H B Q Brand, H T Fitzgerald, S Haggie, J A Prat Matifoll, L Hatton, C Pandit, H Selvadurai, R D'Cruz, J Karpelowsky, D A Fitzgerald
{"title":"The effect of musculoskeletal abnormalities on lung function in children born with congenital diaphragmatic hernia.","authors":"H B Q Brand, H T Fitzgerald, S Haggie, J A Prat Matifoll, L Hatton, C Pandit, H Selvadurai, R D'Cruz, J Karpelowsky, D A Fitzgerald","doi":"10.1016/j.prrv.2026.05.004","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.05.004","url":null,"abstract":"<p><strong>Background: </strong>Chest wall deformities (CWD) and scoliosis are common comorbidities in congenital diaphragmatic hernia (CDH) survivors. Their effects on lung function remain unclear.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148033812","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pierre Goussard, Ernst Eber, Leonore Greybe, Barend Fourie, Jacques Janson, Francois Retief, Jonathan Burke, Celeste Burger, Zane Ismail, Jin Yong Kim, André Gie, Delano Rhode, Jacques du Toit, Savvas Andronikou
{"title":"Complicated tuberculous pleural disease in children.","authors":"Pierre Goussard, Ernst Eber, Leonore Greybe, Barend Fourie, Jacques Janson, Francois Retief, Jonathan Burke, Celeste Burger, Zane Ismail, Jin Yong Kim, André Gie, Delano Rhode, Jacques du Toit, Savvas Andronikou","doi":"10.1016/j.prrv.2026.05.002","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.05.002","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148239847","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Veronica Fasoli, Alessia Mariani, Federica Chironi, Mara Lelii, Barbara Madini, Beatrice Andrenacci, Alessia Rocchi, M Francesca Bedeschi, M Gaffuri, M Pluderi, M Francesca Patria
{"title":"Breathing challenges in achondroplasia: a comprehensive review of pediatric respiratory complications.","authors":"Veronica Fasoli, Alessia Mariani, Federica Chironi, Mara Lelii, Barbara Madini, Beatrice Andrenacci, Alessia Rocchi, M Francesca Bedeschi, M Gaffuri, M Pluderi, M Francesca Patria","doi":"10.1016/j.prrv.2026.05.003","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.05.003","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148056075","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nichkamol Lertamornkitti, Meghan Gunst, Annaleise R Howard-Jones
{"title":"Diagnosis of fungal respiratory infections in immunocompetent children.","authors":"Nichkamol Lertamornkitti, Meghan Gunst, Annaleise R Howard-Jones","doi":"10.1016/j.prrv.2026.05.001","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.05.001","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":3.4,"publicationDate":"2026-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148265259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Youth vaping: an under-recognised risk behaviour in paediatric emergency care.","authors":"Zoe Bothamley, Katharine Harman, Atul Gupta","doi":"10.1016/j.prrv.2026.04.006","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.04.006","url":null,"abstract":"<p><p>The increased prevalence of vaping among UK adolescents over the last three years, driven by youth-targeted marketing from the tobacco industry, represents a growing public health concern. A local service evaluation conducted within a tertiary paediatric emergency department identified significant gaps in current practice: 7.2% of 11-16-year-olds were asked about any smoking habits, and only 1.2% were asked specifically about vaping. This highlights a missed opportunity for early identification and response to youth vaping. We explore potential barriers contributing to these low rates of enquiry, including limited professional confidence and the lack of structured vaping medical education. To address this, we propose a practical framework for clinicians, suitable for use in the emergency department, which includes four targeted screening questions. The review also examines the scarcity of dedicated youth vaping cessation services, while showcasing examples of innovative, community-based interventions. A structured, standardised approach to youth smoking behaviours combined with accessible, youth-oriented support services is essential to mitigate the growing harms associated with youth vaping. Therefore, urgent reform in professional approaches to adolescent vaping behaviours is warranted.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147932292","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Irlanda Alvarado-Amador, Seda Tunca, Chiara Astori
{"title":"Recent advances in pediatric pulmonology and allergy: Articles of interest to fellows. Subclinical small airway dysfunction, anti-inflammatory reliever therapy, and biologic treatment in pediatric asthma.","authors":"Irlanda Alvarado-Amador, Seda Tunca, Chiara Astori","doi":"10.1016/j.prrv.2026.04.004","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.04.004","url":null,"abstract":"<p><p>Recent advances in pediatric asthma continue to refine the understanding of disease mechanisms and to reshape therapeutic strategies. In this brief review, we discuss three recent articles of particular interest to fellows and early-career clinicians. First, we examine evidence showing that subclinical small airway dysfunction may be present in children with well-controlled asthma despite normal FEV<sub>1</sub>, highlighting the potential role of oscillometry as a complementary tool in functional assessment. Second, we review the CARE trial, which demonstrated that as-needed budesonide-formoterol was superior to SABA-only reliever therapy in reducing asthma attacks in children with mild asthma. Third, we summarize recent data on dupilumab in children with uncontrolled moderate-to-severe type 2 asthma, showing sustained benefits across disease severity strata. Together, these studies illustrate how physiological assessment and targeted anti-inflammatory therapies are advancing personalized care in pediatric asthma.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147818622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alessandro Cuozzo, Leopoldo Mauriello, Vitolante Pezzella, Vincenzo Iorio-Siciliano, Elio Ramaglia, Sossio Giuseppe Lanzillo, Lorenzo Marini, Andrea Blasi
{"title":"Oral health status in adult and paediatric patients with cystic fibrosis (CF): A systematic review and meta-analysis.","authors":"Alessandro Cuozzo, Leopoldo Mauriello, Vitolante Pezzella, Vincenzo Iorio-Siciliano, Elio Ramaglia, Sossio Giuseppe Lanzillo, Lorenzo Marini, Andrea Blasi","doi":"10.1016/j.prrv.2026.04.003","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.04.003","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147723377","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Maria Giulia Conti, Rebecca Morelli, Elvira Mazzuca, Lorenzo Lugo, Barbara Luongo, Marco Bianchi, Domenico Paolo La Regina, Laura Petrarca, Raffaella Nenna, Fabio Midulla
{"title":"Targeting neutrophil-driven inflammation in bronchopulmonary dysplasia: From pathogenesis to therapeutic perspectives.","authors":"Maria Giulia Conti, Rebecca Morelli, Elvira Mazzuca, Lorenzo Lugo, Barbara Luongo, Marco Bianchi, Domenico Paolo La Regina, Laura Petrarca, Raffaella Nenna, Fabio Midulla","doi":"10.1016/j.prrv.2026.03.002","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.03.002","url":null,"abstract":"<p><p>Neutrophil-driven inflammation plays a key role in the initiation and progression of the chronic lung disease of preterm infants, also known as bronchopulmonary dysplasia (BPD). Their modulation represents a promising avenue for early risk stratification and targeted intervention. Among its pathogenic mechanisms, neutrophils are central contributors to impaired alveolarization and lung function defects. Preclinical hyperoxia models in neonatal mice confirm a causal link between neutrophil activation and lung injury. Early neutrophil-related biomarkers such as blood neutrophil to lymphocyte ratio are emerging as predictive of BPD onset and severity. Despite these findings, clinical translation in targeted therapies remains limited. Further research should focus on validating neutrophil-derived biomarkers, characterizing neutrophil phenotypes, and ultimately propose clinical trials to evaluate the safety and efficacy of anti-neutrophilic anti-inflammatory therapies in neonates.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147623471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Apolline Gonsard, Marie-Louise Fremond, Alice Hadchouel
{"title":"Immunomodulation in paediatric inflammatory interstitial lung diseases: towards mechanism-based targeted therapies, a focus on the anti-cytokines.","authors":"Apolline Gonsard, Marie-Louise Fremond, Alice Hadchouel","doi":"10.1016/j.prrv.2026.03.001","DOIUrl":"https://doi.org/10.1016/j.prrv.2026.03.001","url":null,"abstract":"<p><p>Childhood interstitial lung diseases (chILDs) associated with inflammatory diseases represent a heterogeneous group of rare conditions. To date, therapeutic approaches predominantly rely on broad-spectrum immunosuppressive agents, often independently of the underlying mechanism. During the last ten years, next generation sequencing techniques allowed the identification of monogenic auto-inflammatory diseases with the subsequent development of mechanism-based targeted immunomodulatory therapies. Among these emerging therapies, anti-cytokine agents occupy an increasingly prominent role in inflammatory chILDs. Janus kinase (JAK) inhibitors have emerged as a promising strategy, with initial efficacy demonstrated in monogenic type I interferonopathies involving the lung, such as STING-associated vasculopathy with onset in infancy (SAVI) and COPA syndrome. Their therapeutic scope has subsequently expanded to include disorders driven by type II interferon signalling, notably systemic juvenile idiopathic arthritis-associated lung disease and refractory pulmonary granulomatosis. In juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5 (MDA5) antibodies, JAK inhibitors address refractory rapidly progressive interstitial lung disease. Further anti-cytokine therapies include tocilizumab (anti-interleukin-6) for juvenile systemic sclerosis-associated interstitial lung disease, anti-interleukin-1β/18 combinations (MAS-825), and anti-tumour necrosis factor-alpha. Whilst evidence remains predominantly limited to case reports and small series, these mechanism-based approaches represent a paradigm shift towards personalised medicine in chILDs. Understanding disease-specific pathophysiology-from interferon activity assessment to genomic sequencing-is essential for optimal therapeutic targeting. Multicentre collaborations are needed to evaluate long-term efficacy and safety of these targeted interventions.</p>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":" ","pages":""},"PeriodicalIF":4.0,"publicationDate":"2026-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147575006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gabriel Bolner, Yohanna Idsabella Rossi, Jonathan Costa Dall’Acqua, Artur Vestena Rossato, Fabiana Dolovitsch de Oliveira, Kauê Bolner, Gilberto Bueno Fischer, Janice Luisa Lukrafka, Helena Teresinha Mocelin
{"title":"Long-acting muscarinic antagonists as add-on treatment for asthma in children under age 12: a systematic review and meta-analysis","authors":"Gabriel Bolner, Yohanna Idsabella Rossi, Jonathan Costa Dall’Acqua, Artur Vestena Rossato, Fabiana Dolovitsch de Oliveira, Kauê Bolner, Gilberto Bueno Fischer, Janice Luisa Lukrafka, Helena Teresinha Mocelin","doi":"10.1016/j.prrv.2025.04.003","DOIUrl":"10.1016/j.prrv.2025.04.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Children under 12 have fewer treatment options for uncontrolled asthma than adolescents and adults. Long-acting muscarinic antagonists<span> (LAMA) can be used as an add-on therapy for asthma in adults, but the evidence in children is unclear.</span></div></div><div><h3>Methods</h3><div><span><span><span>We systematically searched PubMed, </span>Embase, and the </span>Cochrane Library for </span>randomized controlled trials<span><span> (RCTs) and observational studies that evaluated LAMA as an add-on therapy to inhaled corticosteroids in asthmatic children younger than 12. We performed a random-effects meta-analysis for lung function, asthma control, and </span>adverse events. Subgroup analyses were performed for different LAMA dosages.</span></div></div><div><h3>Results</h3><div><span>Four RCTs and two observational studies (n = 1210) were included in our systematic review. The </span>pulmonary function<span> tests indicated that LAMA at any dose significantly improved peak FEV1<span> (MD 86.16 mL; 95 %CI 18.62–153.71; p < 0.01) and FEF25–75 % (MD 0.2518L; 95 %CI 0.1971–0.3064; p < 0.01), while FVC<span> (MD 9.69 mL; 95 %CI −34.57 to 53.95; p = 0.67) remained unchanged. Asthma control measured by the Asthma Control Questionnaire<span> (MD −0.07; 95 %CI −0.08 to −0.06; p < 0.01) also favoured LAMA treatments. Nighttime awakenings and rescue treatment usage showed no significant differences between groups. Using LAMA was also associated with fewer adverse events (RR 0.88; 95 %CI 0.79–0.98; p = 0.021).</span></span></span></span></div></div><div><h3>Conclusion</h3><div>LAMA are safe and effective for moderate/severe asthma for children aged 6–11. Evidence is still slowly evolving for children younger than 6 years.</div></div>","PeriodicalId":19658,"journal":{"name":"Paediatric Respiratory Reviews","volume":"57 ","pages":"Pages 3-10"},"PeriodicalIF":4.0,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144182389","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}