This review demonstrates that incorrect DPI technique with established DPIs is common among patients with asthma and COPD, and suggests that poor inhalation technique has detrimental consequences for clinical efficacy. Regular assessment and reinforcement of correct inhalation technique are considered by health professionals and caregivers to be an essential component of successful asthma management. Improvement of asthma and COPD management could be achieved by new DPIs that are easy to use correctly and are forgiving of poor inhalation technique, thus ensuring more successful drug delivery.

What is already known about this topic? Coronavirus disease 2019 has a mild disease course in children and adolescents. Chronic respiratory conditions, including asthma, have been suggested as risk factors; however, asthma in children is highly variable in both triggers and severity.What does this article add to our knowledge? During the pandemic, pediatric asthma services limited consultations and established virtual clinics. However, respondents perceived their patients' asthma control to be retained or even improved, while treatment adherence was considered increased. Children with asthma were not disproportionately affected by coronavirus disease 2019.How does this study impact current management guidelines? Trigger avoidance and treatment adherence can rapidly improve asthma control in children, even under lockdown pressure. Children/adolescents with asthma do not appear to need additional prophylactic measures from coronavirus disease 2019 when asthma is well-treated.

Omalizumab has been shown to reduce exacerbation rates in moderate to severe allergic asthma. Our aim was to evaluate omalizumab efficacy and safety in a real-life setting in severe asthmatic children.104 children (aged 6-18 years), followed up in paediatric pulmonary tertiary care centres, were included at the beginning of omalizumab treatment. Asthma control levels, exacerbations, inhaled corticosteroid dose, lung function and adverse events were evaluated over 1 year.Children were characterised by allergic sensitisation to three or more allergens (66%), high IgE levels (mean 1125 kU?L -1 ), high rate of exacerbations (4.4 per year) and healthcare use during the previous year, and high inhaled corticosteroid dose (mean 703 mg equivalent fluticasone per day). Asthma control levels defined as good, partial or poor, improved from 0%, 18% and 82% at entry to 53%, 30% and 17% at week 20, and to 67%, 25% and 8% at week 52, respectively (p,0.0001). Exacerbation and hospitalisation rates dropped by 72% and 88.5%, respectively. At 12 months, forced expiratory volume in 1 s improved by 4.9% (p50.023), and inhaled corticosteroid dose decreased by 30% (p,0.001). Six patients stopped omalizumab for related significant adverse events.Omalizumab improved asthma control in children with severe allergic asthma and was generally well tolerated. The observed benefit was greater than that reported in clinical trials. @ERSpublications Omalizumab improves asthma control in children with severe allergic asthma and is generally well tolerated

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We previously reported the French real-life experience of 1 year of add-on treatment with omalizumab in 101 severe allergic asthmatic children (6-18 years), 92 of whom were still receiving the treatment at the end of the first year [1]. The study provided complementary data to the previous randomised trials [2-6]. We showed a marked drop of 72% in the mean rate of severe exacerbations (from 4.4 per patient during the preceding year to 1.25 during the year of treatment) and of 88.5% for hospitalisations (44% of the patients during the preceding year to 6.7% during the year of treatment); a large improvement in asthma control (from 0% at initiation to 67% of well-controlled patients after 1 year); a decrease of 30% of the mean inhaled corticosteroid (ICS) dose (from 703 at initiation to 488 µg fluticasone equivalent per day after 1 year); and a forced expiratory volume in 1 s (FEV1) increase, from a mean of 88% to 92.1% of the predicted value. Treatment was discontinued in six patients due to serious adverse events attributed to omalizumab by the practitioner. Here we report the outcome of this cohort after 2 years of omalizumab treatment.

Methionyl-tRNA synthetase (MARS) catalyzes the ligation of methionine to tRNA and is critical for protein biosynthesis. We identified biallelic missense mutations in MARS in a specific form of pediatric pulmonary alveolar proteinosis (PAP), a severe lung disorder that is prevalent on the island of Réunion and the molecular basis of which is unresolved. Mutations were found in 26 individuals from Réunion and nearby islands and in two families from other countries. Functional consequences of the mutated alleles were assessed by growth of wild-type and mutant strains and methionine-incorporation assays in yeast. Enzyme activity was attenuated in a liquid medium without methionine but could be restored by methionine supplementation. In summary, identification of a founder mutation in MARS led to the molecular definition of a specific type of PAP and will enable carrier screening in the affected community and possibly open new treatment opportunities.

BackgroundInterstitial lung diseases (ILDs) in children represent a heterogeneous group of rare respiratory disorders that affect the lung parenchyma. After the launch of the French Reference Centre for Rare Lung Diseases (RespiRare®), we created a national network and a web-linked database to collect data on pediatric ILD.MethodsSince 2008, the database has been set up in all RespiRare® centres. After patient's parents' oral consent is obtained, physicians enter the data of children with ILD: identity, social data and environmental data; specific aetiological diagnosis of the ILD if known, genetics, patient visits to the centre, and all medical examinations and tests done for the diagnosis and/or during follow up. Each participating centre has a free access to his own patients' data only, and cross-centre studies require mutual agreement. Physicians may use the system as a daily aid for patient care through a web-linked medical file, backed on this database.ResultsData was collected for 205 cases of ILD. The M/F sex ratio was 0.9. Median age at diagnosis was 1.5 years old [0–16.9]. A specific aetiology was identified in 149 (72.7%) patients while 56 (27.3%) cases remain undiagnosed. Surfactant deficiencies and alveolar proteinosis, haemosiderosis, and sarcoidosis represent almost half of the diagnoses. Median length of follow-up is 2.9 years [0–17.2].ConclusionsWe introduce here the French network and the largest national database in pediatric ILDs. The diagnosis spectrum and the estimated incidence are consistent with other European databases. An important challenge will be to reduce the proportion of unclassified ILDs by a standardized diagnosis work-up. This database is a great opportunity to improve patient care and disease pathogenesis knowledge. A European network including physicians and European foundations is now emerging with the initial aim of devising a simplified European database/register as a first step to larger European studies.

ABCA3 (ATP-binding cassette subfamily A, member 3) is expressed in the lamellar bodies of alveolar type II cells and is crucial to pulmonary surfactant storage and homeostasis. ABCA3 gene mutations have been associated with neonatal respiratory distress (NRD) and pediatric interstitial lung disease (ILD). The objective of this study was to look for ABCA3 gene mutations in patients with severe NRD and/or ILD. The 30 ABCA3 coding exons were screened in 47 patients with severe NRD and/or ILD. ABCA3 mutations were identified in 10 out of 47 patients, including 2 homozygous, 5 compound heterozygous and 3 heterozygous patients. SP-B and SP-C expression patterns varied across patients. Among patients with ABCA3 mutations, five died shortly after birth and five developed ILD (including one without NRD). Functional studies of p.D253H and p.T1173R mutations revealed that p.D253H and p.T1173R induced abnormal lamellar bodies. Additionally, p.T1173R increased IL-8 secretion in vitro. In conclusion, we identified new ABCA3 mutations in patients with life-threatening NRD and/or ILD. Two mutations associated with ILD acted via different pathophysiological mechanisms despite similar clinical phenotypes.