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International audience; Rationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1)

Resume L’aide au sevrage tabagique est du devoir de tout professionnel de sante. Les pediatres sont eux-memes largement concernes par cette problematique. Se basant sur le modele d’approche comportementale de Prochaska et DiClemente, le modele anglo-saxon des « 5 A » propose une demarche en cinq etapes afin d’accompagner tout fumeur a travers les differents stades du changement. Les trois premiers A (Ask, Advise, Assess) sont accessibles a tout praticien : il s’agit de depister, conseiller l’arret et evaluer la dependance, ainsi que d’apprecier la motivation a l’arret. Plusieurs outils sont a la disposition du pediatre. Les deux A suivants (Assist et Arrange) correspondent au soutien et au suivi de la personne qui passe a l’action et franchit le pas du sevrage. Le soutien psychologique et la prescription de traitements nicotiniques substitutifs quand necessaire s’inscrivent dans ces deux dernieres etapes. Plus specifiques, celles-ci restent neanmoins abordables en pratique pediatrique courante, et permettront au fumeur de retrouver sa liberte face au tabagisme. Trois situations impliqueront tout specialement le pediatre : la grossesse, l’allaitement et l’adolescent fumeur, avec des specificites qu’il faut connaitre, et pour lesquelles des benefices particulierement importants sont a attendre du sevrage.

Early diagnosis of neuroendocrine cell hyperplasia of infancy (NEHI) is crucial as, conversely to the other causes of intersititial lung disease, corticosteroids are not recommended. Diagnosis is historically based on lung biopsy (NEHI), but in current practice, a clinical and radiological approach is more and more preferred (NEHI syndrome). This national study aimed to address diagnosis and initial management of patients followed up for a NEHI pattern in pediatric centers for rare lung diseases (RespiRare, France). Data on neonatal and familial events, symptoms at diagnosis, explorations performed and results, and therapeutic management were collected by questionnaire. Fifty-four children were included (boys 63%). The mean onset of symptoms was 3.8 ± 2.6 months. The most frequent symptoms at diagnosis were tachypnea (100%), retraction (79.6%), crackles (66.7%), and hypoxemia (59.3%). The mean NEHI clinical score, evocative when ≥ 7/10, was 7.9 ± 1.4 (76% with a score ≥ 7). All chest CT-scans showed ground glass opacities evolving at least the middle lobe and the lingula. Lung biopsy was performed in 38.9% of the cases and was typical of NEHI in only 52.4%, even when the clinical presentation was typical. Initial treatments were oxygen (83.6%) and more curiously intravenous pulses of steroids (83.3%) and azithromycin (70.2%).This national cohort of patients underlines diagnosis difficulties of NEHI. A composite clinical and radiological score should help clinicians for limiting the use of anti-inflammatory drugs.•Neuroendocrine cell hyperplasia of infancy (NEHI) is an interstitial lung disease whose diagnosis is essential to limit corticosteroids therapy.•In this national cohort of 54 patients with a NEHI pattern, diagnosis is mainly based on clinical symptoms and chest CT-scan results. The newly proposed clinical score and, when performed, the lung biopsies are faulted in 25 and 50% of the cases, respectively. •Corticosteroids are widely used. Such results plead for a new composite score to formally diagnose NEHI.

International audience; Our objective was to describe and discuss management of recent cases of drug hypersensitivity in children reported in a pharmacovigilance center. Two pediatric allergy units conducted a collaborative retrospective analysis of 101 adverse drug reactions reported to a regional pharmacovigilance center between January 2016 and July 2019. Time lapse between hypersensitivity reaction onset and allergy consultation varied from 1 month to 12 years. Sixty-two patients (61.4%) presented with immediate reactions, 11 (10.9%) with non-immediate reactions, and 28 (27.7%) had reactions impossible to classify through medical interview. Overall, 92 children (91%) were explored for simultaneously administered drugs. All 113 prick tests were negative, and 2 were uncertain. Among 108 intradermal tests, 2 were positive to penicillin and to an iodinated contrast medium, 105 were negative, and 1 was uncertain. Overall, 129 drug provocation tests were proposed. Nine provocation tests among 80 were positive (11.25%): 6 to penicillin, 1 to sulfonamide antibiotics, and 2 to non-steroidal anti-inflammatory drugs; the remaining 71 were negative. No severe reaction was observed during these tests. Finally, drug allergy was only retained in 11 reported cases (10.9%). Conclusion: These pharmacovigilance reports show the difficulty in defining drug allergy in children only by anamnesis, and that explorations, particularly provocation tests, should take place at a reasonable time lapse after drug hypersensitivity reaction onset. What is Known: True drug allergy is rarely observed in children. Absence of full workup leads to falsely labeling children as ``allergic.'' What is New: Short time lapse between hypersensitivity onset and consultation improves classification of pediatric allergy. Timely allergy consultations are essential, and tests are useful to confirm or exclude pediatric allergy.

Resume Le tabagisme a ete declare « maladie pediatrique » par les instances internationales. Il peut en effet impacter la population pediatrique sous ses trois formes : tabagisme passif (in utero), environnemental (ou expositionnel), et actif a l’adolescence. Ses consequences, avec notamment un risque majore de survenue de pathologies respiratoires et infectieuses sont de mieux en mieux connues, et de nouveaux liens avec differentes pathologies telles que le diabete, la survenue de trouble de l’attention ou encore de certaines tumeurs semblent etablis. Au meme titre que la vaccination ou la prevention des accidents domestiques, le role de prevention du pediatre inclut la lutte anti-tabagique, en informant les parents des risques que represente leur consommation, et en luttant si necessaire contre un certain nombre d’idees recues. Le tabagisme des adolescents est par ailleurs un veritable fleau dont il faut se preoccuper systematiquement. On retrouve chez ces adolescents fumeurs actifs de nombreuses particularites physiologiques, psychologiques et societales quant a la consommation tabagique, au premier rang desquelles un risque de survenue de dependance particulierement rapide. La connaissance de ces particularites permettra de guider les strategies de prevention a leur egard, et ce d’autant plus qu’eux-memes sous-estiment tres largement les risques que leur propre tabagisme peut representer.

From 2015 to 2017, 3197 interpretable Bordetella polymerase chain reaction (PCR) tests were performed for 2760 children presenting to our tertiary university hospital. Requests mainly came from the emergency department (62%) and for children older than 1 year (68%). Only 32 PCR (1%) results were positive, mainly in children younger than 1 year (n = 29/32, 90.6%; p

Management of teenagers with primary spontaneous pneumothorax (PSP) is not consensual. We report our experience over an 11-year period.For each patient under 20 years hospitalised with PSP from 2008 to 2018, demographic data, smoking habits, clinical presentation, hospitalisation unit, radiological management and its results, therapeutic management (observation, needle aspiration, chest tube drainage and surgery), complications, length of stay, given advice at discharge and recurrence were collected.Seventy patients were included in different paediatric or adult surgery or pulmonology wards (82.9% boys; 16.8 ± 1.7 years; one severe presentation; 18/58 smokers). Chest CT-scan (n = 42/70, 60%) revealed blebs/bullae in 18/39 examinations (46.2%). Treatment consisted of observation (14/70, 20%), needle aspiration (2/70, 2.9%), chest tube (53/70, 75.7%) and video-assisted thoracoscopy surgery (27/70, 38.6%). Half patients with interventional procedure presented complications. A median of 10 chest X-rays was noted during a median stay of 8 days. Advice concerning sport practice, flying, smoking, etc., was variably delivered. PSP recurrence concerned 35/70 patients (50%) without identified predictive factors.Compared to recent recommendations of a more conservative approach, chest CT-scan and interventional strategy are overused in our teenagers with PSP. Observation, more or less needle aspiration, should be clearly the first-line treatments.

Introduction Aminoacyl transfer RNA (tRNA) synthetases are associated with diseases when mutations occur in their encoding genes. Pulmonary alveolar proteinosis can be caused by mutation in the methionyl-tRNA synthetase (MARS) gene while mutations in the leucine-tRNA synthetase (LARS) gene lead to infantile liver failure syndrome type 1. We report the case of a patient with LARS1 pathogenics variants and two patients with MARS1 pathogenics variants. The aim of this study was to analyze the phenotypes of our three patients in detail and classify cases in the literature using Human Phenotype Ontology (HPO) terms. Results The first patient has two previously undescribed heterozygous variants in LARS1 (c.1818dup and c.463A>G). The other two patients' MARS1 variants (c.1177G>A and c.1700C>T) have already been described in the literature. All three patients had anemia, hepatomegaly, feeding difficulties, failure to thrive and hypoalbuminemia. Including ours, 65 patients are described in total, for whom 117 phenotypic abnormalities have been described at least once, 41.9% of which both in patients with LARS1 and MARS1 mutations. Conclusion Patients with LARS1 and MARS1 mutations seem to share a common phenotype but further deep phenotyping studies are required to clarify the details of these complex pathologies.

No abstract available

Microbiome and the immune system are constantly shaping each other, in a mutual aim to thrive, defining the unstable equilibrium of the healthy individual. Microbiome is growingly involved in dysimmune conditions such as allergy, asthma, autoimmunity, and primary or acquired immune deficiencies. The current epidemics of allergic diseases and asthma has long been linked to the microbial environment through the hygiene hypothesis. Progress in the understanding of the microbiome-immune system crosstalk has unraveled a tight connection between microbial communities and the development of allergic diseases and asthma. Disruption of the microbiome affects the immune response of the host and paves the way for disease pathogenesis. Conversely, disease and therapeutic interventions affect microbial communities. We aimed at providing the reader with a view of the state-of-the art of microbiome – immune system crosstalk, with special focus on the loopholes giving potential grip to the pathogenesis of microbiome-related dysimmunity. Keywords: Microbiome, Mycobiome, Immune system, Dysbiosis, Allergy, Asthma, Atopy, Atopic dermatitis, Dendritic cell, Macrophage, Mast cell, Chronic rhinosinusitis

Background Severe asthma (SA) in children is a complex, heterogeneous disease, associated with a considerable burden. However, factors influencing asthma severity are poorly described and may differ according to age. Objective To determine whether factors associated with asthma severity differ between preschoolers with severe recurrent wheeze (SRW) and school-age children with SA. Methods Data from the French multicenter prospective observational cohort of preschool (3-6 years) children with SRW and nonsevere recurrent wheeze (NSRW) and school-age (7-11 years) children with SA and nonsevere asthma (NSA) (Pediatric Cohort of Bronchial Obstruction and Asthma) were analyzed. Results A total of 131 preschool children (92 SRW and 49 NSRW) and 207 school-age children (92 SA and 115 NSA) were included. In both univariable and multivariable analysis, SRW was associated with second-hand smoke exposure (multivariable analysis: odds ratio [95% CI], 29.8 [3.57-3910]) and exposure to mold/dampness at home (multivariable analysis: odds ratio [95% CI], 4.22 [1.25-18.2]) compared with NSRW. At school-age, history of atopic dermatitis and food allergy was more frequent in children with SA than in those with NSA. Multivariable analysis confirmed that SA was associated with a history of food allergy (odds ratio [95% CI], 5.01 [2.23-11.9]). Conclusions Our data suggest that factors influencing asthma severity may differ according to age. In preschool children with SRW, second-hand smoke and exposure to mold are predominant, whereas associated allergic disorders are mainly involved in SA at school-age.

BACKGROUND: Gain-of-function mutations in STING1 underlie a type I interferonopathy termed SAVI (STING-associated vasculopathy with onset in infancy). This severe disease is variably characterized by early-onset systemic inflammation, skin vasculopathy, and interstitial lung disease (ILD).OBJECTIVE: To describe a cohort of patients with SAVI.METHODS: Assessment of clinical, radiological and immunological data from 21 patients (17 families) was carried out.RESULTS: Patients carried heterozygous substitutions in STING1 previously described in SAVI, mainly the p.V155M. Most were symptomatic from infancy, but late onset in adulthood occurred in 1 patient. Systemic inflammation, skin vasculopathy, and ILD were observed in 19, 18, and 21 patients, respectively. Extensive tissue loss occurred in 4 patients. Severity of ILD was highly variable with insidious progression up to end-stage respiratory failure reached at teenage in 6 patients. Lung imaging revealed early fibrotic lesions. Failure to thrive was almost constant, with severe growth failure seen in 4 patients. Seven patients presented polyarthritis, and the phenotype in 1 infant mimicked a combined immunodeficiency. Extended features reminiscent of other interferonopathies were also found, including intracranial calcification, glaucoma and glomerular nephropathy. Increased expression of interferon-stimulated genes and interferon α protein was constant. Autoantibodies were frequently found, in particular rheumatoid factor. Most patients presented with a T-cell defect, with low counts of memory CD8+ cells and impaired T-cell proliferation in response to antigens. Long-term follow-up described in 8 children confirmed the clinical benefit of ruxolitinib in SAVI where the treatment was started early in the disease course, underlying the need for early diagnosis. Tolerance was reasonably good.CONCLUSION: The largest worldwide cohort of SAVI patients yet described, illustrates the core features of the disease and extends the clinical and immunological phenotype to include overlap with other monogenic interferonopathies.

International audience; Annual influenza vaccination is recommended for children with chronic diseases. Studies on influenza vaccines, following controversies related to the 2009 H1N1 influenza, are scarce in Europe. Our aim was to evaluate the influenza vaccination coverage in such children in a French tertiary hospital. Secondary objectives were the evaluation of the influenza vaccination coverage trend and the identification of factors influencing the vaccination status. A prospective and descriptive study by questionnaire was performed at the end of 2017 in 402 French hospital outpatients with various underlying chronic diseases eligible to the influenza vaccination. The 2016-2017 vaccination coverage was 46.5%. Figures of 75% or greater were only found in patients with cystic fibrosis and sickle cell disease. CART analysis identified vaccination in the previous year, medical recommendation for vaccination, and maternal influenza vaccination as a child's decisive factors for being vaccinated.Conclusion: Influenza vaccination coverage remains insufficient in children receiving hospital follow-up for chronic diseases. Its implementation clearly depends on pediatricians' recommendation to vaccinate and on the type of chronic disease. What is Known: • Despite health policy recommendations, the rate of annual influenza vaccination in children with chronic diseases is low What is New: • Influenza vaccination coverage depends on the type of chronic disease and on the pediatricians' counseling to vaccine.

We aimed to describe the real-life role of high-flow nasal cannula (HFNC) for bronchiolitis in infants under 3 months of age admitted to three general pediatric departments during the 2017-2018 epidemic period. We retrospectively assessed the clinical severity (Wang score) for every 24-h period of treatment (H0-H24 and H24-H48) according to the initiated medical care (HFNC, oxygen via nasal cannula, or supportive treatments only), the child's discomfort (EDIN score), and transfer to the pediatric intensive care unit (PICU). A total of 138 infants were included: 47±53 days old, 4661±851.9 g, 70 boys (50.7%), 58 with hypoxemia (42%), Wang score of 6.67±2.58, 110 (79.7%) staying for 48 consecutive hours in the same ward. During the H0-H24 period, only patients treated with HFNC had a statistically significant decrease in the severity score (n=21/110; -2 points, P=0.002) and an improvement in the discomfort score (n=15/63; -3.8 points, P0.0001). There was no difference between groups during the H24-H48 period. The rate of admission to the PICU was 2.9% for patients treated for at least 24 h with HFNC (n=34/138, 44% with oxygen) versus 16.3% for the others (P=0.033). Early use of HFNC improves both clinical status and discomfort in infants younger than 3 months admitted for moderately severe bronchiolitis, whatever their oxygen status.

Children are less affected by COVID-19 than adults1 . But, a small proportion of children develop paediatric inflammatory multisystem syndrome2 , with potentially lethal consequences. Studies have also described persistent long COVID symptoms that last more than four weeks from the onset of illness, including fatigue, muscle weakness, dyspnoea, chest pain, cough and anosmia2-5 . However, information on paediatric long COVID remains scarce and studies often describe hospitalised populations2,3 . We report the first 201 paediatric patients under 16 years old that were tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in Marseille public hospitals during the first wave of the COVID-19 pandemic in France. We described the acute symptoms and long-term consequences of COVID-19 in the children, who were mostly outpatients.

International audience

International audience; Non accidental intoxication due to child abuse is rare and its frequency is likely underestimated because it is difficult to diagnose. Here, we report a case of voluntary repeated exposure to lithium in an infant, for whom the clinical manifestations were convulsions. Toxicological analysis was very helpful for documenting lithium exposure during the assumed period of time. Interpreting the results of hair analysis, a simple and minimally invasive examination, is tricky at this age, but it can facilitate the differentiation of acute versus chronic exposure. Although infrequent and underestimated, lithium should be considered as a cause of intoxication in a previously healthy child with acute seizure.

BACKGROUND: It is unclear whether asthma may affect susceptibility or severity of coronavirus disease 2019 (COVID-19) in children and how pediatric asthma services worldwide have responded to the pandemic. OBJECTIVE: To describe the impact of the COVID-19 pandemic on pediatric asthma services and on disease burden in their patients. METHODS: An online survey was sent to members of the Pediatric Asthma in Real Life think tank and the World Allergy Organization Pediatric Asthma Committee. It included questions on service provision, disease burden, and the clinical course of confirmed cases of COVID-19 infection among children with asthma. RESULTS: Ninety-one respondents, caring for an estimated population of more than 133,000 children with asthma, completed the survey. COVID-19 significantly impacted pediatric asthma services: 39% ceased physical appointments, 47% stopped accepting new patients, and 75% limited patients' visits. Consultations were almost halved to a median of 20 (interquartile range, 10-25) patients per week. Virtual clinics and helplines were launched in most centers. Better than expected disease control was reported in 20% (10%-40%) of patients, whereas control was negatively affected in only 10% (7.5%-12.5%). Adherence also appeared to increase. Only 15 confirmed cases of COVID-19 were reported among the population; the estimated incidence is not apparently different from the reports of general pediatric cohorts. CONCLUSIONS: Children with asthma do not appear to be disproportionately affected by COVID-19. Outcomes may even have improved, possibly through increased adherence and/or reduced exposures. Clinical services have rapidly responded to the pandemic by limiting and replacing physical appointments with virtual encounters. (C) 2020 American Academy of Allergy, Asthma & Immunology.

We report the case of an 18-month-old infant with severe serotype 3 adenovirus pneumonia, exceptionally associated with hemophagocytic syndrome. Treatment included cidofovir and mechanical ventilation for 13 days. The child developed chronic respiratory insufficiency due to bronchiectasis and bronchiolitis obliterans. (C) 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.

Despite multiple recommendations, intramuscular epinephrine is poorly prescribed in emergency department receiving pediatric anaphylaxis. To evaluate the role of severity symptoms on this use, we included all admissions for a diagnosis linked to possible allergy in the two pediatric emergency departments of our institution between January 2010 and December 2015. Selection and analysis were restricted to children under 18 years fulfilling Sampson's criteria for anaphylaxis. We retrospectively ranked these admissions with the Ring and Messmer anaphylaxis severity score and compared the use of epinephrine according to this classification. Among 422,483 admissions, 204 (0.05%) fulfilled the anaphylaxis criteria (170 (83.3%) grade II anaphylaxis, and 34 (16.7%) grade III; mean age 7.9 years). Previous allergy, anaphylaxis, and asthma were found in respectively 60.8%, 36.8%, and 35.1%. Food allergy was the main suspected causal trigger. Epinephrine was used in 32.7% (n = 65/199), before admission (11.4% (n = 23/201)) or in the emergency department (22.2% (n = 45/202)). Epinephrine was more frequently prescribed in grade III than in grade II anaphylaxis (84.8% vs 22.3%, p 0.001; OR = 19.05 [7.05-54.10]). Upon discharge, epinephrine auto-injectors prescription and allergy referral were rare (31.7% and 44.2%).Conclusion: Pediatricians intuitively adapt their epinephrine use to the severity of the anaphylaxis and contribute to epinephrine underuse in pediatric anaphylaxis. What is known: • Intramuscular epinephrine is the recommended treatment for pediatric anaphylaxis. However, most of the European and North-American studies show a low prescription rate of epinephrine in both prehospital and pediatric emergency department management. • Reasons for such a low prescription rate are unknown. What is new: • This study confirms that intramuscular epinephrine is poorly prescribed in pediatric anaphylaxis (about one case among 10 before admission and one among 5 in pediatric emergency departments). • Despite recommendations, pediatricians intuitively adapt their prescription to the clinical severity of anaphylaxis, with a fourfold increase prescription in grade III compared to grade II anaphylaxis. This medical behavior ascertainment may be in part explained by the delay between the ED admission/management and the anaphylactic episode onset.

Ethical restrictions are limitations of in vivo inhalation studies, on humans and animal models. Thus, in vitro or ex vivo anatomical models offer an interesting alternative if limitations are clearly identified and if extrapolation to human is made with caution. This work aimed to develop an ex vivo infant-like respiratory model of bronchopulmonary dysplasia easy to use, reliable and relevant compared to in vivo infant data. This model is composed of a 3D-printed head connected to a sealed enclosure containing a leporine thorax. Physiological data and pleural-mimicking depressions were measured for chosen respiratory rates. Homogeneity of ventilation was assessed by 81mkrypton scintigraphies. Regional radioaerosol deposition was quantified with 99mtechnetium-diethylene triamine pentaacetic acid after jet nebulization. Tidal volumes values are ranged from 33.16 ± 7.37 to 37.44 ± 7.43 mL and compliance values from 1.78 ± 0.65 to 1.85 ± 0.99 mL/cmH2O. Ventilation scintigraphies showed a homogenous ventilation with asymmetric repartition: 56.94% ± 9.4% in right lung and 42.83% ± 9.36 in left lung. Regional aerosol deposition in lungs exerted 2.60% ± 2.24% of initial load of radioactivity. To conclude the anatomical model satisfactorily mimic a 3-months old BPD-suffering bronchopulmonary dysplasia and can be an interesting tool for aerosol regional deposition studies.

This article reports an exchange of unbiased arguments between Mr Guy Postiaux speaking in favour of respiratory physiotherapy in acute viral bronchiolitis in the newborn and Prof. Jean-Christoph Dubus arguing against. A review of the literature suggests that traditional methods of physiotherapy should be abandoned because they are not validated and because they have harmful side effects. The latest Cochrane revue (2016) suggests the use of slow expiration techniques that have some validated elements and cause no harmful side effects. Large multicentre studies should be undertaken to confirm or refute the results of the five studies in the Cochrane review. Their analysis would allow extraction of objective evidence for the efficacy of slow expiration techniques on the relief of bronchopulmonary obstruction and the reduction of the degree of severity in the short and medium term. Studies of the effect of ambulatory respiratory physiotherapy for bronchiolitis of a moderate degree not requiring hospitalisation are not available. An evaluation is needed which is based on the pathophysiology of multifactorial bronchial obstruction and on the physical signs, of which auscultation is the cornerstone.

Resume Le piercing se pratique sur des sites corporels divers parmi lesquels les oreilles et notamment le cartilage ont un franc succes. Nous rapportons deux cas d’adolescentes ayant presente une chondrite a Pseudomonas aeruginosa liee a ces piercings.

Introduction La contribution de la reponse IgE aux exacerbations et a la deterioration a long terme de la fonction respiratoire chez les patients atteints de mucoviscidose est acceptee, mais souvent difficile a evaluer. Nous faisons l’hypothese que les nouveaux outils de type biopuce a allergenes peuvent contribuer a mieux caracteriser les reponses IgE pathologiques au niveau pulmonaire. Methodes Nous avons mis au point une methode de detection et de caracterisation des IgE specifiques locales (aspiration bronchique ou crachat) de patients atteints de mucoviscidose. Notre methode consiste en une fluidification puis une lyophilisation des expectorations, avant la realisation d’un biopuce a allergenes (ALEX®, Vienne, Autriche). Resultats Apres la mise au point et optimisation de la methode, nous avons etudie les expectorations de 22 patients mucoviscidosiques, dont 5 transplantes pulmonaires. L’âge etait comparable dans les deux groupes, avec une mediane de 29 ans (extremes 17–72 ans) chez les patients non transplantes et 39 ans (18/53) chez ceux transplantes. Les patients non transplantes presentaient une reponse IgE significativement plus elevee vis-a-vis de plusieurs allergenes environnementaux, notamment les phaneres (p = 0.0004) et les acariens (p = 0,03). La reponse IgE aux moisissures environnementales ne differait pas significativement entre les deux groupes de patients et n’etait pas correlee a l’eventuelle colonisation fongique, cependant les IgE locales vis-a-vis des allergenes fongiques, notamment Cladosporium herbarum, etaient correlees a la presence d’une symptomatologie aigue en cours. Sur le plan methodologique, la richesse en IgE des aspirations bronchiques etait significativement inferieure a celle des crachats. Discussion Nous apportons la preuve de concept d’un outil non invasif pour l’exploration large de l’immunite humorale locale chez des patients mucoviscidosiques. Conclusion L’exploration des prelevements respiratoires par biopuce pourrait apporter des elements importants pour identifier les causes d’exacerbations chez ces patients et contribuer a une prise en charge rapide et adequate.

International audience; Objective To investigate the impact of neighborhood conditions on respiratory-related hospital admissions in the first year after discharge from the neonatal unit in a population of infants born very preterm with bronchopulmonary dysplasia (BPD).Study design Very preterm infants (gestational age

Background Anaphylaxis appears to be heterogeneous with regards to patients’ characteristics, clinical manifestations, triggers, and severity. Objective We set out to define anaphylaxis phenotypes using a non-supervised cluster analysis in children experiencing anaphylaxis. Methods Characteristics of children ( Results (See Fig. 1 ) Two hundre and twenty two (male: 62%, mean age: 7.9 y; SD: 5.1) children were included. Two anaphylaxis clusters have been described. Cluster 1, “Food (nuts) severe anaphylaxis in highly atopic children” (n = 56, 25%): all children had food-induced anaphylaxis, most often induced by peanut (38%) and nuts (36%), and a previous food allergy to the same trigger (89%). These children had allergic comorbidities (multiple food allergy, 77%; asthma, 63%; atopic dermatitis, 36%) and had more severe anaphylaxis reactions (Ring score 3–4, 45%; adrenaline injection, 46%; hospital admission, 36%). Cluster 2, “Mild anaphylaxis with various triggers and few atopic comorbidities” (n = 168, 75%): these children were younger (p = 0.039), had less frequent allergic disease (asthma, 31%; food allergy, 16%; atopic dermatitis, 17%), with various triggers (food, 68%; drugs, 10%; insect venom, 7%) and less severe reactions (Ring 2, 78%; adrenaline injection, 26%; hospital admission, 20%). Conclusion We confirm the heterogeneity of anaphylaxis in children. Children with allergic comorbidities and food triggers (nuts) are at risk of more severe anaphylaxis reactions as compared to children with less allergic disease and other triggers than food. Larger studies are required to confirm these anaphylaxis phenotypes in children.

International audience; A wide spectrum of pathological conditions may result from the interaction of Aspergillus fumigatus and the immune system of its human host. Allergic bronchopulmonary aspergillosis is one of the most severe A.fumigatus-related diseases due to possible evolution toward pleuropulmonary fibrosis and respiratory failure. Allergic bronchopulmonary aspergillosis occurs almost exclusively in cystic fibrosis or asthmatic patients. An estimated 8%-10% of patients with cystic fibrosis experience this condition. The diagnosis of allergic bronchopulmonary aspergillosis relies on criteria first established in 1977. Progress in the understanding of host-pathogen interactions in A.fumigatus and patients with cystic fibrosis and the ongoing validation of novel laboratory tools concur to update and improve the diagnosis of allergic bronchopulmonary aspergillosis.

Resume Introduction L’aerosoltherapie est le traitement privilegie de l’asthme par son action locale et rapide. Cependant, son efficacite depend de la technique d’inhalation apprise aupres des professionnels de sante. Nous avons evalue les connaissances en aerosoltherapie du personnel hospitalier. Methodes Il s’agit d’une etude observationnelle multicentrique effectuee au sein des services de pneumologie et de pediatrie de Marseille. Les pratiques de service, les connaissances pratiques et theoriques sur l’aerosoltherapie, du personnel medical et paramedical etaient evaluees par l’intermediaire d’un questionnaire et d’une demonstration a l’utilisation d’une chambre d’inhalation. Resultats Quarante personnels medicaux et paramedicaux (9 medecins, 14 internes, 16 infirmieres, 1 kinesitherapeute) ont ete interroges dans 8 services de pediatrie et de pneumologie. Tous etaient impliques dans la mise en route de traitements inhales. Une formation a l’aerosoltherapie avait ete suivie par 42,5 %. Concernant les connaissances theoriques, on notait 54 % de reponses justes. On notait en moyenne 1,12 erreurs sur 7 sur la demonstration avec la chambre d’inhalation (45 % sans erreur). Les medecins connaissaient statistiquement mieux l’aerosoltherapie que les internes et les infirmieres. Conclusion Les connaissances parcellaires sur l’aerosoltherapie des personnels hospitaliers plaident pour une meilleure formation de ces personnels.

International audience; Children's asthma is multifactorial. Environmental factors like air pollution exposure, meteorological conditions, allergens, and viral infections are strongly implicated. However, place of residence has rarely been investigated in connection with these factors. The primary aim of our study was to measure the impact of particulate matter (PM), assessed close to the children's homes, on asthma-related pediatric emergency hospital visits within the Bouches-du-Rhône area in 2013. In a nested case-control study on 3- to 18-year-old children, each control was randomly matched on the emergency room visit day, regardless of hospital. Each asthmatic child was compared to 15 controls. PM10 and PM2.5, meteorological conditions, pollens, and viral data were linked to ZIP code and analyzed by purpose of emergency visit. A total of 68,897 visits were recorded in children, 1182 concerning asthma. Short-term exposure to PM10 measured near children's homes was associated with excess risk of asthma emergency visits (adjusted odds ratio 1.02 (95% CI 1.01-1.04; p = 0.02)). Male gender, young age, and temperature were other risk factors. Conversely, wind speed was a protective factor.CONCLUSION:PM10 and certain meteorological conditions near children's homes increased the risk of emergency asthma-related hospital visits in 3- to 18-year-old children in Bouches-du-Rhône. What is Known: • A relationship between short-term exposure to air pollution and increase in emergency room visits or hospital admissions as a result of increased pollution levels has already been demonstrated. What is New: • This study confirms these results but took into account confounding factors (viral data, pollens, and meteorological conditions) and is based on estimated pollution levels assessed close to the children's homes, rather than those recorded at the hospital. • The study area, the Mediterranean, is favorable to creation of secondary pollutants in these sunny and dry seasons.

Resume Introduction La delivrance de fluticasone via une chambre d’inhalation est delicate chez les jeunes nourrissons asthmatiques. Le choix du dispositif est souvent difficile pour le clinicien. Cette etude caracterise in vitro la granulometrie et la delivrance medicamenteuse de la fluticasone par neuf chambres d’inhalation. Methodes Nous avons utilise un modele anatomique in vitro de nourrisson avec deux debits inspiratoires differents (50 et 100 mL/s). La granulometrie de l’aerosol etait evaluee par diffractometrie laser et le depot tracheal par dosage spectrophotometrique. Resultats Des differences granulometriques importantes entre les chambres d’inhalation etaient observees (D50 identique mais D90 de 5,65 ± 0,65 μm a 8,80 ± 1,35 μm). Une fraction respirable superieure a 75 % n’etait obtenue qu’avec cinq chambres d’inhalation. Le debit a 50 mL/s permettait d’obtenir la meilleure delivrance medicamenteuse. Pour ce debit, Optichamber® (62 ± 3 %) et Vortex® (91 ± 8,5 %) avaient un depot tracheal superieur a 50 % de la dose initiale de fluticasone, les sept autres chambres d’inhalation presentant un depot tracheal inferieur a 25 %. Discussion Notre etude montre de fortes variations de delivrance entre les neuf chambres d’inhalation. Un debit inspiratoire faible et une chambre d’inhalation de nature antistatique semblent des elements favorisant cette delivrance.

Objective. A management protocol for infants hospitalized for acute bronchiolitis, established after the study conducted in our unit in 2012, recommends a chest X-ray when the clinical course is unusual or if a differential diagnosis is suspected. The goal of this study was to evaluate professional practices after the introduction of this new management protocol. Study design. Retrospective descriptive study in two pediatric units from October 2013 to March 2015, including infants (0-23 months) hospitalized for their first episode of acute bronchiolitis without any underlying chronic condition. Result. Overall, 599 infants were included (median age, 3.7 months, 54 % boys). Nearly six out of ten (n = 355, 59.3 %) had at least one chest radiograph (38.5 % fewer than in 2012). It was abnormal in 96.3 % of cases, revealing distension and/or bronchial wall thickening (56.7 %), focal opacity (23.5 %), or atelectasis (19.5 %). An X-ray was performed out of the recommendations in 42.5 % of cases. The chest X-ray result led to management changes in 52 infants with prescription of antibiotics for pneumonia (86.5 %) and allowed the diagnosis of heart disease in one case (0.2 %). Management of acute bronchiolitis (X-ray and antibiotics) was statistically different between the two pediatric units. Discussion. This protocol led to a significant decrease in the number of chest X-rays. However, many are still performed out of the recommendations, resulting in an increase of antibiotic use for pneumonia. Conclusion. The decrease in use of chest X-rays in acute bronchiolitis for hospitalized infants was significant but remains insufficient. (C) 2016 Elsevier Masson SAS. All rights reserved.

International audience; Thoracic trauma is a major source of morbi-mortality in injured children. Their pliable chest wall makes pulmonary contusion the most common chest injury. It is most often secondary to blunt trauma caused by traffic accidents. We report a case of severe chest trauma caused by a bumper car collision in an 8-years old girl. She sustained right lung contusion that led to complete atelectasis. After a week of supportive therapy, bronchoscopy removed a mucous plug from the main bronchus, resulting in significant clinical improvement. We aim to raise awareness of the risk of severe chest injuries during bumper car collisions.

Outdoor pollution is a complex mix of more than 200 air contaminants. Among these pollutants, ozone, nitrogen dioxide and fine particles may generate bronchial inflammation and hyperreactivity. The hypothesis that pollution contributes to the development of asthma in children is based on epidemiological, clinical and experimental data. Many risk factors during the in utero and postnatal period have been identified in the aetiology of childhood asthma. During pregnancy, outdoor pollution was identified as a causal factor of respiratory disease in neonatal cohort studies. Several epidemiological studies also demonstrate that outdoor pollution is a trigger of asthma exacerbations. This review aims to highlight the current knowledge on outdoor pollution and asthma.

International audience; Rationale: Lumacaftor-ivacaftor is a CFTR (cystic fibrosis transmembrane conductance regulator) modulator combination recently approved for patients with cystic fibrosis (CF) homozygous for the Phe508del mutation.Objectives: To evaluate the safety and effectiveness of lumacaftor-ivacaftor in adolescents (≥12 yr) and adults (≥18 yr) in a real-life postapproval setting.Methods: The study was conducted in the 47 CF reference centers in France. All patients who initiated lumacaftor-ivacaftor from January 1 to December 31, 2016, were eligible. Patients were evaluated for lumacaftor-ivacaftor safety and effectiveness over the first year of treatment following the French CF Learning Society's recommendations.Measurements and Main Results: Among the 845 patients (292 adolescents and 553 adults) who initiated lumacaftor-ivacaftor, 18.2% (154 patients) discontinued treatment, often owing to respiratory (48.1%, 74 patients) or nonrespiratory (27.9%, 43 patients) adverse events. In multivariable logistic regression, factors associated with increased rates of discontinuation included adult age group, percent predicted FEV1 (ppFEV1) less than 40%, and numbers of intravenous antibiotic courses during the year before lumacaftor-ivacaftor initiation. Patients with continuous exposure to lumacaftor-ivacaftor showed an absolute increase in ppFEV1 (+3.67%), an increase in body mass index (+0.73 kg/m2), and a decrease in intravenous antibiotic courses by 35%. Patients who discontinued treatment had significant decrease in ppFEV1, without improvement in body mass index or decrease in intravenous antibiotic courses.Conclusions: Lumacaftor-ivacaftor was associated with improvement in lung disease and nutritional status in patients who tolerated treatment. Adults who discontinued lumacaftor-ivacaftor, often owing to adverse events, were found at high risk of clinical deterioration.

High-flow nasal cannula (HFNC) is frequently used in infants with acute viral bronchiolitis outside pediatric intensive care units (PICU). A structured questionnaire was sent out to pediatricians of all public French hospitals with pediatric emergency and/or general pediatric departments on their use of HFNC outside PICU (department using HFNC, number of available devices, monitoring, criteria for initiating or stopping HFNC, and personal comments on HFNC). Of the 166 eligible hospitals, 135 answered (96 general and 39 university hospitals; 81.3%), for a total of 217 answering pediatricians. Seventy-two hospitals (53.3%) used HFNC in acute bronchiolitis outside PICU, particularly, general hospitals (59.4% vs 38.5%), and mostly in pediatric general departments (75%). Continuous patient monitoring with a cardiorespiratory monitor was usual (n = 58, 80%). Nursing staff was responsible for 2.7 children on HFNC and checked vital signs 8.6 times per day. Criteria for HFNC initiation and withdrawal were not standardized. Pediatricians had a positive opinion of HFNC and were willing to extend its use to other diseases.Conclusion: Use of HFNC outside PICU in infants with acute bronchiolitis is now usual, but urgently requires guidelines. What is Known: • Acute viral bronchiolitis treatment is only supportive • High-flow nasal cannula (HFNC) is a respiratory support accumulating convincing clinical evidence in bronchiolitis • This latter treatment is usually proposed in pediatric intensive care unit (PICU) What is New: • HFNC are increasingly used outside PICU in bronchiolitis, particularly, in general hospitals and in pediatric general departments • Pediatricians are enthusiastic about this device, but validated criteria for initiation and withdrawal are lacking • Guidelines for the use of HFNC outside PICU are urgently required.

Introduction: Health-related quality of life (HR-QoL) is an understudied area in rare diseases. Interstitial lung diseases in children (chILD) are heterogeneous disorders that are associated with a high morbidity and mortality. The impaired lung function, the nutritional impact and the burden of the treatments can impair the HR-QoL of these children. The aim of this study was to compare the HR-QoL in chILD to an aged-matched healthy population and to identify the factors that were associated to an altered QoL. Methods: Patients were recruited in the French RespiRare network. Patients with chILD of all causes, aged 1 month to 18 years were prospectively included. The children over 8 years old and the 2 parents of all children were asked to complete the PedsQLTM 4.0 Generic Core Scale (0 to 100 points). Results: A total of 78 children were included in the RespiRare centres. Total mean scores appeared to be significantly reduced in child self-report and in parent proxy-report (11.94 points (p=0.0003) and 14.08 points (p Conclusion: The study shows that HR-QoL is significantly impaired in chILD compared to a healthy population, using a validated generic QoL scale. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL. A longitudinal follow-up of HR-QoL in chILD is necessary to follow the impact of the disease and the treatments. To better appreciate chILD QoL, a specific scale is currently under study by the chILD-EU group.