Spirometry in Early Childhood in Cystic Fibrosis Patients

Background:Spirometry data in cystic fibrosis (CF) patients in early childhood is scarce, and the ability of spirometry to detect airways obstruction is debatable.Objective:To evaluate the ability of spirometry to detect airflow obstruction in CF patients in early childhood.Methods:CF children (age range, 2.5 to 6.9 years) in stable clinical condition were recruited from five CF centers. The children performed guided spirometry (SpiroGame; patented by Dr. Vilzone, 2003). Spirometry indices were compared to values of a healthy early childhood population, and were analyzed with relation to age, gender, and clinical parameters (genotype, pancreatic status, and presence of Pseudomonas in sputum or oropharyngeal cultures).Results:Seventy-six of 93 children tested performed acceptable spirometry. FVC, FEV1, forced expiratory flow in 0.5 s (FEV0.5), and forced expiratory flow at 50% of vital capacity (FEF50) were significantly lower than healthy (z scores, mean ± SD: − 0.36 ± 0.58, − 0.36 ± 0.72, − 1.20 ± 0.87; and − 1.80 ± 1.47, respectively; p < 0.01); z scores for FEV1and FVC were similar over the age ranges studied. However, z scores for FEV0.5and forced expiratory flow at 25 to 75% of vital capacity were significantly lower in older children compared to younger children (p < 0.001), and a higher proportion of 6-year-old than 3-year-old children had z scores that were > 2 SDs below the mean (65% vs 5%, p < 0.03). Girls demonstrated lower FEF50than boys (z scores: − 2.42 ± 1.91 vs − 1.56 ± 1.23; p < 0.001). Clinical parameters evaluated were not found to influence spirometric indices.Conclusions:Spirometry elicited by CF patients in early childhood can serve as an important noninvasive tool for monitoring pulmonary status. FEV0.5and flow-related volumes might be more sensitive than the traditional FEV1in detecting and portraying changes in lung function during early childhood.