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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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Nutritional Issues in Cystic Fibrosis.

Missale Solomon1, Molly Bozic2, Maria R Mascarenhas3

  • 1Drexel University Philadelphia, Philadelphia, PA, USA.

Clinics in Chest Medicine
|February 10, 2016
PubMed
Summary

Maintaining adequate nutrition is vital for patients with cystic fibrosis (CF). Early nutritional assessments and a stepwise management approach improve growth, lung function, and overall health outcomes in CF patients.

Keywords:
Cystic fibrosisCystic fibrosis–related diabetesEnteral nutritionMalabsorptionMalnutritionNutritional assessmentNutritional statusPancreatic insufficiency

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Area of Science:

  • Clinical Nutrition
  • Pulmonology
  • Pediatric Medicine

Background:

  • Adequate nutrition is critical for patients with cystic fibrosis (CF).
  • Optimal nutrition and growth correlate with improved lung function in CF.
  • Nutritional challenges are prevalent in CF, impacting overall health.

Purpose of the Study:

  • To emphasize the importance of consistent nutritional assessments in CF.
  • To outline a stepwise approach for managing malnutrition in CF.
  • To highlight nutrition's role throughout the lifespan of individuals with CF.

Main Methods:

  • Regular and comprehensive nutritional assessments.
  • Identification of risk factors for nutritional failure.
  • Stepwise management protocols for malnutrition.

Main Results:

  • Early identification of nutritional deficiencies.
  • Development of individualized nutritional plans.
  • Improved patient outcomes through nutritional intervention.

Conclusions:

  • Nutritional management is a cornerstone of cystic fibrosis care.
  • Consistent assessment and intervention are key to preventing and treating malnutrition.
  • Optimizing nutrition supports better growth and lung function in CF patients.