Heredopathia atactica polyneuritiformis (Refsum's disease). 1. Clinical features and dietary management | Heredopathia atactica polyneuritiformis (Refsum's disease). 1. Clinical features and dietary management. [phytanic acid]
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 a defective enzyme in fatty acid oxidation, has been successfully treated by diet and plasma exchange. In this disease, phytanic acid can no longer be metabolized and its serum concentration rises to dangerous levels. Diet therapy involves feeding phytanic acid-free foods, so that dietary phytanic acid is restricted to a maximum of 10 mg daily. The diet also includes sufficient amounts of linoleic acid and adequate energy and protein to maintain body weight. Foods with high phytanic acid content are not allowed; these include whole milk, beef, lamb, corn, many fruits, all green vegetables, and most fats and oils. Other than forbidden foods, patients are able to eat a varied diet of solid foods which enable them to lead normal lives while managing their disease.
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