Nutrition management in adults with cystic fibrosis

Jessica M. Shape; Marc A. Sala

doi:10.1002/ncp.10842

Nutrition management in adults with cystic fibrosis

Jessica M. Shape, Marc A. Sala^*

^*Corresponding author for this work

Medicine, Pulmonary Division

Research output: Contribution to journal › Review article › peer-review

Abstract

Cystic fibrosis (CF) is a lethal, monogenic, autosomal recessive disease with manifestations in multiple organ systems, including the lungs and gastrointestinal tract, that impact adequate nutrition. This review discusses important aspects of nutrition in adults with CF with a focus on exocrine pancreatic insufficiency, CF-related diabetes, and gastrointestinal motility, as well as how advanced lung disease, CF transmembrane conductance regulator modulators, and aging impact nutrition in CF.

Original language	English (US)
Pages (from-to)	256-264
Number of pages	9
Journal	Nutrition in Clinical Practice
Volume	37
Issue number	2
DOIs	https://doi.org/10.1002/ncp.10842
State	Published - Apr 2022

Keywords

CFTR modulator
cystic fibrosis
diabetes mellitus
exocrine pancreatic insufficiency
gastrointestinal motility
lung diseases
nutrition status
nutrition therapy

ASJC Scopus subject areas

Medicine (miscellaneous)
Nutrition and Dietetics

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1002/ncp.10842

Cite this

@article{6ed1703a64164b61b7faeb46580ebfa2,

title = "Nutrition management in adults with cystic fibrosis",

abstract = "Cystic fibrosis (CF) is a lethal, monogenic, autosomal recessive disease with manifestations in multiple organ systems, including the lungs and gastrointestinal tract, that impact adequate nutrition. This review discusses important aspects of nutrition in adults with CF with a focus on exocrine pancreatic insufficiency, CF-related diabetes, and gastrointestinal motility, as well as how advanced lung disease, CF transmembrane conductance regulator modulators, and aging impact nutrition in CF.",

keywords = "CFTR modulator, cystic fibrosis, diabetes mellitus, exocrine pancreatic insufficiency, gastrointestinal motility, lung diseases, nutrition status, nutrition therapy",

author = "Shape, {Jessica M.} and Sala, {Marc A.}",

note = "Funding Information: None declared. Exocrine pancreatic insufficiency (EPI) is the most common and fundamental nutrition abnormality for the majority of people with CF. An estimated 85%?90% of the CF population is thought to be affected by EPI.8 EPI represents a decrease in the secretion of the normal enzymes (lipase, amylase, and protease) such that normal digestion and growth is impaired. Publisher Copyright: {\textcopyright} 2022 American Society for Parenteral and Enteral Nutrition.",

year = "2022",

month = apr,

doi = "10.1002/ncp.10842",

language = "English (US)",

volume = "37",

pages = "256--264",

journal = "Nutrition in Clinical Practice",

issn = "0884-5336",

publisher = "SAGE Publications Ltd",

number = "2",

}

TY - JOUR

T1 - Nutrition management in adults with cystic fibrosis

AU - Shape, Jessica M.

AU - Sala, Marc A.

N1 - Funding Information: None declared. Exocrine pancreatic insufficiency (EPI) is the most common and fundamental nutrition abnormality for the majority of people with CF. An estimated 85%?90% of the CF population is thought to be affected by EPI.8 EPI represents a decrease in the secretion of the normal enzymes (lipase, amylase, and protease) such that normal digestion and growth is impaired. Publisher Copyright: © 2022 American Society for Parenteral and Enteral Nutrition.

PY - 2022/4

Y1 - 2022/4

N2 - Cystic fibrosis (CF) is a lethal, monogenic, autosomal recessive disease with manifestations in multiple organ systems, including the lungs and gastrointestinal tract, that impact adequate nutrition. This review discusses important aspects of nutrition in adults with CF with a focus on exocrine pancreatic insufficiency, CF-related diabetes, and gastrointestinal motility, as well as how advanced lung disease, CF transmembrane conductance regulator modulators, and aging impact nutrition in CF.

AB - Cystic fibrosis (CF) is a lethal, monogenic, autosomal recessive disease with manifestations in multiple organ systems, including the lungs and gastrointestinal tract, that impact adequate nutrition. This review discusses important aspects of nutrition in adults with CF with a focus on exocrine pancreatic insufficiency, CF-related diabetes, and gastrointestinal motility, as well as how advanced lung disease, CF transmembrane conductance regulator modulators, and aging impact nutrition in CF.

KW - CFTR modulator

KW - cystic fibrosis

KW - diabetes mellitus

KW - exocrine pancreatic insufficiency

KW - gastrointestinal motility

KW - lung diseases

KW - nutrition status

KW - nutrition therapy

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U2 - 10.1002/ncp.10842

DO - 10.1002/ncp.10842

M3 - Review article

C2 - 35118712

AN - SCOPUS:85124141924

SN - 0884-5336

VL - 37

SP - 256

EP - 264

JO - Nutrition in Clinical Practice

JF - Nutrition in Clinical Practice

IS - 2

ER -

Nutrition management in adults with cystic fibrosis

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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