Prospective study of fatty acid supplementation over 3 years in patients with cystic fibrosis.
Intravenous fatty acid supplementation (10% Intralipid, 10 ml/kg body weight) was given to 10 patients with cystic fibrosis (CF), in intervals for 3 years. One year of intensive treatment with supplementation every fortnight was followed by 1 year of intermission and then 1 year of the same treatment given for 2 days every 2 months. Compared with matched CF patients, the clinical course, evaluated by Shwachmann scoring, showed less deterioration in the treated group, especially during the year of intensive treatment. Single parameters, such as pulmonary function tests, pulmonary X-ray films, clinical infections, bacterial growth of sputa, and sweat electrolyte concentration, showed no differences. It is concluded that if there is any benefit of this kind of treatment in CF, it has to be very intensive. At present, the psychological stress involved in repeated parenteral administrations over long periods prevents its use in clinical routine.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000503:physiopathology / Q000628:therapy*; D005217:Fat Emulsions, Intravenous / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010288:Parenteral Nutrition / Q000523:psychology; D011446:Prospective Studies; D013315:Stress, Psychological; D013542:Sweat / Q000032:analysis; D013997:Time Factors
D005217:Fat Emulsions, Intravenous