Çocukluk ve Adölesan Çağı Adrenolökodistrofisinde Beslenme Tedavisinin Etkinliğinin Araştırılması
Özet
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.
