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Spirometry in Early Childhood in Cystic Fibrosis Patients^*

^* From The Edmond and Lili Safra Children’s Hospital (Drs. Vilozni, Efrati, Minuskin, Barak, Szeinberg, Yahav, and Augarten), Sheba Medical Center, Ramat-Gan; Meyer Children’s Hospital (Dr. Bentur), Rambam Medical Center, Haifa; Schneider Children’s Medical Center (Dr. Blau), Petach-Tikva; Shaare Zedek Medical Center (Dr. Picard), Jerusalem; and Hadassah Medical Center (Dr. Kerem), Mount Scopus, Jerusalem, Israel.

Correspondence to: Daphna Vilozni, PhD, Pediatric Pulmonary Unit, The Edmond and Lily Safra Children’s Hospital, Chaim Sheba Medical Center, Tel HaShomer, Ramat-Gan, Israel, 52621; e-mail: avi_vil{at}bezeqint.net

Abstract

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 (FEV_0.5), 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 FEV₁ and FVC were similar over the age ranges studied. However, z scores for FEV_0.5 and 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 FEF₅₀ than 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. FEV_0.5 and flow-related volumes might be more sensitive than the traditional FEV₁ in detecting and portraying changes in lung function during early childhood.

Key Words: airflow obstruction • cystic fibrosis • early childhood • prognosis • pulmonary infection • spirometry