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通过皮下微透析监测生酮饮食中肉碱代谢产物

Monitoring of ketogenic diet for carnitine metabolites by subcutaneous microdialysis.

作者信息

Hack Alexandra, Busch Verena, Pascher Bettina, Busch Raymonde, Bieger Iris, Gempel Klaus, Baumeister Friedrich A M

机构信息

Department of Pediatric Neurology, Children's Hospital, Technical University Munich, Germany.

出版信息

Pediatr Res. 2006 Jul;60(1):93-6. doi: 10.1203/01.pdr.0000219479.95410.79. Epub 2006 May 11.

Abstract

The ketogenic diet (KD) provides ketones from the degradation of free fatty acids for energy metabolism. It is a therapeutic option for pharmacoresistant epilepsies. Carnitine is the carrier molecule that transports fatty acids across the mitochondrial membrane for degradation into ketones. The integrity of this transport system is a prerequisite for an adequate ketogenic response. For monitoring of tissue metabolism with KD, we used the sampling method of s.c. microdialysis (MD), which permits minimally invasive, frequent, and extensive metabolic monitoring independent of blood tests. By using this new method, we monitored changes in carnitine metabolism induced by KD, particularly in free carnitine (C0), acetylcarnitine (C2), and hydroxybutyrylcarnitine (C4OH). Correlation of microdialysate and tissue concentrations for carnitines in vitro was about 85%. Carnitine metabolism was monitored in seven children started on a KD for pharmacoresistant epilepsy after a conventional initial fasting period. Detected metabolic changes consisted of a slight decrease in s.c. C0 and a marked increase in C2/CO and C4OH/CO levels. The levels of s.c. C4OH strongly correlate with beta-hydroxybutyrate (beta-OHB) levels in plasma providing an additional parameter for the carnitine reserve of the body and reflect an optimal ketogenic energy supply. Subcutaneous MD allows close and extensive monitoring of metabolism with a KD.

摘要

生酮饮食(KD)通过游离脂肪酸的降解提供酮类用于能量代谢。它是药物难治性癫痫的一种治疗选择。肉碱是一种载体分子,可将脂肪酸转运穿过线粒体膜以降解为酮类。这种转运系统的完整性是产生足够生酮反应的先决条件。为了用KD监测组织代谢,我们采用了皮下微透析(MD)采样方法,该方法允许进行微创、频繁且广泛的代谢监测,而无需依赖血液检测。通过使用这种新方法,我们监测了KD诱导的肉碱代谢变化,特别是游离肉碱(C0)、乙酰肉碱(C2)和羟基丁酰肉碱(C4OH)的变化。体外微透析液和组织中肉碱浓度的相关性约为85%。在7名因药物难治性癫痫开始采用KD治疗的儿童中,经过常规的初始禁食期后监测了肉碱代谢。检测到的代谢变化包括皮下C0略有下降以及C2/C0和C4OH/C0水平显著升高。皮下C4OH水平与血浆中β-羟基丁酸(β-OHB)水平密切相关,为身体的肉碱储备提供了一个额外参数,并反映了最佳的生酮能量供应。皮下MD允许对KD的代谢进行密切且广泛的监测。

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