Abnormal fatty-acid composition of plasma-lipids in cystic fibrosis. A primary or a secondary defect?
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.
D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000097:blood* / Q000235:genetics / Q000503:physiopathology; D005227:Fatty Acids / Q000097:blood*; D005260:Female; D006579:Heterozygote; D006801:Humans; D008055:Lipids / Q000097:blood*; D008286:Malabsorption Syndromes / Q000097:blood / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009829:Oleic Acids / Q000097:blood; D010169:Palmitic Acids / Q000097:blood; D010179:Pancreas / Q000503:physiopathology; D010743:Phospholipids / Q000097:blood; D014280:Triglycerides / Q000097:blood
D005227:Fatty Acids; D008055:Lipids; D009829:Oleic Acids; D010169:Palmitic Acids; D010743:Phospholipids; D014280:Triglycerides