Using chest computed tomography and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis.

Objectives: Lumacaftor-ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator known to improve clinical status in people with cystic fibrosis (CF). The aim of this study was to assess lung structural changes after 1 year of lumacaftor-ivacaftor treatment and to use unsupervised machine learning to identify morphological phenotypes of lung disease that are associated with response to lumacaftor-ivacaftor.

Methods: Adolescents and adults with CF from a French multicentre real-world prospective observational study evaluating the first year of treatment with lumacaftor-ivacaftor were included if they had pre-therapeutic and follow-up chest computed tomography (CT) scans available.

Eosinophilic granulomatosis with polyangiitis in children: Data from the French RespiRare® cohort.

Objectives: To describe the characteristics of pediatric cases of eosinophilic granulomatosis with polyangiitis (EGPA), a systemic necrotizing vasculitis rarely diagnosed in children, retrieved from the French Reference Center for rare pediatric lung diseases and compared with adult cases included in the French Vasculitis Study Group cohort.

Methods: We collected information on pediatric EGPA disease presentation, management, and outcome. Cases met the Lanham criteria and/or American College of Rheumatology classification criteria.

Pulmonary hemosiderosis in children with Down syndrome: a national experience.

Background: Pulmonary hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary hemosiderosis and DS.

Nutritional Status in the First 2 Years of Life in Cystic Fibrosis Diagnosed by Newborn Screening.

Objective: To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24.

Methods: A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome, and biological nutritional parameters.

Results: One hundred and five infants were recruited and 99 completed the study.

Omalizumab treatment for allergic bronchopulmonary aspergillosis in young patients with cystic fibrosis.

Background: Allergic bronchopulmonary aspergillosis (ABPA) is a severe lung disease complication caused by an Aspergillus fumigatus-induced hypersensitivity that affects 2-15% of patients with cystic fibrosis (CF). The mainstay treatment consists of a combination of corticosteroids and antifungals. However, repeated or long-term corticosteroid therapies can lead to serious side effects.

More Relaxation by Deep Breath on Methacholine- Than on Exercise-Induced bronchoconstriction during the Routine Testing of Asthmatic Children.

Deep inspiration (DI) dilates normal airway precontracted with methacholine. The fact that this effect is diminished or absent in asthma could be explained by the presence of bronchial inflammation. The hypothesis was tested that DI induces more relaxation in methacholine induced bronchoconstriction-solely determined by the smooth muscle contraction-than in exercise induced bronchoconstriction, which is contributed to by both smooth muscle contraction and airway wall inflammation.

What is chronic cough in children?

The cough reflex is modulated throughout growth and development. Cough-but not expiration reflex-appears to be absent at birth, but increases with maturation. Thus, acute cough is the most frequent respiratory symptom during the first few years of life.

[Recommendations for the use of diagnostic testing in low respiratory infections in children older than three months].

Recommendations for the use of diagnostic testing in low respiratory infection in children older than 3 months were produced by the Groupe de Recherche sur les Avancées en Pneumo-Pédiatrie (GRAPP) under the auspices of the French Paediatric Pulmonology and Allergology Society (SP(2)A). The Haute Autorité de santé (HAS) methodology, based on formalized consensus, was used. A first panel of experts analyzed the English and French literature to provide a second panel of experts with recommendations to validate.

Add-on omalizumab in children with severe allergic asthma: a 1-year real life survey.

Omalizumab has been shown to reduce exacerbation rates in moderate to severe allergic asthma. Our aim was to evaluate omalizumab efficacy and safety in a real-life setting in severe asthmatic children. 104 children (aged 6-18 years), followed up in paediatric pulmonary tertiary care centres, were included at the beginning of omalizumab treatment.

Psychological effects of false-positive results in cystic fibrosis newborn screening: a two-year follow-up.

Objective: To evaluate parental stress after a false-positive result at the time of the cystic fibrosis (CF) newborn screening (NBS), attributable to heterozygotism or persistent hypertrypsinemia.

Study Design: A prospective study was conducted in 86 French families at 3, 12, and 24 months after NBS. A psychologist conducted interviews with a questionnaire, the Perceived Stress Scale, and the Vulnerable Child Scale.

QuantiFERON-TB Gold In-Tube as help for the diagnosis of tuberculosis in a French pediatric hospital.

The diagnosis of tuberculosis (TB) is difficult in children. The pediatricians are waiting for new and rapid tests that are easy to realize and that are performed better than tuberculin skin test (TST). We presented here the evaluation of QuantiFERON-TB Gold In-Tube (QFT-G IT) in the Children Hospital of Nancy.

[Difficulties of TB diagnosis in children: QuantiFERON TB Gold In-Tube as useful tool].

Diagnosis of childhood tuberculosis (TB), active TB or latent tuberculosis infection (LTBI), is complicated by uncommon clinical, radiological and bacteriological features. The tuberculin skin test (TST) is imperfect: difficulty of the intradermal injection for the child, lack of sensibility and specificity. The stop of the systematic inoculation by the BCG since July 2007, in France, could lead to an increase of the incidence of the childhood TB.

A new large CFTR rearrangement illustrates the importance of searching for complex alleles.

The p.Val754Met variant, described in 1996 in a CF patient, has been considered a CF mutation. However, biochemical aspects, results of functional studies and, finally, the identification of a complex deletion removing exons 3 to 10 and 14b to 16 in cis of p.

[Flexible bronchoscopy in children. Experience at French centers of pediatric pneumology].

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.

[Chronic obstructive pulmonary diseases and asthma in children: diagnostic and therapeutic strategies].

Asthma and chronic obstructive pulmonary disease are two distinct affections but two chronic inflammatory disorders. Respiratory symptoms are non-specific. There is a possible relationship between airways hyperreactivity and chronic obstructive pulmonary disease.

Follow-up of 452 totally implantable vascular devices in cystic fibrosis patients.

The use and complications of totally implantable vascular access devices (TIVADs) were examined during multiple courses of antibiotics in cystic fibrosis (CF) patients. This retrospective study involved 36 CF centres. Risk factors for removal and septicaemia were sought by survival analysis of censored data.

[Anti-inflammatory therapy in cystic fibrosis].

Cystic fibrosis lung inflammation is early, sustained and severe and would justify an anti-inflammatory treatment. At present, the inhaled corticosteroid treatment did not give evidence of efficacy, contrary to the oral presentation, but at the cost of side effects. Azithromycin gives more encouraging results with a good tolerance.

[Airway inflammation in cystic fibrosis].

Airway inflammation is now recognized as a major factor in the pathogenesis of lung disease in cystic fibrosis. Inflammation is early, sustained and severe. Several anti-inflammatory therapeutic approaches have been developed, but are controversial.

Long-term outcome of idiopathic pulmonary hemosiderosis in children.

We retrospectively analyzed the long-term outcome of idiopathic pulmonary hemosiderosis (IPH) in 15 children. IPH started at a mean age of 5 years, and the mean duration of follow-up was 17.2 years (range, 10-36 yr).

Microbiologic contamination study of nebulizers after aerosol therapy in patients with cystic fibrosis.

Background: To evaluate the contamination of delivery systems after an aerosol therapy session in patients with cystic fibrosis who have chronic Pseudomonas aeruginosa infection.

Methods: Fifty-three patients with cystic fibrosis were enrolled in the study from March 1996 to June 1997. All patients were age 7 years or older and had P aeruginosa infection.