Fatty acid alterations and n-3 fatty acid supplementation in cystic fibrosis. 2007

M Rabie Al-Turkmani, and Steven D Freedman, and Michael Laposata
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, GRB 235, 55 Fruit Street, Boston, MA 02114, USA.

Specific fatty acid alterations have been described in the blood and tissues of cystic fibrosis (CF) patients. The two most consistent alterations include decreased levels of linoleic acid (LA) and decreased levels of docosahexaenoic acid (DHA). Increased arachidonic acid (AA) release from membrane phospholipids, as well as changes in levels of AA and other monounsaturated and polyunsaturated fatty acids (PUFAs) have also been described in CF. Although mechanisms of fatty acid alterations have not yet been determined, these alterations may have an important role in the progression of the CF disease. There have been several clinical trials in which CF patients were supplemented with n-3 fatty acids. Most trials resulted in an increase in the levels of the supplemental fatty acids in the blood of CF patients in the absence of significant clinical improvement. It is recommended that future trials include a larger population of CF patients and measure multiple clinical outcomes.

UI MeSH Term Description Entries
D010743 Phospholipids Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system. Phosphatides,Phospholipid
D003550 Cystic Fibrosis An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION. Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D004281 Docosahexaenoic Acids C22-unsaturated fatty acids found predominantly in FISH OILS. Docosahexaenoate,Docosahexaenoic Acid,Docosahexenoic Acids,Docosahexaenoic Acid (All-Z Isomer),Docosahexaenoic Acid Dimer (All-Z Isomer),Docosahexaenoic Acid, 3,6,9,12,15,18-Isomer,Docosahexaenoic Acid, 4,7,10,13,16,19-(All-Z-Isomer),Docosahexaenoic Acid, 4,7,10,13,16,19-(All-Z-Isomer), Cerium Salt,Docosahexaenoic Acid, 4,7,10,13,16,19-(All-Z-Isomer), Cesium Salt,Docosahexaenoic Acid, 4,7,10,13,16,19-(All-Z-Isomer), Potassium Salt,Docosahexaenoic Acid, 4,7,10,13,16,19-(Z,Z,Z,Z,Z,E-Isomer),Docosahexaenoic Acid, 4,7,10,13,16,19-Isomer,Docosahexaenoic Acid, 4,7,10,13,16,19-Isomer, Sodium Salt,Docosahexaenoic Acid, Sodium Salt,Acid, Docosahexaenoic,Acids, Docosahexaenoic,Acids, Docosahexenoic
D005227 Fatty Acids Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed) Aliphatic Acid,Esterified Fatty Acid,Fatty Acid,Fatty Acids, Esterified,Fatty Acids, Saturated,Saturated Fatty Acid,Aliphatic Acids,Acid, Aliphatic,Acid, Esterified Fatty,Acid, Saturated Fatty,Esterified Fatty Acids,Fatty Acid, Esterified,Fatty Acid, Saturated,Saturated Fatty Acids
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D015525 Fatty Acids, Omega-3 A group of unsaturated fatty acids occurring mainly in fish oils, with three double bonds at particular positions in the hydrocarbon chain. N-3 Fatty Acid,Omega-3 Fatty Acid,Omega-3 Fatty Acids,n-3 Fatty Acids,n-3 Oil,n3 Oil,Omega 3 Fatty Acids,n-3 Oils,n-3 PUFA,n-3 Polyunsaturated Fatty Acid,n3 Fatty Acid,n3 Oils,n3 PUFA,n3 Polyunsaturated Fatty Acid,Acid, N-3 Fatty,Acid, Omega-3 Fatty,Fatty Acid, N-3,Fatty Acid, Omega-3,Fatty Acid, n3,N 3 Fatty Acid,Oil, n-3,Oil, n3,Omega 3 Fatty Acid,PUFA, n-3,PUFA, n3,n 3 Fatty Acids,n 3 Oil,n 3 Oils,n 3 PUFA,n 3 Polyunsaturated Fatty Acid
D016718 Arachidonic Acid An unsaturated, essential fatty acid. It is found in animal and human fat as well as in the liver, brain, and glandular organs, and is a constituent of animal phosphatides. It is formed by the synthesis from dietary linoleic acid and is a precursor in the biosynthesis of prostaglandins, thromboxanes, and leukotrienes. (all-Z)-5,8,11,14-Eicosatetraenoic acid,Arachidonic Acid, (all-Z)-Isomer, 1-(14)C-Labeled,Arachidonic Acid, (all-Z)-isomer, 3H-Labeled,Arachidonic Acid, Ammonium Salt, (all-Z)-Isomer,Arachidonic Acid, Cerium Salt, (all-Z)-Isomer,Arachidonic Acid, Cesium Salt, (all-Z)-Isomer,Arachidonic Acid, Lithium Salt, (all-Z)-Isomer,Arachidonic Acid, Potassium Salt, (all-Z)-Isomer,Arachidonic Acid, Sodium Salt,Arachidonic Acid, Sodium Salt, (all-Z)-Isomer,Arachidonic Acid, Zinc Salt, (all-Z)-Isomer,Sodium Arachidonate,Vitamin F,Arachidonate, Sodium
D019587 Dietary Supplements Products in capsule, tablet or liquid form that provide dietary ingredients, and that are intended to be taken by mouth to increase the intake of nutrients. Dietary supplements can include macronutrients, such as proteins, carbohydrates, and fats; and/or MICRONUTRIENTS, such as VITAMINS; MINERALS; and PHYTOCHEMICALS. Food Supplements,Neutraceuticals,Dietary Supplementations,Food Supplementations,Herbal Supplements,Nutraceuticals,Nutriceuticals,Dietary Supplement,Food Supplement,Herbal Supplement,Neutraceutical,Nutraceutical,Nutriceutical,Supplement, Food,Supplement, Herbal,Supplementations, Dietary,Supplements, Dietary,Supplements, Food,Supplements, Herbal
D019787 Linoleic Acid A doubly unsaturated fatty acid, occurring widely in plant glycosides. It is an essential fatty acid in mammalian nutrition and is used in the biosynthesis of prostaglandins and cell membranes. (From Stedman, 26th ed) 9,12-Octadecadienoic Acid,Linoleate,9-trans,12-trans-Octadecadienoic Acid,Linoelaidic Acid,Linoelaidic Acid, (E,Z)-Isomer,Linoleic Acid, (E,E)-Isomer,Linoleic Acid, (Z,E)-Isomer,Linoleic Acid, (Z,Z)-Isomer,Linoleic Acid, (Z,Z)-Isomer, 14C-Labeled,Linoleic Acid, Ammonium Salt, (Z,Z)-Isomer,Linoleic Acid, Calcium Salt, (Z,Z)-Isomer,Linoleic Acid, Potassium Salt, (Z,Z)-Isomer,Linoleic Acid, Sodium Salt, (E,E)-Isomer,Linoleic Acid, Sodium Salt, (Z,Z)-Isomer,Linolelaidic Acid,cis,cis-9,12-Octadecadienoic Acid,trans,trans-9,12-Octadecadienoic Acid,9 trans,12 trans Octadecadienoic Acid,9,12 Octadecadienoic Acid,Acid, 9,12-Octadecadienoic

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