Parenteral Nutrition in Cystic Fibrosis

  • B. Skeie
  • E. Søreide
  • O. Kirvelä
  • D. P. Katz
  • J. Askanazi
Part of the Current Topics in Rehabilitation book series (CURRENT REHAB)


Patients with cystic fibrosis (CF) often present with malnutrition and pulmonary dysfunction. Pancreatic insufficiency with critical impairment of digestive function can be demonstrated in varying degrees, together with excess energy consumption due to the increased work of breathing and inadequate oral intake. Although pulmonary function has the greatest prognostic significance, Kraemer et al.1


Cystic Fibrosis Parenteral Nutrition Essential Fatty Acid Cystic Fibrosis Patient Gamma Linolenic Acid 
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  1. 1.
    Kraemer R., Rudebery A., Kadain B., et al: Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatric. Scand. 1978; 67:33.CrossRefGoogle Scholar
  2. 2.
    Rochester D.: Malnutrition and the respiratory muscles. In: Askanazi J (Ed) Nutrition and respiratory disease. Clinics in Chest Medicine 1986; 3:91–100.Google Scholar
  3. 3.
    Sahebjami H.: Nutrition and pulmonary parenchyma. In: Askanazi J. (Ed) Nutrition and respiratory disease. Clinics in Chest Medicine 1986; 3:11–126.Google Scholar
  4. 4.
    Kuo P.T., Huang N.N., Basset D.R.: The fatty acid composition of the serum chylomicrons and adipose tissue of children with cystic fibrosis of the pancreas. J. Pediatr. 1962; 60:394–403.PubMedCrossRefGoogle Scholar
  5. 5.
    Caren R., Corbo L.: Plasma fatty acids in pancreatic cystic fibrosis and liver disease. J. Clin. Endocrinol 1966; 26:470–7.CrossRefGoogle Scholar
  6. 6.
    Rosenlund M.L., Kim H.K., Kritchevsky D.: Essential fatty acids in cystic fibrosis. Nature 1974; 251:719.Google Scholar
  7. 7.
    Skeie B., Askanazi J., Rothkopf M.M., et al.: Intravenous fat emulsions and lung function: a review. Crit. Care Med. 1988; 16:183–94.PubMedCrossRefGoogle Scholar
  8. 8.
    Craigh M.C., Faircloth S.A., Teer P.A., et al.: The essential fatty acid deficient chicken as a model for cystic fibrosis._Am. J. Clin. Nutr. 1986; 816-824.Google Scholar
  9. 9.
    Harper T.B., Chase H.P., Henson et al.: Essential fatty acid deficiency in the rabbit as a model of nutritional impairment in cystic fibrosis. Am. Rev. Respir. Dis. 1982; 126:540–7.PubMedGoogle Scholar
  10. 10.
    Elliot R.B.: A therapeutical trial of fatty acid supplementation in cystic fibrosis. Pediatrics 1976; 57:474–9.Google Scholar
  11. 11.
    Davidson G.P., Phelan P.D., Townley R.R.W.: A controlled trial using intravenous infusion of soya oil emulsion in the treatment of children with cystic fibrosis. Austr. Pediatr. J. 1978; 64:207–13.Google Scholar
  12. 12.
    Chase H.P., Cotton E.K., Elliot R.B.: Intravenous linoleic acid supplementation in children with cystic fibrosis. Pediatrics 1979; 64:207–213.PubMedGoogle Scholar
  13. 13.
    Kusoffsky E., Strandvik B., Troell S.: Prospective study of fatty acid supplementation over 3 years in patients with cystic fibrosis. J. Ped. Gastroenter. Nutr. 1983; 2:434–8.CrossRefGoogle Scholar
  14. 14.
    Shepherd R.W., Holt T.L., Thomas B.J., et al.: Nutritional rehabilitation in cystic fibrosis: controlled studies of effects on nutritional growth retardation, body protein turnover, and course of pulmonary disease. J. Pediatr. 1986; 109:788–94.PubMedCrossRefGoogle Scholar
  15. 15.
    Shepherd R., Cooksley W.G.E., Cooke W.D.D.: Improved growth and clinical, nutritional and respiratory changes in response to nutritional therapy in cystic fibrosis. J. Ped. 1980; 97:351–7.CrossRefGoogle Scholar
  16. 16.
    Bertrand J.M., Marin CL., Lasalle R., et al.: Short term clinical, nutritional and functional effects of continuous elemental, enterai alimentation in children with cystic fibrosis._J. Ped. 1984; 104:41.Google Scholar
  17. 17.
    Mansell A.L., Andersen J.C., Muttart C.R., et al.: Short-term pulmonary effects of total parenteral nutrition in children with cystic fibrosis. J. Ped. 1984; 104:700–5.CrossRefGoogle Scholar
  18. 18.
    Askanazi J., Rothkopf M., Rosenbaum S.H., et al.: Treatment of cystic fibrosis with long-term home total parenteral nutrition. Nutrition 1987; 3:277–9.Google Scholar
  19. 19.
    Skeie B., Askanazi J., Rothkopf M.M., et al.: Improved exercise tolerance with long-term parenteral nutrition in cystic fibrosis. Crit. Care Med. 1987; 15:960–2.PubMedCrossRefGoogle Scholar
  20. 20.
    Kirvelä O., Stern R.C., Askanazi J., et al.: Effects of long term parenteral nutrition in cystic fibrosis: A controlled clinical trial (In preparation).Google Scholar

Copyright information

© Springer-Verlag London 1992

Authors and Affiliations

  • B. Skeie
    • 1
  • E. Søreide
    • 1
  • O. Kirvelä
    • 1
  • D. P. Katz
    • 1
  • J. Askanazi
    • 1
  1. 1.Division of Critical Care Medicine, Department of AnesthesiologyAlbert Einstein College of Medicine, Montefiore Medical CenterNew YorkUSA

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