Related Experiment Video
Updated: Aug 6, 2026

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections
Published on: May 8, 2017
Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis
J D Lloyd-Still1, D M Bibus, C A Powers
1Department of Pediatrics, Rush Medical College, Chicago, IL 60612, USA.
Insights
Essential fatty acid (EFA) deficiency is common in cystic fibrosis (CF) patients, particularly infants. This deficiency may increase susceptibility to pulmonary infections and respiratory disease in CF.
Area of Science:
- Biochemistry
- Pediatrics
- Pulmonology
Background:
- Essential fatty acid (EFA) deficiency is linked to increased susceptibility to pulmonary infections by Staphylococcus aureus and Pseudomonas aeruginosa.
- These pathogens are major causes of illness in cystic fibrosis (CF).
Purpose of the Study:
- To assess the essential fatty acid status in cystic fibrosis patients from infancy to 20 years of age.
Main Methods:
- Plasma phospholipid fatty acid profiles were analyzed in CF patients across various age groups (cord, 4 months, 16 months, 3 years, 5-10 years, 10-20 years).
- Data were compared to age-matched control groups.
- Statistical significance was determined using Student's t-test (p < 0.05).
Main Results:
- CF patients, excluding cord blood samples, showed consistent deficiencies in omega-3 and omega-6 EFAs.
- These EFA deficiencies were most severe in infants and more pronounced in CF patients with meconium ileus.
Conclusions:
- EFA deficiency may predispose infants with cystic fibrosis to respiratory diseases.
- This deficiency might also contribute to increased cytotoxic activity observed in bronchoalveolar lavage fluid of young CF infants.
Unlabelled:
Essential fatty acid (EFA) deficiency is a predisposing factor for pulmonary infection with Staphylococcus aureus and Pseudomonas aeruginosa, the two major pathogenic microorganisms in cystic fibrosis (CF).
Objective:
The goal of this study was to investigate the essential fatty acid status of CF patients from infancy to 20 years old.
Materials And Methods:
Plasma fatty acid profiles for phospholipid (PL) were determined for cord (n = 6), 4 months (n = 40), 16 months (n = 25), 3 y (n = 8), 5-10 y (n = 10), and 10-20 y (n = 10) aged CF patients and compared to their respective control; cord (n = 22), 1-36 months (n = 38) and adult (n = 100). Significance was established by Student's t-test (p < 0.05).
Results:
The plasma PL fatty acid profile for all CF patients, except cord, revealed consistent deficiency in omega 3 and omega 6 EFAs. These deficiencies were most marked at infancy and more pronounced for patients with meconium ileus.
Conclusions And Relevance:
EFA deficiency may contribute to the predisposition of CF infants to develop respiratory disease and to the excess cytotoxic activity found in bronchoalveolar lavage fluid at 2 months of age in the majority of screened infants.
More Related Videos
Related Concept Videos
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management
Sinus disease and chronic sinusitis...
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Inflammation
Chronic Obstructive Pulmonary Disease I: Introduction
Chronic Obstructive Pulmonary Disease II: Emphysema
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

