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Resume Introduction Les inhalateurs de poudre seche (IPS) necessitent un effort inspiratoire pour dissocier la poudre et former l’aerosol sec. Une revue de la litterature a ete effectuee afin de savoir si ceci peut, dans certaines situations cliniques (jeunes enfants, sujets âges, syndromes obstructifs aigus ou chroniques de l’asthme et des BPCO), constituer une limite a leur utilisation. Etat des connaissances Les IPS commercialises presentent des caracteristiques techniques differentes. Les facteurs pouvant influencer leur efficacite sont nombreux et l’interet predictif des essais in vitro est limite. Les etudes cliniques publiees indiquent que les IPS peuvent etre efficaces meme a bas debit inspiratoire, ainsi que dans les tests de provocation bronchique non specifique, les crises d’asthme et les syndromes obstructifs des BPCO. Toutefois, ces etudes presentent souvent des limites. Perspectives Les etudes cliniques analysees indiquent que les IPS peuvent etre utilises dans la grande majorite des situations cliniques sous reserve d’une education attentive des patients. Neanmoins, chez l’enfant de moins de six ans, les recommandations internationales preconisent de preferer l’aerosol-doseur couple a une chambre d’inhalation. Conclusions De nouvelles etudes cliniques bien conduites sont encore souhaitables pour etablir l’efficacite des IPS dans les situations cliniques extremes.

Hemolymphangioma is a benign malformation of the lymphatic vessels. Cervical localisation is the most frequent. Mediastinal localisation is rare, but may be life-threatening because of airway compression. We report on a four-month-old boy who presented, in a context of epidemics, with clinical signs of acute bronchiolitis requiring mechanical ventilation for several days. Chest X-rays showed an important mediastinal mass with airways compression. Diagnosis was evoked on CT-scan aspects and confirmed by histology. Surgery allowed complete resolution, but dysphonia and oropharyngeal disorders persisted for several months. This rare congenital malformation is reviewed.

INTRODUCTION Fibreoptic bronchoscopy (FB) is an important diagnostic examination in paediatric pulmonology. In 2002 the Paediatric Pulmonology and Allergy Club undertook a retrospective study to establish the current status of fibreoptic bronchoscopy among its members. METHODS In 2001 sixty five paediatric pulmonologists carried out an average of 116 examinations (+/- 111) in 35 paediatric centres. FB was performed either in an operating theatre (15 centres), a dedicated bronchoscopy suite (6 centres) or an endoscopy suite shared with gastro-enterologists (7 centres). Other examinations were performed in areas dedicated to, or associated with intensive care. General anaesthesia was routinely used in 18 centres. The others used sedation including an equimolar mixture of oxygen and nitrous oxide in 14 centres. Ten centres performed less than 50 examinations, 12 between 51 and 100, 4 between 101 and 200 and 8 centres more than 200 in the year. Seventy two per cent of the children were less than 6 years old. The washing and disinfection procedures were manual in 20 centres and automatic in 15. RESULTS Three principal indications were reported: persistent wheezing, suspicion of a foreign body and ventilatory difficulties. Cough, desaturation and fever were the most frequently reported side effects. CONCLUSIONS This is the first survey in paediatric pulmonology in France. It shows a wide variation in the practice of fibreoptic bronchoscopy in children.

Resume Introduction L’asthme, affection chronique la plus frequente en pediatrie, se caracterise par un processus inflammatoire impliquant les voies aeriennes proximales et distales, avec des phenomenes de remodelage des voies aeriennes et d’alterations de la fonction respiratoire. Etat des connaissances Les travaux recemment realises chez l’enfant mettent en evidence une atteinte des petites voies aeriennes identique a celle de l’adulte. En effet, la presence de cellules inflammatoires ainsi que des alterations structurales des voies aeriennes distales ont ete retrouvees soit sur des biopsies bronchiques de l’enfant soit sur des produits de lavage bronchoalveolaire de nourrissons et jeunes enfants. Sur le plan fonctionnel, cette atteinte est objectivee par une diminution du debit expiratoire maximal median entre 25 et 75 % de la capacite vitale. Celle-ci s’exprime egalement par l’existence de bronches a parois epaisses ou de plages hypodenses a la tomodensitometrie thoracique en haute resolution. Il semble que les traitements inhales conventionnels restent sans effet sur les petites voies aeriennes. Enfin, les principaux facteurs pronostiques de cette atteinte distale sont representes par les infections respiratoires et l’exposition tabagique in-utero. Perspectives L’atteinte des petites voies aeriennes dans l’asthme de l’enfant plaide en faveur de la necessite de nouvelles therapeutiques ciblees sur cette partie de l’appareil respiratoire. Conclusion L’inflammation et le remodelage des petites voies aeriennes sont precoces et durables chez l’enfant asthmatique dont l’appareil respiratoire est en pleine croissance.

Unilateral primitive agenesia of pulmonary artery is a rare congenital disorder. Experience of three hospital-based pulmonology pediatric units including eight children (three boys and five girls) with such anomaly is reported (11 years median follow-up, range 6 months to 10 years). Median age at diagnosis was 4 years. Clinical features included recurrent respiratory tract infections (75%), effort dyspnea (50%) and, curiously, asthmatic symptoms (75%). Diagnosis was evoked on chest X rays and confirmed by angiography in all cases. Agenesis of the right pulmonary artery was more frequent (five cases). Abnormal vascular ring was associated in two cases of agenesis of the left pulmonary artery. Pulmonary function tests were performed in six children, with bronchial obstruction in two. Regular clinical follow-up, up to adult age in four cases, showed no complication, such as hemoptysis or pulmonary hypertension in our patients.

Objectifs L’objectif principal est de mesurer l’impact des particules en suspension (PM), estime a proximite du domicile des enfants, sur les admissions pour exacerbation d’asthme aux urgences dans le departement des Bouches-du-Rhone en 2013 chez l’enfant. Materiel et methodes Un cas temoins emboite dans une cohorte est realise chez les enfants de 3 a 18 ans. L’appariement etait aleatoire sur le jour de la consultation aux urgences. L’exposition aux PM 10 et PM 2,5, les donnees meteorologiques, polliniques et virologiques du jour de consultation etaient correlees au code geographique du domicile du patient et analysees par rapport au motif de consultation. Resultats principaux Notre etude concernait 68 897 consultations, dont 1182 asthmatiques. L’exposition a court terme aux PM 10 etait associee a un exces de risque de consultation pour asthme (RR = 1,02, IC 95 % :1,01–1,04 ; p = 0,02). Le sexe masculin, le jeune âge et la temperature etaient egalement des facteurs de risque. Par contre, le vent etait retrouve comme facteur protecteur. Conclusion Les PM 10 et certains facteurs meteorologiques, estimes au plus proche du domicile des enfants, augmentent le risque de consultation aux urgences pour exacerbation d’asthme chez les enfants de 3 a 18 ans dans les Bouches-du- Rhone.

Determining which drug is suitable for, and which patient can benefit from, a once-daily dose of prophylactic treatment is important for practitioners who want to improve therapeutic compliance in children with asthma. According to the literature, once-daily delivery of cromolyn sodium, nedocromil or beclomethasone dipropionate must be avoided. On the other hand, switching from a twice-daily to a once-daily regimen is efficient and safe only in children with well-controlled asthma using nebulized or dry-powder budesonide, dry-powder fluticasone propionate, flunisolide, or sustained-release theophylline. Such information is not available for long-acting β2-agonists, except for oral bambuterol. Initiating a once-daily treatment in previously untreated children can only be based on low doses of inhaled budesonide or on an oral drug, montelukast. Further studies in children with severe asthma or treated with metered-dose inhalers and spacer devices are required before recommending a once-daily drug delivery in such situations.

beta 2-agonists, by inducing a fast and long relaxation of the bronchial smooth muscle, are considered as the more potent bronchodilators. beta 2-receptors are present from the 16th gestational week, explaining a possible bronchial response in the youngest children. beta 2-agonists do not induce any bronchodilator response in healthy children. Short-acting beta 2-agonists (salbutamol or albuterol, terbutaline) are indicated for asthma attacks, as needed in chronic asthma, and for prevention of symptoms during effort. They are safe and secure. The more efficient route of administration in preschool children is pressurized metered-dose inhaler used with a spacer device. Therefore, whatever the route of inhalation chosen (inhalation, injection, or continuous nebulization in acute asthma attack), more specified indications and doses are needed in young children. Long-acting beta 2-agonists (formoterol, salmeterol) are not authorized in France in children under 4 to 5 years of age depending on the drug used. Because of new oral formulations and recent considerations about their use in asthma attack, instead of short-acting beta 2-agonists, their indication in preschool asthmatic children might be reconsidered.

Inhaled way is the most efficient method to deliver drugs for the treatment of respiratory diseases. Pulmonary deposition depends upon the inhalation system and technique. The evaluation of the drug deposition is difficult, particularly in children. Because of irradiation, scintigraphic studies which permit the best approach of the pulmonary deposition are rare in children. Various inhalation systems are available: nebuliser, metered-dose inhaler with or without spacer device, dry powder inhaler. Whatever the system used, the control of asthma will be only possible with the regular evaluation of the child inhalatory technique.

Introduction La pollution atmospherique a des effets nefastes sur la sante et l’environnement. Les particules en suspension, le dioxyde d’azote (NO2) et l’ozone (O3) sont reconnus comme etant les 3 polluants les plus significatifs en termes d’incidence sur la sante. L’asthme est multifactoriel avec notamment l’exposition a la pollution atmospherique, les conditions meteorologiques, les allergenes, les infections virales qui sont fortement impliques. Cependant, le lieu de residence des patients est rarement etudie en rapport avec ces facteurs. L’objectif principal de notre etude est de mesurer l’impact de la pollution atmospherique, au plus proche du domicile des patients, sur les consultations pour asthme dans les services d’accueil des urgences (SAU) dans la region Provence-Alpes-Cote-D’azur (PACA) en 2013. Methodes Initialement, les variables ont ete selectionnees par un arbre de classification et de regression concernant les patients asthmatiques et traumatologiques, âges de 3 a 99 ans, ayant consulte dans un SAU en PACA du 01 janvier au 31 decembre 2013. Puis, dans une etude cas-temoins emboitee dans une cohorte, chaque controle traumatologique a ete randomise de maniere aleatoire sur le jour, quel que soit le SAU de consultation. Les facteurs environnementaux et la charge virale ont ete relies au code INSEE de residence des patients. Resultats Un total de 794 884 consultations a ete reporte chez les patients de 3 a 99 ans dont 6250 pour asthme et 278 192 pour traumatisme. L’exposition a court terme au NO2, mesuree au plus proche du domicile des patients, est associee a un exces de risque de consultation aux urgences pour asthme (OR ajuste : 1,35 [5 % CI : 1,17–1,57 ; p =

Introduction La reponse IgE specifique est un element crucial du faisceau d’arguments diagnostiques d’une allergie, a l’inverse de la reponse IgG correspondante. Aspergillus fumigatus (Af) est un champignon aeroporte ubiquitaire, pathogene opportuniste en cas de mucoviscidose (MV), asthme ou immunodepression. La pertinence clinique des IgG et IgE anti-aspergillaires est presumee dans l’aspergillose broncho-pulmonaire allergique (ABPA). Nous avons etudie la reponse IgG vis-a-vis d’Af et de ses allergenes et ses relations avec les IgE et IgG4 de meme specificite. Methodes Les IgG, IgE et IgG4 contre l’extrait d’Af et les allergenes Asp f 1, Asp f 2, Asp f 3, Asp f 4 et Asp f 6 ont ete quantifiees par ImmunoCAP (ThermoFisher) chez 59 patients dont 49 MV (25 enfants ; 24 adultes) et 10 ABPA (2 enfants ; 3 MV). Resultats La double sensibilisation IgG et IgE vis-a-vis de l’extrait et de Asp f 1 est preponderante (90 a 100 % des patients). Des discordances IgG/IgE sont observees pour les autres allergenes, avec une reponse IgE isolee plus frequente que la reponse IgG isolee (27 vs 11 patients), notamment dans le groupe ABPA. La prevalence des IgG Asp f 3 et Asp f 4 est plus elevee dans le groupe ABPA (56 % vs 22 % et 78 % vs 38 %, p = 0,004 et 0,003). Quantitativement, les IgG vis-a-vis de l’extrait et des allergenes sont plus elevees chez les patients ABPA (p = 0,004 a 0,0003), sauf pour Asp f 2. Ce dernier est le seul allergene pour lequel les titres IgG et IgE sont correles quantitativement, avec ou sans ABPA. Les titres IgG et IgG4 sont directement correles pour tous les allergenes sauf Asp f 6 chez les patients indemnes d’ABPA et Asp f 3 et 4 chez les patients ABPA. Discussion Les reponses IgG et IgE specifiques d’Af et des allergenes aspergillaires correspondants permettent de distinguer entre ABPA et sensibilisation banale. Les allergenes different par les reponses IgG et IgE, suggerant des proprietes immunogeniques differentes. Les anomalies en IgG, IgE et IgG4 interessent les memes allergenes Asp f 3, 4 et 6. Conclusion L’exemple d’Af suggere que l’exploration de la reponse IgG anti-allergenes peut etre informative dans certaines situations cliniques.

Introduction Les anomalies hereditaires du metabolisme du surfactant representent 15 % des causes de syndrome respiratoire associees a une pathologie interstitielle chez l’enfant. Le facteur de transcription NKX2-1 (NK2 homeobox 1) est crucial au niveau pulmonaire pour reguler les genes codant les proteines du surfactant SP-A, B, C et D et ABCA3. NKX2-1 est aussi exprime dans la thyroide et le systeme nerveux. Chez l’homme, des mutations de ce gene NKX2-1 causent le syndrome « cerveau-poumon-thyroide » associant generalement une maladie respiratoire avec des symptomes neurologiques et une hypothyroidie, avec une grande variabilite phenotypique. Plus recemment, des atteintes respiratoires isolees ont ete rapportees. Methodes Nous avons recueilli les donnees cliniques de 15 patients presentant une atteinte respiratoire liee a une mutation de NKX2-1 . Resultats La triade clinique est observee chez 8 patients et 4 n’ont que l’atteinte pulmonaire. L’âge median au diagnostic est de 21 mois (IQ 3–100). Une detresse respiratoire neonatale est retrouvee chez 9 patients dont 5 evoluent vers une pneumopathie infiltrative diffuse (PID), 3 patients ont eu une PID diagnostiquee avant 1 an et 3 a l’âge adulte. Douze patients ont eu des examens paracliniques : scanner thoracique ( n = 12), fibroscopie bronchique ( n = 5), biopsie pulmonaire ( n = 3) et des explorations fonctionnelles respiratoires ( n = 6). Une oxygenotherapie a ete administree chez 6 patients pour une duree mediane de 18 mois (IQ 2,5–29). Tous les patients souffrant de PID ont ete traite par corticoides per os ou en bolus ( n = 10), azithromycine ( n = 8) et/ou hydroxychloroquine ( n = 4). Conclusion L’atteinte pulmonaire liee aux mutations de NKX2-1 est heterogene et l’analyse genetique doit etre realisee meme en absence de symptomes neurologique ou thyroidien.

Resume La botryomycose est une infection bacterienne rare. Elle se presente sous 2 formes : cutanee ou viscerale. Moins de 30 cas pediatriques ont ete rapportes. Nous presentons le cas, pour la premiere fois chez un nouveau-ne de 14 j, d’une botryomycose revelee par 4 tumefactions de localisation inguinale et poplitee, sans syndrome inflammatoire biologique. L’examen anatomopathologique du prelevement etait en faveur de cette pathologie. La culture du prelevement retrouvait un Staphylococcus aureus. L’evolution etait favorable sous antibiotherapie adaptee prolongee. Un terrain sous-jacent a type de deficit immunitaire ou mucoviscidose a ete ecarte.

Exposure to tobacco smoke has been not evaluated in children with bronchopulmonary dysplasia (BPD). We evaluate the association of in utero smoking (IUS) and environmental tobacco smoke (ETS) with the respiratory events of BPD and non-BPD children. Two hundred sixty-two children born before 35 weeks of gestational age (GA) and regularly followed up in our regional network for preterms were enrolled. They were paired according to their BPD status, their gestational age and birth weight (131 children with BPD and 131 without BPD, 28 mean weeks GA; mean weight 1000 g). Respiratory data were obtained prospectively during their first 2 years of life. A complementary questionnaire was completed by the parents about their child's respiratory health at the age of 2, their home environment, and tobacco status. IUS concerned 12.6 %; ETS, 48.8 % (67 % in BPD children treated with oxygen at home). No further influence on respiratory outcome could be found by exposure to intrauterine smoke or extrauterine tobacco smoke in this patient sample.IUS and ETS exposures are as high in preterm children as in a general pediatric population. The highest exposure occurs among BPD infants treated with oxygen at home.• Environmental tobacco smoke (ETS) and in utero smoking (IUS) are responsible for many morphological, functional, and clinical changes in children. • Children with bronchopulmonary dysplasia (BPD) have more respiratory events in their first years of life than preterm children without BPB, maybe triggered by ETS and IUS. What is New: • The exposition to ETS and IUS is high in preterm children with and without BDP, as high as in a general. • Pedaitric population, particularly in children with BPD and treated with oxygen at home. • No further influence on respiratory outcome could be found by exposure to ETS or IUS in our studied population.

Ceftazidime is particularly efficient against Pseudomonas aeruginosa in cystic fibrosis patients. Thus, the spontaneous production of pyridine, which is a toxic product, raises some concern. Our aim was to examine the kinetics of degradation of ceftazidime in portable infusion pumps either at 4°C, 22°C, or 33°C and to propose some recommendations in order to reduce the pyridine exposure. Two administration models were studied in vitro . In model 1, we administered 12 g of ceftazidime infused over 23 h (once-daily infusion) compared to 6 g infused over 11.5 h in model 2 (twice-daily regimen). Samples were collected at 0 h and then every 4 and 2 h after the shaping of portable infusion pumps in models 1 and 2, respectively. Both ceftazidime and pyridine were analyzed using an ultraviolet high-performance liquid chromatograph. Production of pyridine is highly depending on the temperature. The in situ production of pyridine per day of treatment decreases at a ratio close to 1/6 and 1/3 between 33°C and 4°C in models 1 and 2, respectively. Regardless of the conditions, the production of pyridine is significantly lower in model 2, whereas the total delivery amount of ceftazidime is significantly higher at 4°C and 33°C compared to that in model 1. According to a the precautionary principle, these findings lead to three major recommendations: (i) exposing a solution of ceftazidime to over 22°C should be strictly avoided, (ii) a divided dose of 6 g over 11.5 h instead of a once-daily administration is preferred, and (iii) infusion should be administered immediately after reconstitution.

International audience; BACKGROUND: Idiopathic eosinophilic pneumonia is extremely rare in children and adults. We present herein the first series describing the specificities of idiopathic chronic (ICEP) and acute (IAEP) eosinophilic pneumonia in children. METHODS: We retrospectively analyzed all cases of ICEP and IAEP in children that were retrieved from French Reference Centers for rare pediatric lung diseases. RESULTS: Five cases of pediatric ICEP were identified. Corticosteroid or immunosuppressive therapy dramatically improved the outcome in three cases. The remaining two cases had a persistent interstitial pattern with progressive development of cystic airspace lesions. Three cases of IAEP in adolescents were reported, with one requiring four days of extracorporeal membrane oxygenation. CONCLUSION: ICEP is a rare disease with a polymorphic clinical presentation in children. We identified patients with persistent interstitial patterns progressing to cystic airspace regions, for which the boundaries with idiopathic interstitial pneumonias are difficult to establish. We therefore propose a specific pediatric definition and classification algorithm. IAEP in children remains an inflammatory reaction of the lung to an acute toxic exposure, mainly tobacco, as in adults. International studies are required to comprehensively assess the various clinical forms of the disease as well as the appropriate therapeutic regimens.

Background: The objective was to investigate the local side-effects of inhaled corticosteroids (ICS) in daily life in asthmatic children, particularly the younger ones, by an observational prospective cross-sectional cohort study. Methods: Asthmatic children (n=639, 75.9±48.9 months, 61.3% boys), treated with beclomethasone dipropionate (BDP) (721.0±287.3 µg per day) or budesonide (BUD) (835.5±684.9 µg per day) for at least 1 month, were recruited at the time of a scheduled visit. Local side-effects were researched by questionnaire (cough during inhalation, hoarseness, dysphonia, and thirsty feeling) and clinical examination (perioral dermatitis, oral candidiasis, and tongue hypertrophy). Results: Exactly 63.3% of the children aged under 6 years and 59.5% of the older ones reported one local side-effect. Cough (39.7%) was dependent on young age, use of BDP, and mainly use of spacer device, with an OR of 4.7 (95% CI: 2.7–8.2). Thirsty feeling (21.9%) and hoarseness (14.1%) occurred in children using ICS and long-acting β2-agonists. Dysphonia (11.1%) was favored by high doses of BDP and BUD, and by inhalation from spacer devices or nebulizers. No factor favored oral candidiasis (10.7%). Perioral dermatitis (2.9%) and tongue hypertrophy (0.1%) were associated with nebulization. Conclusions: Local side-effects of ICS are common in asthmatic children of all_ ages, and the device used constitutes the most influential factor.

Intralobar pulmonary sequestration (ILPS) and bronchogenic cyst are rare congenital diseases. We present the first case of the association between an ILPS located in the upper lobe and a bronchogenic cyst. This association has been discovered antenatally in a girl. The cystic lesion has been shown by a fetal MRI and confirmed by a CT scan at 3 weeks of life. She underwent a left upper lobectomy by thoracoscopy at the age of 5 months. Postoperative course was uneventfull. This case confirms the congenital origin of pulmonary sequestration and shows the interest of an early surgery.

Resume La crise d'asthme aigu grave de l'enfant se definit comme une crise d'asthme qui ne repond pas au traitement d'urgence bronchodilatateur et qui est responsable d'une insuffisance respiratoire aigue potentiellement fatale. La severite de la crise d'asthme doit etre apprehendee rapidement, supposant une connaissance parfaite des criteres cliniques de gravite. La prise en charge therapeutique doit etre faite sans delai. Elle repose sur l'utilisation des β2-agonistes de courte duree d'action, de preference par voie inhalee et a fortes doses. Ladjonction d'anticholinergiques aux nebulisations de β2-agonistes peut s'averer utile chez les enfants les plus obstructifs. La corticotherapie par voie generale doit etre administree precocement en raison de son delai d'action retarde. Les mesures symptomatiques associeront une hospitalisation rapide medicalisee, la correction d'une hypoxemie et le maintien d'une hydratation. L'absence de signes objectifs d'amelioration impose l'emploi de β2-agonistes par nebulisation continue ou par voie veineuse sous couvert d'une surveillance cardiaque imperative. Le transfert en unite de soins intensifs pediatriques permettra, lors d'obstruction bronchique persistante, d'associer les traitements bronchodilatateurs voire de recourir si necessaire a la ventilation mecanique. Une fois l'episode aigu resolu, il convient dans tous les cas de prescrire un traitement de fond par corticoides inhales, d'organiser un suivi clinique et fonctionnel respiratoire et, enfin, de realiser une education du patient et de sa famille.

Background: Studies of the effects of tobacco smoke often rely on reported exposure to cigarette smoke, a measure that is subject to bias. We describe here the relationship between parental smoking exposure as assessed by urinary cotinine excretion and lung function in children with asthma.Methods: We studied 90 children 4–14 years of age, who reported a confirmed diagnosis or symptoms of asthma. In each child, we assessed baseline pulmonary function (spirometry) and bronchial responsiveness to carbachol stimulation. Urinary cotinine was measured by HPLC with ultraviolet detection.Results: Urinary cotinine concentrations in the children were significantly correlated (P Conclusion: Passive smoke exposure increases the bronchial responsiveness to carbachol in asthmatic children.

Resume Le concept d'immuno-incompetence du nouveau-ne a cede la place a la nolion d'une competence immunitaire reelle mais differente de celle de l'adulte. Ces differences se retrouvent a tous les niveaux du systeme immunitaire. L'immunite non specifique du nouveau-ne est caracterisee par la diminution de la phagocytose, liee a la diminution du pool post-mitotique des polynucleaires. Les macrophages, presents en quantite normale, ont une production de eytokines reduite, entrainant une diminution de production d'interferon-γ par les lymphocytes T. L'immunite specifique se distingue par un defaut de production d'inlerleukines par les lymphocytes T auxiliaires, un defaut de cooperation cellulaire et une diminution de production des immunoglobulines. Cette difference pourrait etre liee a l'absence d'exposition prealable aux antigenes. L'immunisation en periode neonalale entraine une reponse mais celle-ci est de type TH2. Dans cette orientation TH2. l'environnement du fœtus pendant la gestation joue un role fondamental.

We report the first case in the literature of thoracoscopic bronchial reimplantation in 5 month-old boy. The child was born with congenital cystic adenomatoid malformation of the right lower lobe associated with lower intralobar pulmonary sequestration diagnosed prenatally. An iatrogenic middle lobar bronchus injury was detected per-operatively during thoracoscopic lobectomy. Management and follow-up were exposed.

Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.

The revival of nebulization as a drug delivery route is real. The current delivery systems respond to the new European norms, the new mesh-vibrating nebulizers allow delivering drugs more quickly, other nebulizers, more performant because of less drug losses and of a better lung deposition of the drug, are in progress. Only 12 drugs are commercialized for nebulization. All are available in dispensaries, some requiring a first prescription by a physician working in a hospital (cystic fibrosis drugs), others requiring a prescription from only some specialists as paediatricians or pulmonologists (bronchodilators). Works are in progress concerning the diameter and shape of the drug particles (nanotechnology) and also concerning the use of nebulized drugs for a systemic effect (vaccines, insulin, cyclosporine, anticancerous agents, etc.).

Summary Toxocara canis, one of the most frequent parasites worldwide, rarely triggers respiratory symptoms. We report the case of a 5-year-old girl hospitalized for a unilateral eosinophilic pleural effusion due to Toxocara canis. Besides the fact that she was living in a squat, no other medical condition was reported. There was no other site of infection caused by the parasite and she was successfully treated with albendazole. This case report is obviously unique as very few cases of pleural effusion due to Toxocara canis are reported in literature, all in adult patients. Pediatr Pulmonol. 2015; 50:E35–E36. © 2015 Wiley Periodicals, Inc.

A formulation of a eutectic mixture of lidocaine-prilocaine (EMLA) changes basal skin perfusion. Its use for alleviating pain associated with the Mantoux test may modify the recruitment of sensitised lymphocytes and then the response to tuberculin test. Twenty-four healthy BCG-vaccinated volunteers (26.7+/-4.1 years) received on each forearm an intradermal injection of 10IU tuberculin, one of the forearms being randomly pre-treated for 1h with EMLA-patch 5%. Pain associated with the Mantoux test was evaluated using a visual analogue scale. The transversal diameter of the induration was read at 72h. Subjects with 6mm difference between diameters (i.e. twice the usual variation for a Mantoux test) were recorded. Results were compared using a paired t-test. When using lidocaine-prilocaine prior to the test, a three-fold decrease in pain was noted (p0.0001). Reading of the test were not affected by the lidocaine-prilocaine application (p=0.26). Four subjects had 6mm or more difference between their two tests, two of them having an induration greater than 15mm with lidocaine-prilocaine. Lidocaine-prilocaine reduces significantly pain associated with the Mantoux test but does not normally affect the test reading. However, when the induration is more than 15mm, a control without lidocaine-prilocaine has to be considered.

The emergence of multidrug-resistant (MDR) bacteria in cystic fibrosis (CF) patients has led to the use of colistin drug and the emergence of colistin-resistant Gram-negative bacteria. The aim of this study was to compare the disk diffusion and Etest methods for colistin susceptibility testing on MDR bacteria associated with CF from Marseille, France. Forty-nine MDR clinical isolates (27 Stenotrophomonas maltophilia, 22 Achromobacter xylosoxidans) were used in this study. Disk diffusion and Etest assays were used to assess the reliability of these two techniques. For S. maltophilia, 25 out of 27 isolates had low minimum inhibitory concentrations (MICs, 0.125-0.75 mg/L), whereas two isolates displayed high MICs (32 mg/L). Similarly, 19 out of 22 A. xylosoxidans isolates had low MICs (0.75-3.0 mg/L), whereas three isolates had high MICs (32-256 mg/L). The diameters of zone inhibition with a 50-μg colistin disk displayed a good correlation with the MICs obtained by the Etest. Susceptible and resistant strains were eventually separated using a disk diffusion assay at a cut-off of ≤ 12 mm for a 50-μg disk. Colistin displayed excellent activity against S. maltophilia and A. xylosoxidans and the disk diffusion assay could be confidently used to determine the susceptibility to colistin for MDR Gram-negative bacteria in the context of CF.

Objective To investigate the risk factors of empyema after acute viral infection and to clarify the hypothesized association(s) between empyema and some viruses and/or the use of nonsteroidal anti-inflammatory drugs (NSAIDs). Study design A case-control study was conducted in 15 centers. Cases and controls were enrolled for a source population of children 3-15 years of age with acute viral infections between 2006 and 2009. Results Among 215 empyemas, 83 cases (children with empyema and acute viral infection within the 15 preceding days) were included, and 83 controls (children with acute viral infection) were matched to cases. Considering the intake of any drug within 72 hours after acute viral infection onset and at least 6 consecutive days of antibiotic use and at least 1 day of NSAIDs exposure, the multivariable analysis retained an increased risk of empyema associated with NSAIDs exposure (aOR 2.79, 95% CI 1.4-5.58, P = .004), and a decreased risk associated with antibiotic use (aOR 0.32, 95% CI 0.11-0.97, P = .04). The risk of empyema associated with NSAIDs exposure was greater for children not prescribed an antibiotic and antibiotic intake diminished that risk for children given NSAIDs. Conclusions NSAIDs use during acute viral infection is associated with an increased risk of empyema in children, and antibiotics are associated with a decreased risk. The presence of antibiotic-NSAIDs interaction with this risk is suggested. These findings suggest that NSAIDs should not be recommended as a first-line antipyretic treatment during acute viral infections in children.

International audience; Background: The use of valved holding chambers (VHCs) with pressurized metered dose inhalers (pMDIs) is reported to reduce the oral deposition of inhaled drugs and to facilitate the handling of these devices by patients, especially children. Although the number of commercially available VHCs is increasing, the correct choice of VHC in clinical practice is important, because VHCs are not equally effective regarding medication delivery. Hence, we aimed to evaluate the use of three small-volume VHCs-Vortex (R), AeroChamber (R) Plus (ACP), and Able Spacer (TM) (AS)-along with a commercial pMDI containing a combination of beclomethasone and formoterol (Innovair (R)) frequently used by asthma patients. Methods: Evaluation of the delivered dose of both drugs and analysis of particle size distribution of aerosols emitted for the inhaler were performed using the Next Generation Impactor with and without the tested VHCs. Results: The VHCs retained significant quantities of both drugs and dramatically reduced the quantity of drugs deposited in the throat of the impactor, indicating that particles with large size were preferably retained in the VHCs. Interestingly, although the delivered dose of both drugs was reduced by the use of VHCs, the use of the Vortex and the ACP resulted in comparable fine particle doses (FPDs) to that obtained when the pMDI was used alone, whereas the AS VHC significantly reduced the FPDs of both drugs. This may be due to the fact that, unlike the AS VHC, the Vortex and the ACP VHCs are made of antistatic materials that minimize the electrostatic interaction with emitted aerosols, enhancing medication delivery. Conclusion: The Vortex and the ACP VHCs present interesting advantages over the AS VHC to be used with Innovair pMDI. However, these results are based on an in vitro evaluation and need to be validated in an in vivo study in order to clinically assess the performance of these VHCs.

The emergence of multidrug-resistant Gram-negative bacteria that cause nosocomial infections is a growing problem worldwide. Colistin was first introduced in 1952 and was used until the early 1980s for the treatment of infections caused by Gram-negative bacilli. In vitro, colistin has demonstrated excellent activity against various Gram-negative rod-shaped bacteria, including multidrug-resistant Pseudomonas aeruginosa, Acinetobacter baumannii and Klebsiella pneumoniae. Recent clinical findings regarding colistin activity, pharmacokinetic properties, clinical uses, emerging resistance, toxicities and combination therapy have been reviewed. Recent approaches to the use of colistin in combination with other antibiotics hold promise for increased antibacterial efficacy. It is probable that colistin will be the 'last-line' therapeutic drug against multidrug-resistant Gram-negative pathogens in the 21st century.

Staphylococcus aureus is one of the most frequently encountered species in Cystic Fibrosis (CF) lung microbiota. However its frequent presence on agar plates from sputum samples of CF patients likely impaired the culture of other minority bacterial species that may be responsible for lung exacerbations. In order to discover this minority bacterial population, we examined the sputum sample of a CF patient using conventional culture dependent with or without inhibition of S. aureus growth with vancomycin and compare our results to culture independent molecular techniques. Using standard cultures as well as selective media at different physico-chemical conditions, we isolate Staphylococcus aureus in the majority of our culture conditions and media. Interestingly, after pre-incubation of the sample with Vancomycin to inhibit Staphylococcus aureus growth, we isolate new bacterial species (Granulicatella, Streptococcus, Rothia, Lactobacillus and Propionibacterium species), especially Actinomyces radicidentis, a rare bacterial species associated with endodontic infections never described in the context of CF. Using 16S rDNA PCR amplification and sequencing of subsequent clones, we identified Streptococcus, Granulicatella, Rothia, Abiotropha, Peptostreptococcus, Lachnospiraceae and Actinomyces along with S. aureus confirming the culture dependent findings. In conclusion, the use of various bacterial inhibitors to inhibit the growth of the dominant bacteria may help to discover the minority bacterial population that could be new or emerging species in the context of CF.

Spacer devices are used to optimize airway aerosol deposition from pressurized metered-dose inhalers (pMDI). The in vitro performance of the combination fluticasone/salmeterol pMDI alone and connected to 3 different valved holding chambers (VHC) was compared by measuring impactor entry port (“throat”) deposition and fine particle dose (FPD) of each medication. Salmeterol (SX) and Fluticasone (FP) throat deposition was reduced over 90 % by all VHC compared to pMDI alone (p < 0,001). The FPD obtained from pMDI alone and connected to VHCs Vortex®, AeroChamber Plus® and Able Spacer® for Salmeterol (25 μg nominal dose) were 12.2 ± 0.7, 12.5 ± 0.5, 11.6 ± 0.8, and 7.9 ± 0.9 μg, respectively. For Fluticasone (125 μg nominal dose) the FPD were 42.5 ± 2.6, 36.3 ± 3.1, 39.8 ± 2.4, and 22.8 ± 3.5 μg, respectively. There were no statistical differences in FPD between devices, except for AbleSpacer® that delivered a lower FPD for both drugs (p < 0.001)., Colegio de Farmacéuticos de la Provincia de Buenos Aires

Background Mycobacterium chimaera is a recently described species within the Mycobacterium avium complex. Its pathogenicity in respiratory tract infection remains disputed. It has never been isolated during cystic fibrosis respiratory tract infection. Case presentation An 11-year-old boy of Asian ethnicity who was born on Réunion Island presented to our hospital with cystic fibrosis after a decline in his respiratory function over the course of seven years. We found that the decline in his respiratory function was correlated with the persistent presence of a Mycobacterium avium complex organism further identified as M. chimaera. Conclusion Using sequencing-based methods of identification, we observed that M. chimaera organisms contributed equally to respiratory tract infections in patients with cystic fibrosis when compared with M. avium subsp. hominissuis isolates. We believe that M. chimaera should be regarded as an emerging opportunistic respiratory pathogen in patients with cystic fibrosis, including young children, and that its detection warrants long-lasting appropriate anti-mycobacterial treatment to eradicate it.

We evaluated the suitability of an aminosterol derivative (ASD), possessing interesting in vitro antimicrobial activities against various resistant pathogens involved in lung infections of cystic fibrosis patients, for aerosol drug delivery.The suitability of 2 and 10 mg/mL ASD solutions for aerosol delivery was evaluated and compared with that of a commercial inhalable solution of tobramycin using Pari LC Plus and eFlow rapid nebulizers. Physicochemical properties of ASD solutions, including pH and osmolarity, were assessed. The particle size distribution of the aerosols was analysed using a laser diffraction method. Effects of mucin on the in vitro antibacterial activities of ASD solutions and tobramycin were assessed using the broth dilution method for MIC determination.MICs of ASD and tobramycin for Pseudomonas aeruginosa ATCC 27853 were 4 and 1 mg/L, respectively, and those for Staphylococcus aureus ATCC 25923 were 1 and 0.5 mg/L, respectively. MICs of tobramycin increased at least 4- and 16-fold for both bacteria after addition of mucin at 1 and 10 mg/mL, respectively, while MICs of ASD remained unchanged. ASD solutions should be prepared in 0.9% NaCl solution in order to produce an isotonic state, and need the addition of NaOH to give a suitable pH value for inhalation. ASD solutions were successfully nebulized using both nebulizers, as reflected by the similarity of the aerodynamic parameters to those of a commercial tobramycin solution.This introductory study demonstrates the suitability of ASDs for aerosol delivery and calls for further work to evaluate such formulations using a lung-infected animal model.

Objective To report pediatric cases of paradoxical respiratory adverse drug reactions (ADRs) after exposure to oral mucolytic drugs (carbocysteine, acetylcysteine) that led to the withdrawal of licenses for these drugs for infants in France and then Italy. Design The study followed the recommendations of the European guidelines of pharmacovigilance for medicines used in the paediatric population. Setting Cases voluntarily reported by physicians from 1989 to 2008 were identified in the national French pharmacovigilance public database and in drug company databases. Patients The definition of paradoxical respiratory ADRs was based on the literature. Exposure to mucolytic drugs was arbitrarily defined as having received mucolytic drugs for at least 2 days (>200 mg) and at least until the day before the first signs of the suspected ADR. Results The non-exclusive paradoxical respiratory ADRs reported in 59 paediatric patients (median age 5 months, range 3 weeks to 34 months, 98% younger than 2 years old) were increased bronchorrhea or mucus vomiting (n = 27), worsening of respiratory distress during respiratory tract infection (n = 35), dyspnoea (n = 18), cough aggravation or prolongation (n = 11), and bronchospasm (n = 1). Fifty-one (86%) children required hospitalization or extended hospitalization because of the ADR; one patient died of pulmonary oedema after mucus vomiting. Conclusion Parents, physicians, pharmacists, and drug regulatory agencies should know that the benefit risk ratio of mucolytic drugs is at least null and most probably negative in infants according to available evidence.

Background: In Cystic Fibrosis (CF) lung function testing is a useful tool for monitoring progression of lung disease. Studies in CF children have shown that lung clearance index (LCI) is significantly greater than healthy controls. Aim: The objectives of this study were: (a) To compare LCI among CF preschoolers (aged 2 to 5 years) and healthy controls and (b) to assess the effect of two-week iv antibiotic treatment on LCI among CF preschoolers with CF. Method: Twenty-eight children with CF (20 were infected with Pseudomonas aeruginosa) and 27 healthy controls performed Multiple-Breath Washout (MBW) measurements and LCI was assessed. The ones with Ps. aeruginosa infection were measured again one month following a two-week course of iv antibiotics. Results: Children with CF had significantly higher LCI compared to controls (mean difference for CF vs control [95%CI] 2.6 [1.8, 3.7], p< 0.001). Children with CF who were infected with Ps. aeruginosa had significantly higher LCI, when compared with Ps. Negative CF children (mean difference [95%CI]: 1.75 [0.38, 3.5], p< 0.05). LCI was reduced significantly after two-weeks course of iv antibiotics (mean difference [95%CI]: −1.97 [1.2, 3.6], p< 0.001). Conclusions: LCI is a sensitive marker to detect lung function abnormalities among preschoolers with CF. It can be used to assess severity of the disease and the effect of treatment.

International audience; We describe what we believe is an entirely novel case of a 15-year-old boy with idiopathic acute eosinophilic pneumonia and unusual, resistant hypoxaemia which necessitated extracorporeal membrane oxygenation. Response to corticosteroids was excellent and a full recovery was observed. Smoking cigarettes and cannabis on the day the symptoms began may have contributed to the occurrence of this rare disease.

Background Systematic flexible bronchoscopy is advocated in the initial management of childhood pulmonary tuberculosis. Methods Our aim was to describe and to compare the systematic flexible bronchoscopy findings of 53 children (6.5±4.4 years; 52.8% boys) with active pulmonary tuberculosis to their initial clinical and radiological (chest x-ray, n=53; CT, n=45) features in an 11-year retrospective study. Results Flexible bronchoscopy was normal in 51% of cases. A severe tracheobronchial involvement (extrinsic compression >50% or obstructive endoluminal mass >25% of the lumen) was found in 10 patients. Flexible bronchoscopy guided therapy in 13 cases (steroids n=12, bronchoscopic extraction of a granuloma n=1) and permitted isolation of Mycobacterium tuberculosis in three patients (5.7%). No useful information was obtained by flexible bronchoscopy in 73.5% of cases. No correlation was found between flexible bronchoscopy findings and clinical features or x-ray findings. Conversely, a strong correlation was found between severe bronchoscopic involvement and tracheobronchial luminal narrowing on CT. The CT negative predictive value was 100% (95% CI 91% to 100%). Based on CT results, flexible bronchoscopy could have been avoided in about 60% of our patients. Conclusions Flexible bronchoscopy remains a very relevant tool in the diagnostic and therapeutic management of childhood pulmonary tuberculosis but resulted in treatment modification or microbiological proof in a minority of our patients. We propose that flexible bronchoscopy in children with pulmonary tuberculosis be limited to those who show tracheobronchial luminal narrowing on an initial CT scan.

International audience; Non-invasive ventilation (NIV) is used increasingly in children with chronic respiratory insufficiency. For best results NIV requires a nasal or face mask adapted to the paediatric patient. Other devices used with masks, such as nebulisers in cystic fibrosis (CF) patients or spacer devices in asthmatics, can be microbiologically contaminated. As we found no literature data about contamination of NIV nasal masks, we conducted a pilot study to evaluate the methods of cleaning used by patients at home and their rate of microbial contamination.

Introduction Respiratory tract infections are the major causes of morbidity and mortality in patients with cystic fibrosis. Nocardia are rarely implicated in these infections and few reports of the involvement of this species are found in the literature. Case presentation We describe a case of lung infection followed by chronic colonization of trimethoprim and sulfamethoxazole resistant Nocardia farcinica in a patient with cystic fibrosis. The chronic colonization of this uncommon bacterium in patients with cystic fibrosis was proved using a newly developed real-time polymerase chain reaction assay, which indicates that this bacterium, despite treatment, is difficult to eradicate. Conclusion Our case report confirms that this organism can be recovered in persons with cystic fibrosis. Its eradication is necessary especially if the patient is to undergo lung transplantation.

Respiratory tract colonization with these bacteria may be common in this population., An increasing body of evidence indicates that nondiphtheria corynebacteria may be responsible for respiratory tract infections. We report an outbreak of Corynebacterium pseudodiphtheriticum infection in children with cystic fibrosis (CF). To identify 18 C. pseudodiphtheriticum strains isolated from 13 French children with CF, we used molecular methods (partial rpoB gene sequencing) and matrix-assisted laser desorption ionization time-of-flight (MALDI-TOF) mass spectrometry. Clinical symptoms were exhibited by 10 children (76.9%), including cough, rhinitis, and lung exacerbations. The results of MALDI-TOF identification matched perfectly with those obtained from molecular identification. Retrospective analysis of sputum specimens by using specific real-time PCR showed that ≈20% of children with CF were colonized with these bacteria, whereas children who did not have CF had negative test results. Our study reemphasizes the conclusion that correctly identifying bacteria at the species level facilitates detection of an outbreak of new or emerging infections in humans.

Recent studies using 16S rRNA gene amplification followed by clonal Sanger sequencing in cystic fibrosis demonstrated that cultured microorganisms are only part of the infecting flora. The purpose of this paper was to compare pyrosequencing and clonal Sanger sequencing on sputum. The sputum of a patient with cystic fibrosis was analysed by culture, Sanger clone sequencing and pyrosequencing after 16S rRNA gene amplification. A total of 4,499 sequencing reads were obtained, which could be attributed to six consensus sequences, but the length of reads leads to fastidious data analysis. Compared to clonal Sanger sequencing and to cultivation results, pyrosequencing recovers greater species richness and gives a more reliable estimate of the relative abundance of bacterial species. The 16S pyrosequencing approach expands our knowledge of the microbial diversity of cystic fibrosis sputum. The current lack of phylogenetic resolution at the species level for the GS 20 sequencing reads will be overcome with the next generation of pyrosequencing apparatus.