Abnormal fatty-acid composition of plasma-lipids in cystic fibrosis. A primary or a secondary defect?

Lancet. 1977 Dec;2(8052-8053):1302-4. doi: 10.1016/s0140-6736(77)90359-2.

Abstract

The percentage fatty-acid composition of the various plasma-lipid fractions from cystic-fibrosis patients with and without pancreatic insufficiency, obligate heterozygotes, and normal subjects was determined. Only the cystic-fibrosis patients with pancreatic insufficiency had significantly abnormal fatty-acid composition. This general observation did not correlate with vitamin-E deficiency, age, sex, or severity of the disease. Thus the altered fatty-acid composition of the different plasma-lipid fractions appears to be a secondary consequence of the pancreatic insufficiency commonly associated with cystic fibrosis and not a direct metabolic defect related to the cystic-fibrosis gene itself.

MeSH terms

  • Adolescent
  • Adult
  • Cystic Fibrosis / blood*
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology
  • Fatty Acids / blood*
  • Female
  • Heterozygote
  • Humans
  • Lipids / blood*
  • Malabsorption Syndromes / blood
  • Malabsorption Syndromes / etiology
  • Male
  • Middle Aged
  • Oleic Acids / blood
  • Palmitic Acids / blood
  • Pancreas / physiopathology
  • Phospholipids / blood
  • Triglycerides / blood

Substances

  • Fatty Acids
  • Lipids
  • Oleic Acids
  • Palmitic Acids
  • Phospholipids
  • Triglycerides