Melegh B, Kerner J, Bieber L L
Pediatric Clinic, University Medical School, Pecs, Hungary.
Biochem Pharmacol. 1987 Oct 15;36(20):3405-9. doi: 10.1016/0006-2952(87)90318-2.
Pivampicillin treatment of seven children (five boys and two girls) for 7 days significantly reduced the amounts of total acid-soluble carnitine, free carnitine, and long-chain acylcarnitines and increased the amounts of acid-soluble acylcarnitine in plasma. The fasting plasma levels of 3-hydroxybutyrate at the end of treatment were 15% of the control value. The levels of free fatty acids were decreased, whereas triglyceride levels were unaffected, indicating impaired fat metabolism. Daily urinary excretion of total carnitine was four to five times higher than controls after the first day of treatment, although the amounts of free carnitine and acetylcarnitine were decreased. The urinary acylcarnitines were isolated and characterized by gas chromatography/electron impact mass spectrometry and fast-atom bombardment mass spectrometry. Pivaloylcarnitine was the predominant urinary acylcarnitine; it represented greater than 96% of the increased excretion of total carnitine and 75-80% of the total conjugated pivalic acid. The renal clearance of acylcarnitines was comparable to that of creatinine, indicating no reabsorption of pivaloylcarnitine. These data suggest a detoxification function of carnitine for pivalic acid in humans.
用匹氨西林治疗7名儿童（5名男孩和2名女孩）7天，可显著降低血浆中总酸溶性肉碱、游离肉碱和长链酰基肉碱的含量，并增加酸溶性酰基肉碱的含量。治疗结束时，空腹血浆3-羟基丁酸水平为对照值的15%。游离脂肪酸水平降低，而甘油三酯水平未受影响，表明脂肪代谢受损。治疗第一天后，每日尿总肉碱排泄量比对照组高4至5倍，尽管游离肉碱和乙酰肉碱的量有所减少。通过气相色谱/电子轰击质谱和快原子轰击质谱对尿酰基肉碱进行分离和表征。新戊酰肉碱是主要的尿酰基肉碱；它占总肉碱排泄量增加的96%以上，占总共轭新戊酸的75-80%。酰基肉碱的肾清除率与肌酐相当，表明新戊酰肉碱无重吸收。这些数据表明肉碱对人体新戊酸具有解毒功能。
