Bonner C M, DeBrie K L, Hug G, Landrigan E, Taylor B J
Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock.
J Pediatr. 1995 Feb;126(2):287-92. doi: 10.1016/s0022-3476(95)70562-7.
The effects of parenteral L-carnitine supplementation on fat metabolism, nutrient intake, and plasma and erythrocyte carnitine concentrations were studied in 43 very low birth weight infants. Infants were randomly assigned to control or carnitine-supplemented (50 mumol/kg per day) groups within two weight categories: group 1, 750 to 1000 gm, and group 2, 1001 to 1500 gm. Plasma total, free, and acyl carnitine levels, erythrocyte carnitine levels, serum beta-hydroxybutyrate and triglyceride levels, and total fat intake were monitored weekly until 50% of total caloric intake was met enterally. Neonates receiving carnitine had higher plasma carnitine levels than control groups (total carnitine: group 1, 75.2 +/- 22.9 vs 9.6 +/- 2.7 mmol/ml; group 2, 61.6 +/- 31.2 vs 13.0 +/- 9.2 nmol/ml). Levels of beta-OH-butyrate decreased from baseline in control neonates (group 1, 0.12 +/- 0.06 to 0.03 +/- 0.02 mmol/L; group 2, 0.11 +/- 0.03 to 0.05 +/- 0.02 mmol/L); they remained unchanged in supplemented groups. Thus ketogenesis appeared less impaired in infants receiving supplements. Supplemented group 2 tolerated more fat than control group 2; triglyceride levels remained acceptable in all groups. Carnitine group 2 had greater weight gain than control group 2 during the first 2 weeks of life. We conclude that very low birth weight infants requiring prolonged parenteral nutrition have carnitine deficiency with impaired ketogenesis. Parenteral administration of carnitine appears to alleviate this metabolic disturbance.
在43名极低出生体重儿中研究了肠外补充左旋肉碱对脂肪代谢、营养摄入以及血浆和红细胞肉碱浓度的影响。婴儿按体重分为两类，随机分为对照组或补充肉碱组（每天50μmol/kg）：第1组，体重750至1000克；第2组，体重1001至1500克。每周监测血浆总肉碱、游离肉碱和酰基肉碱水平、红细胞肉碱水平、血清β-羟基丁酸和甘油三酯水平以及总脂肪摄入量，直至经肠道摄入的热量达到总热量摄入的50%。接受肉碱补充的新生儿血浆肉碱水平高于对照组（总肉碱：第1组，75.2±22.9 vs 9.6±2.7mmol/ml；第2组，61.6±31.2 vs 13.0±9.2nmol/ml）。对照组新生儿的β-羟基丁酸水平从基线下降（第1组，0.12±0.06至0.03±0.02mmol/L；第2组，0.11±0.03至0.05±0.02mmol/L）；补充组中该水平保持不变。因此，补充肉碱的婴儿生酮作用受损程度似乎较小。补充组2比对照组2能耐受更多脂肪；所有组的甘油三酯水平均保持在可接受范围内。肉碱组2在出生后的前2周体重增加比对照组2更多。我们得出结论，需要长期肠外营养的极低出生体重儿存在肉碱缺乏并伴有生酮作用受损。肠外给予肉碱似乎可缓解这种代谢紊乱。
