RESUMEN
OBJECTIVE: To assess whether elevated resting energy expenditure (REE) in female patients with cystic fibrosis (CF) persists longitudinally during late childhood and puberty. STUDY DESIGN: REE and body composition were measured 3 times in 2 years in 86 children with CF. Pubertal status, bacterial colonization, liver disease, pancreatic and pulmonary function, and genotype were determined, and linear mixed model analyses were used to determine predictors and changes in REE longitudinally. RESULTS: REE did not change with time allowing for fat free mass, pancreatic insufficiency (PI), or severe mutations. Pulmonary function and liver disease were not significant predictors of REE. Percentage predicted REE compared with control data was higher (P = .002) in female patients with CF (109.5%) and lower in male patients with CF (104%) and persisted with time. In post-menarchal female patients with CF, REE adjusted for fat free mass was 366 kJ/d lower than in pre-menarchal female patients, but still 112% predicted. CONCLUSIONS: This longitudinal study demonstrates that REE is elevated in patients with CF with PI and severe mutations. The elevation of percentage predicted REE was greater in female patients than male patients and persisted for 2 years, and during pubertal maturation, independent of pulmonary and liver disease. These results highlight the need for a high-energy diet throughout childhood and adolescence, particularly in female patients with PI.
Asunto(s)
Fibrosis Quística/metabolismo , Metabolismo Energético/fisiología , Descanso/fisiología , Adolescente , Composición Corporal , Estudios de Casos y Controles , Niño , Preescolar , Fibrosis Quística/complicaciones , Fibrosis Quística/fisiopatología , Femenino , Humanos , Estudios Longitudinales , Masculino , Factores Sexuales , Factores de TiempoRESUMEN
OBJECTIVES: To evaluate which factors might contribute to raised resting energy expenditure (REE) in patients with cystic fibrosis (CF). STUDY DESIGN: REE and anthropometry were measured in 134 (males = 68) children with CF and 100 (males = 51) controls (range, 3-18.7 years) in an outpatient setting. Bacterial colonization, liver disease, inhaled steroid use, pancreatic and pulmonary function, sex, and genotype were determined and regression analysis was used to determine the predictors of REE in the group with CF. RESULTS: REE for children with CF was increased on average by 7.2% compared with controls. This increase was greater for females than for males. REE in males was positively associated with fat-free mass (FFM), pancreatic insufficiency (PI), and liver disease, and negatively associated with pulmonary function, whereas in females, REE was positively associated with FFM and PI. REE (adjusted for FFM) was higher in children with a severe mutation (5495 +/- 47 kJ) compared with a mild mutation (5,176 +/- 124 kJ, P <.02). CONCLUSIONS: PI, severe mutations, and female sex are the main contributing factors to elevated REE in patients with CF with near normal pulmonary function.