Original ArticleBetter Nutritional Status in Early Childhood Is Associated with Improved Clinical Outcomes and Survival in Patients with Cystic Fibrosis
Section snippets
Methods
This is a prospective, observational study of patients with CF in the US. Data were extracted from the CFF Patient Registry (“registry”) for patients born between 1989 and 1992. This range was chosen to allow for maximum follow-up of patients born in the era of a modern approach to nutrition management. The registry contains information on over 25 500 patients who receive their care in a CFF-accredited care center; we analyzed data from 3142 patients born during the time period of interest.
Results
The cohort consists of 3142 patients, mostly Caucasian, with a slight male predominance (Table I; available at www.jpeds.com). Most patients were diagnosed by 5 months of age, primarily with gastrointestinal manifestations of disease. The frequency of the F508del allele in the cohort was 72% among those who had a genotype entered in the registry.
Discussion
Several short-term studies have shown associations between various measures of nutritional status in young children and lung function or survival.8, 9 Longer-term studies have demonstrated that either improvement or worsening of nutritional status correlated with either better or worse lung function,10 that short stature was negatively correlated with survival,11 and that children who recovered their birth weight z-scores by age 2 years had better lung function at age 6 years.12 Our lung
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Funded by Cystic Fibrosis Foundation (BOROWI03CS0 to D.B. and OCONNO01E0 to H.Q.). The authors declare no conflicts of interest.