Frances Payne Bolton School of Nursing, Case Western Reserve University, USA.
J Cyst Fibros. 2013 Jul;12(4):345-51. doi: 10.1016/j.jcf.2012.11.008. Epub 2013 Jan 3.
Altered ventilatory pattern and increased energy expenditure are facets of the complex cystic fibrosis (CF) phenotype. It is not known whether these are inherent attributes of CF, secondary consequences of lung infection or other disease complications.
Studies were performed in congenic C57BL/6J, F508del (Cftr((tm1kth))) and CF gut-corrected (F508del) mice. Ventilatory patterns were measured using whole-body plethysmography. Indirect calorimetry was used to determine oxygen consumption, carbon dioxide production and resting energy expenditure.
CF mice (F508del and F508del gut-corrected) have a significantly faster respiratory rate and increased ventilatory pattern variability as compared to non-CF mice. F508del but not CF gut-corrected mice had significantly increased energy expenditure per gram body weight.
CF mice exhibit a faster, more variable ventilatory pattern. These changes were present in the absence of detectable infection or illness due to gastrointestinal obstruction. Increased resting energy expenditure does not completely account for these differences.
呼吸模式的改变和能量消耗的增加是复杂囊性纤维化（CF）表型的特征。目前尚不清楚这些是 CF 的固有属性，还是肺部感染或其他疾病并发症的继发后果。
在同源 C57BL/6J、F508del（Cftr（tm1kth））和 CF 肠道校正（F508del）小鼠中进行了研究。使用全身 plethysmography 测量呼吸模式。通过间接量热法测定耗氧量、二氧化碳产生量和静息能量消耗。
CF 小鼠（F508del 和 F508del 肠道校正）的呼吸频率明显快于非 CF 小鼠，呼吸模式变异性增加。F508del 但不是 CF 肠道校正的小鼠每克体重的能量消耗显著增加。
CF 小鼠表现出更快、更可变的呼吸模式。这些变化在没有检测到由于胃肠道梗阻引起的感染或疾病的情况下存在。静息能量消耗的增加并不能完全解释这些差异。
