Zachwieja J, Duran M, Joles J A, Allers P J, van de Hurk D, Frankhuisen J J, Donckerwolcke R A
Institute of Paediatrics, Poznan, Poland.
Pediatr Nephrol. 1994 Dec;8(6):739-43. doi: 10.1007/BF00869107.
Plasma carnitine, amino acids and lipids levels were studied in ten uraemic children treated with haemodialysis and given amino acid supplementation with and without carnitine. As carnitine is synthesised from lysine and methionine and has a significant influence on lipid metabolism, the relationship between these was examined. Amino acid supplementation (0.25 g/kg body weight) was started with the intention of improving the plasma amino acid pattern in these children and increasing the concentration of lysine, which is the substrate for carnitine synthesis. Amino acids were administered i.v. during dialysis and carnitine (25 mg/kg body weight i.v.) was administered after dialysis three times a week. Concentrations of most essential amino acids were decreased in these patients. The first period of amino acid supplementation did not increase plasma levels of the essential amino acids, with the exception of tyrosine (P < 0.01). After the second period of supplementation, methionine was increased (P < 0.01), isoleucine was decreased (P < 0.01), but tyrosine normalised and was significantly lower than after the first period (P < 0.05). Thus overall amino acid supplementation did not improve amino acid levels; it was inconsistently associated with a further decrease in high-density lipoprotein-cholesterol and an increase in total protein levels. Lysine concentrations after amino acid supplementation remained low. Paradoxically, before carnitine supplementation a positive correlation between free carnitine and triglycerides was observed.(ABSTRACT TRUNCATED AT 250 WORDS)
对10名接受血液透析并补充或不补充肉碱的氨基酸的尿毒症儿童的血浆肉碱、氨基酸和脂质水平进行了研究。由于肉碱由赖氨酸和蛋氨酸合成且对脂质代谢有显著影响,因此研究了它们之间的关系。补充氨基酸(0.25g/kg体重)旨在改善这些儿童的血浆氨基酸模式并提高赖氨酸浓度,赖氨酸是肉碱合成的底物。氨基酸在透析期间静脉注射,肉碱(25mg/kg体重静脉注射)在透析后每周三次给药。这些患者中大多数必需氨基酸的浓度降低。第一个氨基酸补充期除酪氨酸外(P<0.01)并未提高必需氨基酸的血浆水平。第二个补充期后,蛋氨酸升高(P<0.01),异亮氨酸降低(P<0.01),但酪氨酸恢复正常且显著低于第一个补充期后(P<0.05)。因此,总体而言,补充氨基酸并未改善氨基酸水平;它与高密度脂蛋白胆固醇的进一步降低和总蛋白水平的升高不一致相关。补充氨基酸后赖氨酸浓度仍然较低。矛盾的是,在补充肉碱之前,观察到游离肉碱与甘油三酯之间呈正相关。(摘要截短于250字)