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Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?
Author(s) -
Johnson JoAnne,
Bush Andrew,
Buchdahl Roger
Publication year - 2010
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.21271
Subject(s) - medicine , meconium ileus , cystic fibrosis , spirometry , vital capacity , pulmonary function testing , anthropometry , pediatrics , confounding , sweat test , meconium , lung , lung function , pregnancy , asthma , fetus , genetics , diffusing capacity , biology
Abstract It is a matter of debate as to what extent the long‐term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long‐term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non‐MI) in an era of non new‐born‐screening (NBS). We collected annual lung function data between the ages of 8–15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2–15 years annual height and weight Z‐scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period. Thirty‐eight MI and 76 non‐MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non‐MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non‐MI groups apart from WtZ score in the non‐MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non‐significant trend for subjects presenting with MI to do better in all four parameters. We conclude that babies presenting with MI have no worse long‐term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF. Pediatr Pulmonol. 2010; 45:951–958. © 2010 Wiley‐Liss, Inc.