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口服L-肉碱补充剂对以母乳为食的低出生体重早产儿的影响。

Effects of oral L-carnitine supplementation in low-birth-weight premature infants maintained on human milk.

作者信息

Melegh B, Kerner J, Sándor A, Vincellér M, Kispál G

出版信息

Biol Neonate. 1987;51(4):185-93. doi: 10.1159/000242650.

DOI:10.1159/000242650
PMID:3580423
Abstract

Effects of oral L-carnitine supplementation on fat and protein metabolism have been studied in 20 low-birth-weight premature infants (mean weight at birth 1.519 g, range 1,200-1,880 g) fed with pooled pasteurized human milk. Throughout 7 consecutive days, started at various postnatal ages (range 10-33 days) infants were fed exclusively with milk containing 300 nmol/ml L-carnitine as added supplement. The amount of extra carnitine intake ranged from 42.6 to 72.0 mumol/kg/day. Until day 5 of supplementation there was a continuous increase in the daily urinary excretion of total carnitine, which levelled off thereafter, corresponding approximately to 50% of the extra L-carnitine intake, indicating that a part of the supplement was retained by the body. Total, free and esterified carnitine levels were significantly elevated in the plasma at the end of the study period. The increased levels of acylcarnitines in plasma and urine indicate that the carnitine supplement was taken up by tissues and entered the intermediary metabolism. Plasma triglyceride level was decreased, whereas 3-hydroxybutyrate level was increased at the end of supplementation, indicating an enhanced fat utilization. Plasma and urine analysis also revealed an altered nitrogen handling. There was a marked decrease in plasma urea level as well as a significant fall in the urea and total N excretion, with a trend of decrease in excretion of 3-methylhistidine, suggesting a reduced amino acid and protein catabolism during L-carnitine supplementation.

摘要

在20名低出生体重早产儿(出生时平均体重1519克,范围1200 - 1880克)中,研究了口服补充L - 肉碱对脂肪和蛋白质代谢的影响,这些婴儿喂养的是混合巴氏消毒人乳。在出生后不同年龄(范围10 - 33天)开始,连续7天,婴儿仅喂食添加了300 nmol/ml L - 肉碱的牛奶。额外摄入肉碱的量为42.6至72.0 μmol/kg/天。在补充的第5天之前,总肉碱的每日尿排泄量持续增加,此后趋于平稳,约相当于额外L - 肉碱摄入量的50%,这表明一部分补充剂被身体保留。在研究期末,血浆中总肉碱、游离肉碱和酯化肉碱水平显著升高。血浆和尿液中酰基肉碱水平的升高表明肉碱补充剂被组织摄取并进入中间代谢。补充结束时,血浆甘油三酯水平降低,而3 - 羟基丁酸水平升高,表明脂肪利用率提高。血浆和尿液分析还显示氮代谢发生改变。血浆尿素水平显著下降,尿素和总氮排泄量也显著降低,3 - 甲基组氨酸排泄量有下降趋势,表明补充L - 肉碱期间氨基酸和蛋白质分解代谢减少。

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Curr Mol Med. 2020;20(5):336-354. doi: 10.2174/1566524019666191113120828.
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Identification of SLC22A5 Gene Mutation in a Family with Carnitine Uptake Defect.一个患有肉碱摄取缺陷的家族中SLC22A5基因突变的鉴定。
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Acylcarnitine esters profiling of serum and follicular fluid in patients undergoing in vitro fertilization.
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Randomised controlled trial of L-carnitine as a nutritional supplement in preterm infants.左旋肉碱作为早产儿营养补充剂的随机对照试验。
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