Aslıhan DEMİR, Ayşegül ÇAKMAK, Gülden KÖKSAL

Çocukluk ve Adolesan Çağı Adrenolökodistrofisinde Beslenme Tedavisinin Etkinliği

Amaç: Bu araştırma, çocukluk ve adolesan çağı adrenolökodistrofisinde (ALD) beslenme tedavisinin hastaların plazma çok uzun zincirli yağ asitleri (VLCFA) düzeyleri üzerine etkisinin olup olmadığını saptamak amacıyla planlanmış ve yürütülmüştür. Bireyler ve Yöntem: Bu araştırma Hacettepe Üniversitesi İhsan Doğramacı Çocuk Hastanesi, Pediatrik Nöroloji Anabilim Dalı tarafından izlenen, yaşları 5-15 yıl arasında değişen, tedavi edilmekte olan ALDli 4 çocuk üzerinde 2 ay süre ile yürütülmüştür. Hastalara uygulanan diyet tedavisinde Lorenzonun yağı (enerjinin %20si olacak şekilde) ve gliserol trioleat (GTO) yağı (enerjinin %10-15i olacak şekilde) kullanılmış, C26:0 yağ asidi kısıtlı (0.05). Bireylerin çalışma öncesi ve çalışma sonrası plazma C22:0 düzeyleri arasında istatistiksel olarak anlamlı bir fark bulunmamıştır (p>0.05). Çalışma öncesi ve sonrası C26:0/ C22:0 ve C24:0/C22:0 oranları da değerlendirilmiş ve bu değerler arasında da anlamlı bir fark bulunmamıştır (p>0.05). Hastaların önerilen Lorenzonun yağı miktarlarına uyumu ile plazma çok uzun zincirli yağ asitlerindeki değişim arasındaki ilişki incelenmiş, pozitif belirgin korelasyon olmasına rağmen istatistiksel olarak anlamlı bir ilişki bulunamamıştır (p>0,05). Sonuç: Çocukluk ve adolesan çağı adrenolökodistrofide diyet tedavisinin etkin olabileceği saptanmıştır. Enerjinin %20si Lorenzonun yağından, %10-15i GTO yağı ve diyet yağından gelmek üzere,

The Efficacy of Nutrition Therapy in Childhood and Adolescence Adrenoleukodystrophy

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 İhsan Doğramacı Childrens Hospital, Department of Pediatric Neurology. In the diet therapy, Lorenzos 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 (<3 mg) was limited and banned saturated fats were eliminated. The patients were given omega-3 fatty acid supplement (2 g/day). During the research, the daily food consumption of the patients were recorded once for 2 weeks. VLCFA plasma levels of the individuals were assessed at the beginning and at the end of the research. Results: Statistically significant changes (p<0.05) were found in the amount of energy, fat, C26:0, Lorenzos oil and glycerol trioleate oil consumption of the individuals before and during the research but there was not any significant change in protein intake (p>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 Lorenzos 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 Lorenzos Oil, 10-15% coming from GTO and diet oil, could be recommended.

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