Çocukluk ve Adölesan Çağı Adrenolökodistrofisinde Beslenme Tedavisinin Etkinliğinin Araştırılması

Abstract

This research was carried out on 5 children with Adrenoleukodystrophy whose ages range between 5-18 and who have been treated and examined by Hacettepe University İhsan Doğramacı Children’s Hospital, Department of Pediatric Neurology and Şişli Etfal Training and Research Hospital, Department of Pediatrics. The daily energy and protein needs of the patients were calculated. In the diet theraphy, Lorenzo’s Oil (as the 20 percent of the energy) and glycerol trioleate oil (as the 10-15 percent of the energy) were added, a diet which limited C26:0 fatty acid (<3 mg) and banned saturated fats was arranged. The patients were given omega-3 fatty acid support as essantial fatty acid. The all children and their parents under the comprehension of the research were provided with specific nutrition education. It was seen that there were statistical significant changes (p<0.05) in the amount of energy, fat, C26:0, Lorenzo’s oil and glycerol trioleate oil consumption of the individuals before and during the research but there were not any significant changes in protein intake (p>0.05). The level of plasma very long chain fatty acid of the four patient who are monitoring in Hacettepe University and one patient who is monitoring in Şişli Etfal Training and Research Hospital was examined before and during the research seperately. For 4 patients before the research, the values C26:0, C24:0, C22:0, C26:0/C22:0, C24:0/C22:0 can be shown consecutively as 2,96 ± 1,16 μmol/L, 39,47 ± 14,51 μmol/L, 29,22 ± 8,31 μmol/L, 0,10 ± 0,01 1,33 ± 0,13. After the research they were seen as 2,06 ± 0,91 μmol/L, 38,49 ± 12,73 μmol/L, 28,79 ± 6,82 μmol/L, 0,68 ± 0,28, 1,31 ± 0,19. For one patient, before the research, the values C26:0, C24:0, C22:0, C26:0/C22:0, C24:0/C22:0 can be shown consecutively as 1,15 μmol/L, 38,40 μmol/L, 23,50 μmol/L, 0,05 and 1,63. After the research they were seen as 20,62 μmol/L, 23,40 μmol/L, 13,30 μmol/L, 0,05 and 1,76. Plasma C26:0 level of the 4 out of 5 individuals in the research decreased. No statistical significant difference (p>0.05) was seen between the first and last levels of C26:0. A decrease was also observed in plasma C24:0 level of the 4 individuals. No statistical significant difference (p>0.05) was seen between the first and last levels of C24:0. No statistical significant difference was seen in plasma C22:0 level before the research and the level after the research of the individuals. The proportions of C26:0/C22:0 and C24:0/C22:0 before and after the research were assessed and also no statistical significant difference (p>0.05) was encountered. The correlation between the adaptation to Lorenzo’s oil and the change in plasma very long chain fatty acids was examined. Despite the positive significant correlation, no statistical significant relation (p>0.05) was found. According to the findings of this study, nutrition therapy (Lorenzo’s oil (as the 20 percent of the energy) and glycerol trioleate oil (as the 10-15 percent of the energy), a diet which limited C26:0 fatty acid (<3 mg)) should be recommended.