Defective essential-fatty-acid metabolism in cystic fibrosis

Lancet. 1975 Oct 4;2(7936):642-3. doi: 10.1016/s0140-6736(75)90121-x.

Abstract

Cystic fibrosis (C.F.) is characterised by low serum levels of essential fatty acids (E.F.A.). However, the fatty-acid pattern does not totally resemble that of dietary E.F.A. deficiency. The differences suggest a reduction in the desaturation of E.F.S. It is not known whether this defect is the primary lesion in C.F. or is the result of tissue damage in the disease. It is proposed that C.F. patients might have increased linoleic-acid requirements, and possibly specific requirements for its desaturation products.

Publication types

  • Review

MeSH terms

  • Animals
  • Cystic Fibrosis / metabolism*
  • Dietary Fats / metabolism
  • Fatty Acid Desaturases / metabolism
  • Fatty Acids, Essential / deficiency
  • Fatty Acids, Essential / metabolism*
  • Humans
  • Parenteral Nutrition
  • Phospholipids / metabolism

Substances

  • Dietary Fats
  • Fatty Acids, Essential
  • Phospholipids
  • Fatty Acid Desaturases