Çocukluk ve Adolesan Çağı Adrenolökodistrofisinde Beslenme Tedavisinin Etkinliği

Aim: This research has been planned and conducted in the purpose of defining whether nutrition therapy of children and adolescents with adrenoleukodystrophy (ALD) has an effect on blood very long chain fatty acid level (VLCFA) level of the patients. Subjects and Methods: This research was conducted on 4 children with adrenoleukodystrophy whose ages range between 5-15 years and who have been treated and followed for 2 months by Hacettepe University Ihsan Dogramaci Children’s Hospital, Department of Pediatric Neurology. In the diet therapy, Lorenzo’s Oil (as the 20 percent of the energy) and glycerol trioleate oil (as the 10-15 percent of the energy) were used, C26:0 fatty acid ( 0.05). Plasma C26:0 levels of the 3 individuals were decreased. No statistically significant difference (p>0.05) was seen between C26:0 levels. A decrease was also observed in plasma C24:0 levels of the 3 individuals. No statistically significant difference (p>0.05) were seen between plasma C24:0 and C22:0 levels before and after the treatment The ratios of C26:0/C22:0 and C24:0/C22:0 before and after the treatment and also no statistical significant difference (p>0.05) was seen. The correlation between the adaptation of the patients to proposed Lorenzo’s oil amount and the change in plasma very long chain fatty acids was examined. Despite the positive significant correlation, no statistical significant relationship (p>0.05) was found. Conclusion: It has been determined that diet therapy could be effective in the treatment of childhood and adolescent period adrenoleukodystrophy. A diet therapy including <3 mg/day C26:0, as the 20% of the energy coming from Lorenzo’s Oil, 10-15% coming from GTO and diet oil, could be recommended.