long term complications. New environmental hazards include vapes and other devices designed to produce a new generation of nicotine addicts; gas flaring which ruthlessly maximises profit at the expense of the environment and those living close by; and climate change, perhaps the greatest threat of all. Compounding these challenges is the effects of displacement by war or natural disasters on increasing numbers of children. On the other hand, encouragingly, two new highly effective strategies for the prevention of respiratory syncytial virus infection are becoming widely available, namely maternal immunisation with a pre-fusion vaccine in the third trimester of pregnancy and a long-acting monoclonal antibody. We await data as to whether these strategies will prevent impairment of lung function and later asthma. However, strong political action is essential if the expanding range of threats to the health of children and young people are to be averted. Awareness of new threats and advocacy are important roles for all paediatricians.
Early childhood asthma is one of the most common chronic diseases in children, and its course may be modified by coexisting upper airway obstruction. It remains uncertain whether adenoidectomy or adenotonsillectomy can improve asthma control or reduce exacerbations in children with asthma and obstructive symptoms. Emerging evidence links asthma phenotype, type 2 inflammatory biomarkers (fractional exhaled nitric oxide, blood eosinophilia, periostin, chitinase) and changes in upper airway microbiota with variable clinical responses to surgery. This narrative review summarises studies evaluating the impact of adenoidectomy and adenotonsillectomy on asthma outcomes in paediatric populations, including secondary analyses of major randomised controlled trials (CHAT, PATS). Across heterogeneous cohort and database studies, surgery is associated with improved symptom control and reductions in hospitalisations and emergency visits in selected children, particularly younger patients with severe obstruction and poorly controlled disease despite pharmacotherapy. A post-hoc analysis of the CHAT trial demonstrated a significant reduction in wheezing after adenotonsillectomy, and a secondary analysis of the PATS trial showed a trend toward improved asthma symptom burden, although neither trial was designed with asthma as a primary endpoint. Conversely, some large population-based analyses suggest a neutral effect or even an increased long-term risk of asthma in specific subgroups, underscoring substantial uncertainty. Critically, no randomised controlled trial has evaluated asthma control or exacerbation rate as a primary outcome after adenotonsillectomy, and overall evidence quality is low to very low. Adenotonsillectomy should therefore be considered a targeted option for a carefully selected subgroup of young children with documented upper airway obstruction and poorly controlled asthma, rather than a routine intervention. Well-designed multicentre prospective studies with standardised asthma endpoints, detailed phenotyping and microbiome and biomarker assessment are needed to clarify which patients derive durable benefit and to define the role of surgery within personalised asthma management.
Acute respiratory infections (ARIs) are the most common infectious diseases during childhood, responsible for significant morbidity and mortality in the pediatric population worldwide. Metabolomics, the global analysis of small metabolites, is a novel tool that offers insights into diagnostics and prognostics of viral respiratory infections. This review summarizes the existing literature on metabolomic studies in viral acute respiratory infections (ARIs) within the pediatric population. Metabolomic research on patients with bronchiolitis has addressed several clinical questions, such as discrimination by causative agent, i.e. respiratory syncytial virus or rhinovirus, and most importantly prognosis, predicting disease severity and long-term outcomes. In pneumonia, studies have focused on discriminating viral from bacterial etiology and from healthy controls but also predicting disease severity. There is a growing number of studies on metabolomics in ARIs providing a strong foundation for addressing remaining challenges and allowing for more comparable results and greater impact on clinical practice.
BACKGROUND:Chest wall deformities (CWD) and scoliosis are common comorbidities in congenital diaphragmatic hernia (CDH) survivors. Their effects on lung function remain unclear. METHODS:We retrospectively analysed 184 CDH patients treated in our clinic between 1999 and 2025, of whom 166 (90.2%) survived until discharge. We matched patients with varying degrees of musculoskeletal abnormalities to controls from our cohort and compared spirometry data. RESULTS:6.7% of patients had scoliosis and 57.9% had CWD, most commonly asymmetry of the chest (46.4%) and pectus excavatum (42.1%). CWD were usually ipsilateral (95.4%) but the convexity of scoliosis varied. 5/12 scoliosis patients received treatment, and none of the CWD patients. Moderate CWD was associated with lower forced vital capacity in univariate (Z-score effect size -2.08, p < 0.01) and multivariate (-1.53, p = 0.015) analyses. CONCLUSIONS:Chest wall deformities are common in children born with congenital diaphragmatic hernia. More severe chest wall deformities in CDH were associated with a lower forced vital capacity.
Pleural tuberculosis (TB) is the most common presentation of extrapulmonary TB and the most common cause of pleural effusion worldwide. Effusion due to TB is rare in children younger than 5 years and more common in adolescent boys. Pleural disease can range from small effusions associated with parenchymal disease in young children to large effusions seen mostly in older children and adolescents. The effusion can progress from a simple effusion to complicated pleural disease with the development of TB empyema or involvement of the chest wall. There is a paucity of information on the management of complicated TB pleural disease. Although most children will be treated conservatively, surgical intervention may be required to prevent post-TB lung disease and functional loss. Bronchopleural fistula (BPF) is a rare complication in children but can be very difficult to manage, especially in children with severe chronic or cystic lung disease, leading to prolonged hospitalization. Interventional bronchoscopy may have a role in the management of persistent BPF with the placement of either blood clots or valves, but there is limited evidence for these procedures in paediatrics.
Fungal respiratory infections are rare in immunocompetent children, and their recognition remains challenging since clinical features are often subacute and overlapping with other causes of respiratory disease. A structured diagnostic approach is fundamental to facilitating earlier diagnosis and prompt targeted therapy, thereby improving outcomes. Fungal spores are ubiquitous in the environment and fungal colonisation of skin and mucosal membranes is common, hence differentiation of infection from colonisation is key to an accurate diagnosis and a prudent approach to management. The diagnosis of invasive fungal infection should prompt consideration of underlying immunocompromise where appropriate. Approaches to the diagnosis of invasive fungal infections in children with known immune defects, particularly in the haematology and oncology setting, are covered extensively elsewhere and are not the subject of this review.
Achondroplasia is the most common skeletal dysplasia, characterized by disproportionate short stature and associated craniofacial, musculoskeletal, and neurological abnormalities. Respiratory complications are frequent and represent a major contributor to morbidity and mortality. Sleep-disordered breathing (SDB), encompassing obstructive (OSA) and central sleep apnea (CSA), is the most prevalent respiratory complication with pediatric prevalence reported between 22% and 93%. OSA results primarily from multilevel upper-airway obstruction related to craniofacial abnormalities and adenotonsillar hypertrophy, whereas CSA is often linked to cervicomedullary compression secondary to foramen magnum stenosis (FMS). Early sleep evaluation, particularly during infancy, is essential. Management strategies include adenotonsillectomy, maxillofacial interventions, cervicomedullary decompression, and non-invasive ventilation (NIV), according to underlying pathophysiology. Restrictive lung disease, pulmonary hypertension (PH), airway malacia and conductive hearing loss (CHL) further contribute to respiratory burden. Over the last fifteen years, major advances in this field have included improved recognition of the heterogeneity of SDB, the role of dynamic airway abnormalities and residual postoperative obstruction, and the emergence of FGFR3-targeted therapies. However, major gaps remain regarding optimal surveillance strategies and the impact of novel therapies on respiratory outcomes. Early identification, multidisciplinary management and follow-up are critical to optimize long-term outcomes.
The aim of this study was to evaluate oral health in paediatric and adult patients affected by cystic fibrosis (CF). A systematic review was conducted following PRISMA guidelines. Electronic search was performed on scientific databases to identify studies reporting relevant oral health parameters. Eleven studies met inclusion criteria, mostly cross-sectional and from tertiary care settings. Paediatric CF cohorts showed caries risk comparable or lower than controls but a higher prevalence of developmental enamel defects (DDE) and altered salivary function. Adult CF patients exhibited increased DMFT scores with more untreated decay, while severe periodontitis (PD ≥ 6 mm) remained rare despite high plaque accumulation. CF is associated with a distinctive oral health profile, characterized by age-dependent caries risk, frequent enamel anomalies, altered salivary physiology, and relatively preserved periodontal status. Standardized longitudinal studies are needed to clarify these associations, evaluate CFTR modulators effects, and support the integration of dental care within multidisciplinary CF management.
The availability of highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulators has transformed outcomes for people with cystic fibrosis (pwCF). Eligibility, conferred initially by clinical trial data, has evolved through advances in in vitro 'theratyping' and use of real‑world evidence. Since ivacaftor's approval for a single gating variant in 2012, eligibility has broadened to over 180 variants with the highly effective therapies elexacaftor/tezacaftor/ivacaftor and recently launched vanzacaftor/tezacaftor/deutivacaftor. Regulatory authorities have increasingly accepted non‑traditional evidence, notably the FDA's 2017 ivacaftor extension based on cell‑based assays and, in 2025, the EMA's decision to extend elexacaftor/tezacaftor/ivacaftor to people ≥2 years with at least one non class I variant. However, clinical response remains variable, influenced in part by baseline characteristics, pharmacokinetics, pharmacogenetics, and environmental exposures. We outline evolving approaches to determining eligibility and strategies to improve methods to predict, verify, and monitor clinical response in an era of personalised medicine.
