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Background Child exposure to cigarette smoke is harmful. It should be reduced through parental smoking cessation interventions. The aim of our study was to determine the impact of simple advice provided by the pediatrician on the smoking habits of parents of children with cystic fibrosis (CF), diabetes mellitus (DM) and infants hospitalized for bronchiolitis. Methods Parents were interviewed on their smoking habits. Smoking cessation advice was provided by the pediatrician. A new smoking habits assessment was done at three months by phone interviews. Results Two hundred and sixty parents were interviewed (91 in the CF group, 136 in the DM group and 33 in the bronchiolitis group). Seventy parents were active smokers: 33% of parents of children with CF, 23.5% of parents of children with DM and 24.2 % for those with infants hospitalized for bronchiolitis (p = 0.42). In the CF group, smoking cessation had been significantly more frequently discussed with the medical team previously. Sixty-seven smoking parents (95.7%) answered the 3-month assessment: 29.8% reported having started a smoking cessation process; 10.4% had quit smoking. The quitting rate was significantly higher in the groups of patients followed for a respiratory disorder (37.5% for bronchiolitis, 15% for CF versus 0% for DM, p= 0.005). Conclusion This study shows the important role that information and simple advice from pediatricians can have in initiating smoking cessation in parents of patients followed in specialized clinics or who are hospitalized, with a greater efficiency in parents of patients suffering from lung disorders. This article is protected by copyright. All rights reserved.

Resume Introduction Le scanner thoracique est essentiel pour le suivi respiratoire des enfants atteints de mucoviscidose, mais sa repetition expose a la toxicite potentielle des radiations ionisantes. L’objectif de cette etude etait de comparer un protocole scanographique complet (acquisition en inspiration helicoidale puis expiration sequentielle) a un protocole ne comprenant que les coupes expirees, en termes d’analyse d’image et de dose delivree de radiations ionisantes. Methodes Sur une cohorte monocentrique de 57 enfants âges de 5 a 18 ans, 78 examens ont ete scores par deux radio-pediatres sur le protocole complet puis uniquement sur les coupes expirees. Resultats La correlation entre les scores radiologiques globaux obtenus selon les deux protocoles etait tres bonne (r = 0,90 pour les deux radiologues) ainsi que pour les scores de bronchectasies (r = 0,72 et 0,86), impactions mucoides (r = 0,87 et 0,83) et trappage expiratoire (r = 0,96 et 0,92). Les parametres d’irradiation etaient reduits, avec un produit dose longueur (DLP) passant de 103,31 mGy.cm (protocole complet) a 3,06 mGy.cm (protocole expiration seule) (p Conclusion Afin d’espacer la realisation de scanners complets plus irradiants, l’utilisation d’un protocole avec seulement des coupes expirees permettrait un diagnostic tomodensitometrique de qualite tout en reduisant l’irradiation chez les enfants atteints de mucoviscidose.

To assess the diagnostic and therapeutic difficulties as well as the long-term complications of prolonged endobronchial foreign body retention.Between January 2000 and May 2021, 794 patients with suspected foreign body aspiration (FBA) were hospitalized in our department. A total of 12 patients with a delayed diagnosis of over 1 month were included. FBAs were confirmed by flexible or rigid endoscopy. A retrospective analysis of medical records was performed.Six male patients and six female patients were hospitalized due to prolonged FBA. The average age was 6.90 years (range: 1-13 years). The average duration of the foreign body retention was 2.60 years (2 months to 9 years). A choking event was found in eight cases. Coughing and wheezing were the main symptoms and signs. A misdiagnosis of asthma was made for five patients. Two atypical clinical presentations led to diagnosis of endobronchial foreign body, unilateral pleurisy, and hemoptysis. We report one case of an occult foreign body externalized spontaneously through a pneumo-pleuro-cutaneous fistula. The most common clinical and radiological findings were of pneumonia and atelectasis. Computed tomography showed localized bronchiectasis in three patients. FBAs were removed with a rigid bronchoscope in eight cases. Other extractions were carried out with a flexible endoscope. The foreign bodies were most frequently of vegetable origin, such as seeds and peanuts. A granulation tissue was observed in seven cases. Bronchial stenosis and bronchiectasis are the most common late complications. Only one patient needed a surgical intervention.FBA should always be considered in the differential diagnosis of chronic or recurrent respiratory diseases, even in the absence of a previous choking event. Clinical and radiological findings should be carefully evaluated for a possible FBA. Delay in diagnosis and treatment of FBA should be avoided in order to prevent complications. Open surgery may be required when lung abscess has occurred.

Resume Le depistage neonatal (DNN) de la mucoviscidose, grâce a une prise en charge multidisciplinaire tres precoce des nourrissons, est optimal en termes de pronostic pour les patients. Depuis 20 ans, il a connu une expansion internationale spectaculaire. Les performances du DNN national francais realise depuis 2002 sont en accord avec les standards europeens pour la valeur predictive positive (0,31 pour un minimum de 0,30) et la specificite (0,95 pour un minimum de 0,95) ; nous soulignons le nombre tres faible de cas non conclus, un pourcentage tres eleve de test de la sueur realises (95,5 %) et d’identification des mutations (96,6 %), un ratio de cas de mucoviscidose par rapport aux cas de diagnostic incertain de 6,3:1, ainsi qu’une strategie efficace pour reperer les faux negatifs. Une nouvelle organisation du DNN francais vient de se mettre en place, il est capital de maintenir l’efficacite du processus, du nouveau-ne en maternite jusqu’au diagnostic dans des centres de reference ou de competences de la mucoviscidose avec un recueil exhaustif des donnees et leur validation. Par ailleurs, une proposition de changement d’algorithme introduisant le dosage du polypeptide d’activation pancreatique est soumise aux nouvelles instances du DNN. L’annonce d’un diagnostic positif reste difficile et peu standardisee, si bien qu’une plateforme de simulation est en cours de mise en place. Nous detaillons ici les bonnes pratiques et les difficultes de realisation du test de la sueur, avec les seuils de chlorures recemment redefinis, ainsi que les modalites du conseil genetique.

In cystic fibrosis (CF), cystic fibrosis transmembrane regulator (CFTR) dysfunction leads to digestive disorders that promote intestinal inflammation and dysbiosis enhancing gastrointestinal symptoms. In pancreatic insufficiency CF patients, both intestinal inflammation and dysbiosis, are associated with an increase in the fecal calprotectin (FC) level. However, associations between the FC level, gastrointestinal symptoms, and quality of life (QoL) remain poorly studied. We aimed to assess such associations in pancreatic insufficiency CF children. The FC level was measured in pancreatic insufficiency CF children&rsquo, s stool samples. Children and their parents completed two questionnaires: The Gastrointestinal Symptoms Scales 3.0-PedsQLTM and the Quality of Life Pediatric Inventory 4.0-PedsQLTM. Lower scores indicated worse symptomatology or QoL. Thirty-seven CF children were included. A FC level above 250 &micro, g/g was associated with worse gastrointestinal symptoms and QoL scores. The FC level was inversely correlated with several gastrointestinal scores assessed by children (i.e., Total, &ldquo, Heart Burn Reflux&rdquo, &ldquo, Nausea and Vomiting&rdquo, and &ldquo, Gas and Bloating&rdquo, ). Several QoL scores were correlated with gastrointestinal scores. The FC level was weakly associated with clinical parameters. Some gastrointestinal and QoL scores were related to disease severity associated parameters. In CF, the FC level, biomarker previously related to intestinal inflammation and dysbiosis, was associated with worse digestive symptoms and QoL scores.

Background Whereas Burkholderia infections are recognized to impair prognosis in cystic fibrosis (CF) patients, there is no recommendation to date for early eradication therapy. The aim of our study was to analyse the current management of initial colonisations with Burkholderia cepacia complex (BCC) or B. gladioli in French CF Centres and its impact on bacterial clearance and clinical outcome. Methods We performed a retrospective review of the primary colonisations (PC), defined as newly positive sputum cultures, observed between 2010 and 2018 in five CF Centres. Treatment regimens, microbiological and clinical data were collected. Results Seventeen patients (14 with BCC, and 3 with B. gladioli) were included. Eradication therapy, using heterogeneous combinations of intravenous, oral or nebulised antibiotics, was attempted in 11 patients. Six out of the 11 treated patients, and 4 out of the 6 untreated patients cleared the bacterium. Though not statistically significant, higher forced expiratory volume in 1 second and forced vital capacity at PC and consistency of treatment with in vitro antibiotic susceptibility tended to be associated with eradication. The management of PC was shown to be heterogeneous, thus impairing the statistical power of our study. Large prospective studies are needed to define whom to treat, when, and how. Conclusions Pending these studies, we propose, due to possible spontaneous clearance, to check the presence of Burkholderia 1 month after PC before starting antibiotics, at least in the milder cases, and to evaluate a combination of intravenous beta-lactam + oral or intravenous fluoroquinolone + inhaled aminoglycoside.

Whereas Burkholderia infections are recognized to impair prognosis in cystic fibrosis (CF) patients, there is no recommendation to date for early eradication therapy. The aim of our study was to analyse the current management of initial colonisations with Burkholderia cepacia complex (BCC) or B. gladioli in French CF Centres and its impact on bacterial clearance and clinical outcome. We performed a retrospective review of the primary colonisations (PC) observed between 2010 and 2018 in five CF Centres. Treatment regimens, microbiological and clinical data were collected. Seventeen patients (14 with BCC, and 3 with B. gladioli) were included. Eradication therapy, using heterogeneous combinations of intravenous, oral or nebulised antibiotics, was attempted in 11 patients. Six out of the 11 treated patients, and 4 out of the 6 untreated patients cleared the bacterium. Though not statistically significant, higher FEV1 and FVC at PC and consistency of treatment with in vitro antibiotic susceptibility tended to be associated with eradication. Thus, the management of PC was shown to be heterogeneous, and the statistic power of our study insufficient. Large prospective studies are needed to define who to treat, when, and how. Pending these studies, we propose, due to possible spontaneous clearance, to check the presence of Burkholderia one month after PC before starting antibiotics, at least in the milder cases, and to evaluate a combination of IV beta-lactam + oral or IV fluoroquinolone + inhaled aminoglycoside.

Chest computed tomography (CT) is essential to monitor lung disease in children with cystic fibrosis, but it involves recurrent exposure to ionizing radiation. The aim of this study was to compare the current complete CT protocol (volumetric end-inspiratory plus sequential expiratory acquisition) to a sequential expiratory acquisition protocol alone in terms of image analysis and ionizing radiation dose.Seventy-eight CT scans from 57 children aged 5 to 18 years old were scored on the complete protocol images and on the expiratory sequential images only. Each CT protocol was scored independently, using the Brody scoring system, by two paediatric radiologists.Correlations between the Brody global scores of the two different CT protocols were very good (r=0.90 for both observers), for the bronchiectasis score (r=0.72 and 0.86), mucus plugging score (r=0.87 and 0.83), and expiratory trapped air (r=0.96 and 0.92). Total ionizing radiation dose was reduced, with the measured dose length product (DLP) reduced from 103.31mGy.cm (complete protocol) to 3.06mGy.cm (expiratory protocol) (P0.001).An expiratory chest CT protocol was accurate in diagnosing early signs of CF disease and permitted significant reduction of radiation dose. This protocol would allow spacing out of complete CT scanning with its higher radiation dose and should be considered for the monitoring of lung disease severity in children with CF.

Objectives: In France, chest physiotherapy for cystic fibrosis (CF) patients is partly performed by liberal physiotherapists at home or at the professional's office. The first lockdown, due to the COVID-19 pandemic, impaired the possibility to practice liberal physiotherapy. We aimed to describe, in our paediatric CF centre, the changes in physiotherapy and sport practice induced by this lockdown, and to correlate it with respiratory symptoms and pulmonary function evolution. Methods: During the lockdown, 68 CF patients >6 years old and their parents answered a questionnaire on time spent in physiotherapy, with or without a liberal professional, and sport practice before and during lockdown. Respiratory symptoms and FEV1 were collected at the venues before and after the lockdown. Results: This study showed a clear reduction of time spent with a liberal physiotherapist from 73 min/week before lockdown to 30 min/week during lockdown. Time of physiotherapy practised alone or with parents increased from 2 min/week to 26 min/week. Thus, the overall physiotherapy time per week was only slightly reduced from 90 min/week before lockdown to 80 min/week during lockdown. Time spent in sport practice was not significantly decreased on average (233 min/week before lockdown vs 225 min/week during lockdown). The standard deviation of sport practice evolution was large (–8 min ± 225 min), reflecting a wide variation of sport practice changes from one patient to another. No deterioration in respiratory symptoms nor FEV1 was observed. Conclusions: During the lockdown in France, we noted that our patients drastically decreased their time spent with a liberal physiotherapist, without enough counterbalance with auto-drainage or sport pratice increase. Nevertheless, their respiratory status was not impaired, suggesting some other confounding factors, such as prevention from viral infection, time to rest or improvement of other treatment observance.

International audience; Pseudomonas aeruginosa and Staphylococcus aureus toothbrush contamination in cystic fibrosis (CF) is unknown. This pilot study aimed to determine their prevalence and the potential involvement of toothbrushes in pulmonary infection. Methods. Toothbrush bacteriological analysis for children aged 8–18 years was conducted on 27 CF patients, 15 healthy siblings, and 15 healthy children from the general population. Results : S. aureus was detected on 22% of the patients’ toothbrushes, and 13% of healthy children's toothbrushes and P. aeruginosa on 15% of patients’ toothbrushes and 0–13% of healthy children's toothbrushes. There was no statistical correlation between pulmonary colonization and toothbrush contamination. P. aeruginosa genotyping showed two identical clones on the patients’ toothbrushes and in their sputum, and between one patient's sputum and his sibling's toothbrush. Conclusion : S. aureus and P. aeruginosa can colonize CF patients’ toothbrushes. The impact on pulmonary colonization remains unknown. Toothbrush decontamination methods need to consider these bacteria in CF patients.

Resume Les fistules arterio-veineuses pulmonaires sont des vaisseaux anormaux reliant l’artere aux veines pulmonaires. Elles entrainent un shunt extracardiaque droite-gauche a l’origine d’une hypoxemie refractaire a l’oxygenotherapie. Nous rapportons le cas d’une fillette de 7 ans presentant une volumineuse fistule arterio-veineuse (FAV) pulmonaire avec une hypoxemie refractaire decouverte a l’occasion d’une anesthesie generale pour adenoidectomie. Cette FAV a ete diagnostiquee par l’angioscanner thoracique. Une occlusion percutanee a ete realisee en deux temps au cours d’un catheterisme cardiaque interventionnel. Ce fait clinique illustre les circonstances de decouverte, la demarche diagnostique face a une hypoxemie refractaire de l’enfant et les avantages et inconvenients de la fermeture percutanee de FAV pulmonaire.

Objectifs Decrire l’âge de menarche et le deroulement pubertaire des filles atteintes de mucoviscidose. Patients, methodes Il s’agit d’une etude transversale, multicentrique, chez 164 jeunes filles de plus de 8 ans suivies pour mucoviscidose dans 7 CRCM. L’âge median aux premieres regles a ete estime par la methode de Kaplan-Meier chez 164 filles dont 97 reglees. Nous avons compare certains parametres lies a la maladie entre le groupe des filles avec une puberte retardee (PR) et le groupe avec une puberte normale (PN). Resultats L’âge median aux premieres regles est estime a 13 ans [11–21 ans]. Ces resultats correspondent a ceux observes dans la population generale, mais la distribution des âges montre une proportion plus importante de pubertes retardees (17,3 % versus 5 %, p Conclusion Il s’agit de la plus grande cohorte de suivi pubertaire de patientes atteintes de mucoviscidose montrant un âge de menarche comparable a la population generale. Les precedentes etudes sont anciennes et decrivaient un retard de menarche de 2 ans. Cependant, il faut rester vigilant sur le depistage des retards pubertaires qui demeurent plus frequents que dans la population generale.

In advanced cirrhosis, decreased lung transfer for carbon monoxide (TLCO) and increased alveolar-arterial oxygen tension difference (PA-aO2≥15 mmHg while breathing ambient air) are frequently detected. Pulmonary membrane diffusion capacity for CO (DmCO) and pulmonary capillary blood volume (Vcap) can be derived from the simultaneous measurement of TLCO and lung transfer for nitric oxide (TLNO). Measurements of single-breath TLNO and TLCO were performed in 49 cirrhotic patients with advanced liver cirrhosis and in 35 healthy controls to derive Vcap, DmCO, and TLNO:TLCO ratio. Twenty-five patients had increased PA-aO2, of whom 11 had hepatopulmonary syndrome (HPS). Compared with controls, non-HPS patients with normal PA-aO2had a significant ∼10% decrease in TLCO, DmCO, and Vcap but similar TLNO:TLCO ratios. Compared with non-HPS patients with normal PA-aO2, non-HPS patients with increased PA-aO2had lower Vcap and higher TLNO:TLCO ratio but similar DmCO. HPS patients had lower Vcap and higher TLNO:TLCO ratios than both subgroups of non-HPS patients. In cirrhotic patients, TLNO:TLCO ratios correlated positively, and TLCO (percentage of the predicted value) and Vcap (percentage of the predicted value) correlated negatively with PA-aO2( r2= 0.25, P = 0.0003, r2= 0.48, P < 0.0001 and r2= 0.57, P < 0.0001, respectively). We concluded that, in cirrhotic patients, lower TLCO and increased PA-aO2are associated with lower Vcap. In addition, high TLNO:TLCO ratios in patients with increased PA-aO2suggest a decreased thickness of the capillary blood layer in these patients.

In cirrhotic patients, alveolar nitric oxide (NO) concentration is increased. This may be secondary to increased output of NO produced by the alveoli (V'(A,NO)) and/or to decreased lung transfer factor of NO. In advanced liver cirrhosis, NO produced by the alveoli may play a role in abnormalities of pulmonary haemodynamics and gas exchanges. In cirrhotic patients, we aimed to measure V'(A,NO) and to compare V'(A,NO) with pulmonary haemodynamics and gas exchange parameters. Measurements were performed in 22 healthy controls and in 29 cirrhotic patients, of whom eight had hepatopulmonary syndrome. Exhaled NO concentrations were measured at multiple expiratory flow rates to derive alveolar NO concentration. V'(A,NO) was the product of alveolar NO concentration by single breath lung transfer factor for NO. V'(A,NO) was increased in patients (median (range) 260 (177-341) nL x min(-1)) compared with controls (79 (60-90), p0.0001). Alveolar-arterial oxygen tension difference failed to correlate with V'(A,NO). However, cardiac index correlated positively and systemic vascular resistance correlated negatively with V'(A,NO) (r = 0.56, p = 0.001 and r = -0.52, p = 0.004, respectively). In cirrhotic patients, NO was produced in excess by the alveolar compartment of the lungs. Alveolar NO production was associated with hyperdynamic circulatory syndrome but not with arterial oxygenation impairment.

Resume Introduction La frequence elevee des manifestations respiratoires d’allure asthmatique a ete clairement demontree chez les athletes de haut niveau. Les donnees chez les sportifs occasionnels et sur les autres manifestations atopiques (rhinite allergique, sensibilisations aux allergenes) sont plus contradictoires. Methodes Une association entre le temps d’entrainement et la presence d’une rhinite allergique ou de critere d’atopie a ete recherchee chez 153 sujets de niveaux sportifs differents. Dans un sous-groupe de sujets s’entrainant intensement (au moins dix heures hebdomadaires), l’influence du mode de pratique (exterieur/interieur, endurance/resistance) a ete analysee. Resultats Aucune association n’a ete retrouvee entre le temps de pratique sportive et l’existence de rhinite allergique ou d’atopie. Chez les sportifs s’entrainant intensivement, la pratique en milieu interieur etait significativement associee a la rhinite allergique. Parmi les sportifs s’entrainant intensivement, 32,1 % etaient porteurs de rhinite allergique. Soixante-cinq pour cent des rhinites allergiques responsables d’un inconfort nasal n’etaient pas traitees. Conclusions Le temps de pratique sportive n’est pas directement lie a la presence de rhinite allergique ou d’atopie. Le lieu de pratique sportive semble jouer un role. La rhinite allergique reste frequente et negligee chez le sportif.

Objectif Paecilomyces lilacinus (PL) et variotii (PV) sont des champignons opportunistes emergents, non etudies dans la mucoviscidose. Methode Etude retrospective aux CRCM enfants et adultes de Toulouse sur les donnees informatisees du 01/01/2008 au 01/09/2014. Sont etudiees les caracteristiques des patients au 1 er isolement de Paecilomyces (P) dans l’expectoration, et leur evolutivite : VEMS et IMC en % de la norme, a 1 an et 2 ans avant et apres, et le type de colonisation bronchique chronique, intermittente ou ponctuelle. Resultat Sur cette periode sont realisees 3337 analyses microbiologiques de l’expectoration chez 98 enfants (de plus de 5 ans) et 164 adultes ; 39 (1,1 %) sont positifs pour P. Vingt patients ont au moins une fois du P : 2 colonisations chroniques (PL), 4 intermittentes (PL) et 14 ponctuelles (12 PL, 2 PV). Au 1 er isolement, l’âge median est de 13 ans (9 a 36), le VEMS median de 82 % (28 a 112) et le BMI median de 94 % (79 a 113). Tous sont colonises a Pseudomonas aeruginosa , 17 a S. aureus , et 14 a Aspergillus . La charge therapeutique semble elevee : l’annee precedente, 11 (55 %) ont de l’itraconazole, 13 une corticotherapie (12 inhalees, 1 par voie orale), 14 de l’azithromycine, 10 une antibiotherapie inhalee, 10 des ATB IV. Le profil des patients selon le type de colonisation ne differe pas, hormis l’itraconazole prescrit chez 100 % des patients chroniques et intermittents (vs 35 % chez les colonises ponctuels). P ne semble pas influencer l’evolutivite : VEMS et BMI restent stables. Conclusion P semble peu pathogene, mais survient chez des patients avec une charge therapeutique elevee. La sensibilite diminuee de P a l’itraconazole peut evoquer une pression de selection par ce traitement.

The authors present the case of a 48-year-old man with diffuse pulmonary lymphangiomatosis. This rare lymphatic disorder is characterized by proliferation of anastomosing lymphatic vessels varying in size. Clinical presentation and imaging findings are highly suggestive. Bronchoscopic examination of this patient showed, for the first time to our knowledge, vesicles disseminated throughout the bronchial tree. Histopathological examinations are necessary to differentiate lymphangiomatosis from lymphangiectasis. The diagnosis can be made by transbronchial biopsy without performing open lung biopsy which was, until now, considered necessary for diagnosis.

But Evaluer l’impact du depistage neonatal de la mucoviscidose sur l’evolution nutritionnelle precoce. Methode Etude retrospective monocentrique realisee du 1/10/02 au 31/12/12 qui a evalue au moment du diagnostic et vers un an les parametres nutritionnels cliniques et biologiques. L’analyse a ete faite en 3 sous-groupes en fonction de la valeur de l’elastase fecale initiale : suffisant (SP), intermediaire et insuffisant pancreatique (IP), respectivement elastase > 200, 15–200 et t de Student non apparie pour les variables continues, exprimees en moyenne ± DS, et en un test de Chi 2 pour les variables categorielles, exprimees en pourcentage. Resultats Cinquante-deux enfants ont ete diagnostiques durant cette periode dont 43 par depistage neonatal (7 pathologies chirurgicales neonatales et 2 faux negatifs). Ils etaient repartis en 8 SP (17 %), 10 intermediaires (21 %) et 28 IP (59 %). Lors de l’annonce, a 37 ± 14 jours, le poids etait significativement plus bas dans le groupe IP (−1,13 ± 1,18 DS, p p Au niveau biologique, les valeurs au diagnostic de l’albumine et du zinc serique etaient plus basses dans le groupe IP. Au cours du suivi, a ete notee une augmentation des taux d’acide eicosapentaenoique et une diminution du taux d’acide docosahexaenoique quel que soit le statut pancreatique. Concernant les vitamines liposolubles, la principale difference concernait la vitamine E dont le taux etait significativement plus bas au diagnostic dans le groupe IP vs. SP, respectivement de 5,8 ± 4,2 vs. 11,1 ± 3,4 mg/L ( p = 0,01). Conclusion Le depistage neonatal de la mucoviscidose a permis une correction a un an des anomalies nutritionnelles presentes au moment du diagnostic. Toutefois, une attention particuliere doit etre portee aux nourrissons ayant une fonction pancreatique « intermediaire », dont le statut nutritionnel a un an etait moins bon.

Pseudomonas aeruginosa or Staphylococcus aureus toothbrushes contamination in cystic fibrosis (CF) patients is unknown. The objective of this pilot study was to determine prevalence of those germs on toothbrushes of CF and healthy children, and define if toothbrushes may be involved in pulmonary infection.

Pulmonary arteriovenous fistulas are abnormal vessels joining the right pulmonary artery to the pulmonary veins. They lead to an extracardiac right-to-left shunt with refractory hypoxemia. We report the case of a 7-year-old girl with a large pulmonary arteriovenous fistula discovered with refractory hypoxemia diagnosed during general anesthesia for adenoidectomy. Radio-opacity was observed on the upper lobe of the right lung. The diagnosis was made using thoracic angiotomography. The proximal arterial vessel arose from the lobar pulmonary artery. The fistula had developed in the entire right upper lobe and drained into two veins flowing into the right superior pulmonary artery. Given the marked hypoxemia, the potential risks of pulmonary hemorrhage and pulmonary infection, an occlusion of the fistula was indicated. After discussion between surgeons and interventional cardiologists, catheterization was indicated. The occlusion of the fistula was successful at the second attempt after release of a vascular plug in the main proximal vessel. This case illustrates the clinical circumstances of diagnosis of arteriovenous fistula, the diagnostic algorithm for refractory hypoxemia and the therapeutic options, with discussion of the benefits and drawbacks of a catheterization procedure.

Resume Une jeune fille presente un tableau de choc anaphylactique, apres etre entree dans une creperie, mais… avant toute consommation alimentaire ! Le bilan allergologique montre une sensibilisation aux acariens et au sarrasin. L’histoire clinique et le bilan allergologique font suspecter les allergenes de sarrasin aeroportes dans la genese de cette reaction anaphylactique. En immuno-empreinte, le serum de la patiente reagit contre une albumine 2S, allergene majeur du sarrasin, mais aussi contre une globuline 13S, dont le role dans la reaction par voie aeroportee reste a determiner. Le sarrasin est repandu en Asie dans l’alimentation et pour usages domestiques. En Occident, sa consommation revient a la mode. Notre cas clinique est un nouvel exemple de reaction severe a cet allergene, deja decrit comme responsable de reactions graves par voie inhalee comme par voie ingeree.

Objectives: Oxidative stress is associated with the condition of cystic fibrosis (CF), but no guidelines exist for its assessment or treatment. Our aim was to evaluate a test that measures the blood antioxidant capability in CF children. Methods: This antioxidant capability was assessed by the Kit Radicaux Libres (KRL) test in 44 CF children (24 boys). We recorded also anthropometric measures, pulmonary function, CF severity scores, and plasma nutritional and inflammatory parameters (proteins, vitamins, erythrocyte fatty acids, and micronutrients). We performed univariate and multivariate analyses with linear regression models. Results: The mean age at the first KRL assessment was 12.2 ± 3.8 years. Factors that correlated with decreased antioxidant capacity were mostly related to the severity of pulmonary disease [ forced expiratory volume at 1 second (FEV1), acute exacerbation, and congestion. In multivariate analysis, the correlation between Brasfield score and erythrocyte antioxidant potential remained significant (β = - 0.611, p < 0.001). Among nutritional factors, the ω6/ω3 ratio was significantly correlated to erythrocyte antioxidant potential (β = - 1.213, p = 0.01). Conclusion: The blood antioxidant capability, measured by the KRL test, appears to be an interesting biomarker to evaluate oxidative stress in CF. This study suggests that the ω6/ω3 ratio should be regarded as a nutritional marker in antioxidant management in CF children.

Background: Allergic bronchopulmonary aspergillosis (ABPA) is a common complication among patients with CF that produces significant respiratory morbidity. Standard therapy for ABPA includes systemic corticosteroids for a prolonged period of time with potentially severe side effects and use of antifungal agents. Omalizumab is a humanized monoclonal antibody directed against IgE that prevents its binding to receptors on effector cells. It has been shown to be effective in improving asthma control in patients with a strong allergic component. Recently, sporadic reports of omalizumab use against ABPA have been reported. Methods: We present our long term experience of omalizumab in three children with CF and ABPA who were steroid dependent. Results: Five (3.4%) out of 148 children and adolescents with CF followed in our Unit over a seven year period, developed ABPA (75% females, mean age at start of therapy 14.3 years). Omalizumab was given to 3 of them (2 males, mean age 17.1 years, mean FEV1 35%). All three were already experiencing significant side effects from chronic steroid therapy (invasive aspergillosis, diabetes mellitus, depression, relapse of ABPA and severe osteoporosis) and received regular antifugal therapy. Omalizumab treatment was given for a period of 12−18 months. Oral steroids were gradually stopped. Omalizumab resulted in significant improvement of respiratory symptoms and lung function (mean FEV1 52%, p: 0.01). Conclusion: According to our results in a small number of patients, omalizumab has a significant potential as a strategy to control ABPA in steroid dependent patients. However, more studies are needed to confirm these findings.

Introduction: Physical activity is beneficial for cystic fibrosis (CF) patients. This induces to stimulate their habitual physical activity (HAP) from childhood. Thus, for HAP evaluation, we need methods that are simple, reliable, objective and well accepted by the patients. Accelerometry is one of the possibilities for evaluating HAP. We studied its feasibility, acceptability and correlation with other HAP evaluation methods in a population of paediatric CF patients. Methods: 34 CF patients, aged between 9 and 18 years, were proposed to wear accelerometers. The reasons for not wearing the accelerometer were evaluated. A correlation was measured between the data produced by accelerometry, the answers to HAES and Veerschuur questionnaires, and evaluations by medical staff. Results: – 14 children out of 34 (41%) did not wear the accelerometer, 5 of them simply refused it; 6 children (17%) have worn the accelerometer less than 5 days. Finally, 14 recordings were interpretable, i.e. 41% of the 34 children to whom the measurement was proposed. – The answers to the questionnaires appeared not to be correlated to the accelerometry data. But there was a good correlation of 62.1% (p = 0.017) between the data obtained by accelerometry and the medical evaluation. Conclusion: The feasibility of the HAP evaluation by accelerometry in a pediatric CF population is only around 40%. Therefore it cannot be used in every-day clinical practice. However, in clinical trials, the objective evaluation by accelerometry should be preferred because of the discrepancies observed with answers to standardized questionnaires on physical activity.

Introduction : Chez les patients cirrhotiques, la concentration alveolaire de monoxyde d’azote (CA, NO) est augmentee mais pas le debit bronchique de NO (J’maxbr,NO), Le monoxyde d’azote (NO) pourrait etre un facteur clef dans l’hemodynamique pulmonaire et les anomalies des echanges gazeux de ces patients. Methodes : Les objectifs de notre travail ont ete, dans une population de patients cirrhotiques, (1) de mesurer la production de NO par le compartiment alveolaire pulmonaire (V’A,NO) et (2) de comparer V’A,NO avec l’hemodynamique pulmonaire et la difference alveolo-arterielle en oxygene (PA-a,O2). Les concentrations de NO exhale ont ete mesurees par la methode des debits multiples afin de calculer CA,NO. La mesure du transfert pulmonaire du monoxyde d’azote (TL,NO) a ete mesuree par une technique « en apnee », et V’A,NO a ete calculee comme le produit de CA,NO par TL,NO Un catheterisme droit a ete pratique dans un sous-groupe de patients. Resultats : Le NO exhale et le TL,NO ont ete mesures chez 26 sujets normaux et chez 40 patients cirrhotiques, dont 9 porteurs d’un syndrome hepato-pulmonaire. On retrouvait une augmentation de V’A,NO chez les patients compares au groupe controle (408 ± 160 vs 238 ± 126 nLmin-1 respectivement, p

Introduction : Une diminution du transfert pulmonaire du monoxyde de carbone (TL,CO) et une augmentation de la difference alveolo-arterielle en oxygene (PA–a,O 2 ) sont deux anomalies frequentes chez les patients atteints de cirrhose severe. L’equation de Roughton et Forster permet de calculer la capacite de diffusion membranaire du CO (Dm,CO) et le volume sanguin capillaire pulmonaire (Vcap) a partir de la mesure simultanee de TL,CO et du transfert pulmonaire du monoxyde d’azote (TL,NO). L’objectif de ce travail est de determiner quelle composante de TL,CO (Vcap et/ou Dm,CO) est associee a la baisse de TL,CO et a l’augmentation de (PA–a,O 2 ). Methodes : La mesure simultanee de TL,CO et TL,NO a ete realisee par une technique « en apnee » chez une serie de 49 patients cirrhotiques en attente de transplantation hepatique. La PA-a,O 2 a ete calculee a partir de la mesure des gaz du sang arteriels preleves en air ambiant. Les correlations entre PA–a,O 2 d’une part, et TL,CO, Vcap, Dm,CO et le rapport TL,NO/TL,CO d’autre part ont ete recherchees. Resultats : 25 patients presentaient une hypoxemie (PA-a,O 2 > 15mmHg), et 11 patients presentaient un syndrome hepato-pulmonaire (SHP). Les patients ayant un SHP avaient un TL,CO et un Vcap (exprimes en pourcentage de leur valeur theorique) plus bas (63 ± 18 % vs 89 ± 15 %, p vs 90 ± 16 %, p vs 4,3 ± 0,4, p = 0,005) que les patients n’ayant pas de SHP. En revanche, le Dm,CO des patients ayant un SHP n’etait pas significativement different du Dm,CO des patients sans SHP (76 ± 27 % vs 87 ± 25, p = 0,22). Pour l’ensemble des patients, on retrouvait une correlation negative entre TL,CO et PA–a,O 2 (r2 = 0,48, p 2 (r2 = 0,25, p 2 (r2 = 0,57, p Conclusion : Les anomalies d’oxygenation du sang arteriel rencontrees chez les patients cirrhotiques sont associees a une diminution de la perfusion d’alveoles ventiles. Ce resultat est compatible (1) avec l’existence d’une obstruction arteriolaire touchant certains territoires pulmonaires et (2) des territoires pulmonaires a bas rapport ventilation/perfusion realisant un effet shunt voire, dans le cas du SHP, un shunt anatomique.