title: [Early lung disease in infants with cystic fibrosis. Diagnostic tools and possible therapeutic pathways].
abstract: The lungs of infants with cystic fibrosis (CF) have been considered to be normal at birth. However, recent data indicates that this is unlikely to be true in most cases. Animal CF-models developed in the early 2000s have shown that constitutional airway narrowing may be present at birth, and is associated with both functional and structural abnormalities. Longitudinal birth cohort studies have shown that 25 % of CF infants followed in specialized centers, while being asymptomatic, showed decreased lung function at 3months of age. Air trapping was present in 68 % and bronchiectasis in 28 % of patients at the same age. The presence of neutrophil elastase in the bronchoalveolar lavage at 3months of age tripled the risk of bronchiectasis at the age of 3years. Currently available tools such as infant pulmonary function tests (both the jacket and multiple breath washout) as well as high-resolution volume controlled chest-computed tomography or functional magnetic resonance imaging will facilitate early intervention trials in the very near future. The role of such tools for the routine follow-up of patients, and the ability of early therapeutic interventions to alter the natural history of CF-lung disease should soon be established.
authors: UMR5558, centre de référence de la mucoviscidose, hospices civils de Lyon, 59, boulevard Pinel, 69677 Bron, France. Electronic address: philippe.reix@chu-lyon.fr.
authors: P
authors: P
authors: Reix
authors: Unité d'exploration fonctionnelle pédiatrique, Inserm 1046, université de Montpellier I, CHU de Montpellier, 371, avenue du Doyen-Gaston-Giraud, 34295 Montpellier cedex 5, France.
authors: S
authors: S
authors: Matecki
authors: Centre de ressources de compétences pédiatriques de la mucoviscidose, centre d'investigation clinique (CIC 0005), hôpital Pellegrin-Enfants, CHU de Bordeaux, place Amélie-Raba-Léon, 33076 Bordeaux, France.
authors: M
authors: M
authors: Fayon
mesh_terms: D000818:Animals; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis; D004195:Disease Models, Animal; D042241:Early Diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis* / Q000628:therapy*; D008171:Lung Diseases / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy*; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests
keywords: Computed tomography; Cystic fibrosis; Fonction respiratoire; Infant; Lung function; Mucoviscidose; Nourrisson; Scanner