Energy balance and obesity in individuals with cystic fibrosis

Marina Litvin; John C Yoon; Julio Leey Casella; Scott M Blackman; Amanda L Brennan

doi:10.1016/j.jcf.2019.08.015

Energy balance and obesity in individuals with cystic fibrosis

J Cyst Fibros. 2019 Oct:18 Suppl 2:S38-S47. doi: 10.1016/j.jcf.2019.08.015.

Authors

Marina Litvin¹, John C Yoon², Julio Leey Casella³, Scott M Blackman⁴, Amanda L Brennan⁵

Affiliations

¹ Division of Endocrinology, Metabolism, and Lipid Research, Department of Internal Medicine, Washington University School of Medicine, St. Louis, MO, USA. Electronic address: [email protected].
² Division of Endocrinology, Diabetes, and Metabolism, Department of Internal Medicine, University of California School of Medicine, Sacramento, CA, USA.
³ Division of Endocrinology, Diabetes, and Metabolism, University of Florida School of Medicine, Gainesville, FL, USA.
⁴ Division of Pediatric Endocrinology and Diabetes, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
⁵ Manchester Adult Cystic Fibrosis Center, University Hospital South Manchester, Wythenshawe, Manchester, UK.

PMID: 31679728
DOI: 10.1016/j.jcf.2019.08.015

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.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Cystic Fibrosis / complications*
Cystic Fibrosis / metabolism*
Energy Metabolism*
Humans
Nutritional Status
Obesity / complications*
Obesity / metabolism*
Obesity / therapy