Dietary treatment of adrenoleukodystrophy.
We treated two patients with adrenoleukodystrophy (ALD) with a diet that contained 0.636% lipids as soybean oil, and less than 0.01% of the total fatty acids were very-long-chain fatty acids (VLCFA). Although clinical improvement was not evident in either patient, the VLCFA levels of erythrocyte membranes and plasma decreased in patient 1; in particular, the C25:0 in erythrocyte membranes became normal. Therefore, the abnormal accumulation of VLCFA in ALD arises partially from the diet. The VLCFA-free diet should be beneficial in ALD.
D000326:Adrenoleukodystrophy / Q000097:blood / Q000178:diet therapy* / Q000503:physiopathology; D000328:Adult; D002648:Child; D004041:Dietary Fats / Q000008:administration & dosage; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000178:diet therapy*; D004910:Erythrocyte Membrane / Q000378:metabolism; D005227:Fatty Acids / Q000097:blood / Q000145:classification / Q000378:metabolism; D005526:Food, Formulated; D006801:Humans; D008297:Male; D009420:Nervous System / Q000503:physiopathology
D004041:Dietary Fats; D005227:Fatty Acids