title: Effect of a dietary intervention on growth and energy expenditure in children with cystic fibrosis.
abstract: BACKGROUND
  The study aim was to determine the effect of a dietary intervention on growth, body composition and resting energy expenditure (REE) in children with cystic fibrosis (CF) and pancreatic insufficiency (PI) in a randomized, double blind, placebo-controlled trial.
  
  
  METHODS
  Subjects (5 to 17 yrs) participated in a 12-month trial of the organized lipid matrix LYM-X-SORB™ (LXS) vs. placebo dietary supplements with similar calories, total fat and fatty acids. Dietary intake was assessed using 3-day weighed food records. Height (HAZ), weight (WAZ), BMI (BMIZ), mid-upper arm muscle (UAMAZ) and fat area (UAFAZ) Z-scores were calculated. Fat mass (FM) and fat-free mass (FFM) were obtained by whole body DXA. REE (kcal/d) was evaluated by indirect calorimetry at baseline, 3 and 12 months and %REE calculated using Schofield equations. No growth or REE differences were observed between LXS and placebo groups so data were pooled for analysis.
  
  
  RESULTS
  63 children (57% males, age 10.6 ± 2.9 yr, 43% receiving LXS) completed REE measurements. Caloric intake increased from a median of 2502 [1478, 4909] to 2616 [1660, 4125] kcal/d at 12 months. HAZ, WAZ and UAMAZ increased (p < 0.05) over 12 months. Mean REE was 109 ± 8% predicted at baseline and 107 ± 9% at 12 months (p < 0.05). REE (kcal/d) adjusted for FFM and FM decreased over 12 months ([mean ± SE] -31 ± 12 kcals, p < 0.01), significant only in males (-49 ± 16 kcals, p < 0.01).
  
  
  CONCLUSIONS
  Over a 12 month nutrition intervention with either LXS or placebo, the growth status, muscle stores and REE improved. Sustained increased energy intake improved energy metabolism, growth and nutritional status in school age children with CF, PI and mild lung disease.
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA; Division of Gastroenterology, Hepatology and Nutrition, Ste-Justine University Hospital Center, Montreal, QC, Canada; University of Montreal, Montreal, QC, Canada. Electronic address: veronique.groleau@umontreal.ca.
authors: Veronique
authors: V
authors: Groleau
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA.
authors: Joan I
authors: JI
authors: Schall
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA; University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
authors: Kelly A
authors: KA
authors: Dougherty
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA.
authors: Norma E
authors: NE
authors: Latham
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA; University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
authors: Asim
authors: A
authors: Maqbool
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA; University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
authors: Maria R
authors: MR
authors: Mascarenhas
authors: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3535 Market Street, Room 1558, Philadelphia, PA 19104, USA; University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
authors: Virginia A
authors: VA
authors: Stallings
mesh_terms: D000293:Adolescent; D001823:Body Composition; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000178:diet therapy* / Q000503:physiopathology*; D004311:Double-Blind Method; D002149:Energy Intake; D004734:Energy Metabolism; D010188:Exocrine Pancreatic Insufficiency / Q000178:diet therapy; D005260:Female; D006128:Growth; D006801:Humans; D008171:Lung Diseases / Q000178:diet therapy; D008297:Male
keywords: Children; Cystic fibrosis; Dietary intervention; Resting energy expenditure