R. Gauthier

Mucoviscidose : du bon usage des explorations fonctionnelles respiratoires

Resume Introduction Le depistage neonatal de la mucoviscidose permet de proposer aux patients atteints un suivi multidisciplinaire dedie et precoce. Etat des connaissances L’exploration fonctionnelle respiratoire est un outil obligatoire de cette surveillance clinique. Le but de cet article est de decrire les anomalies les plus frequemment observees et de faire le point des recommandations en fonction du type d’examen, de l’âge du patient et des principales situations cliniques. Perspectives Il apparait que seule la frequence d’utilisation de la spirometrie, avec ses limites d’interpretation, fait l’objet d’un large consensus. Conclusion L’effort d’evaluation de la riche panoplie des tests fonctionnels respiratoires doit donc se poursuivre dans le cadre de la mucoviscidose, en particulier chez le petit enfant.

A simplified, semi-quantitative structural lung disease computed tomography outcome during quiet breathing in infants with cystic fibrosis

Chest tomography (CT) using the controlled ventilation technique (CTCV) is a sensitive method to detect features of lung cystic fibrosis (CF) disease in infants with CF. However, this technique needs sedation and is not easily applied for the clinician who may need, in the follow-up, to evaluate more precisely lung disease in infants with CF. Thus, our study aims to evaluate if CT assessment of lung disease, without the need of sedation, during quiet breathing, using a semi-quantitative scoring system, is reproducible and may discriminate infants with CF from control infants at an early stage of the lung disease. 39 infants with CF underwent a first CT at 10.3 [9.4, 11.4] weeks of age. Among them, 33 underwent a second CT at 56.1 [53.1, 59.6] weeks of age. CF scoring images of the different scanner variables, i.e. bronchial wall thickening, bronchiectasis, mucus plugging and air trapping were compared to CT scoring obtained in 2 different groups of control infants of similar age without lung disease. Among all the constituents of the scoring, air trapping is the only parameter discriminating infants with CF from control infants at both ages in our study (p≤0.01). Moreover, air trapping explains 90% of the total score variability with r2=0.89 with a good concordance after re-scoring in blind, 6months apart, by the same operator for both infant populations: ICC=0.98 [0.97, 0.99]. In this study, we propose that CT during quiet breathing could be a useful clinical tool to evaluate the early presence of gas trapping in infants with CF.