+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Prospective study of fatty acid supplementation over 3 years in patients with cystic fibrosis



Prospective study of fatty acid supplementation over 3 years in patients with cystic fibrosis



Journal of Pediatric Gastroenterology and Nutrition 2(3): 434-438



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 2 weeks was followed by 1 year of intermission and 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 (e.g., 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. (author)

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 001240354

Download citation: RISBibTeXText

PMID: 6413667

DOI: 10.1097/00005176-198302030-00007


Related references

Polyunsaturated fatty acid supplementation reverses cystic fibrosis-related fatty acid abnormalities in CFTR-/- mice by suppressing fatty acid desaturases. Journal of Nutritional Biochemistry 26(1): 36-43, 2015

A comprehensive evaluation of omega-3 fatty acid supplementation in cystic fibrosis patients using lipidomics. Journal of Nutritional Biochemistry 63: 197-205, 2019

Fatty acid supplementation in cystic fibrosis. Monographs in Paediatrics 10: 1-4, 1979

The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients - A pilot study. Prostaglandins Leukotrienes and Essential Fatty Acids 108: 45-50, 2016

Is there a case for n-3 fatty acid supplementation in cystic fibrosis?. Current Opinion in Clinical Nutrition and Metabolic Care 8(2): 153-159, 2005

Fatty acid alterations and n-3 fatty acid supplementation in cystic fibrosis. Prostaglandins Leukotrienes and Essential Fatty Acids 77(5-6): 309-318, 2007

A therapeutic trial of fatty acid supplementation in cystic fibrosis. Pediatrics 57(4): 474-479, 1976

Epidemiologic study of cystic fibrosis: design and implementation of a prospective, multicenter, observational study of patients with cystic fibrosis in the U.S. and Canada. Pediatric Pulmonology 28(4): 231-241, 1999

Essential fatty acid deficiency and nutritional supplementation in cystic fibrosis. Journal of Pediatrics 141(2): 157-159, 2002

Effect on renal function of essential fatty acid supplementation in cystic fibrosis. Journal of Pediatrics 115(2): 242-250, 1989

Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation. American Journal of Clinical Nutrition 34(1): 1-7, 1981

Liver function and morphology during long-term fatty acid supplementation in cystic fibrosis. Liver. 14(1): 32-36, 1994

Negative effects of oral fatty acid supplementation on sweat chloride in cystic fibrosis. Pediatrics 64(1): 50-52, 1979

Fatty acids in blood and intestine following docosahexaenoic acid supplementation in adults with cystic fibrosis. Journal of Cystic Fibrosis 5(2): 77-84, 2006

Paradoxical effects of essential fatty acid supplementation on lipid profiles and sweat electrolytes in cystic fibrosis. British Journal of Nutrition 63(2): 259-271, 1990