Dahlström K A, Ament M E, Moukarzel A, Vinton N E, Cederblad G
Department of Pediatrics, Karolinska Institute, Huddinge University Hospital, Sweden.
J Pediatr Gastroenterol Nutr. 1990 Oct;11(3):375-9. doi: 10.1097/00005176-199010000-00016.
Total and free carnitine and acylcarnitine concentrations were analyzed in whole blood and plasma in 12 children with a mean age of 68.4 +/- 42.9 months who had received carnitine-free total parenteral nutrition (TPN) for an average of 4 years. The purpose of the study was to see if the children had become carnitine deficient and, if so, whether this correlated with poor lipid clearance. Compared to controls, the TPN-dependent children had significantly decreased concentrations of total and free carnitine in blood (26.6 +/- 9.4 (SD) mumols/L vs. 43.3 +/- 9.1 mumols/L, p less than 0.001, and 17.1 +/- 7.7 mumols/L vs. 35.2 +/- 8.1 mumols/L, p less than 0.001, respectively). Similar results were found in plasma (total carnitine of 19.0 +/- 8.0 mumols/L vs. 41.9 +/- 5.2 mumols/L, p less than 0.001, and free carnitine of 15.7 +/- 7.3 mumols/L vs. 36.1 +/- 5.2 mumols/L, p less than 0.001, respectively). The acylcarnitine concentration in plasma was decreased in the TPN children (3.3 +/- 1.5 mumols/L vs. 5.8 +/- 3.0 mumols/L, p less than 0.01) compared to controls. Despite the low carnitine concentrations, serum triglyceride levels and serum free fatty acid levels were within the normal range. There was no correlation between carnitine concentrations in plasma and serum triglyceride and free fatty acid levels. Our data show that children receiving carnitine-free TPN for many years developed markedly decreased concentrations of carnitine in blood and plasma. However, no adverse effects of the low carnitine levels were found on triglyceride and free fatty acid metabolism under stable conditions.
对12名平均年龄为68.4±42.9个月的儿童进行了全血和血浆中总肉碱、游离肉碱及酰基肉碱浓度的分析，这些儿童接受无肉碱的全胃肠外营养（TPN）平均达4年。本研究的目的是观察这些儿童是否出现肉碱缺乏，以及如果出现缺乏，这是否与脂质清除不良相关。与对照组相比，依赖TPN的儿童血液中总肉碱和游离肉碱浓度显著降低（分别为26.6±9.4（标准差）μmol/L对43.3±9.1μmol/L，p<0.001；以及17.1±7.7μmol/L对35.2±8.1μmol/L，p<0.001）。血浆中也发现了类似结果（总肉碱为19.0±8.0μmol/L对41.9±5.2μmol/L，p<0.001；游离肉碱为15.7±7.3μmol/L对36.1±5.2μmol/L，p<0.001）。与对照组相比，TPN儿童血浆中的酰基肉碱浓度降低（3.3±1.5μmol/L对5.8±3.0μmol/L，p<0.01）。尽管肉碱浓度较低，但血清甘油三酯水平和血清游离脂肪酸水平仍在正常范围内。血浆中肉碱浓度与血清甘油三酯及游离脂肪酸水平之间无相关性。我们的数据表明，多年接受无肉碱TPN的儿童血液和血浆中的肉碱浓度显著降低。然而，在稳定条件下，低肉碱水平对甘油三酯和游离脂肪酸代谢未发现不良影响。
