Skip to main content
Log in

Relationships between essential fatty acid levels, pulmonary function and fat absorption in pre-adolescent cystic fibrosis children with good clinical scores

  • Metabolic Diseases
  • Published:
European Journal of Pediatrics Aims and scope Submit manuscript

Abstract

Twenty-one pre-adolescent cystic fibrosis (CF) children with good clinical scores had significant (P<0.001) deficiencies of arachidonic (mean 3.8% of total plasma fatty acids ± SD 1.4) and linoleic (18.1±6.3) acids compared with controls (6.0±1.0, and 27.6±3.9, respectively). Despite the presence of pulmonary involvement of varying severity in all the CF children, neither arachidonic nor linoleic acid levels correlated significantly with pulmonary function measured by spirometry. All children had adequate caloric and fat intakes, and the coefficient of fat absorption correlated with none of the deficient fatty acid levels. These findings suggest that deficiencies of arachidonic and linoleic acids are of minor importance in the early development of pulmonary involvement in CF, and that factors other than fat malabsorption and decreased dietary intake probably contribute to fatty acid deficiency.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

Abbreviations

CF:

Cystic fibrosis

EFA:

Essential fatty acid

PUFA:

polyunsaturated fatty acid

FVC:

forced vital capacity

FEV1 :

forced expiratory volume in 1 s

FEF50, FEF75 :

forced expiratory flows at 50% and 75% of the expired vital capacity

FEF25–75 :

forced expiratory flow measured between 25% and 75% of the expired vital capacity

References

  1. Chase HP, Dupont J (1978) Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis. Lancet II:236–238

    Google Scholar 

  2. Chase HP, Cotton EK, Elliott RB (1979) Intravenous linoleic acid supplementation in children with cystic fibrosis. Pediatrics 64: 207–213

    Google Scholar 

  3. Corey M, Levison H, Crozier D (1976) Five-to seven-year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis 114: 1085–1092

    Google Scholar 

  4. Crawford MA (1983) Background to essential fatty acids and their prostanoid derivatives. Br Med Bull 39:210–213

    Google Scholar 

  5. Darling PB, Lapage G, Leroy C, Roy CC (1985) Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatr Res 19: 578–582

    Google Scholar 

  6. Davidson GP, Kirubakaran CP, Ratcliffe G, Cooper DM, Robb TA (1986) Abnormal pancreatic electrolyte secretion in cystic fibrosis. Acta Paediatr Scand 75:145–150

    Google Scholar 

  7. Dodge JA (1985) The nutritional state and nutrition. Acta Paediatr Scand [Suppl] 317:31–37

    Google Scholar 

  8. Farrell PM, Mischler EH, Engle MJ, Brown DJ, Lau SM (1985) Fatty acid abnormalities in cystic fibrosis. Pediatr Res 19:104–109

    Google Scholar 

  9. Friedman Z, Rosenberg A (1979) Abnormal lung surfactant related to essential fatty acid deficiency in the neonate. Pediatrics 63:855–859

    Google Scholar 

  10. Gaskin K, Gurwitz D, Durie P, Corey M, Levison H, Forstner G (1982) Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr 100:857–862

    Google Scholar 

  11. Gibson RA, Teubner JK, Haines K, Cooper DM, Davidson GP (1986) Relationships between pulmonary function and plasma fatty acid levels in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 5:408–415

    Google Scholar 

  12. Holt TL, Ward LC, Francis PJ, Isles A, Cooksley WGE, Shepherd RW (1985) Whole body protein turnover in malnourished cystic fibrosis patients and its relationship to pulmonary disease. Am J Clin Nutr 41:1061–1066

    Google Scholar 

  13. Hopkins DT, Witter RL, Nesheim MC (1963) A respiratory disease syndrome in chickens fed essential fatty acid free diets. Proc Soc Exp Biol Med 114:82–86

    Google Scholar 

  14. Hubbard VS (1983) What is the association of essential fatty acid status with cysicc fibrosis? Eur J Pediatr 141:68–70

    Google Scholar 

  15. Hughes RL, Sahgal V, Craig R (1985) Potential effects of nutritional state on pulmonary function in cystic fibrosis. Acta Paediatr Scand [Suppl] 317:42–47

    Google Scholar 

  16. Jones DL, Hemphill W, Meyers ESA (1973) Height, weight and other physical characteristics of New South Wales children. New South Wales: New South Wales Department of Health; New South Wales Government Printer (parts 1 and 2)

    Google Scholar 

  17. Kuo PT, Huang NN (1965) The effect of medium chain triglyceride upon fat absorption and plasma lipid and depot fat of children with cystic fibrosis of the pancreas. J Clin Invest 44:1924–1933

    Google Scholar 

  18. Lloyd-Still JD, Johnson SB, Holman RT (1981) Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation. Am J Clin Nutr 34:1–7

    Google Scholar 

  19. Mischler EH, Parrell SW, Farrell PM, Raynor WJ, Lemen RJ (1986) Correction of linoleic acid deficiency in cystic fibrosis. Pediatr Res 20:36–41

    Google Scholar 

  20. Rogiers V, Dab I, Crockaert R, Vis HL (1980) Long chain nonessential fatty acid pattern in plasma of cystic fibrosis patients and their parents. Pediatr Res 14:1088–1091

    Google Scholar 

  21. Rosenlund ML, Kim HK, Kritchevsky D (1974) Essential fatty acids in cystic fibrosis. Nature 251:719–720

    Google Scholar 

  22. Shepherd R, Cooksley WGE, Cooke WDD (1980) Improved growth and clinical, nutritional, and respiratory changes to nutritional therapy in cystic fibrosis. J Pediatr 97:351–357

    Google Scholar 

  23. Sinclair HM (1984) Essential fatty acids in perspective. Hum Nutr Clin Nutr 38C:245–260

    Google Scholar 

  24. Taussig L, Kattwinkel J, Friedewald WT, di Sant'Agnese PA (1973) A new prognostic scoring system for cystic fibrosis. J Pediatr 82:380–390

    Google Scholar 

  25. Van de Kamer JH, ten Bokkell Juinink H, Weijers HA (1949) A rapid method for the determination of fat in feces. J Biol Chem 177:347–355

    Google Scholar 

  26. Wenlock RW, Disselduff MM, Skinner RK, Knight I (1986) The diets of British schoolchildren. Her Majesty's Stationery Office, London

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Thompson, G.N. Relationships between essential fatty acid levels, pulmonary function and fat absorption in pre-adolescent cystic fibrosis children with good clinical scores. Eur J Pediatr 148, 327–329 (1989). https://doi.org/10.1007/BF00444126

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00444126

Key words

Navigation