Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: Analysis of the Cystic Fibrosis Foundation National CF Patient Registry

Objective: To determine prospectively the relationship among growth, nutritional status, and pulmonary function over a 4-year period in a large cohort of children with cystic fibrosis (CF). Study design: CF Foundation National CF Patient Registry data collected from 1991 to 1995 for 968 children (507 male) aged 5 to 8 years with pancreatic insufficiency and forced expiratory volume in 1 second within 60% to 140% of predicted values (FEV1%) were analyzed longitudinally. Variables hypothesized to affect FEV1% included age, sex, z scores for height, weight, percent of height-appropriate body weight, and annual number of days hospitalized. Results: The significant decline in FEV1% was curvilinear and dependent on baseline FEV1%; children with initial FEV1% ≥90 declined 2.6 U/y more than those with initial FEV1%