Tags

Type your tag names separated by a space and hit enter

Growth and Nutrition in Cystic Fibrosis.
Semin Respir Crit Care Med. 2019 12; 40(6):775-791.SR

Abstract

Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

Authors+Show Affiliations

Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.Department of Pediatrics, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania. Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

31659726

Citation

Brownell, Jefferson N., et al. "Growth and Nutrition in Cystic Fibrosis." Seminars in Respiratory and Critical Care Medicine, vol. 40, no. 6, 2019, pp. 775-791.
Brownell JN, Bashaw H, Stallings VA. Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 2019;40(6):775-791.
Brownell, J. N., Bashaw, H., & Stallings, V. A. (2019). Growth and Nutrition in Cystic Fibrosis. Seminars in Respiratory and Critical Care Medicine, 40(6), 775-791. https://doi.org/10.1055/s-0039-1696726
Brownell JN, Bashaw H, Stallings VA. Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 2019;40(6):775-791. PubMed PMID: 31659726.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Growth and Nutrition in Cystic Fibrosis. AU - Brownell,Jefferson N, AU - Bashaw,Hillary, AU - Stallings,Virginia A, Y1 - 2019/10/28/ PY - 2019/10/30/pubmed PY - 2020/7/21/medline PY - 2019/10/30/entrez SP - 775 EP - 791 JF - Seminars in respiratory and critical care medicine JO - Semin Respir Crit Care Med VL - 40 IS - 6 N2 - Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes. SN - 1098-9048 UR - https://www.unboundmedicine.com/medline/citation/31659726/Growth_and_Nutrition_in_Cystic_Fibrosis_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-0039-1696726 DB - PRIME DP - Unbound Medicine ER -