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.

• Authors: A Masters-Thomas, J Bailes, J D Billimoria, M E Clemens, F B Gibberd, N G Page
• Journal: Journal of human nutrition (J Hum Nutr) Vol. 34 Issue 4 Pg. 245-50 (Aug 1980) ISSN: 0308-4329 [Print] England
• PMID: 6157716 (Publication Type: Case Reports, Journal Article)

Chemical References

• Dietary Proteins
• Linoleic Acids
• Phytanic Acid

Topics

• Adipose Tissue (analysis)
• Adult
• Diet
• Dietary Proteins (administration & dosage)
• Humans
• Linoleic Acids
• Male
• Phytanic Acid (administration & dosage, blood)
• Refsum Disease (blood, diet therapy)
• Retinitis Pigmentosa (etiology)