195 Pulmonary function in cystic fibrosis children younger than 3 years old

Abstract

Studies suggest that the decline in pulmonary function begins early in CF infants even in those patients without respiratory symptoms. Objective: to describe pulmonary function in CF children younger than 3 years old and to determine if there is any association between pulmonary function and clinical features. Methods: observational and retrospective, study. We collected data from CF patients who underwent an Infant Pulmonary Function Test (IPFT) before the age of three years. IPFTs were assessed by partial flow/volume curves with the rapid thoracicabdominal compression technique to obtain the maximal flow at the functional residual capacity (V′maxFRC). Clinical issues recorded were: age, gender, genetics, pancreatic sufficiency, nutritional status and microbiological findings. Results: 46 patients (31 males) with a median (interquartile range) age of 8 months (5 to 15) were included. Overall V′maxFRC Z score was −0.8 (−1.4 to 0.3), −0.1 (−1.1 to 0.7) in males and −0.9 (−1.8 to 0.3) in females (p =NS). V′maxFRC Z score was −0.3 (−1.1 to 0.2) in children younger than 6 months and −0.1 (−1.3 to 1) in older ones (p =NS). In patients homozygous p.508del V′maxFRC Z score was −0.5 (−1.2 to 0.8) while in patients with other mutations it was −0.2 (−1.2 to 0.6) (p =NS). There was also no statistical difference in V′maxFRC between well-nourished and bad-nourished patients, and between patients with and without Pseudomonas aeruginosa (PA) in sputum. Conclusion: IPFT assessed by partial flow/volume curves was normal in our CF population less than 3 years old. We have not found any difference in pulmonary function comparing CF children with different clinical features.