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Gastrointestinal pathophysiology and nutrition in cystic fibrosis.
Expert Rev Gastroenterol Hepatol. 2018 Sep; 12(9):853-862.ER

Abstract

INTRODUCTION

Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Optimizing nutrition is critical, as higher growth parameters are associated with better pulmonary function and outcomes, but unfortunately patients with this disease are prone to malnutrition, growth failure, and vitamin deficiencies. The purpose of this review is to provide a timely highlight of the physiologic processes and outcome data to support today's management strategies, as well as review these principles themselves. Areas covered: This review covers the background of the importance of vigilant attention to nutrition and growth in these patients, the underlying physiology leading to an abnormal gastrointestinal tract and its role in CF malnutrition, and current evaluation and management strategies to address nutrition in CF. Analysis of up-to-date relevant literature was performed using PubMed. Expert commentary: Advances in research and clinical developments over the years have improved knowledge of this disease as well as patient outcomes. Of particular importance is optimizing nutrition especially in the early stages of life, as well as accounting for the markedly abnormal CF intestinal milieu when addressing the gastrointestinal and nutritional needs of these patients.

Authors+Show Affiliations

a Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition , Saint Louis University School of Medicine , Saint Louis , Missouri , USA.a Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition , Saint Louis University School of Medicine , Saint Louis , Missouri , USA.a Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition , Saint Louis University School of Medicine , Saint Louis , Missouri , USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

30019967

Citation

Ratchford, Thomas L., et al. "Gastrointestinal Pathophysiology and Nutrition in Cystic Fibrosis." Expert Review of Gastroenterology & Hepatology, vol. 12, no. 9, 2018, pp. 853-862.
Ratchford TL, Teckman JH, Patel DR. Gastrointestinal pathophysiology and nutrition in cystic fibrosis. Expert Rev Gastroenterol Hepatol. 2018;12(9):853-862.
Ratchford, T. L., Teckman, J. H., & Patel, D. R. (2018). Gastrointestinal pathophysiology and nutrition in cystic fibrosis. Expert Review of Gastroenterology & Hepatology, 12(9), 853-862. https://doi.org/10.1080/17474124.2018.1502663
Ratchford TL, Teckman JH, Patel DR. Gastrointestinal Pathophysiology and Nutrition in Cystic Fibrosis. Expert Rev Gastroenterol Hepatol. 2018;12(9):853-862. PubMed PMID: 30019967.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Gastrointestinal pathophysiology and nutrition in cystic fibrosis. AU - Ratchford,Thomas L, AU - Teckman,Jeffrey H, AU - Patel,Dhiren R, Y1 - 2018/08/03/ PY - 2018/7/19/pubmed PY - 2018/12/12/medline PY - 2018/7/19/entrez KW - Cystic fibrosis transmembrane conductance regulator (CFTR) KW - Pancreatic enzyme replacement therapy (PERT) KW - cystic fibrosis KW - enteral nutrition KW - failure to thrive KW - growth failure KW - malabsorption KW - nutrition KW - pancreatic insufficiency KW - vitamin deficiency SP - 853 EP - 862 JF - Expert review of gastroenterology & hepatology JO - Expert Rev Gastroenterol Hepatol VL - 12 IS - 9 N2 - INTRODUCTION: Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Optimizing nutrition is critical, as higher growth parameters are associated with better pulmonary function and outcomes, but unfortunately patients with this disease are prone to malnutrition, growth failure, and vitamin deficiencies. The purpose of this review is to provide a timely highlight of the physiologic processes and outcome data to support today's management strategies, as well as review these principles themselves. Areas covered: This review covers the background of the importance of vigilant attention to nutrition and growth in these patients, the underlying physiology leading to an abnormal gastrointestinal tract and its role in CF malnutrition, and current evaluation and management strategies to address nutrition in CF. Analysis of up-to-date relevant literature was performed using PubMed. Expert commentary: Advances in research and clinical developments over the years have improved knowledge of this disease as well as patient outcomes. Of particular importance is optimizing nutrition especially in the early stages of life, as well as accounting for the markedly abnormal CF intestinal milieu when addressing the gastrointestinal and nutritional needs of these patients. SN - 1747-4132 UR - https://www.unboundmedicine.com/medline/citation/30019967/Gastrointestinal_pathophysiology_and_nutrition_in_cystic_fibrosis_ L2 - https://www.tandfonline.com/doi/full/10.1080/17474124.2018.1502663 DB - PRIME DP - Unbound Medicine ER -