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Plasma lipid concentrations in children with cystic fibrosis: the value of a high-fat diet and pancreatic supplementation

Published online by Cambridge University Press:  10 October 2007

Graham C. Burdge ,
Alissa J. Goodale ,
Catherine M. Hill ,
Penny J. Halford ,
Elizabeth J. Lambert ,
Anthony D. Postle  and
Christopher J. Rolles
Graham C. Burdge
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
Alissa J. Goodale
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
Catherine M. Hill
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
Penny J. Halford
Affiliation:
University of Southampton, Bassett Crescent East, Southampton SO9 3TU
Elizabeth J. Lambert
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
Anthony D. Postle
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
Christopher J. Rolles
Affiliation:
Departments of Child Health. Southampton General Hospital, Tremona Road, Southampton SO9 4XY and Human Nutrition
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Abstract

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Impaired digestion of dietary fat is an almost universal feature of cystic fibrosis (CF) which results in low concentrations of essential fatty acids in plasma lipids. We have evaluated the effect of a high-lipid diet and pancreatic enzyme supplementation, using enteric-coated microsphere preparations, on plasma lipid concentrations in paediatric CF patients. Absorption of dietary lipid was comparable between control and CF subjects. This resulted in plasma cholesterol, triacylglycerol, total phosphatidylcholine and individual phosphatidylcholine molecular species concentrations in CF patients which were in the same range as those in controls. Normal values for these variables were also found in patients with clinically detectable liver disease. These results show that present dietary management of CF patients supports normal plasma lipid concentrations.

Keywords

Cystic fibrosisPlasma lipidsPlasma phospholipidsChildhood
Type
High Diets in Cystic Fibrosis
Information
British Journal of Nutrition , Volume 71 , Issue 6 , June 1994 , pp. 959 - 964
Copyright
Copyright © The Nutrition Society 1994

References

Bligh, E. G. & Dyer, W. S. (1959). A rapid method of total lipid extraction and purification. Canadian Journal of Biochemistry 31, 911–923.Google Scholar
Caesar, P. A., Wilson, S. J., Normand, I. C. S. & Postle, A. D. (1988). A comparison of the specificity of phosphatidylcholine synthesis by human fetal lung maintained in either organ or organotypic culture. Biochemical Journal 253, 451–457.Google ScholarPubMed
Exton, J. H. (1990). Signalling through phosphatidylcholine breakdown. Journal of Biological Chemistry 265, 1–4.Google Scholar
Farrell, P. M., Mischler, E. H., Engle, M. J., Brown, D. J. & Lau, S.-M. (1985). Fatty acid abnormalities in cystic fibrosis. Pediatric Research 19, 104–109.Google Scholar
Hubbard, V. S., Dunn, G. D. & Di Sant' Agnese, P. A. (1977). Abnormal fatty acid composition of plasma lipids in cystic fibrosis. Lancet ii, 1302–1304.CrossRefGoogle Scholar
Kang, J. X., Man, S. F. P., Brown, N. E., Labrecque, P. A. & Clandinin, M. T. (1992). The chloride channel blocker anthracene 9-carboxylate inhibits fatty acid incorporation into phospholipid in cultured human airway epithelial cells. Biochemical Journal 285, 725–729.CrossRefGoogle ScholarPubMed
Kuo, P. T., Huang, N. N. & Bassett, D. R. (1962). The fatty acid composition of the serum chylomicrons and adipose tissue of children with cystic fibrosis of the pancreas. Journal of Pediatrics 60, 394–403.CrossRefGoogle ScholarPubMed
Nagel, R. A., Westaby, D., Javaid, A., Kavani, J., Meire, H. B., Lombard, M. G., Wise, A., Williams, R. & Hodson, M. E. (1989). Liver disease and bile duct abnormalities in adults with cystic fibrosis. Lancet ii, 1422–1424.CrossRefGoogle Scholar
Postle, A. D. (1987). Method for the analysis of individual molecular species of phosphatidylcholine by high- performance liquid chromatography with post-column fluorescence detection. Journal of Chromatography 415, 41–51.Google Scholar
Rosenlund, M. L., Kim, H. K. & Kritchevsky, D. (1974). Essential fatty acids in cystic fibrosis. Nature 251, 719.Google Scholar
Scott-Jupp, R., Lama, M. & Tanner, M. S. (1991). Prevalence of liver disease in cystic fibrosis. Archives of Disease in Childhood 66, 698–701.CrossRefGoogle ScholarPubMed
Stern, R. C., Stevens, D. P., Boat, T. F., Doershuk, C. F., Izant, R. J. & Matthews, L. D. (1976). Symptomatic hepatic disease in cystic fibrosis: incidence, course and outcome of portal systemic shunting. Gastroenterology 70, 645–649.CrossRefGoogle Scholar
Vaughan, W. J., Lindgren, F. T., Whalen, J. B. & Abraham, S. (1978). Serum lipoprotein concentrations in cystic fibrosis. Science 199, 783–785.CrossRefGoogle ScholarPubMed