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.

Cystic fibrosis (CF) is one of the most common rare, genetic diseases. Newly available, highly effective modulator therapy (HEMT) improves many domains of CF pathophysiology. Thereby, HEMT challenges current standards of care and prediction models of disease outcomes. We established a public patient involvement (PPI) group including experts and caregivers of children with CF and collated a narrative review on CF management. This review is based on the views of healthcare professionals and caregivers and summarizes important features of the disease, prevention and treatment which are currently considered relevant for caregivers and Austrian healthcare settings.

Asthma is the most common chronic respiratory disease in children and adolescents. While most patients achieve good control with guideline-based treatment, a significant proportion experience persistent symptoms, frequent exacerbations, and impaired quality of life.This guideline aims to define severe and difficult-to-treat asthma in children and adolescents, support diagnostic precision, and provide practical, evidence-based recommendations for assessment and management, including biological therapies.The S1 guideline was developed under the coordination of the German Society for Pediatric Pulmonology following AWMF procedures. A structured consensus process involving experts from pediatric pulmonology, allergology, and general pediatrics was conducted. Existing national and international guidelines and new evidence were systematically reviewed and adapted.Key elements include a stepwise diagnostic algorithm to distinguish difficult-to-treat from truly severe asthma, guidance on assessing adherence, comorbidities, and inflammation biomarkers, and recommendations for targeted biological treatment. This guideline addresses monitoring tools, transition to adult care, and the role of rehabilitation.Children and adolescents with severe asthma require early referral to specialized centers and a structured, interdisciplinary approach. Personalized treatment strategies-including biologics-should be guided by phenotyping and biomarkers. Registry data are essential to improve care quality and generate real-world evidence.

INTRODUCTION:Neonatal lung biopsy guides management of unusually severe, diffuse lung disease with an uncertain diagnosis. Childhood interstitial lung disease (chILD) constitutes a diverse group of uncommon respiratory diseases which are associated with major morbidity and mortality. The incidence, outcome and mortality of chILD and other severe respiratory diseases in resource-limited settings (RLS) are unclear. OBJECTIVE:This retrospective, descriptive study examined lung biopsy in an RLS on infants up to 3 months of age to diagnose and facilitate management. This study included neonates with severe respiratory distress not responding to surfactant replacement. RESULTS:Lung biopsy was diagnostic in 94% (29/31) of patients without procedure-related mortality. The mean gestational age at birth was 35.8 weeks (SD ± 4.6). The mean presentation age was 20 days of life (IQR 1-28). Of 31 participants, 16% (n = 5) had histological findings in keeping with pulmonary interstitial glycogenosis (PIG) only. Sixteen percent (n = 5) of biopsies showed findings in keeping with adenosine triphosphate (ATP)-binding cassette subfamily A member 3 (ABCA3) deficiency in combination with features of PIG on electron microscopy. Surfactant protein (SFTP)-B deficiency and undefined surfactant deficiency were equally prevalent (6% (n = 2). Pulmonary hypertension (PHT) featured in 61% (n = 17) of infants. Genetic testing was performed on 29% (n = 9) of infants, with 56% (n = 5) showing normal results. The cohort mortality was 42% (n = 13). CONCLUSIONS:ChILD should be considered in neonates with severe respiratory distress and PHT not responding to surfactant replacement. Lung biopsy is safe and diagnostic and may prevent long and futile treatment. Histopathological diagnosis frequently assists treatment decisions. Future multicentre studies should include children from an RLS to collect information on diagnoses, genetics and treatment responses.

INTRODUCTION:Pneumocystis jirovecii pneumonia (PJP) is a significant cause of morbidity and mortality in children with advanced HIV disease (AHD) and other immunosuppressive conditions. Acquired tracheal stenosis in children living with HIV (CLHIV) has not been described. CASE PRESENTATION:A 4-month and 3-week-old child living with HIV presented with persistent respiratory symptoms after mechanical ventilation for 10 days for confirmed PJP and cytomegalovirus (CMV) pneumonia at the age of 3 months and 1 week. She tested positive for HIV at 3 months of age and had a high viral load of log 2.7 copies/mL. She was re-admitted to the PICU with multilobar pneumonia, requiring non-invasive ventilation with metapneumovirus identified from nasopharyngeal aspirate. Persistent wheeze and stridor were noted. During hospitalization, the mother was diagnosed with confirmed tuberculosis (TB). The child was referred for bronchoscopy due to the possibility of pulmonary TB and airway compression. A chest CT scan revealed short segment tracheal stenosis of >50% but no signs of TB as a possible cause. Bronchoscopy demonstrated significant narrowing occurring in the midtracheal region with the acquired nature configuration. The stenosis was successfully dilated twice, first with rigid bronchoscopy, followed by dilatation with flexible bronchoscopy and an angioplasty balloon. CONCLUSION:Acquired tracheal stenosis in CLHIV is not well documented, although many young children with HIV infection have been ventilated for severe pneumonia. Bronchoscopy should be considered in children with persistent respiratory symptoms, and endoscopic procedures can be safely performed in immunosuppressed children.

Die bronchopulmonale Dysplasie (BPD) ist eine schwerwiegende Komplikation der Frühgeburtlichkeit, die neben den parenchymatösen Lungenveränderungen auch mit pulmonalen Gefäßveränderungen und der Entwicklung einer pulmonalen Hypertonie (BPD-PH) einhergehen kann. Beeinträchtigungen der alveolären Diffusion, abnorme Gefäßumbauprozesse und eine Rarefizierung der Lungengefäße (vaskulärer Wachstumsstillstand) führen zu erhöhtem pulmonalvaskulären Widerstand und Rechtsherzinsuffizienz. Bei etwa einem Viertel aller Säuglinge mit mittelschwerer bis schwerer BPD stellt sich eine BPD-PH, die mit einer hohen Morbidität und Mortalität verbunden ist, ein. Die Entwicklung PH-spezifischer Pharmakotherapien hat neue Behandlungsmöglichkeiten für Säuglinge mit BPD-PH eröffnet. Sildenafil ist zum Hauptbestandteil der modernen BPD-PH-Therapie geworden. Weitere Medikamente wie Endothelinrezeptorantagonisten und Prostazyklinanaloga/Mimetika werden zunehmend bei Säuglingen mit einer PH untersucht. Pädiatrische Daten aus randomisierten kontrollierten Studien sind jedoch bisher kaum verfügbar.

BACKGROUND:Convergent selection has been identified in the IgE antibody repertoires of peanut-allergic individuals, primarily targeting the 2S albumin Ara h 2 and cross-reacting with two other major allergens, the vicilin Ara h 1 and the legumin Ara h 3. In this study, we aimed to investigate the structural and functional basis of this cross-reactivity and its contribution to the co-sensitization to tree nuts often observed in peanut-allergic subjects. METHODS:Six convergent antibodies, targeting the immunodominant Ara h 2-DPYSPS motif-associated sequence, and their reverted germline version, were produced as human IgG1 and IgE. Antibody specificity to natural and recombinant peanut and tree nut allergens and allergen-derived peptides was evaluated using ELISA, immunoblotting, inhibition tests, and basophil activation assays. RESULTS:The six antibodies showed reactivity to Ara h 1, Ara h 2, Ara h 3 and weak reactivity to tree nut legumins, especially from almond, walnut and Brazil nut. The germline antibody exclusively recognized Ara h 2. Basophils sensitized with the individual antibodies were activated by Ara h 2 at a concentration of 10 ng/ml and at 100-fold higher concentrations by Ara h 1 and Ara h 3, but not by tree nut legumins. The three Ara h 1- and two Ara h 3-derived antibody-binding peptides, with one from each group previously identified as immunodominant, are in close proximity and may contribute to conformational epitopes. CONCLUSION:The biological activity of affinity-matured cross-reactive antibodies with Ara h 2-associated sequence convergence may explain the high allergenic potency of peanut and clinically irrelevant co-sensitizations to tree nuts commonly observed in peanut-allergic patients.

Asthma bronchiale ist die häufigste chronische Atemwegserkrankung im Kindes- und Jugendalter. Während der Großteil der Patient:innen unter leitliniengerechter Therapie gut behandelt werden kann, leidet eine relevante Subgruppe an schwerem oder schwer behandelbarem Asthma mit eingeschränkter Lebensqualität und hoher Krankheitslast. Diese Leitlinie zielt darauf ab, schweres Asthma im Kindes- und Jugendalter klar zu definieren, eine strukturierte diagnostische und therapeutische Herangehensweise zu fördern und konkrete Empfehlungen für das Management, einschließlich des Einsatzes von Biologika, zu geben. Die Leitlinie wurde unter Federführung der Gesellschaft für Pädiatrische Pneumologie (GPP) gemäß dem AWMF-Regelwerk erstellt. Grundlage war ein systematischer Literaturreview relevanter nationaler und internationaler Empfehlungen. Ein multidisziplinäres Expertengremium stimmte alle Empfehlungen konsensbasiert ab. Im Mittelpunkt steht ein mehrstufiges diagnostisches Vorgehen zur Differenzierung zwischen schwer behandelbarem und genuin schwerem Asthma. Es werden Kriterien zu Therapieadhärenz, Komorbiditätserfassung und phänotypspezifischer Auswahl von Biologika dargestellt. Weitere Themen sind Monitoring, Rehabilitation, Transition und zukünftige Therapien. Empfehlungen zur Anwendung bestehender und neuer monoklonaler Antikörper basieren auf aktueller Evidenz. Kinder mit schwerem Asthma benötigen eine strukturierte, interdisziplinäre Versorgung. Eine frühe phänotypbasierte Therapie mit Biologika sowie flächendeckende Registerdaten und patientenorientierte Versorgungspfade sind erforderlich, um die Prognose dieser Patientengruppe zu verbessern.

Bronchopulmonary dysplasia (BPD) is a severe complication of premature birth that can be associated with pulmonary vascular changes and the development of pulmonary hypertension (BPD-PH). Impairment of alveolar diffusion, abnormal vascular remodeling and rarefication of pulmonary vessels (vascular growth cessation) lead to increased pulmonary vascular resistance and right heart failure. Approximately one quarter of all infants with moderate to severe BPD develop BPD-PH, which is associated with a high morbidity and mortality. The ongoing development of PH-specific pharmacotherapy provides new treatment options for infants with BPD-PH. Sildenafil has become the mainstay of modern BPD-PH treatment, while other medications, such as endothelin receptor antagonists and prostacyclin analogues/mimetics are increasingly being investigated; however, pediatric data from randomized controlled trials are currently scarce.

Objectives To investigate the role of both diagnostic and interventional paediatric bronchoscopy in the management of respiratory diseases in children in low- and middle-income countries (LMICs). Design A review of published English literature from January 2014 to February 2024. Results Indications for bronchoscopy in LMICs will vary depending on the burden of infectious diseases like tuberculosis (TB) and HIV, and the expertise and equipment available. TB diagnosis in children remains challenging due to the paucibacillary nature of the disease and its overlap with other infectious diseases like actinomycosis and echinococcosis. Acquired conditions, such as foreign body (FB) inhalation, present late with a high complication rate, making them challenging to manage. Paediatric bronchoscopy has an important role in the diagnoses, management and follow-up of many of these conditions. Interventional procedures like endobronchial ultrasound (EBUS), radial EBUS and cryotherapy enhance diagnostic and management capabilities. Conclusion Children in LMICs are affected by both infectious and acquired conditions. Bronchoscopy remains expensive with limited training offered in LMICs but is increasingly recognised for its important diagnostic and therapeutic role.

OBJECTIVE:RSV bronchiolitis is a leading cause of hospitalization in infants and young children. We aimed to document the economic burden and epidemiology of RSV over seven seasons in Southern Austria. PATIENTS AND METHODS:All RSV-associated hospitalized (PCR-proven) children ≤ 5 years of age between 1 October 2015 and 30 April 2022 were collected retrospectively. Demographic and epidemiologic data, along with hospitalization costs (direct and indirect), were calculated. RESULTS:Among 976 children hospitalized due to RSV infection, 87% were healthy term infants, and 79% were < 12 months old. Prematurity (13%) and pre-existing conditions (11%) significantly impacted older children-59% of cases in the 2nd compared with 68% in the 1st year of live. RSV-related hospital costs were approximately €2.0 millions per year (of a total of 60 millions per year). RSV accounted for 19% of hospitalizations due to acute respiratory illness (ARI) in children ≤ 5 years, 37% of all ARI < 6 months, 28% of all ARI < 12 and 6.3% of all-cause hospitalizations < 12 months of age, respectively. CONCLUSIONS:Every 5th hospitalization due to respiratory illness in children ≤ 5 years of age was associated with RSV, representing 7.9% of all hospitalizations and 3.3% of all paediatric hospitalization costs.