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Study Objective To describe human papilloma virus (HPV) vaccination practice among adolescent girls with cystic fibrosis (CF) and to identify reasons for non-vaccination. Design Cross-sectional multicentric study. Setting and Participants Girls aged 9-17 years, attending 7 French pediatric CF centers, and their accompanying adult. Interventions Administration of a self-report questionnaire. Main Outcome Measures The proportion of girls having received or receiving HPV vaccination, compliance with the vaccination schedule, factors associated with vaccination, and reasons for vaccination and for non-vaccination. Results A total of 113 girls and 104 accompanying adults participated. The mean age was 13.6 years (standard deviation 2.5; range 9-17). A total of 34 (30.9%) patients reported having received HPV vaccination. Among the 34 girls aged 15 years or older, 15 (44.1%) were vaccinated. Most patients (58.8%) started vaccination between 11 and 14 years of age (mean age 13.9). Most vaccine prescriptions (67.6%) were made by a CF center health care provider. Factors associated with vaccination were older age (odds ratio [OR] = 1.27, 95% confidence interval [CI] = 1.01-1.6, P = .037 for each year older), previous vaccination by the accompanying parent of one of their children for hepatitis B (OR = 8.01, 95% CI = 0.96-67.02), P = .055), and parental influence on decision-making (OR = 2.77, 95% CI = 0.97-7.95, P = .058). Health care providers’ positive advice and fear of HPV-related disease were the main reasons given to justify vaccination decisions. Insufficient knowledge and concerns about potential side effects were the main barriers. Conclusion HPV vaccination remains insufficient among girls with CF. CF health care providers may play a crucial role in HPV vaccination acceptance, and their sensitization to cervical cancer prevention is mandatory.

The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of -4.1% predicted forced expiratory volume in 1s (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV1 were more at risk of FEV1 decrease.

Actinomycosis is a rare bacterial disease caused by Actinomyces spp., an anaerobic bacteria from the oropharynx, digestive, and female genital tracts. Initial clinical presentation often mimics malignancy, which can lead to a delay in diagnosis. Cervico-facial, genitourinary, digestive, and respiratory features are the most frequent. Few cases are reported in children and risk factors are not well known in this population. We report on the case of an 8-year-old boy with disseminated actinomycosis with cervico-facial, pulmonary, and bone involvement caused by Actinomyces israelii. The infiltrative appearance initially suggested malignancy and the patient was started on chemotherapy for presumed histiocytosis. Evaluation of subsequent tissue samples demonstrated the presence of filamentous structures consistent with fungal or filamentous bacterial infection. Prolonged culture yielded the correct diagnosis. The patient had a severe allergic reaction to piperacillin/tazobactam and was therefore transitioned to clindamycin to complete a 9-month course. This treatment, which has not been reported in children, led to a favorable clinical, biological, and radiological response, with a good clinical tolerance.

With the increasing life expectancy of patients with cystic fibrosis (CF), prevalence of late complications such as CF-related bone disease (CFBD) has increased. It was initially described in 24% of the adult population with CF and has also been reported in the pediatric population. CFBD is multifactorial and progresses in different steps. Both decreased bone formation and increased bone resorption (in different amounts) are observed. CFBD is likely primitive (directly related to the CFTR defect itself), but is also worsened by acquired secondary factors such as lung infections, chronic inflammation, denutrition, vitamin deficiency, and decreased physical activity. CFBD may be clinically apparent (i.e., mainly vertebral and costal fractures), or clinically asymptomatic (therefore corresponding to abnormalities in bone density and architecture). CFBD management mainly aims to prevent the occurrence of fractures. Prevention and regular monitoring of bone disease as early as 8 years of age is of the utmost importance, as is the control of possible secondary deleterious CFBD factors. New radiological tools, such as high-resolution peripheral quantitative computed tomography, allow an accurate evaluation of cortical and trabecular bone micro-architecture in addition to compartmental density; as such, they will likely improve the assessment of the bone fracture threat in CF patients in the near future.

Resume Vingt-cinq ans apres l’identification du gene responsable de la mucoviscidose, les retombees de sa decouverte commencent (enfin) a profiter aux patients. La meilleure connaissance des anomalies genetiques et de leurs consequences, l’avenement de techniques de criblage a haut debit des molecules ont permis de realiser des avancees therapeutiques importantes au cours de ces dernieres annees avec la mise a disposition recente pour les patients de traitements modifiant de facon significative les manifestations immediates, et probablement l’histoire de la maladie. Cet aspect de la medecine personnalisee au cours de la MV, c’est-a-dire basee sur des caracteristiques genetiques propres a l’individu, n’en est vraisemblablement qu’a ses debuts. Il est probable que les nombreux essais cliniques en cours, avec des molecules activatrices et/ou potentiatrices permettent rapidement d’elargir l’offre de ces traitements a un plus grand nombre de patients. Au-dela de ces traitements de reparation proteique, des avancees ont egalement ete faites sur les dispositifs d’administration des antibiotiques par voie inhalee ; certains nouveaux « fluidifiants » et des molecules anti-inflammatoires sont en cours d’evaluation. Il ne faut pas abandonner la piste des traitements symptomatiques, car ce sont bien eux qui ont permis de faire progresser l’esperance de vie des malades au cours de ces dernieres decennies. Les annees a venir devraient voir se developper les essais therapeutiques chez de tres jeunes enfants atteints de MV, dont on sait maintenant que l’atteinte fonctionnelle et structurale respiratoire est tres precoce.

Resume Introduction Malgre une incidence en lente decroissance, la morbi-mortalite de la tuberculose (TB) reste importante. L’emergence et la diffusion de souches multi-resistantes (MR) constituent actuellement un probleme mondial majeur de sante publique. Les cas pediatriques de TB-MR sont un evenement sentinelle indiquant une circulation de souches au sein d’une communaute. L’infection tuberculeuse latente (ITL) precede les cas de TB-MR. La detection et le suivi des sujets contacts sont des axes cles de la lutte anti-TB. Methodes Autour de 20 cas adultes atteints de TB-MR pris en charge dans notre institution 46 enfants contacts ont ete recenses. Resultats Durant le suivi, le nombre d’enfants perdus de vue etait eleve (80 % a 1 an) attestant de la difficulte de suivre une jeune population « reservoir » issue de l’immigration. Le taux d’incidence d’une infection tuberculeuse secondaire etait de 11 % : un enfant a developpe une TB-maladie (virage tuberculinique associe a des anomalies tomodensitometriques thoraciques) ; quatre enfants ont developpe une ITL (virage tuberculinique a tomodensitometrie thoracique normale). Deux enfants ont recu un traitement adapte a la souche du cas index (preventif ou curatif). Discussion Dans ce contexte d’emergence de la TB-MR, la substitution de l’IDR par les tests de liberation d’interferon (TLI), aussi sensibles et plus specifiques (independants du BCG), semble pertinente. De meme, la TDM thoracique optimise la detection de lesions infra-radiographiques. La strategie de traitement de l’ITL en post-exposition d’une TB-MR avec une combinaison adaptee a la souche du cas index est debattue au profit d’une strategie proposee par l’OMS de surveillance prolongee avec traitement curatif d’une TB active le cas echeant.

Resume Introduction Le syndrome d’Allgrove ou « syndrome Triple A » comporte une insuffisance surrenalienne (Adrenal Insufficiency) par resistance a l’hormone adrenocorticotrope (ACTH), une achalasie et une alacrymie, souvent associees a des signes neurologiques. Nous rapportons un nouveau cas de cette maladie genetique rare, interessant par son mode de decouverte dermatologique. Observation Une enfant de 4 ans, nee de parents consanguins au premier degre, presentait une hyperpigmentation buccale et une melanodermie diffuse acquise, ainsi qu’un syndrome sec oculaire ancien. Une insuffisance surrenalienne basse etait confirmee biologiquement. L’association alacrymie-insuffisance surrenalienne faisait rechercher un syndrome d’Allgrove, confirme par l’analyse genetique mettant en evidence une mutation c.1331 + 1G > A a l’etat homozygote dans l’intron 14 du gene de la proteine ALADIN. Les deux parents etaient heterozygotes pour la meme mutation. Deux ans plus tard, l’apparition de vomissements faisait suspecter une achalasie, confirmee par la manometrie œsophagienne. L’enfant etait traitee symptomatiquement par supplementation en hydrocortisone et dilatations œsophagiennes. Discussion Cette observation rappelle que le dermatologue peut etre amene a diagnostiquer un syndrome d’Allgrove. La prise en charge des patients est multidisciplinaire, reposant sur le traitement medical de l’insuffisance surrenalienne et les larmes artificielles en cas d’alacrymie. L’achalasie est traitee par la dilatation œsophagienne ou par chirurgie.

The ECFS-CTN Standardisation Committee has undertaken this review of lung clearance index as part of the group's work on evaluation of clinical endpoints with regard to their use in multicentre clinical trials in CF. The aims were 1) to review the literature on reliability, validity and responsiveness of LCI in patients with CF, 2) to gain consensus of the group on feasibility of LCI and 3) to gain consensus on answers to key questions regarding the promotion of LCI to surrogate endpoint status. It was concluded that LCI has an attractive feasibility and clinimetric properties profile and is particularly indicated for multicentre trials in young children with CF and patients with early or mild CF lung disease. This is the first article to collate the literature in this manner and support the use of LCI in clinical trials in CF. © 2013 European Cystic Fibrosis Society. Published by Elsevier B.V.

Les pneumopathies interstitielles diffuses (PID) de l’enfant representent un groupe heterogene de pathologies respiratoires chroniques rares et severes. La maladie elle-meme, le poids des traitements (oxygenotherapie, bolus de corticotherapie, nutrition enterale), ainsi que les hospitalisations repetees alterent la qualite de vie (QoL) de ces enfants. Le but de cette etude etait de comparer leur QoL a celle d’enfants sains et de determiner les facteurs predictifs d’une QoL degradee. Nous avons inclus prospectivement des enfants de 1 mois a 18 ans atteints de PID d’etiologie connue ou inconnue. Le questionnaire generique PedsQL™ 4.0 a ete rempli par les parents et les enfants de plus de 8 ans, puis converti en score de 0 a 100 points. Au total, 78 enfants ont ete recrutes dans 13 centres francais. La difference de scores entre enfants atteints de PID et enfants sains etait de 11,94 points en faveur de la population saine pour le score total rapporte par l’enfant (p = 0,0003) et de 14,08 points pour celui rapporte par les parents (p La QoL des enfants atteints de PID etait statistiquement degradee par rapport a des enfants sains. Les facteurs predictifs d’un score de QoL plus faible constituent un signe d’alerte pour le clinicien qui peut reperer ces enfants a risque.

Resume Staphylococcus aureus represente 2 a 5 % des etiologies des pneumopathies communautaires. Ces infections surviennent principalement chez des patients âges avec comorbidite, dans un contexte post-grippal. S. aureus est egalement responsable de pneumonie necrosante, heureusement rare, touchant des adolescents ou adultes jeunes, egalement dans un contexte post-grippal. Les pneumonies necrosantes sont associees a la production d’une toxine particuliere, la leucocidine de Panton-Valentine, impliquee dans la necrose pulmonaire, la survenue d’hemoptysies, d’une leucopenie, et d’une mortalite elevee. En Europe, ces souches restent majoritairement sensibles a la penicilline M, qui doit etre utilisee en intraveineuse a forte dose en association avec un antibiotique « anti-toxinique » tel que la clindamycine, et a des immunoglobulines intraveineuses polyvalentes dans les formes graves. Par ailleurs, S. aureus est l’un des pathogenes implique le plus precocement dans les infections respiratoires des patients porteurs de mucoviscidose, ou la resistance a la meticilline joue un role pronostique important. En revanche, l’implication de S. aureus dans les exacerbations de BPCO semble rare. Enfin, S. aureus represente 20 a 30 % des causes de pneumopathies nosocomiales, notamment acquises sous ventilation mecanique. La resistance a la meticilline est alors frequente, peut jouer un role pronostique, et necessite l’utilisation de glycopeptides ou de linezolide. La place des nouveaux anti-staphylococciques tels que les cephalosporines de nouvelle generation ou encore la tigecycline reste a definir.

Resume La pneumopathie necrosante a Staphylococcus aureus secreteur de toxine leucocidine de Panton-Valentine (SA-LPV+) represente moins de 1 % des pneumopathies communautaires de l’enfant et de l’adulte jeune. Les cas neonatals sont exceptionnels. Nous rapportons les observations de deux enfants nouveau-nes, des jumelles non allaitees, qui ont successivement presente une pneumopathie necrosante due a la meme souche de SA-LPV+ malgre les mesures de decolonisation nasale instaurees. Ces deux cas mettent en lumiere la possibilite d’infections pulmonaires severes a SA-LPV+ chez de jeunes nourrissons, et leur mode de transmission intrafamiliale stricte pour lesquelles les mesures d’eradication se sont averees inefficaces.

Resume Objectif Les troubles du metabolisme glucidique (TMG) aggravent la morbimortalite au cours de la mucoviscidose. Au sein d’un reseau regroupant 4 centres de ressources et de competences de la mucoviscidose, une etude sur la conformite des pratiques de depistage et de prise en charge des TMG a ete realisee suite a l’implementation du referentiel reseau. Methodes En 2005 puis en 2007, 60 dossiers de patients de plus de 10 ans ont ete tires au sort. Le referentiel recommandait une glycemie aleatoire a chaque bilan standard, un dosage de l’hemoglobine glyquee (HbA1c) annuel et une hyperglycemie provoquee par voie orale (HGPO) a 10, 15 et 18 ans puis par 2 a 3 ans, a la recherche d’un diabete ou d’une intolerance aux hydrates de carbone. Resultats Le depistage par glycemie aleatoire etait realise de facon annuelle pour 82 % des patients en 2005 et 91,5 % en 2007. L’HbA1c etait realisee annuellement chez 77 % des patients en 2005 et 90 % en 2007 ( p Conclusion Nous observons une tendance a l’amelioration des pratiques de depistage entre 2005 et 2007 pouvant s’expliquer par l’appropriation des recommandations par les professionnels, cette etude ayant permis de les sensibiliser afin d’ameliorer leurs pratiques.

Respiratory function of the cricothyroid muscle (CT) is virtually unknown in the neonatal period. This study was aimed at assessing CT electrical activity (EMG) during respiration and central apneas in non-sedated lambs. Seven full-term and four preterm lambs were instrumented for polysomnographic recording, including EMG of the diaphragm, thyroarytenoid (TA, a glottal constrictor), posterior cricoarytenoid (PCA, the primary glottal dilator) and CT. Phasic CT EMG was usually observed during inspiration and late expiration, whereas phasic TA EMG was observed during early expiration. While TA EMG virtually disappeared in REM sleep, both inspiratory and expiratory CT EMG increased. Overall, while CT EMG was not frequently observed during central apneas in either full-term (10% of apneas, but never simultaneously with TA EMG) or preterm lambs (30% of apneas), it was associated with decreased lung volume and subglottal pressure when present alone or with PCA EMG. Our results concur with the assumption that CT behaves as a laryngeal dilator in the neonatal period.

Resume Objectifs. – Evaluer l'aptitude physique et la tolerance de l'exercice chez des enfants transplantes renaux et preciser les indications et contre-indications de la pratique du sport. Patients et methodes. – L'etude a porte sur 32 enfants et adolescents ayant beneficie d'une transplantation renale depuis plus d'un an. Le poids, la taille, l'indice de masse corporelle (IMC), la fonction respiratoire, l'echocardiographie, la frequence cardiaque et la pression arterielle systolique (PAS) et diastolique (PAD) au repos et a l'exercice, les capacites aerobies au cours d'une epreuve triangulaire d'effort ont ete etudies. Resultats. – Il existait globalement un retard de taille et une augmentation de l'IMC. Les parametres ventilatoires etaient normaux de meme que l'echocardiographie (sauf dans un cas). Globalement l'epreuve d'effort menee jusqu'a l'epuisement a ete parfaitement toleree ; chez quatre enfants seulement la PAS etait anormalement augmentee en fin d'exercice. Les puissances maximales ( P max ) et les consommations maximales d'oxygene (VO2 max ) rapportees a la taille et au poids etaient inferieures a celles des enfants sains, en particulier chez les filles. Conclusion. – La tolerance de l'exercice est bonne chez la grande majorite des enfants transplantes renaux ; lorsque l'epreuve d'effort est normale il n'y a pas de contre-indication a la participation a l'education physique et sportive (EPS) scolaire ou a la pratique d'un sport si cela est souhaite (en prenant en compte le risque traumatique pour le greffon).

Ondine syndrome is the central congenital hypoventilation syndrome (CCHS) caused by the mutation of the PHOX2B gene. In late onset cases, the symptomatology often appears after an acute event (infection, general anesthesia, drug intake), increasing hypoventilation. We report a case of late onset Ondine curse in a 9-year-old girl. The diagnosis was made based on a hypercapnic coma complicating a respiratory infection caused by Mycoplasma pneumoniae and was confirmed by genetic testing. In the patient's history we found symptoms that had not been noted (e.g., enuresis, morning headache, adynamia), attesting to chronic hypoventilation. Through this observation, we review the literature on CCHS, notably late onset cases, which are rare and insidious, emphasizing the pre-existing hypoventilation symptoms in this child. This case underlines the need for all practitioners not to trivialize these symptoms so as to decrease the current delay in diagnosis for late onset CCHS and to introduce optimal care as soon as possible.

The primary aim of the study was to explore cardiorespiratory reflexes originating from laryngeal C fiber endings in the neonatal period. Seventeen lambs were instrumented for recording glottal adductor and diaphragm EMG, heart rate, systemic arterial pressure and respiratory movements. C fiber blockade was induced in eight lambs by 30 mg/kg capsaicin, the remaining nine lambs serving as controls. Cardiorespiratory reflexes were induced in non-sedated lambs by flowing air, menthol or 13% CO2, or by injecting water or 50 microg capsaicin in the laryngeal inlet through an endoscope. Responses to all stimuli but capsaicin were similar between the two groups. While cardiorespiratory responses were induced by capsaicin in control lambs, the responses were significantly inhibited in lambs with C fiber blockade. We conclude that laryngeal C fiber endings are functional and responsible for laryngeal chemoreflexes in newborn lambs.

Swallowing is a powerful inhibitor of respiratory rhythm in infants. The present study was aimed at investigating the influence of states of alertness on non-nutritive swallowing (NNS) frequency, on NNS and respiration coordination, and on bursts of NNS frequency in newborn lambs. Six full term newborn lambs were instrumented for electroencephalogram, eye movement, diaphragm and thyroarytenoid muscle electromyogram, nasal flow and electrocardiogram. Polysomnographic recordings were performed in non-sedated lambs, using radiotelemetry. NNS frequency was significantly higher during quiet wakefulness (W) and active sleep (AS) than during quiet sleep (QS). NNS mainly interrupted inspiration and the transition phases between expiration and inspiration, especially in W and AS. Bursts of NNS occurred significantly more often during AS. This study highlights the relevance of the ovine model to study ontogeny of NNS during sleep, and documents the influence of sleep on NNS and respiration coordination.

Introduction Un lien entre les infections respiratoires par le virus respiratoire syncytial (VRS) et la primocolonisation precoce par Pseudomonas aeruginosa (PA) a ete etabli pour les jeunes patients atteints de mucoviscidose. A ce titre, l’immunoprophylaxie par palivizumab (Synagis ® ), anticorps monoclonaldirige contre la proteine F du VRS, chez ces patients, est actuellement controversee. Objectif L’objectif principal de notre etude etait de determiner l’interet du palivizumab a retarder la primocolonisation par PA. Les objectifs secondaires etaient de preciser l’interet de cette molecule a retarder la primocolonisation par Staphylococcus aureus (SA), Haemophilus influenzae (Hp) et a reduire le nombre d’hospitalisation pour infections respiratoires aigues par VRS. Materiel et methodes Nous avons realise une etude retrospective, cas-temoins comparant des nourrissons ayant recu du palivizumab au centre de ressources et de competences de Besancon (CRCM) ( n = 40) et des temoins n’en ayant pas recu au CRCM de Lyon ( n = 140). Les patients ont ete suivis pendant leurs trois premieres annees de vie. Resultats Nous n’avons pas montre de difference statistiquement significative pour l’âge de primocolonisation par PA entre les cas (16,6 ± 16,5 mois) et les temoins (12,9 ± 8,8 mois ; p = 0,143). Aux termes des trois premieres annees de vie, 41,4 % des nourrissons–cas avaient ete primocolonises par PA et 40 % des nourrissons–temoins ( p = 0,384). En comparaison, ce taux est de 37,2 % dans la cohorte du registre national francais ( n = 2171). Nous n’avons pas observe de difference statistiquement significative pour les âges moyens de primocolonisation par SA et Hp entre les cas et les temoins (respectivement, 10,0 ± 9,1 mois pour les cas et 8,2 ± 9,6 mois pour les temoins [ p = 0,397] ; 7,7 ± 6,1 pour les cas et 10,1 ± 8,7 mois pour les temoins [ p = 0,127]). Les taux d’hospitalisations au cours des trois premieres annees de vie n’etaient pas differents entre les deux centres (17,5 % pour les cas, 22,9 % pour les temoins ; p = 0,06). Les infections respiratoires basses a VRS etaient rares dans les deux groupes (5 % pour les cas ; 2,8 % pour les temoins [ p = 0,634]). Conclusion Les resultats de notre etude indiquent que les benefices cliniques et microbiologiques ne sont pas en faveur d’une utilisation systematique du palivizumab chez les nourrissons atteints de mucoviscidose.

Introduction Les pneumopathies d’hypersensibilite ou alveolites allergiques extrinseques representent un groupe de maladies pulmonaires inflammatoires a mecanisme immunologique liees a l’inhalation repetee de poussieres organiques [1] . Elles sont surtout observees chez l’adulte en contexte professionnel mais existent egalement chez l’enfant et sont moins bien connues [2] . Notre objectif est de decrire les cas francais pediatriques afin d’en observer les caracteristiques diagnostiques, les traitements, les resultats cliniques et fonctionnels. Methodes Il s’agit d’une etude observationnelle descriptive retrospective multicentrique francaise portant sur seize enfants atteints d’alveolite allergique extrinseque. Les caracteristiques cliniques et fonctionnelles au diagnostic, les allergenes en cause, les aspects radiologiques, les resultats du lavage bronchoalveolaire, la recherche de precipitines, la prise en charge therapeutique et la reponse au traitement sont decrits dans cette etude. Resultats La forme subaigue est majoritaire (88 %). Les symptomes cliniques comprennent principalement une dyspnee au repos ou a l’effort. Les seize patients sont sensibilises a des antigenes aviaires ou issus de moisissures. Le principal traitement est l’eviction allergenique, frequemment associee a une corticotherapie orale ou en bolus. L’evolution est le plus souvent favorable mais certains patients peuvent evoluer vers une forme chronique. Conclusion Le diagnostic repose sur un ensemble d’arguments evocateurs. Les differents protocoles therapeutiques ne sont pas standardises mais sont bases sur les donnees observees dans la litterature adulte ou pediatrique. Le pronostic est favorable dans la majorite des cas.

Recent data has shown that lung inflammation and infection subvene very early in very young infants with Cystic Fibrosis (CF). This leads to impaired lung function and structural damage, even in asymptomatic children. In the CF-pig model constitutional airway narrowing is present at birth, and is associated with defective mucus migration, and impaired bacterial clearance. At the age of 3 months, 25% of screened CF infants show decreased lung function. Air trapping is also present in 68% and bronchiectasis in 28% of patients. At the same age, the presence of neutrophil elastase in the bronchoalveolar lavage is an ominous sign since it triples the risk of bronchiectasis at the age of 3 years. Since only very few drug therapies have been validated in the preschool children, adapted clinical trials are warranted in this age group. Early interventions may have a huge impact on the natural history of CF, on the condition of not interfering with normal lung growth.

Since the discovery of chloride secretion by the Cystic Fibrosis Transport regulator CFTR in 1983, and CFTR gene in 1989, knowledge about CFTR synthesis, maturation, intracellular transfer and function has dramatically expanded. These discoveries have led to the distribution of CF mutations into 6 classes with different pathophysiological mechanisms. In this article we will explore the state of art on CFTR synthesis and its chloride secretion function. We will then explore the consequences of the 6 classes of mutations on CFTR protein function and we will describe the new therapeutic developments aiming at correcting these defects.

Community-acquired pneumonia is complicated by purulent pleural effusion in approximately 28% of affected children. Its incidence has increased over the last decade. Streptococcus pneumoniae is the most frequent causal microorganism, encountered in roughly 80% of cases. We report the case of an immunocompetent 13-year-old teenager wearing orthodontic braces who suffered from pleuropneumonia due to Capnocytophaga sputigena. To date, this Gram-negative bacillus, a commensal of the buccal cavity, had never been reported to be responsible for pleural or lung infection. The clinical presentation is similar to that observed with usual bacteria, while bacterial sensitivity to betalactam antibiotics is excellent.

The 2000 French consensus conference for acute viral bronchiolitis management underlined the fundamental role of chest physiotherapy. According to Chalumeau and al., rib fractures were found out in 1/1000 children hospitalized for bronchiolitis or pneumonia. However, such complication of chest physiotherapy is exceptional. We report 2 cases with third to sixth lateral rib fractures after chest physiotherapy in infants with bronchiolitis. Despite the rarity of these complications, clinicians must keep in mind this etiology while facing rib fractures in infants.

Objective: CFRD is the most common comorbidity in CF. The recommended treatment of CFRD is Insulin, but other options are also in use. Only few data exist related to other treatment options. Therefore we analyzed the German CFregister for the documented therapy of CFRD. Methods: Data from the German CF-register were used from patients with CFRD in 2010 in a retrospective observational study. Delta FEV1% and delta BMI-Zscore from 2 year before diagnosis of CFRD to year of diagnosis and from year of diagnosis to 2 years after were calculated for each treatment group (Insulin, oral anti-diabetic drugs, no therapy with drugs) and compared using ANOVA-analysis. Results: 798 patients with CFRD were documented in 2010. 51.9%.were female; age (mean±SD) at diagnosis 23.8±9.3 years; CFRD duration 5.8±4.5 years. Treatment: 57.6% Insulin, 9.8% oral anti-diabetic drugs, 2.1% with both and 30.2% without any drug treatment. The mean (±SD) CFRD duration in the non-treated patients was 4.5±4.0 years. ANOVA tests showed no differences between treatment groups regarding changes in FEV1% and BMI-Z-score from 2 years before to the year of diagnosis and delta FEV1 and delta BMI-Z-score and from year of diagnosis to 2 years later. Conclusions: The percentage of patients treated with oral anti-diabetic drugs is in the international published range. We know the weakness of retrospective studies. Nevertheless the numbers are high and the observation time is long. Our data point to the question if insulin is the only successful initial treatment of CFRD in all CF patients. At least a part of patients with CFRD seems to be well treated with other regimes. Supported by German CF Foundation.

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.

Resume Chez un nourrisson de cinq mois un acces de toux asphyxiante sur fond de dyspnee moderee conduit a la decouverte d'images kystiques pulmonaires bilaterales sur la radiographie thoracique. L'exploration chirurgicale et l'examen anatomopathologique decouvrent un pleuropneumoblastome, une tumeur embryonnaire rare de l'enfant a potentiel malin. Ce cas radioclinique illustre la necessite de realiser l'exerese chirurgicale de lesions pulmonaires kystiques d'aspect malformatif.

Background In 2008, ECFS-CTN selected 6 multisite centres out of the 7 French inter-regions. Objectives The French CF Society (SFM) and the patients’ association setup (2009) a NPRC in order to optimize the contribution to Clinical Research (CR) of the 45 CF centres following 6200 patients recorded in the French CF Registry. Methods Establishment of i. a Steerco including the principal investigator and a clinical research coordinator (CRC) of each inter-region, SFM and patients’ association representatives; ii. a website, http://www.recherchecliniquemuco.fr/ ; iii. the CRC in each inter-region has the following main tasks: – collect/enter data related to patients’ inclusions into a software – run CR protocols within the multisite centres – assist the development of clinical studies – prepare investiqator's responses to CTN requests Results Advices by the Steerco for the setup of CR projects. The Webcast CR data show 31 industrial and 75 academic projects either carried out or in progress since 2010. Intervention of Steerco to setup a Vertex LEAD action for LCI training in France. Regular improvement in ECFS assessments of French multisite CTN centres. Conclusion The NPCR may help meet ECFS-CTN multisite centres challenges by: – giving an overview of CR projects – implementing tools for patient's screening and inclusion – sharing effective experiences of multisite centres – reinforcing the link between centres by greater involvement into a project regarding a priority topic (e.g. antibiotic treatment/LCI/physical activity) – identifying projects that have resulted in a publication and sometimes in a change in care practices – coordinating the ECFS eQUIP project deployment.

Objectives The aim of DIAMUCO study is to describe the natural history of the glucose tolerance (GT) and to identify predictive factors of the changes in GT in cystic fibrosis (CF) patients for a three-year period. Methods We used a cross-sectional study design and included a total of 228 patients, 111 children (between 10 and 18 years) and 117 adults between 2009 and 2011. All patients had an annual screening. Patients were classified as having normal glucose tolerance (NGT), impaired glucose tolerance (IGT), or CF-related diabetes mellitus (CFRD) using the 2-h oral glucose tolerance test (OGTT). Results At baseline, 67.1% (n = 153) patients were classified as NGT, 25.4% (n = 58) as IGT and 7.4% (n = 17) as CFRD. Over half of patients changed at least once of glucose tolerance status during follow-up. The mean decline of FEV1 was 1.54 per year without difference between female and male. A decrease in FEV1 (p TableClinical characteristicsTotalCFRDIGTNTpAge20.1±8.124.3±9.319.3±7.420.0±8.20.1FEV172.4±24.165.0±22.173.8±23.972.6±24.50.4Weight z-score0.5±1.5−0.70±1.24−0.79±1.18−0.37±1.50.1 Conclusion We confirm the variability of glucose tolerance status over time. The impact of glucose tolerance status on respiratory deterioration seems to concern only patients with permanently CFRD status. The early treatment decision must therefore be careful.