228 Pulmonary and nutritional outcomes in children with cystic fibrosis diagnosed by meconium ileus in Argentina

Abstract

Introduction: Early diagnosis in Cystic Fibrosis (CF) still remains a challenge, because it allows the implementation of appropriate therapeutic strategies. It can reach through neonatal screening (NS) an early detection or by meconium ileus a premature clinical manifestation associated with poorer growth. Objective: To compare clinical status and lung function in children with CF diagnosed with meconium ileus and those detected by NS. Methods: We compare two groups: one detected by NS and one diagnosed by meconium ileus (MI) since 1995 (beginning of the program of NS in the state of Buenos Aires). We assessed: Z score Weight/age, Z score Height/age from 6 months up to 6 years of age, Forced Expiratory Volume in 1st (FEV1). We also registered first isolation of Pseudomonas aeruginosa (Pa). Student’s t-test was performed to compare the means of the indicators. Results: We included 26 patients in NS group and 31 in MI, all with pancreatic insufficiency. p.PHE508del/p.PHE508del was present in 52% of NS and 41.4% in MI. In MI group were four deaths and one in NS. The NS group showed better nutritional parameters than MI, as seen in the table. We found no significant differences in first isolation of Pa (p = 0.55) and FEV1 (p = 0.11) between the two groups.