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.
Abstract

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