Dietary essential amino acids are highly anabolic in pediatric patients with cystic fibrosis.
Dietary essential amino acids are highly anabolic in pediatric patients with cystic fibrosis.
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囊性纤维化儿科患者的膳食必需氨基酸具有高度合成代谢作用。
DOI：
10.1016/j.jcf.2012.12.011
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Deutz,NicolaasEP
中科院分区：
文献类型：
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作者：
Engelen,MariëllePKJ;Com,Gulnur;Wolfe,RobertR;Deutz,NicolaasEP
目前的营养治疗方法在囊性纤维化（CF）中已经部分成功。具有高亮氨酸水平（EAA）的必需氨基酸混合物在分解代谢条件下具有合成代谢特性，但在CF中缺乏数据。方法根据随机交叉设计，15例小儿CF患者在两天内摄入6.7 g EAA和乳清中存在的总氨基酸混合物。采用稳定同位素方法评价了全体蛋白质和精氨酸代谢（EAA缺乏精氨酸）。结果EAA后蛋白质合成高于对照组（P< 0.05），蛋白质分解高于对照组（P< 0.001），净合成代谢高于对照组（70%）。EAA后精氨酸周转率降低（P< 0.001）， de novo Arginine synthesis有降低趋势（P= 0.09）。一氧化氮的合成没有什么不同。结论scf患者对EAA摄入的反应与营养状况无关。在CF中，在EAA混合物中添加精氨酸是有必要的。
BackgroundCurrent nutritional approaches have been partially successful in Cystic Fibrosis (CF). Essential amino acids mixtures with high Leucine levels (EAA) have anabolic properties in catabolic conditions, however data in CF are lacking.MethodsOn two days according a randomized crossover design, 15 pediatric CF patients ingested 6.7 g EAA versus mixture of total amino acids as present in whey. Whole body protein and Arginine metabolism (as EAA lack Arginine) were assessed by stable isotope methodology.ResultsProtein synthesis (P< 0.05) but not protein breakdown was higher after EAA and 70% higher values for net anabolism (P< 0.001)were found both in patients with and without nutritional failure. Arginine turnover was lower (P< 0.001) and de novo Arginine synthesis tended lower (P= 0.09) after EAA. Nitric oxide synthesis was not different.ConclusionsCF patients are highly responsive to EAA intake independent of their nutritional status. Addition of Arginine to the EAA mixture may be warranted in CF.