Coulter D L
Department of Pediatrics, Boston University School of Medicine, MA.
J Child Neurol. 1991 Jan;6(1):7-14. doi: 10.1177/088307389100600102.
Carnitine is an important nutrient that is present in the diet (particularly in meat and dairy products) and is synthesized from dietary amino acids. It functions to assist long-chain fatty acid metabolism and to regulate the ratio of free coenzyme A to acylcoenzyme A in the mitochondrion. Carnitine deficiency occurs in primary inborn errors of metabolism, in nutritional deficiency, and in various other disorders including antiepileptic drug therapy. Valproate therapy is often associated with decreased carnitine levels and occasionally with true carnitine deficiency. Some experimental and clinical evidence links valproate-induced carnitine deficiency with hepatotoxicity, but this evidence is limited and inconclusive. Carnitine supplementation has been useful in some studies, but these data are also limited. Young children with neurologic disabilities taking multiple antiepileptic drugs may have the greatest risk for carnitine deficiency. Measurement of carnitine levels appears warranted in these patients and in patients with symptoms and signs of possible carnitine deficiency.
肉碱是一种重要的营养素，存在于饮食中（特别是肉类和奶制品），并由膳食氨基酸合成。它的作用是协助长链脂肪酸代谢，并调节线粒体中游离辅酶A与酰基辅酶A的比例。肉碱缺乏发生在原发性先天性代谢缺陷、营养缺乏以及包括抗癫痫药物治疗在内的各种其他疾病中。丙戊酸盐治疗常与肉碱水平降低有关，偶尔也会导致真正的肉碱缺乏。一些实验和临床证据将丙戊酸盐诱导的肉碱缺乏与肝毒性联系起来，但这些证据有限且尚无定论。在一些研究中，补充肉碱是有用的，但这些数据也很有限。服用多种抗癫痫药物的神经残疾幼儿可能患肉碱缺乏症的风险最大。对于这些患者以及有肉碱缺乏可能症状和体征的患者，似乎有必要检测肉碱水平。
