Background: Disturbances of fatty acid metabolism have been reported in PA. It is not known whether the enzymes of LCPUFA synthesis are also affected.

Subjects: Five clinically stable children with PA(age: 6.9±2.5 years, fibroblast propionyl CoA carboxylase activity: 1.0±0.3%, congnitive IQ: 78±7, mean±SD).

Interventions: Fatty acid composition of plasma phospholipids was determined by high-resolution capillary gas-liquid chromatography and the data obtained were compared to those of age-matched healthy controls (CO; n=18).

Results: Major phospholipid fatty acids [% wt/wt, median (IQR)] Table

Table 1
Full size table

Conclusion: Substrates and products of ω-6 andω-3 LCPUFA synthesis in children with PA were not indicative of disturbances of LCPUFA metabolism.