Johnson Mark R, Ferkol Thomas W, Shepherd Ross W
Department of Pediatrics, Washington University School of Medicine. St. Louis, MO 63011, USA.
J Cyst Fibros. 2006 Jan;5(1):53-8. doi: 10.1016/j.jcf.2005.10.001. Epub 2005 Dec 15.
In cystic fibrosis (CF), perturbations of total daily energy expenditure (TDEE) may be a major determinant of altered nutrition and growth. Measurement of TDEE is problematic, though the flex-heart rate method (FHRM) provides a close estimation of TDEE, as compared to the cost-prohibitive, gold standard, the double-labeled water method, and permits estimates of the energy cost of daily activities (ECA) above resting energy expenditure (REE). We hypothesize that alterations in ECA affects TDEE in CF.
To measure components of TDEE in adolescents with CF and normal lung function compared with controls, and to determine whether ECA can be improved by diet and exercise.
Clinically stable CF subjects (aged 9-13, n=12) and age- and gender-matched controls (n=13) had repeated measurements of TDEE by FHRM, REE, and maximal cardiopulmonary exercise testing (CPET) during a 6-week exercise and diet program.
While the mean REE was similar in both groups, ECA was significantly lower in CF adolescents as compared to controls (p=0.02). During CPET, maximal exercise in CF was characterized by hyperventilation, which was unrelated to ventilation-perfusion mismatching. There were no changes in REE after dietary intervention.
ECA in CF adolescents with normal lung function is lower when compared to healthy controls. These findings support the hypothesis that clinically stable patients with CF have inefficient energy metabolism or alternatively conserve energy during activities of daily living.
在囊性纤维化(CF)中,每日总能量消耗(TDEE)的紊乱可能是营养和生长改变的主要决定因素。TDEE的测量存在问题,尽管与成本高昂的金标准双标记水法相比,心率变异性方法(FHRM)能提供对TDEE的近似估计,并且可以估计静息能量消耗(REE)之上的日常活动能量消耗(ECA)。我们假设ECA的改变会影响CF患者的TDEE。
测量肺功能正常的CF青少年与对照组相比的TDEE组成部分,并确定饮食和运动是否能改善ECA。
临床稳定的CF受试者(9 - 13岁,n = 12)和年龄及性别匹配的对照组(n = 13)在为期6周的运动和饮食计划期间,通过FHRM、REE和最大心肺运动测试(CPET)对TDEE进行重复测量。
虽然两组的平均REE相似,但与对照组相比,CF青少年的ECA显著更低(p = 0.02)。在CPET期间,CF患者的最大运动以过度通气为特征,这与通气 - 灌注不匹配无关。饮食干预后REE没有变化。
肺功能正常的CF青少年的ECA低于健康对照组。这些发现支持了这样的假设,即临床稳定的CF患者能量代谢效率低下,或者在日常生活活动中节约能量。