THE CYSTIC-FIBROSIS GENE AND RESTING ENERGY-EXPENDITURE
THE CYSTIC-FIBROSIS GENE AND RESTING ENERGY-EXPENDITURE
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DOI：
10.1016/s0022-3476(05)83042-2
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发表时间：
1991-12-01
影响因子：
5.1
通讯作者：
PENCHARZ, PB
中科院分区：
文献类型：
--
作者：
FRIED, MD;DURIE, PR;PENCHARZ, PB
为了确定囊性纤维化患者静息能量消耗的增加是否与原发性遗传缺陷（DELTA-F508）或肺功能下降相关，或两者兼而有之，我们对32名男性受试者（年龄7至39岁）进行了前瞻性静息能量消耗测试，这些受试者营养正常，肺功能良好。根据是否存在DELTA-F508和胰腺功能，将其分为三个基因型组。平均静息能量消耗为预测值的104%，与基因型无关。当29名营养状况正常但肺功能可变的受试者加入该组时，肺功能下降与静息能量消耗增加之间存在很强的相关性。我们的结论是，在营养正常的男孩和男性囊性纤维化患者中，静息能量消耗增加似乎与肺功能下降的关系比与基因型的关系更密切。
To determine whether the increase in resting energy expenditure in cystic fibrosis is associated with the primary genetic defect (DELTA-F508) or with declining pulmonary function, or both, we tested resting every energy expenditure prospectively in 32 male subjects (aged 7 to 39 years) who were normally nourished and had good pulmonary function. They were categorized into three genotype groups on the basis of the presence or absence of DELTA-F508 and pancreatic function. Mean resting energy expenditure was 104% of the predicted value and was not associated with genotype. When 29 subjects with normal nutritional status but variable lung function were added to the group, there was a strong correlation between declining pulmonary function and increased resting energy expenditure. We conclude that increased resting energy expenditure in normally nourished boys and men with cystic fibrosis appears to be more closely associated with declining pulmonary function than with genotype.