title: Heredopathia atactica polyneuritiformis (Refsum's disease): 1. Clinical features and dietary management.
abstract: In a patient with Refsum's disease successful dietary control of the disease has been shown to depend on adequate energy and protein intake from phytanic acid-free sources and restriction of dietary phytanic acid to a maximum of 10 mg per day, with the provision of a generous amount of the essential fatty acid, linoleic acid. The present work gives data on the phytanic-acid content of various foodstuffs and suggests dietary manipulation of the patient. Although the role that chlorophyll-bound phytol plays in Refsum's disease is uncertain, it is advisable that this is eliminated until its role is more clearly identified.
mesh_terms: D000273:Adipose Tissue / Q000032:analysis; D000328:Adult; D004032:Diet; D004044:Dietary Proteins / Q000008:administration & dosage; D006801:Humans; D008041:Linoleic Acids; D008297:Male; D010831:Phytanic Acid / Q000008:administration & dosage / Q000097:blood; D012035:Refsum Disease / Q000097:blood / Q000178:diet therapy*; D012174:Retinitis Pigmentosa / Q000209:etiology
chemical_list: D004044:Dietary Proteins; D008041:Linoleic Acids; D010831:Phytanic Acid