Energy balance in cystic fibrosis when stable and during a respiratory exacerbation

Abstract

There was no difference in TEE and energy intake when patients were unwell in hospital compared to when they were stable at home despite an increase in REE.

Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2

“…Among the five genes, MC3R has been implicated in weight maintenance and regulation of energy balance in animals and humans 34-36 . Variation in resting energy expenditure has been correlated with lung function measurements, lung tissue damage and lung disease exacerbation in CF patients 37,38 . MC3R has also been implicated as a modulator of neutrophil accumulation in a murine model of lung inflammation 39 , a key feature of CF lung disease, as noted above.…”

Effect of exocrine pancreatic function on resting energy expenditure in cystic fibrosis

“…It is known that energy imbalance in CF results from increased total energy expenditure (TEE) and decreased energy intake (1). It has been reported that increased REE in CF may account for 70% of TEE (2).…”

“…It has been reported that increased REE in CF may account for 70% of TEE (2). However, high REE is not always associated with increased TEE (1,2). The cause of increased REE in CF has not been clarified (3,4).…”

Characteristics of Resting Metabolic Rate in Critically Ill, Mechanically Ventilated Adults With Cystic Fibrosis

“…These factors might be expected to have opposing effects on resting metabolic rate, with illness hypermetabolism raising the rate and with sedation and decreased work of breathing due to mechanical assistance lowering it. In fact, our critically ill, mechanically ventilated and sedated patients with cystic fibrosis had resting metabolic rates that were similar to spontaneously breathing patients with cystic fibrosis in acute exacerbation (body weight, 37 vs 40 kcal/kg), 19 even though it can be presumed that our subjects were more severely ill at the time of measurement. On a kcal/kg basis, our patients with cystic fibrosis appeared to have a higher resting metabolic rate than that of their cohort without cystic fibrosis (38 vs 31 kcal/kg), even controlling for age (37 vs 32 kcal/kg).…”

“…McCloskey et al 19 measured the resting metabolic rate of patients with cystic fibrosis when stable in clinic and during exacerbation when hospitalized. The resting metabolic rate during exacerbation was elevated versus baseline (1984 vs 1745 kcal/d).…”