Search Results (165)

Background: Pediatric thoracic magnetic resonance imaging (MRI) has evolved into a valuable diagnostic modality that offers high-resolution morphological and functional assessment. While conventional radiography and computed tomography (CT) remain standard, their radiation exposure poses significant risks in children requiring repeated imaging. Technological innovations have addressed prior MRI limitations such as low lung proton density and motion artifacts, expanding its role in pediatric thoracic imaging. Methods: A review of the recent literature was performed, focusing on technical advancements, key MRI sequences and clinical applications in pediatric thoracic imaging. Emphasis was placed on ultrashort echo time (UTE), phase-resolved functional lung (PREFUL) MRI, hyperpolarized xenon-129 MRI, radial imaging, compressed sensing, parallel imaging and respiratory gating techniques. Results: Modern MRI sequences provide both detailed anatomic visualization and quantitative functional assessment of the pediatric thorax. UTE and PREFUL enable evaluation of lung parenchyma, ventilation, and perfusion, while hyperpolarized gas imaging offers high-resolution functional mapping. Radial, compressed sensing and parallel imaging reduce motion artifacts and acquisition times, enhancing feasibility in uncooperative children. Clinical indications include assessment of congenital malformations, chronic lung disease like cystic fibrosis, infectious and inflammatory disorders, tumors and selected traumatic injuries. Conclusions: Recent technical advances have established pediatric thoracic MRI as a versatile, patient-friendly alternative, as well as a complementary method to CT in selected clinical scenarios. Ongoing developments in acquisition speed, motion compensation and functional imaging are expected to further improve diagnostic accuracy and clinical utility, supporting broader adoption in routine pediatric thoracic evaluation. Full article

Pediatric interstitial lung diseases (chILD) are a diverse and complex group of rare but impactful disorders characterized by heterogeneous etiologies and variable clinical courses. Traditional diagnosis-based management often delays targeted treatment, underscoring the need for a more precise therapeutic approach. The “treatable traits” framework, originally developed in adult respiratory medicine, offers a novel paradigm for personalized care by focusing on identifying and modifying discrete, clinically relevant features in each child. This narrative review synthesizes existing evidence and expert consensus to define key treatable traits in pediatric ILD, encompassing genetic and surfactant dysfunction, immune dysregulation, pulmonary hypertension, hypoxemia, aspiration, growth deficits, and environmental exposures. For each trait, we describe diagnostic pathways—including genetic testing, bronchoalveolar lavage, imaging, and functional assessments—and outline targeted management strategies. The implementation of a trait-based approach necessitates multidisciplinary collaboration, standardized protocols, and ongoing research to validate biomarkers and optimize therapies. By adopting this personalized strategy, clinicians can improve early diagnosis, tailor interventions, and potentially alter disease trajectories. Our discussion highlights the current limitations and future priorities, emphasizing the importance of pediatric-specific studies and international networks to fully realize the promise of precision medicine in pediatric ILD. Full article

Background/Objectives: To evaluate the prevalence, age at diagnosis, non-invasive ventilation pressures used in management, and clinical predictors for sleep disordered breathing (SDB) in pediatric patients with muscular dystrophies (MDs). Methods: A retrospective analysis of 195 polysomnography (PSG) studies conducted over 20 years for 98 children with different MDs was performed. Diagnosis of SDB was established if a child met the diagnostic criteria for one or more of the following conditions: obstructive sleep apnea (OSA), central apnea, nocturnal hypoxemia, or nocturnal hypoventilation. Outcomes were assessed and compared between MDs. Positive and negative predictive values (PPV, NPV), sensitivity, and specificity for detecting SDB were calculated for certain clinical parameters. Results: SDB was diagnosed in 73.6% of children with MDs, including OSA in 67%, followed by nocturnal hypoxemia (15.3%), nocturnal hypoventilation (7.7%), and central apnea (6.6%). The age at diagnosis and BiPAP pressures used varied between MDs. Patients with Congenital MD had the lowest mean age and required higher pressures (p < 0.05). PPV was high for maximum inspiratory or expiratory pressures (MIP, MEP) < 40% or <60%, forced vital capacity < 50% or <80%, total lung capacity < 60%, left ventricular ejection fraction < 50%, non-ambulation, and body mass index ≥ 95% for the presence of SDB. However, NPV, sensitivity, and specificity varied. Conclusions: SDB is common in pediatric patients with MDs, with OSA being the most prevalent disorder. The age at diagnosis and required BiPAP pressures for management differ among MD groups. Certain clinical measures may help identify some patients with the disease given the high PPV. Full article

Lung ultrasound (LUS) has emerged as a valuable diagnostic modality for the evaluation of respiratory disorders in neonates, infants and children. LUS has high diagnostic accuracy for identification of lung lesions in neonates, infants and children, where most lung lesions abut the pleura. Furthermore, LUS has the advantage of rapid execution and ease of use, and does not require ionizing radiation. Its sensitivity, cost-effectiveness, and clinical efficiency make it an important tool for supporting clinical decision-making and improving patient management. Moreover, LUS may represent a reliable alternative to chest radiography for the assessment of pediatric lung conditions and, in selected cases, could potentially replace routine chest X-rays (CXRs). Because LUS is a user-friendly technique that enables real-time imaging without radiation, it has increasingly been used in clinical practice in recent years. Here, we discuss the diagnostic role of LUS for the accurate identification of pulmonary lesions in pediatric patients. In addition, we present LUS sonographic findings associated with common pediatric lung diseases, including signs and artifacts that can be used during diagnosis and evaluation of pediatric patients. Full article

Objectives: This systematic review aimed to evaluate the association between oral health—particularly dental caries and dysbiosis of the oral microbiome—and respiratory diseases across different age groups and clinical settings, with emphasis on microbial overlap, clinical outcomes, and preventive strategies. Methods: A systematic search was conducted in PubMed, Scopus, Embase, Web of Science, and the Cochrane Library up to June 2025. Eligible studies included randomized controlled trials, cohort, case–control, and cross-sectional investigations examining the relationship between oral diseases or microbiome alterations and respiratory outcomes. Data on study design, population, oral health parameters, microbial taxa, and respiratory endpoints were extracted. Study quality was assessed using the Mixed Methods Appraisal Tool (MMAT, 2018). Results: Twenty studies met the inclusion criteria, encompassing pediatric, adult, and elderly populations. Poor oral health, reflected by higher caries indices and periodontal inflammation, was consistently associated with increased risk of lower respiratory tract infections (LRTI), aspiration events, ventilator-associated pneumonia (VAP), and impaired pulmonary function. Oral microbiome analyses revealed enrichment of Veillonella, Prevotella, Klebsiella, and Pseudomonas species in both oral and airway samples, supporting the oral cavity as a reservoir for respiratory pathogens. Interventional evidence from intensive care and nursing home settings demonstrated that structured oral care—particularly daily toothbrushing and chlorhexidine-based plaque control—significantly reduced pneumonia incidence. Conclusions: This review confirms a clinically relevant and biologically plausible link between oral dysbiosis, dental caries, and respiratory disease. Oral biofilms contribute to infection risk through microaspiration and microbial seeding of the lower airways. Integrating oral screening, hygiene maintenance, and treatment of active oral disease into respiratory care pathways may reduce respiratory morbidity and mortality, particularly among high-risk populations such as ICU patients, older adults, and individuals with chronic lung disease. Full article

Background: Acute Respiratory Distress Syndrome (ARDS) was first described in 1967 by Ashbaugh et al. as a severe acute hypoxemic respiratory failure with reduced lung compliance, representing a common end-path of severe pulmonary endothelial inflammation from diverse etiologies. Since then, several definitions for the adult syndrome have been proposed, culminating in the 2024 “New Global Definition” (Berlin 2.0). In pediatrics, dedicated criteria (pediatric ARDS, PARDS) have been established over the past decade, with the most recent update published by the Second Pediatric Acute Lung Injury Consensus Conference (PALICC-2) in 2023. Methods: We performed a narrative literature review of consensus statements and key studies defining ARDS in adult and pediatric (non-neonatal) populations. Primary sources included the full Berlin 2.0 and PALICC-2 documents, supplemented by PubMed, Embase, and society guidelines. Definitions were compared across major diagnostic domains: timing of onset, imaging requirements, oxygenation thresholds, inclusion of patients with chronic comorbidities, ventilatory support modalities, and applicability in resource-limited settings. Results: Both definitions show convergence in incorporating non-invasive oxygenation indices and adaptability to resource-limited contexts. Key distinctions include the use of the Oxygenation Index (OI) or Oxygen Saturation Index (OSI) in invasively ventilated pediatric patients—metrics that integrate mean airway pressure and correlate more strongly than PaO₂/FIO₂ with short-term outcomes—and PALICC-2’s explicit inclusion of patients with chronic lung disease or cyanotic congenital heart disease when acute deterioration is documented. Imaging criteria differ, with Berlin 2.0 requiring bilateral opacities (and permitting lung ultrasound) versus PALICC-2’s acceptance of unilateral findings. Conclusions: Berlin 2.0 and PALICC-2 represent substantial progress toward globally applicable ARDS definitions, but physiologic and structural differences remain. These distinctions have prognostic and research implications, and harmonization will be critical to improve cross-age comparability, optimize clinical trial design, and ultimately enhance patient outcomes. Full article

Background: Lung resections in children are rare but critical for congenital lung malformations (CLMs) and acquired pathologies; few studies have analyzed the full spectrum of indications. This study evaluated indications, complications, outcomes, and temporal trends in a tertiary pediatric center. Methods: We retrospectively analyzed patients who underwent lung resection (2012–2024), focusing on indications, approaches, complications, and outcomes. Comparisons between pathologies (CLMs vs. acquired pathologies), approaches (thoracoscopy vs. thoracotomy), an temporal trends were evaluated. Results: Among 160 patients (mean age: 7.8 years), acquired lesions (68.6%) were more common than CLMs (31.4%), predominating in children under 8 years. Compared with thoracotomy, thoracoscopy (72.8% of cases, conversion rate: 22.8%) was correlated with shorter operative times (p < 0.001) and hospital stays (p = 0.001). The complication rate was 19.5%, with 71.9% of patients achieving disease-free, asymptomatic status at follow-up. Risk factors for conversion from thoracoscopy to open surgery included intraoperative adhesions (p = 0.003), underlying pathology (p = 0.013), and age < 8 years (p = 0.017). Compared with acquired lesions, CLMs were associated with fewer complications (14.3% vs. 23.1%, p = 0.041) and more favorable outcomes (89.2% vs. 64.7%; p < 0.05). Over time, the use of thoracoscopy increased (p = 0.012), with reduced operative time (p = 0.005); complication and outcome rates remained stable. Conclusions: Pediatric lung resections address diverse pathologies; outcomes are linked to the pathology, and CLMs are associated with lower complication rates in our cohort. Thoracoscopy has progressively become the preferred approach in the last decade, offering advantages particularly in postoperative recovery, though its success depends on careful, pathology-driven patient selection. Full article

Asthma management in children aims to prevent ongoing symptoms, preserve lung function and support normal daily activities. Impulse oscillometry (IOS) represents a modern approach to evaluating lung function that is also suitable for performing in the pediatric asthma population. Further research is warranted to clarify the role of IOS in the early identification of small airway disease (SAD) as a potential treatable asthma trait and to understand its implications for personalized treatment strategies. Before the integration of IOS into routine clinical protocols, it is necessary to establish population-specific reference values. Further studies in the pediatric population are needed to evaluate the added value of IOS in combination with conventional spirometry and fractional exhaled nitric oxide (FeNO). Future pediatric asthma management guidelines may consider incorporating the assessment of SAD with IOS as a possible tool for its evaluation. Full article

Background: Asthma is the most common chronic disease during childhood. Spirometry is recommended as a reliable lung function test. Several studies have demonstrated the lack of use of spirometry for both diagnostic confirmation and monitoring. Using subjective symptom control tests alone may underestimate the risk for future asthma attacks. Methods/Objectives: We conducted a retrospective, observational study in a single pediatric centre in Romania. The main objectives of the study were to analyse the quality of spirometry in children and to emphasise the importance of performing accurate spirometry for asthma monitoring. The secondary objective was to evaluate if forced expiratory volume in the first second (FEV1) and mid-maximum expiratory flow (MMEF) values are predictive markers for future exacerbations in children with asthma. Results: The study group included 416 patients between 5 and 18 years who performed at least one spirometry. The success rate for spirometry in our study was 66.3%. In a subsequent study group of 88 patients we monitored spirometry initially and after 12 months. We found a statistically significant difference between FEV1 and MMEF in the controlled, partially controlled and uncontrolled groups (p = 0.0102 and p = 0.0001). Our study showed no association between FEV1 and risk for exacerbations (Rs = −0.156, p = 0.146) and an acceptably negative (Rs = −0.30) and statistically significant (p = 0.040) correlation between initial MMEF values and the number of exacerbations. Conclusions: Low initial MMEF values correlate with the number of exacerbations in a 12-month follow-up period. This suggests that evaluating MMEF alongside FEV1 in children with asthma could contribute to better identification of the risk of exacerbation. Full article

The introduction of CFTR modulators has dramatically shifted the clinical management of cystic fibrosis (CF) from a life-limiting pediatric condition to a chronic disease with broader health implications. This review explores the impact of these advancements on lung immunology and the emerging spectrum of health challenges. While these modulators have reduced traditional pulmonary complications by mitigating inflammation and infection, they also introduce new considerations for long-term health management. As patients experience longer lives, issues such as the increased risk of certain cancers and other systemic complications like CF-related diabetes and liver disease are gaining attention. Understanding the interplay between CFTR modulators, immune response, and the development of these conditions is essential for optimizing patient outcomes. This review highlights the importance of integrated care strategies that address both the respiratory improvements and emerging health risks associated with longer life expectancy in CF patients. By fostering a comprehensive approach, we aim to enhance the overall quality of life and address the complex needs of individuals navigating CF in the modern therapeutic landscape. Full article

Obesity and asthma are increasingly prevalent chronic conditions that often coexist in the pediatric population and may influence each other through shared pathophysiological mechanisms. Obesity can affect asthma expression and severity via mechanical effects on the lungs, systemic inflammation, altered adipokine levels, and metabolic dysregulation. These mechanisms contribute to a distinct asthma phenotype in children with obesity that is often less responsive to standard therapy. Nutrition plays a critical role in this context by influencing immune function, inflammation, and respiratory outcomes. Specific dietary patterns, such as the Mediterranean diet, along with nutrients including vitamin D, antioxidants, and polyunsaturated fatty acids, have been associated with the modulation of airway inflammation and asthma risk. Additionally, early-life nutritional exposures and gut microbiota composition may influence immune development and the propensity for allergic diseases. This narrative review aims to synthesize current evidence on the interplay between obesity, asthma, and nutrition in the pediatric population, highlighting potential dietary interventions and targets for improved asthma management in children with obesity. Full article

Background/Objectives: This study involved an analysis of clinical data, histological types, genetic predisposition, treatment and outcomes in PPB in children. Patients and methods: We conducted a retrospective review of children treated for PPB at Polish pediatric oncology centers between 2011 and 2024. Results: A total of fifteen children (seven boys, eight girls; median age of 39 months; range: 27–64 months) were included. Type II solid/cystic PPB and type III solid PPB were diagnosed in six and eight children, respectively (one not known). Overall, 93% of patients were diagnosed at up to 4 years of age. Metastatic disease at diagnosis was confirmed in three (20%) patients, localized in bones, bone marrow and lymph nodes. Diagnosis was confirmed via central pathology review in 11 patients (73%). DICER1 pathogenic variants were identified in eight patients. All children presented with respiratory symptoms. The tumor dimensions were >10 cm (n = 7), 5–10 cm (n = 5) and <5 cm (n = 2). No bilateral lung involvement was observed. Tumor biopsy was performed in six children (40%), with subsequent resection (R0) in five patients. Primary resection (R0) was achieved in three patients (20%) with type II (n = 1) or type III (n = 2). In the other six patients, non-radical resection was performed: R1 in four (27%) children (with a tumor rupture in one patient) and R2 (subtotal resection) in two children (13%). All patients received postoperative chemotherapy. Maintenance chemotherapy was given to two patients. No patient received radiotherapy as first-line treatment. Progressive disease occurred in two patients in the CNS and lungs. Relapsed disease appeared in three patients, all with CNS involvement. Conclusions: PPB is a rare, malignant tumor of early childhood with an uncertain prognosis. Despite multimodal treatment, patients remain at risk of progression or CNS relapse. Complete surgical resection remains a key prognostic factor. Full article

Background: Recent hypotheses suggest that mutations associated with alpha1 antitrypsin (AAT) deficiency (AATD) may influence the clinical presentation and progression of cystic fibrosis (CF). This study employs a longitudinal design to determine the prevalence of AATD mutations and assess their impact on CF. Methods: The study Finding AAT Deficiency in Obstructive Lung Diseases: Cystic Fibrosis (FADO-CF) is a retrospective cohort study evaluating people with CF from November 2020 to February 2024. On the date of inclusion, serum levels of AAT were measured and a genotyping of 14 mutations associated with AATD was performed. Historical information, including data on exacerbations, microbiological sputum isolations, and lung function, was obtained from the medical records, aiming at a temporal lag of 10 years. Results: The sample consisted of 369 people with CF (40.9% pediatrics). Of these, 58 (15.7%) cases presented at least one AATD mutation. The AATD allelic combinations identified were PI*MS in 47 (12.7%) cases, PI*MZ in 5 (1.4%) cases, PI*SS in 3 (0.8%) cases, PI*SZ in 2 (0.5%) cases, and PI*M/P_lowell in 1 (0.3%) case. The optimal cutoff value for AAT levels to detect AATD-associated mutation carriers was 129 mg/dL in the overall cohort (sensitivity of 73.0%; specificity 69.2%) and 99.5 mg/dL when excluding PI*MS cases (sensitivity 98.0%; specificity 90.9%), highlighting the need for lower thresholds in clinically severe genotypes to improve case detection. The number of mild exacerbations during the follow-up appeared to be associated with AATD mutations. Conclusions: AATD mutations are prevalent in CF and may impact certain clinical outcomes. If systematic screening was to be planned, we recommend considering the proposed cut-off points to select the population for genetic studies. Full article

In recent years, the global prevalence of pediatric allergic diseases—including atopic dermatitis, allergic rhinitis, and asthma—has increased significantly. Accumulating evidence underscores the pivotal role of the microbiota–immune axis in the regulation of immune tolerance, wherein microbial dysbiosis is a critical driver in the onset and progression of these conditions. Notably, reduced microbial diversity and imbalanced proportions can also cause immune dysregulation and cross-organ signaling. The skin–lung–gut axis has emerged as a key conduit for multi-organ immune communication. Microbial communities at barrier sites not only mediate local immune homeostasis but also influence distant organs through metabolite production and immune signaling pathways, forming a complex network of organ crosstalk. This mechanism is integral to the maintenance of both innate (e.g., epithelial barrier integrity and phagocytic activity) and adaptive (e.g., the Type 1/Type 2 cytokine balance and regulatory T cell function) immunity, thereby suppressing allergic inflammation. Early microbial colonization is crucial for immune system maturation, and its perturbation is strongly linked to abnormal allergic immune responses. As such, the skin–lung–gut axis functions as a cross-organ microecological–immune regulatory network that is particularly relevant in the context of infantile allergic disorders. Intervention strategies targeting the microbiota—including probiotics, prebiotics, synbiotics, and postbiotics—have demonstrated potential in modulating host immunity. Furthermore, emerging approaches such as engineered probiotics, advanced delivery systems, and fecal microbiota transplantation (FMT) offer promising therapeutic avenues. This review provides a comprehensive overview of microbiota development in early life, its association with allergic disease pathogenesis, and the current progress in microbiota-targeted interventions, offering a theoretical foundation for individualized prevention and treatment strategies. Full article

Even with significant advances in therapeutic interventions and monitoring protocols, cystic fibrosis (CF) remains a critical pediatric health challenge affecting respiratory function and long-term patient outcomes. CF, caused by mutations in the CFTR gene, disrupts normal mucociliary clearance and creates conditions for chronic respiratory infections. The disorder affects individuals globally, with pediatric patients facing particularly complex microbial challenges that evolve throughout childhood growth. CF poses significant risks with progressive lung function decline and increased mortality, leading to potential short- and long-term respiratory complications. There is a growing concern among clinicians about the dynamic nature of airway microbial communities, with classical pathogens like Pseudomonas aeruginosa and Staphylococcus aureus showing sequential emergence patterns that complicate treatment strategies, highlighting an urgent need for microbiome-informed therapeutic approaches. Our review aims to provide a comprehensive overview of airway microbiome evolution in pediatric CF patients. We outline the molecular and ecological mechanisms involved in microbial community progression, as well as the age-related trajectories leading to pathogen-dominated ecosystems and the subsequent complications associated with microbial dysbiosis. Given the widespread implications of disrupted microbial balance on disease progression, our review also presents the temporal landscape of airway microbiome changes, including age-related microbial succession patterns, and explores the underlying mechanisms driving these ecological shifts. The progressive nature of microbial simplification frequently leads to treatment challenges, emphasizing the importance of investigating microbiome-targeted therapeutic interventions. Therefore, in this review, we also explore established therapeutic strategies, including CFTR modulators and probiotics, which could offer promising approaches to maintaining microbial balance and improving outcomes in pediatric CF patients. Full article