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.
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