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Biomedical Research Unit (Dr. Martinez), Maternity-Children's Hospital, Valle de Hebron; the Departments of Neuropediatrics (Dr. Pineda) and Electrophysiology (Dr. Conill), Hospital San Juan de Dios; and the Neuropediatrics Unit (Dr. Vidal) and Gastroenterology Unit (Dr. Martin), Hospital de Tarrasa, Barcelona, Spain.
Docosahexaenoic acid (DHA, 22:6
3) is a major constituent of brain membrane phospholipids and photoreceptor cells. Patients with generalized peroxisomal disorders have extremely low levels of DHA in the brain and other tissues. Since a DHA deficiency could explain some basic symptoms in peroxisomal-disorder patients, we tested the possible beneficial effects of DHA in two patients with neonatal adrenoleukodystrophy (NALD). Before the treatment, both patients had very low DHA levels in plasma and erythrocytes. We first gave DHA in the form of fish oil and, in both patients, the rapid increase in red-cell DHA levels indicated that this fatty acid was being absorbed and incorporated into membrane phospholipids very fast. However, a low ratio 22:63/22:53 was still present in erythrocyte membranes, and the content of 20:53 (eicosapentaenoic acid) was too high with the fish oil diet. We then began treatment with pure DHA ethyl ester and, after a few weeks, erythrocyte 3 polyunsaturated fatty acids were normal. There was an increase in the 18:0 molecular species of plasmalogens in both patients, most significantly in the child with affected plasmalogen biosynthesis in cultured fibroblasts. In the less severely affected NALD patient, treatment with DHA produced a very significant decrease in the ratios 24:1/22:0 and 26:1/22:0, and this child improved neurologically. The present data suggest that DHA deficiency may be the cause for some of the most characteristic abnormalities in peroxisomal-disorder patients and open new therapeutic possibilities for these patients.
Docosahexaenoic acid ethyl ester was provided by Charleston Laboratory, through a grant from the Fish Oil Test Materials Program (NIH/ADAMHA/DOC).
Address correspondence and reprint requests to Dr. Manuela Martinez, Gran Via 750, 6-1, 08013 Barcelona, Spain.
Received August 3, 1992. Accepted for publication in final form November 4, 1992.
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 M. Martinez, E. Vazquez, M T. Garcia-Silva, J. Manzanares, J. M Bertran, F. Castello, and I. Mougan Therapeutic effects of docosahexaenoic acid ethyl ester in patients with generalized peroxisomal disorders1 Am. J. Clinical Nutrition, January 1, 2000; 71(1): 376S - 385S. [Abstract] [Full Text] [PDF]
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 D. P. Gaposchkin and R. A. Zoeller Plasmalogen status influences docosahexaenoic acid levels in a macrophage cell line: insights using ether lipid-deficient variants J. Lipid Res., March 1, 1999; 40(3): 495 - 503. [Abstract] [Full Text]
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