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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)

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.

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

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; 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.

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.

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.

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; Fifty-two meshes of e-flow rapid® were characterized for tobramycin delivery with a laser diffractometer after 6 months of home use by cystic fibrosis patients treated with various nebulized drugs. Three meshes were out of order and 30 considered to be defective for tobramycin delivery. The use of the specific mesh cleaning shower system permitted 14 defective meshes to be in the expected range of nebulized volume.

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.

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.

International audience; Introduction: Interstitial lung disease in children (chILD) is a highly heterogeneous group of rare and severe respiratory disorders. The disease by itself, the burden of the treatments (oxygen therapy, corticosteroid pulses, nutritional support) and recurrent hospitalizations may impair the quality of life (QoL) of these children. The aim of the study was to compare the health-related QoL (HR-QoL) in chILD compared to a healthy population and to find out the predictive factors of an altered QoL.Methods: Patients aged 1 month to 18 years with ILD of known or unknown etiology were prospectively included. Parents and children over 8 years old were asked to fill the PedsQL 4.0 Generic Core Scale ranging from 0 to 100 points.Results: A total of 78 children were recruited in 13 French pediatric centers. Total scores were 11.94 points (P = 0.0003) less for child self-report and 14.08 points ( P < 0.0001) less for parent proxy-report with respect to the healthy population. The clinical factors associated with a lower total score were: extrapulmonary expression of the disease, higher Fan severity score, long-term oxygen therapy, nutritional support, and a number of oral treatments.Conclusion: Using a validated quality of life (QoL) scale, we showed that health-related-QoL is significantly impaired in chILD compared with a healthy population. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL

Bronchiectasis is a rare chronic airway disease arising from several respiratory and systemic diseases. The grade of evidence for specific treatment of childhood bronchiectasis unrelated to cystic fibrosis (CF) is low with very few randomized controlled trials. Treatment has been based mainly on evidence from studies in adults with non-cystic fibrosis bronchiectasis and patients with cystic fibrosis. Recently, long-term treatment with macrolides has been proposed. These molecules offer the advantage of anti-inflammatory and immunomodulatory properties in addition to their antibacterial properties. A total of three randomized double-blind placebo-controlled trials conducted in adults showed that macrolides taken for 6-12 months led to a significant reduction in exacerbation rates. Only one long-term, randomized double-blind placebo-controlled trial was conducted in the pediatric population. It showed that azithromycin administered weekly for up to 24 months reduced pulmonary exacerbations. Further randomized controlled studies are needed to determine the optimal dose and duration of treatment with macrolides. The clinical profile of children who would benefit from this treatment also needs to be determined.

Viral infections are known to lead to serious respiratory complications in cystic fibrosis (CF) patients. Hypothesizing that CF patients were a population at high risk for severe respiratory complications from SARS-CoV-2 infection, we conducted a national study to describe the clinical expression of COVID-19 in French CF patients. This prospective observational study involves all 47 French CF centers caring for approximately 7500 CF patients. Between March 1st and June 30th 2020, 31 patients were diagnosed with COVID-19: 19 had positive SARS-CoV-2 RT-PCR in nasopharyngeal swabs, 1 had negative RT-PCR but typical COVID-19 signs on a CT scan, and 11 had positive SARS-CoV-2 serology. Fifteen were males, median (range) age was 31 (9&ndash, 60) years, and 12 patients were living with a lung transplant. The majority of the patients had CF-related diabetes (n = 19, 61.3%), and a mild lung disease (n = 19, 65%, with percent-predicted forced expiratory volume in 1 s (ppFEV1) &gt, 70). Three (10%) patients remained asymptomatic. For the 28 (90%) patients who displayed symptoms, most common symptoms at admission were fever (n = 22, 78.6%), fatigue (n = 14, 50%), and increased cough (n = 14, 50%). Nineteen were hospitalized (including 11 out of the 12 post-lung transplant patients), seven required oxygen therapy, and four (3 post-lung transplant patients) were admitted to an Intensive Care Unit (ICU). Ten developed complications (including acute respiratory distress syndrome in two post-lung transplant patients), but all recovered and were discharged home without noticeable short-term sequelae. Overall, French CF patients were rarely diagnosed with COVID-19. Further research should establish whether they were not infected or remained asymptomatic upon infection. In diagnosed cases, the short-term evolution was favorable with rare acute respiratory distress syndrome and no death. Post-lung transplant patients had more severe outcomes and should be monitored more closely.

Introduction. Even if there are HAS (French National Health Authority) guidelines on shaken baby syndrome, many other child abuse situations are not included in these recommendations. The aim of this study was to invent the complementary exams in cases of child abuse in France and compare the practice to existing guidelines. Material and methods. This was a multicenter study by email to 128 French hospitals (35 university hospitals and 93 general hospitals) that receives children in emergency and hospitalization settings. Three child abuse clinical situations were included in a clinical case multiple-choice format concerning the further explorations. We described the main results and evaluated their adherence to the HAS protocol for case 1. Results. Of 128 hospitals surveyed, 104 responded, for an 81% response rate, which corresponded to 274 doctors. Analysis of the results showed great heterogeneity in practices. The majority of physicians (99%) performed systematic explorations in the situation of physical abuse, while only 27% undertook such exams in situations of serious neglect. The situation of sexual abuse was the most consensual in terms of diagnostic tests for the detection of sexually transmitted diseases, but other types of associated abuse were not sought. In the first case, the HAS guidelines were respected in less than half of the cases for all complementary exams except the eye fundus exam. Abdominal imaging was insufficiently performed (40% of cases). Examinations that were not indicated were still prescribed. Moreover, siblings under 2 years of age were examined in only one-third of cases (n = 88/274; 32%). Practices were not influenced by the age of the child. Conclusion. This study illustrates the heterogeneity in the use of complementary exams in cases of child abuse in France. Common protocols throughout the country would be useful, standardizing the most relevant exams for potential medical-legal issues, and facilitating exchanges concerning practices between different centers. (C) 2016 Elsevier Masson SAS. All rights reserved.

We report the case of a 21-month-old child suffering from pulmonary fibrosis, who presented with acute respiratory distress and liver damage, due to an accidental overdose of intravenous lipid emulsion. This poisoning is a rare entity, whose potential severity and almost exclusive iatrogenic effect deserve to be remembered.

Pediatric eosinophilic pneumonias (EPs) are characterized by a significant infiltration of the alveolar spaces and lung interstitium by eosinophils, with conservation of the lung structure. In developed countries, EPs constitute exceptional entities in pediatric care. Clinical symptoms may be transient (Loffler syndrome), acute ( 1 month). Diagnosis relies on demonstration of alveolar eosinophilia on bronchoalveolar lavage, whether or not associated with blood eosinophilia. EPs are a heterogeneous group of disorders divided into: (i) secondary forms (seen mainly in parasitic infections, allergic bronchopulmonary aspergillosis, and drug reactions); and (ii) primary forms (eosinophilic granulomatosis with polyangiitis, hypereosinophilic syndrome, idiopathic chronic eosinophilic pneumonia, and idiopathic acute eosinophilic pneumonia). Despite their rarity, the etiological approach to EP must be well-defined as some causes can be rapidly life-threatening without initiation of the proper treatment. This approach (i) eliminates secondary forms, with comprehensive history taking and minimal biological assessment, (ii) is oriented in primary forms by the acute or chronic setting, and the existence of extrapulmonary symptoms. Treatment of primary forms has traditionally relied on corticosteroids, usually with a dramatic response. Specific treatments or the adjunction of corticosteroid-sparing treatment or immunosuppressors are currently being evaluated in order to improve the prognosis and the side effects associated with corticosteroid treatment in a pediatric setting.

International audience; Objectives To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort. Methods Results We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria. Fourteen cases of pediatric EGPA were included, from 1980 to 2012, with a median follow-up of 58.5 months. Median age at diagnosis was 12.3 years. All cases had respiratory involvement. The organ systems most frequently involved were the upper airway (85%), skin (71%), digestive tract (64%), and heart (57%). Neurological and renal involvement were rare. Four of the fourteen children were positive for ANCA (30.7%). During follow-up, three children required intensive care and one child died. The relapse rate was 64%. In comparison with an adult cohort, we found more ENT, heart, and digestive-tract involvement, and fewer neurological manifestations. In children, the delay between asthma onset and diagnosis was shorter, and biopsies showed fewer features of vasculitis. Conclusion This French cohort is the biggest pediatric EGPA series described to date, with a long follow-up period. The findings confirm that pediatric EGPA has specific clinical, radiological, and histological characteristics that differ from adult EGPA. Development of systemic symptoms, and consequently diagnosis, occur with a shorter delay in children, mainly during the eosinophilic phase and leading to a specific presentation.

28th International Congress of the European-Respiratory-Society (ERS), Paris, FRANCE, SEP 15-19, 2018

Homozygous or compound heterozygous for frameshift or nonsense mutations in the ATP–binding cassette transporter A3 (ABCA3) is associated with neonatal respiratory failure and death within the first year of life without lung transplantation. We report the case of a newborn baby girl who developed severe respiratory distress soon after birth. She was diagnosed with compound heterozygous frameshift mutation of the ABCA3 gene. Despite extensive treatment (intravenous corticosteroids pulse therapy, oral corticosteroids, azithromycin, and hydroxychloroquine), she developed chronic respiratory failure. As the parents refused cardio-pulmonary transplantation and couldn't resolve to an accompaniment of end of life, a tracheostomy was performed resulting in continuous mechanical ventilation. A neurodevelopmental delay and an overall muscular dystrophy were noted. At the age of 5 years, after 2 episodes of pneumothorax, the patient died from severe respiratory failure. To our knowledge, this was the first case of a child with compound heterozygous frameshift mutation who posed such an ethical dilemma with a patient surviving till the age of five years. Keywords: ABCA3 deficiency, Compound heterozygous frameshift mutation, Neonatal respiratory failure, Tracheostomy, Mechanical ventilation, Ethical dilemma

International audience; Introduction: Particulate matter, nitrogen dioxide (NO2) and ozone are recognized as the three pollutants that most significantly affect human health. Asthma is a multifactorial disease. However, the place of residence has rarely been investigated. We compared the impact of air pollution, measured near patients' homes, on emergency department (ED) visits for asthma or trauma (controls) within the Provence-Alpes Cote-d'Azur region. Methods: Variables were selected using classification and regression trees on asthmatic and control population, 3-99 years, visiting ED from January 1 to December 31, 2013. Then in a nested case control study, randomization was based on the day of ED visit and on defined age groups. Pollution, meteorological, pollens and viral data measured that day were linked to the patient's ZIP code. Results: A total of 794,884 visits were reported including 6250 for asthma and 278,192 for trauma. Factors associated with an excess risk of emergency visit for asthma included short-term exposure to NO2, female gender, high viral load and a combination of low temperature and high humidity. Conclusion: Short-term exposures to high NO2 concentrations, as assessed close to the homes of the patients, were significantly associated with asthma-related ED visits in children and adults. (c) 2017 Elsevier Ltd. All rights reserved.

Resume Introduction L’obesite de l’enfant expose a des complications multiples, notamment cardiovasculaires et metaboliques a l’âge adulte. Elle peut aussi occasionner des troubles respiratoires, encore insuffisamment recherches. L’objectif de cette etude etait de faire une evaluation des pratiques pour le depistage et le diagnostic des troubles respiratoires des enfants obeses adresses dans un centre specialise pediatrique de l’obesite. Patients et methodes Etude retrospective des enfants atteints d’obesite severe non syndromique ou explores en hospitalisation (duree de recueil : 21 mois). Les caracteristiques cliniques (antecedents, parametres anthropometriques, habitudes alimentaires, acanthosis nigricans, symptomes de syndrome d’apnees obstructives du sommeil [SAOS]), paracliniques (bilan endocrinien, gazometrie, enregistrement du sommeil) et la prise en charge ont ete relevees. Resultats Parmi 102 enfants inclus (âge moyen de 10,5 ± 3,3 ans, Z-score d’indice de masse corporelle [IMC] moyen de 4,52 DS ± 1,5), 30 presentaient une symptomatologie de SAOS et 9 avaient une polysomnographie pathologique confirmant le SAOS. Huit d’entre eux ont ete ventiles : ces enfants avaient une obesite plus severe avec un rebond d’adiposite plus precoce (2,17 ± 1,2 ans vs 4,0 ± 2,2 ans, p = 0,01) et un Z-score plus eleve (7,2 DS ± 2,3 vs 4,3 DS ± 1,1, p = 0,027). Un syndrome obesite-hypoventilation (SOH) a ete diagnostique pour 4 d’entre eux. Conclusion Les diagnostics du SAOS et du SOH, souvent meconnus chez l’enfant obese, necessitent une demarche systematique et precoce de depistage clinique des troubles respiratoires. Une meilleure accessibilite aux explorations respiratoires permettrait d’ameliorer la qualite de prise en charge de ces enfants particulierement preoccupants.

We report on the case of a 3-year-old child presenting bilateral bronchiectasis due to recurrent pneumonia with esophageal achalasia. The final diagnosis was triple A syndrome. This presentation is particularly atypical and rare at this age.

Resume Des criteres d’hospitalisation pour la bronchiolite aigue du nourrisson ont ete etablis lors de la conference de consensus de 2000. Cette etude avait pour objectif d’evaluer leur impact sur l’hospitalisation dans les services d’accueil des urgences pediatriques (SAUP). Materiel et methodes Il s’est agi d’une enquete observationnelle multicentrique un jour donne dans 66 SAUP repartis sur l’ensemble du territoire. Le questionnaire portait sur la structure evaluee et etudiait le parcours de soin, l’etat clinique, la prise en charge et le devenir de chaque nourrisson presentant un premier episode de dyspnee sifflante. Une question ouverte permettait de detailler sur quel(s) critere(s) l’hospitalisation etait decidee. Le modele de regression logistique a ete utilise pour identifier des criteres cliniques independants conduisant a l’hospitalisation. Les donnees ont ete comparees par le test t de Student ou le test de Chi 2 . Resultats Pour 338 enfants inclus, 145 ont ete hospitalises (45,1 %). Les criteres ayant le plus de poids dans le choix d’hospitalisation ont ete un aspect toxique (OR: 3 [IC 95 %: 1,0–8,5], p = 0,04), une frequence respiratoire superieure a 60/minute (OR: 3,4 [IC 95 %: 1,3–8,8], p = 0,02), un âge inferieur a 6 semaines (OR: 29,8 [IC 95 %: 7,0–125,4], p p p p = 0,007). Conclusion Les criteres cliniques de gravite de la conference de consensus de 2000 influencent la decision d’hospitalisation. Cependant d’autres criteres sont retenus dans pres de 1/3 des cas.

In adults, congenital pulmonary malformations are candidates for surgery due to symptoms. A pre-natal diagnosis is simple and effective, and allows an early thoracoscopic surgical treatment. A retrospective study was performed to assess management in two different populations of adults and children to define the best strategy. Subjects and Methods: Pulmonary malformations followed at the University Hospital from 2000 to 2012 were reviewed. Clinical history, malformation site, duration of hospitalisation, complications and pathology examinations were collected. Results: A total of 52 cases (33 children, 19 adults) were identified. In children, 28 asymptomatic cases were diagnosed pre-natally and 5 during the neonatal period due to infections. Surgery was performed on the children between the ages of 2 and 6 months. Nineteen adults underwent surgery, 16 because of symptoms and 3 adults for anomalies mimicking tumours. The mean age within the adult group was 42.5 years. In children, there was one thoracotomy and 32 thoracoscopies, with 7 conversions for difficult exposure, dissection of vascular pedicles, bleeding or bronchial injury. In the adults, there were 15 thoracotomies and 4 thoracoscopies, with one conversion. Post-operative complications in the adults were twice as frequent than in children. The mean time of the children's hospitalisation was 7.75 days versus 7.16 days for the adults. Pathological examinations showed in the children: 7 sequestrations, 18 congenital cystic pulmonary malformations (CPAM), 8 CPAM associated sequestrations; in adults: 16 sequestrations, 3 intra-pulmonary cysts. Conclusion: Early thoracoscopic surgery allows pulmonary parenchyma conservation with pulmonary development, reduces respiratory and infectious complications, eliminates a false positive cancer diagnosis later in life and decreases risks of thoracic parietal deformation.

International audience; Purpose of the study: Vibrating-mesh nebulizers are widely used at home for cystic fibrosis (CF) treatment, with a therapeutic efficiency closely linked to the mesh performance. This national study looks at the maintenance at home by CF patients and their families of the mesh of the eFlow (R) rapid nebulizer. Ninety-two patients from 34 French CF centers, treated at home with inhaled drugs delivered with a vibrating-mesh nebulizer, answered to a phone standardized questionnaire specifying the different techniques of maintenance of the nebulizer. Principal results: Patients were aged from 2 to 68 years (58.7% of children). They inhaled a mean of 1.8 nebulizations per day. Maintenance was assumed by patients in 36% of the cases. All steps of the theoretical maintenance of a nebulizer were respected in 66% of the cases. The mesh was not cleaned in 45.6%, not disinfected or not thermically disinfected in 32.5%, and not rinsed after chemical disinfection in 45.5% of the case. Only 49% of the patients knew the role of the MeshCare (R) system in the mesh maintenance, and only 39% had already used it when the nebulization duration reached 10 min. Conclusions: Efforts about education, particularly for the maintenance of the mesh, are needed for CF patients using a vibrating-mesh nebulizer at home. (C) 2017 Elsevier Ltd. All rights reserved.

We retrospectively studied the clinical presentation, treatment modalities and outcome in 16 patients with heterozygous NKX2-1 mutation associated with chronic lung disease. Twelve different NKX2-1 mutations, including 4 novel mutations, were identified in the 16 patients. Nine patients presented with brain-lung-thyroid syndrome, 3 had neurological and lung symptoms and 4 had only pulmonary symptoms. Ten patients had neonatal respiratory distress, and 6 of them developed interstitial lung disease (ILD). The other patients were diagnosed with ILD in childhood (n = 3) or in adulthood (n = 3). The median age at diagnosis was 36 months (IQ 3.5–95). Patient testing included HRCT (n = 13), BALF analysis (n = 6), lung biopsies (n = 3) and lung function tests (n = 6). Six patients required supplemental oxygen support with a median duration of 18 months (IQ 2.5–29). All symptomatic ILD patients (n = 12) benefited from a treatment consisting of steroids, azithromycin (n = 9), and/or hydroxychloroquine (n = 4). The median follow-up was 36 months (IQ 24–71.5). One patient died of respiratory failure at 18 months and another is waiting for lung transplantation. In summary, the initial diagnosis was based on clinical presentation and radiological features, but the presentation was heterogeneous. Definitive diagnosis required genetic analysis, which should be performed, even in absence of neurological or thyroid symptoms.

International audience; We report the case of a 14-month-old girl suffering from cough and wheeze around Christmas. She was treated with anti-asthmatic drugs with no success, and 3 weeks later a chest X-ray revealed a LED bulb in the left main bronchus. This LED bulb came from a Christmas light garland decorating the Christmas tree. We discuss the different Christmas objects that can be inhaled by young children, the challenge to diagnose bronchial inhalation during this winter period, and the emergence of new foreign bodies, such as LED bulbs, with a particularly aerodynamic shape.

Asthma is the most common chronic disease in childhood. With its high economic burden, it is considered a disease of major public health importance by the World Health Organization. The link between respiratory tract infections and acute exacerbation has been recognized for a long time. The aim of this retrospective study in routine care was to evaluate our practices concerning microbiological prescriptions in children hospitalized for asthma exacerbation.All children aged from 2 to 15 years hospitalized for asthma exacerbation between January 2010 and December 2011 in our unit were included in the study. Microbiological prescriptions, their indications, their results, and their cost were studied.One hundred ninety-seven children were included in the study. A potential causative agent was sought in 79.7% of the children (n=157) by immunofluorescence assay (IFA) and/or polymerase chain reaction (PCR). The main indications were upper airway infections, hypoxemia, and pneumonia. Viruses were detected in 23.8% of them (30/126). Mycoplasma pneumoniae was detected by PCR in only 3.2% of these patients (4/125). No other bacterial agent was identified. There was no correlation between the severity of asthma exacerbation and the microbiological diagnosis of infection. The results did not influence the therapy given. These prescriptions represented a substantial cost for each child.These analyses do not seem to have a real advantage for the patient except for epidemiology. It would be important to conduct a new study analyzing the role of rhinovirus, and of other viruses such as coronavirus, bocavirus, and enterovirus, not routinely investigated in our hospital, and to question the value of these costly microbiological tests.

Resume Nous rapportons le cas d’un adolescent hospitalise pour une hemoptysie massive secondaire a des anastomoses systemo-pulmonaires directes traitees avec succes par embolisation. A notre connaissance, aucun autre cas n’a ete rapporte dans la litterature, probablement du fait du caractere letal de ce type de malformation.

Depuis quelques annees, de nouvelles molecules administrees par voie inhalee et de nouveaux dispositifs d’inhalation sont apparus pour le traitement de la mucoviscidose. Les nebuliseurs synchronises sur l’inspiration permettent un depot pulmonaire plus important, moins variable, et une duree de traitement raccourcie. Les nouvelles formulations de tobramycine, colistine ou mannitol en poudre seche modifient la technique d’inhalation a travers l’inhalateur de poudre seche qui doit maintenant etre lente et profonde. Dans le domaine des fluidifiants inhales, le serum sale hypertonique et le mannitol semblent interessants chez certains patients. En antibiotherapie, si la tobramycine et colistine en poudre peuvent etre substituees a la meme molecule delivree en nebulisation, la place de l’aztreonam nebulise reste a preciser. Ces traitements representent une avancee certaine pour les patients et meritent donc d’etre connus. Leur position dans l’arsenal therapeutique reste donc a definir.

Recommendations for the use of diagnostic testing in low respiratory infection in children older than 3 months were produced by the Groupe de Recherche sur les Avancees en Pneumo-Pediatrie (GRAPP) under the auspices of the French Paediatric Pulmonology and Allergology Society (SP(2)A). The Haute Autorite de sante (HAS) methodology, based on formalized consensus, was used. A first panel of experts analyzed the English and French literature to provide a second panel of experts with recommendations to validate. Only the recommendations are presented here, but the full text is available on the SP(2)A website.

Resume Nous decrivons le cas d’un adolescent de 14 ans qui a presente un syndrome de Nicolau du membre inferieur droit. Ce syndrome faisait suite a une injection intramusculaire gluteale homolaterale de benzathine-penicilline associee a de la lidocaine, liee a une suspicion de rhumatisme articulaire aigu. Outre la rarete de ce syndrome, nous rapportons une association particuliere a une torsion testiculaire aigue.

International audience; A 5-year-old girl with failure to thrive and multiorgan disease was referred to our center for chronic hypoxemia. On evaluation, we noted tachypnea (respiratory rate 35/min), supraclavicular retractions, median diurnal oxygen saturation as measured by pulse oximetry (Sp O 2) = 91.7% at rest, percentage of time below Sp O 2 90% at 26% during sleep, and clubbing. A computed tomography scan showed diffuse interstitial lung disease (Figure 1). Spirometry was normal (TLC, 83% of predicted; FEV 1 , 83% of predicted; FEV 1 /FVC, 98%; and forced expiratory flow, midexpiratory phase, 142% of predicted), but it was not possible to measure DL CO .