Outcomes of SARS-CoV-2 infection post-solid organ transplantation in the cystic fibrosis population In March of 2020, coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection was declared a pandemic by the World Health Organization.1 It was...

Outcomes of SARS-CoV-2 infection post-solid organ transplantation in the cystic fibrosis population www.cysticfibrosisjournal.com/article/S156... #hvhebron #pneuped

Management and Long-Term Outcomes of Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective Study Significant differences in treatment strategies for PTI/NEHI were observed across European countries, highlighting the need for evidence-based guidelines. While long-term prognosis is generally favora...

Management and Long-Term Outcomes of Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy: A European Multicenter Retrospective Study journal.chestnet.org/article/S001... #hvhebron #pneuped

Revisión de alcance de las herramientas de medición de uso de teléfonos inteligentes Los patrones de uso de pantallas han cambiado de manera significativa en los últimos años, con un aumento notable en el uso de los teléfonos inteligen…

Revisión de alcance de las herramientas de medición de uso de teléfonos inteligentes www.sciencedirect.com/science/arti... #hvhebron #pneuped [Text complet]

Impact on Body Composition After Two Years of Elexacaftor-Tezacaftor-Ivacaftor Therapy in Children with Cystic Fibrosis www.mdpi.com/2227-9067/12... #hvhebron #pneuped [Text complet]

Protocol for an open labelled observational study in children & adolescents with severe asthma with an eosinophilic phenotype treated with mepolizumab (CASAM) Introduction Children with severe eosinophilic asthma are at an increased risk of severe exacerbations, medication side effects, impaired lung function, and significantly reduced quality of life. The ...

Protocol for an open labelled observational study in children & adolescents with severe asthma with an eosinophilic phenotype treated with mepolizumab (CASAM) journals.plos.org/plosone/arti... #hvhebron #pneuped [Text complet]

SUROVA study: global real-world treatment strategies and mortality risk prediction in advanced ovarian cancer This study aimed to compare 5-year overall survival between primary debulking surgery and neoadjuvant chemotherapy followed by interval surgery in pat…

SUROVA study: global real-world treatment strategies and mortality risk prediction in advanced ovarian cancer www.sciencedirect.com/science/arti... #hvhebron #pneuped

Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort AbstractBackground and Aims. Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD). PAH following CHD repair (re

Paediatric pulmonary arterial hypertension following congenital heart defect repair: Enhanced risk stratification and outcomes in a national cohort academic.oup.com/ehjqcco/adva... #hvhebron #pneuped

Cardiorespiratory fitness in patients with post-infectious bronchiolitis obliterans: a cross-sectional study BackgroundPost-infectious bronchiolitis obliterans (PIBO) arises after lower respiratory tract infection during childhood and is marked by impaired lung function. Despite possible impacts on exercise capacity, cardiorespiratory fitness in PIBO patients remain largely unknown. This study aimed to examine cardiorespiratory fitness and identify possible demographic and clinical–functional associations.MethodsThe following variables were examined in a multicentre, cross-sectional study conducted in clinically stable PIBO patients aged 6–20 years: cardiorespiratory fitness (exercise testing), lung function (spirometry and impulse oscillometry), muscle strength (handgrip, upper and lower extremities), functional capacity (30-s sit-to-stand (STS) test), physical activity level (physical activity questionnaire adolescents and children; PAQ-A-C), body mass index (BMI), quality of life (Saint George's respiratory questionnaire; SGRQ) and dyspnoea perception (modified Medical Research Council scale).Results51 patients were enrolled (mean age 13.2±4.2 years, 49% female). BMI z-score was 0.06±1.20. Lung function tests indicated moderate-to-severe obstruction (forced expiratory volume in 1 s (FEV1) z-score −3.55±1.30) and elevated airway resistance (R5 Hz 165.8±53.64%). Peak oxygen consumption (V′O2peak) was 36.8±8.58 mL·kg−1·min−1 (47% ≤10 percentile) and breathing reserve 7.08±8.29%, indicating ventilatory limitation. Handgrip strength was 0.49±0.14 kg·kg−1. The 30-s STS test was 27.2±5.7 repetitions. PAQ-A/PAQ-C was 1.89±0.46 indicating a low physical activity level. SGRQ was 20.1±10.8%. There was no resting dyspnoea. Multivariate regression identified significant associations between V′O2peak and FEV1 (p<0.001), BMI (p<0.001), SGRQ (p=0.005), female sex (p=0.002) and age (p=0.033).ConclusionsIn these patients, cardiorespiratory fitness was impaired mainly due to ventilatory limitations. Lung function, upper and lower limb muscle strength, BMI, quality of life, sex and age were all associated with fitness level.

Cardiorespiratory fitness in patients with post-infectious bronchiolitis obliterans: a cross-sectional study publications.ersnet.org/content/erjo... #hvhebron #pneuped [Text complet]

European Respiratory Society and American Thoracic Society guidelines for the diagnosis of Primary Ciliary Dyskinesia Primary ciliary dyskinesia (PCD) is caused by pathogenetic variants in >55 genes. PCD is associated with early-onset chronic wet cough and rhinosinusitis, laterality defects, middle ear disease, and reduced fertility. The clinical presentation is heterogeneous, and diagnosis often relies on multiple tests. The American Thoracic Society (ATS) and European Respiratory Society (ERS) have previously developed separate guidelines for diagnosis. Here, ERS and ATS members systematically reviewed the literature on diagnostic tools used in practice and developed unified evidence-based guidelines for PCD diagnosis using GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology, and a transparent process of decision-making using Evidence-to-Decision (EtD) frameworks. The Task Force panel formulated three PICO (Patients, Intervention, Comparison, Outcomes) questions and three narrative questions. The accuracies of high-speed video microscopy (HSVM), immunofluorescence (IF), and nasal nitric oxide (nNO) were compared to a reference test of transmission electron microscopy (TEM) and/or genetics. The panel gives strong recommendation for use of HSVM, IF, and nNO as adjunct tests to TEM and/or genetics for PCD diagnosis. However, no adjunct test is suitable as a standalone test to diagnose PCD and no single adjunct or reference test is suitable to exclude PCD. Pursuing a genetic diagnosis is encouraged due to the implication on management. The panel emphasizes that tests should meet a minimum standard and proposes evaluation of patients at a referral centre experienced in diagnosis. The pretest probability based on symptoms should be considered when interpreting results.

European Respiratory Society and American Thoracic Society guidelines for the diagnosis of Primary Ciliary Dyskinesia publications.ersnet.org/content/erj/... #hvhebron #pneuped

First results from the international paediatric bronchiectasis registry (Child-BEAR-Net Registry) describing multicountry variations in childhood bronchiectasis and its management: a multicentre, cros... Despite increasing recognition of bronchiectasis worldwide, there are no multicountry data characterising bronchiectasis in children. We aimed to desc…

First results from the international paediatric bronchiectasis registry (Child-BEAR-Net Registry) describing multicountry variations in childhood bronchiectasis and its management: a multicentre, cross-sectional study www.sciencedirect.com/science/arti... #hvhebron #pneuped

Impact of asthma age of onset or duration on efficacy of dupilumab in moderate-to-severe type 2 asthma Age of asthma onset is critical for determining heterogeneous asthma phenotypes. How onset and duration affect therapeutic response is not well understood. Phase 3 QUEST (NCT02414854) and open-labe...

Impact of asthma age of onset or duration on efficacy of dupilumab in moderate-to-severe type 2 asthma www.tandfonline.com/doi/full/10.... #hvhebron #pneuped [Text complet]

Efficacy of a Digitally Supported Intervention for Children and Adolescents With Difficult-to-control Asthma (INSPIRINGKIDS): Results From a Multicentre Randomized Controlled Trial

Efficacy of a Digitally Supported Intervention for Children and Adolescents With Difficult-to-control Asthma (INSPIRINGKIDS): Results From a Multicentre Randomized Controlled Trial www.sciencedirect.com/science/arti... #hvhebron #pneuped

Severe Paediatric Asthma Collaborative in Europe: real-world data on children on biologics IntroductionReal-world data on children with severe asthma is scarce. We report characteristics of children with severe asthma already on biologics, enrolled in the Severe Paediatric Asthma Collaborative in Europe, a clinical research collaboration of the European Respiratory Society.MethodsWe describe patient's characteristics including asthma control assessed with Global Initiative for Asthma (GINA) criteria, composite asthma severity index (CASI), exacerbations, unscheduled medical attendances, lung function and quality of life in children on biologic treatment because of severe asthma. We also assessed previous biologics use. Forced expiratory volume in 1 s, CASI, GINA, Paediatric Asthma Quality of Life Questionnaire score, exacerbations, unscheduled medical attendance and hospital admission comparisons in patients treated with different biologics were adjusted by age, sex and biologic therapy duration.ResultsAmong the 250 children (median age 13.2 years) recruited, 56.8% used omalizumab, 21.6% mepolizumab and 21.6% dupilumab. At enrolment, the dupilumab group was older (median 15.0 years), while the omalizumab group had been on biologic treatment the longest (median 622 days). Overall, 27% and 8% had partly controlled and uncontrolled asthma respectively, according to GINA. In the last 12 months, 52% and 29% had at least one and two exacerbations, respectively; airflow obstruction was found in 33%. 10% were admitted to hospital due to exacerbation. A previous switch from another biologic was recorded in 16%, predominantly due to nonresponse.ConclusionsMost children on biologics obtained good symptom control, but many still experienced asthma attacks. Switching between biologics was substantial. There is still an unmet need in severe paediatric asthma.

Severe #Paediatric #Asthma Collaborative in Europe: real-world data on children on biologics publications.ersnet.org/content/erjo... #hvhebron #pneuped [Text complet]

Natural variability of lung function in primary #ciliary #dyskinesia: longitudinal analysis from the PROVALF-PCD cohort publications.ersnet.org/content/erjo... #hvhebron #pneuped [Text complet]

First results from the international paediatric bronchiectasis registry (Child-BEAR-Net Registry) describing multicountry variations in childhood bronchiectasis and its management: a multicentre, cros... Despite increasing recognition of bronchiectasis worldwide, there are no multicountry data characterising bronchiectasis in children. We aimed to desc…

First results from the international paediatric #bronchiectasis registry (Child-BEAR-Net Registry) describing multicountry variations in childhood bronchiectasis and its management: a multicentre, cross-sectional study www.sciencedirect.com/science/arti... #hvhebron #pneuped

WS06.01Prenatal CFTR Modulator Exposure: a Review of the European and U.S. Experience Evaluate clinical outcomes, ethical considerations, and emerging data from U.S. and European cases of prenatal elexacaftor-tezacaftor-ivacaftor (ETI) …

WS06.01Prenatal CFTR Modulator Exposure: a Review of the European and U.S. Experience www.sciencedirect.com/science/arti... #hvhebron #pneuped

Efficacy of a Digitally Supported Intervention for Children and Adolescents With Difficult-to-control Asthma (INSPIRINGKIDS): Results From a Multicentre Randomized Controlled Trial

Efficacy of a Digitally Supported Intervention for Children and Adolescents With Difficult-to-control Asthma (INSPIRINGKIDS): Results From a Multicentre Randomized Controlled Trial www.sciencedirect.com/science/arti... #hvhebron #pneuped

An update on diagnosis and treatments of childhood interstitial lung diseases Childhood interstitial lung diseases (chILDs) are rare and heterogeneous disorders associated with significant morbidity and mortality. The clinical presentation of chILD typically includes chronic or recurrent respiratory signs and symptoms with diffuse radiographic abnormalities on chest imaging. Diagnosis requires a structured, multi-step approach. Treatment options are limited, with disease-specific therapies available only in selected cases and management relying primarily on supportive care. Awareness of chILDs has been steadily increasing. New diagnoses, advanced diagnostic tests, and novel treatments are emerging each year, highlighting the importance of collaborative, multidisciplinary teams in providing comprehensive care for children and families affected by these complex conditions. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review provides an updated overview of the diagnostic approach and management strategies for chILDs.

An update on diagnosis and treatments of childhood interstitial lung diseases publications.ersnet.org/content/brea... #hvhebron #pneuped [Text complet]

Efficacy of a digitally supported intervention for children and adolescents with difficult-to-control asthma (INSPIRINGKIDS): results from a multicentre randomized controlled trial

Efficacy of a digitally supported intervention for children and adolescents with difficult-to-control asthma (INSPIRINGKIDS): results from a multicentre randomized controlled trial www.sciencedirect.com/science/arti... #hvhebron #pneuped

The In-between: Time to Talk About Bronchiectasis in Adolescents and Their Transition to Adult Care Paediatric and adult bronchiectasis patients have been addressed in the literature as two different populations due to several differences, but there …

The In-between: Time to Talk About Bronchiectasis in Adolescents and Their Transition to Adult Care www.sciencedirect.com/science/arti... #hvhebron #pneu #pneuped

DIAGNOSTIC EVALUATION AND CLINICAL FINDINGS IN CHILDREN WITH PERSISTENT TACHYPNEA OF INFANCY/NEUROENDOCRINE CELL HYPERPLASIA OF INFANCY: A EUROPEAN MULTICENTER RETROSPECTIVE STUDY Persistent tachypnea of infancy (PTI)/neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease (chILD) that …

DIAGNOSTIC EVALUATION AND CLINICAL FINDINGS IN CHILDREN WITH PERSISTENT TACHYPNEA OF INFANCY/NEUROENDOCRINE CELL HYPERPLASIA OF INFANCY: A EUROPEAN MULTICENTER RETROSPECTIVE STUDY www.sciencedirect.com/science/arti... #hvhebron #pneuped

L’amélioration de la fonction respiratoire est associée à un meilleur contrôle de l’asthme chez les enfants atteints d’asthme de type 2 modéré à sévère : étude VOYAGE Dans l’étude VOYAGE, dupilumab améliore la fonction respiratoire et le contrôle de l’asthme chez les enfants (6–11 ans) atteints d’asthme modéré à sév…

L’amélioration de la fonction respiratoire est associée à un meilleur contrôle de l’asthme chez les enfants atteints d’asthme de type 2 modéré à sévère : étude VOYAGE www.sciencedirect.com/science/arti... #hvhebron #pneuped