Heredopathia atactica polyneuritiformis (Refsum's disease). 2. Estimation of phytanic acid in foods
1980
Masters-Thomas, Anne | Bailes, Janet | Billimoria, J.D. | Clemens, M.E. | Gibberd, F.B. | Page, N.G.R.
Refsum's disease, a metabolic disorder characterized by an enzyme defect in fatty acid oxidation, is treatable by restricting intake of foods with high phytanic acid content. Dietary phytanic acid is kept to a maximum of 10 mg per day. The phytanic acid concentration in patients' plasma and in allowed foods must be carefully monitored. Phytanic acid in plasma can be readily quantitated by gas liquid chromatography. Estimation of the phytanic acid content of foodstuffs is achieved using gas liquid chromatography of food lipid extracts on an APL column. The phytanic acid content of a wide range of commonly eaten foods has been analyzed, generating lists of permitted vs. forbidden foods which can be used in dietary management of the disease.
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