Energy balance and obesity in individuals with cystic fibrosis

Abstract

This article reviews the significance of nutritional status in patients with cystic fibrosis (CF), and sheds light on the reasons behind the intense focus placed on perpetual weight gain and increased caloric intake by CF patients and their providers. The manuscript explores the potential mechanisms by which aberrant CFTR may contribute to increased resting energy expenditure (REE), and how correcting and potentiating its activity, possibly by reducing REE, among other intended and off-target effects, can contribute to weight gain in this patient population. The commentary also examines what is currently known about metabolic and vascular complications of obesity in patients with CF, and presents dietary, nutritional, pharmacologic and surgical approaches that may help obese patients with CF lose weight and build more lean body mass.

Keywords: Cystic fibrosis; Cystic fibrosis related diabetes (CFRD); Energy balance; Fat free mass; Lean body mass; Nutrition; Obesity; Overnutrition; Overweight; Resting energy expenditure.