Pediatric Cystic Fibrosis and Fat-Soluble Vitamins

Kacie Beckett, Karyn Shaw, Meghana Sathe

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Citation (Scopus)

Abstract

Cystic Fibrosis (CF) is a genetic disorder that affects approximately 70,000 people worldwide. CF is caused by a mutation in the Cystic Fibrosis conductance Transmembrane Regulator (CFTR) gene. Mutations in CFTR result in thick, sticky mucus within the lungs, pancreas, intestines, and liver. Thick secretions within the pancreas result in destruction of pancreatic exocrine cells that are responsible for the production of pancreatic enzymes essential to the breakdown of fat, protein, and carbohydrates into absorbable nutrients. This also affects the absorption of fat-soluble vitamins A, D, E, and K. These vitamins are integral to physiologic processes within the body. Deficiencies result in significant morbidity and mortality. Thus, it is critical that patients with CF are screened at least annually for fat-soluble vitamin deficiencies. Once deficiencies are identified, they should be treated with appropriate supplementation, and treatment should be monitored to ensure adequate levels and prevent toxicity.

Original languageEnglish (US)
Title of host publicationDiet and Exercise in Cystic Fibrosis
PublisherElsevier Inc.
Pages125-130
Number of pages6
ISBN (Print)9780128005880, 9780128000519
DOIs
StatePublished - Aug 12 2014

Fingerprint

Cystic Fibrosis
Vitamins
Fats
Pediatrics
Pancreas
Avitaminosis
Cystic Fibrosis Transmembrane Conductance Regulator
Mutation
Inborn Genetic Diseases
Vitamin K
Mucus
Regulator Genes
Vitamin A
Vitamin D
Intestines
Carbohydrates
Morbidity
Food
Lung
Mortality

Keywords

  • CFTR (cystic fibrosis conductance transmembrane regulator)
  • Cystic fibrosis
  • Fat-soluble vitamins
  • Pancreatic insufficiency
  • Vitamin A
  • Vitamin D
  • Vitamin E
  • Vitamin K

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Beckett, K., Shaw, K., & Sathe, M. (2014). Pediatric Cystic Fibrosis and Fat-Soluble Vitamins. In Diet and Exercise in Cystic Fibrosis (pp. 125-130). Elsevier Inc.. https://doi.org/10.1016/B978-0-12-800051-9.00016-X

Pediatric Cystic Fibrosis and Fat-Soluble Vitamins. / Beckett, Kacie; Shaw, Karyn; Sathe, Meghana.

Diet and Exercise in Cystic Fibrosis. Elsevier Inc., 2014. p. 125-130.

Research output: Chapter in Book/Report/Conference proceedingChapter

Beckett, K, Shaw, K & Sathe, M 2014, Pediatric Cystic Fibrosis and Fat-Soluble Vitamins. in Diet and Exercise in Cystic Fibrosis. Elsevier Inc., pp. 125-130. https://doi.org/10.1016/B978-0-12-800051-9.00016-X
Beckett K, Shaw K, Sathe M. Pediatric Cystic Fibrosis and Fat-Soluble Vitamins. In Diet and Exercise in Cystic Fibrosis. Elsevier Inc. 2014. p. 125-130 https://doi.org/10.1016/B978-0-12-800051-9.00016-X
Beckett, Kacie ; Shaw, Karyn ; Sathe, Meghana. / Pediatric Cystic Fibrosis and Fat-Soluble Vitamins. Diet and Exercise in Cystic Fibrosis. Elsevier Inc., 2014. pp. 125-130
@inbook{48b00ab47cd84063a23f189b49266818,
title = "Pediatric Cystic Fibrosis and Fat-Soluble Vitamins",
abstract = "Cystic Fibrosis (CF) is a genetic disorder that affects approximately 70,000 people worldwide. CF is caused by a mutation in the Cystic Fibrosis conductance Transmembrane Regulator (CFTR) gene. Mutations in CFTR result in thick, sticky mucus within the lungs, pancreas, intestines, and liver. Thick secretions within the pancreas result in destruction of pancreatic exocrine cells that are responsible for the production of pancreatic enzymes essential to the breakdown of fat, protein, and carbohydrates into absorbable nutrients. This also affects the absorption of fat-soluble vitamins A, D, E, and K. These vitamins are integral to physiologic processes within the body. Deficiencies result in significant morbidity and mortality. Thus, it is critical that patients with CF are screened at least annually for fat-soluble vitamin deficiencies. Once deficiencies are identified, they should be treated with appropriate supplementation, and treatment should be monitored to ensure adequate levels and prevent toxicity.",
keywords = "CFTR (cystic fibrosis conductance transmembrane regulator), Cystic fibrosis, Fat-soluble vitamins, Pancreatic insufficiency, Vitamin A, Vitamin D, Vitamin E, Vitamin K",
author = "Kacie Beckett and Karyn Shaw and Meghana Sathe",
year = "2014",
month = "8",
day = "12",
doi = "10.1016/B978-0-12-800051-9.00016-X",
language = "English (US)",
isbn = "9780128005880",
pages = "125--130",
booktitle = "Diet and Exercise in Cystic Fibrosis",
publisher = "Elsevier Inc.",

}

TY - CHAP

T1 - Pediatric Cystic Fibrosis and Fat-Soluble Vitamins

AU - Beckett, Kacie

AU - Shaw, Karyn

AU - Sathe, Meghana

PY - 2014/8/12

Y1 - 2014/8/12

N2 - Cystic Fibrosis (CF) is a genetic disorder that affects approximately 70,000 people worldwide. CF is caused by a mutation in the Cystic Fibrosis conductance Transmembrane Regulator (CFTR) gene. Mutations in CFTR result in thick, sticky mucus within the lungs, pancreas, intestines, and liver. Thick secretions within the pancreas result in destruction of pancreatic exocrine cells that are responsible for the production of pancreatic enzymes essential to the breakdown of fat, protein, and carbohydrates into absorbable nutrients. This also affects the absorption of fat-soluble vitamins A, D, E, and K. These vitamins are integral to physiologic processes within the body. Deficiencies result in significant morbidity and mortality. Thus, it is critical that patients with CF are screened at least annually for fat-soluble vitamin deficiencies. Once deficiencies are identified, they should be treated with appropriate supplementation, and treatment should be monitored to ensure adequate levels and prevent toxicity.

AB - Cystic Fibrosis (CF) is a genetic disorder that affects approximately 70,000 people worldwide. CF is caused by a mutation in the Cystic Fibrosis conductance Transmembrane Regulator (CFTR) gene. Mutations in CFTR result in thick, sticky mucus within the lungs, pancreas, intestines, and liver. Thick secretions within the pancreas result in destruction of pancreatic exocrine cells that are responsible for the production of pancreatic enzymes essential to the breakdown of fat, protein, and carbohydrates into absorbable nutrients. This also affects the absorption of fat-soluble vitamins A, D, E, and K. These vitamins are integral to physiologic processes within the body. Deficiencies result in significant morbidity and mortality. Thus, it is critical that patients with CF are screened at least annually for fat-soluble vitamin deficiencies. Once deficiencies are identified, they should be treated with appropriate supplementation, and treatment should be monitored to ensure adequate levels and prevent toxicity.

KW - CFTR (cystic fibrosis conductance transmembrane regulator)

KW - Cystic fibrosis

KW - Fat-soluble vitamins

KW - Pancreatic insufficiency

KW - Vitamin A

KW - Vitamin D

KW - Vitamin E

KW - Vitamin K

UR - http://www.scopus.com/inward/record.url?scp=84942878143&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84942878143&partnerID=8YFLogxK

U2 - 10.1016/B978-0-12-800051-9.00016-X

DO - 10.1016/B978-0-12-800051-9.00016-X

M3 - Chapter

AN - SCOPUS:84942878143

SN - 9780128005880

SN - 9780128000519

SP - 125

EP - 130

BT - Diet and Exercise in Cystic Fibrosis

PB - Elsevier Inc.

ER -