Essential fatty acids in clinically stable children with propionic acidaemia

Journal of Inherited Metabolic Disease
T DecsiB Koletzko

Abstract

Disturbances of fatty acid metabolism with accumulation of odd-chain fatty acids have been reported in propionic acidaemia (PA). It is not known whether the synthesis of long-chain polyunsaturated fatty acids (LCPUFA) is also affected. In five clinically stable children with PA (median age 8 years, range 3.5-9.5 years; median percentage fibroblast propionyl-CoA carboxylase activity 0.8, range 0.8-1.5), we determined the fatty acid composition of plasma phospholipids, triglycerides and sterol esters and compared the results with those of 18 age-matched healthy controls. Odd-numbered fatty acids were found in all samples of PA patients but in controls median values were zero. Percentage contributions of substrate (linoleic acid, C18:2 omega-6) and principal product (arachidonic acid, C20:4 omega-6) of omega-6 LCPUFA synthesis did not differ between patients and controls. Similarly, there were no differences between both groups in the substrate (alpha-linolenic acid, C18:3 omega-3) and principal product (docosahexaenoic acid, C22:6 omega-3) of omega-3 LCPUFA formation. We conclude that disturbances of fatty acid metabolism in clinically stable children with PA do not affect LCPUFA synthesis.

References

Apr 1, 1986·European Journal of Pediatrics·B KoletzkoH J Bremer
Jan 30, 1985·Clinica Chimica Acta; International Journal of Clinical Chemistry·T SuormalaE R Baumgartner
Apr 1, 1994·Acta Paediatrica. Supplement·T Decsi, B Koletzko
Jul 1, 1996·European Journal of Pediatrics·M GiovanniniE Riva

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Citations

May 25, 2012·The British Journal of Nutrition·Mercedes Gil-Campos, Pablo Sanjurjo Crespo
Apr 8, 2006·Journal of Inherited Metabolic Disease·L Aldámiz-EchevarríaJ Rodríguez-Soriano
Sep 1, 2010·Nutrients·Katalin Fekete, Tamás Decsi

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