Enomoto T M, Isichei C, VanderJagt D J, Fry D E, Glew R H
Department of Biochemistry, School of Medicine, University of New Mexico, Albuquerque 87131, USA.
J Trop Pediatr. 1998 Feb;44(1):28-34. doi: 10.1093/tropej/44.1.28.
The purpose of this study was to determine if the growth retardation often associated with sickle cell anaemia could be related in part to a deficiency of essential fatty acids. We reported recently that children with sickle cell disease in Jos, Nigeria have lower levels of serum amino acids and higher levels of urinary amino acids than their healthy counterparts. In the current study, we determined that the serum phospholipids of children with sickle cell anaemia did not deviate in the proportions of the essential fatty acids, linoleic and alpha-linolenic they contain compared to controls. However, their serum phospholipid profiles were significantly different in the proportions of four other fatty acids. Specifically, the phospholipids of children with sickle cell anaemia contained 19 per cent more palmitic acid (P = 0.006), 22 per cent more oleic acid (P = 0.014), 18 per cent less arachidonic acid (P = 0.008), 51 per cent less eicosapentaenoic acid (P = 0.0008), and 43 per cent less decosahexaenoic acid (P = 0.001). These data show that children with sickle cell anaemia are not deficient in essential fatty acids, but that the fatty acid elongation and desaturation pathway is somehow disturbed in this disease.
本研究的目的是确定镰状细胞贫血常伴有的生长发育迟缓是否部分与必需脂肪酸缺乏有关。我们最近报道，尼日利亚乔斯患有镰状细胞病的儿童与健康儿童相比，血清氨基酸水平较低，尿氨基酸水平较高。在当前研究中，我们确定镰状细胞贫血儿童血清磷脂中所含必需脂肪酸亚油酸和α-亚麻酸的比例与对照组相比没有偏差。然而，他们血清磷脂谱中其他四种脂肪酸的比例有显著差异。具体而言，镰状细胞贫血儿童的磷脂中棕榈酸含量多19%（P = 0.006），油酸含量多22%（P = 0.014），花生四烯酸含量少18%（P = 0.008），二十碳五烯酸含量少51%（P = 0.0008），二十二碳六烯酸含量少43%（P = 0.001）。这些数据表明，镰状细胞贫血儿童不存在必需脂肪酸缺乏，但这种疾病中脂肪酸延长和去饱和途径受到了某种程度的干扰。
