Parsons H G, Hill R, Pencharz P, Kuksis A
Biochim Biophys Acta. 1986 Aug 21;860(2):420-7. doi: 10.1016/0005-2736(86)90538-9.
A semi-synthetic diet (Vivonex) was administered via nasogastric tube to three cystic fibrosis patients with pancreatic exocrine deficiency for 14 days to gain weight. Dietary essential fatty acids were provided as safflower oil, which constituted 1.3% of total calories. Plasma and red blood cells were analyzed for the content and composition of lipids at the start of the diet and at days 7 and 14 of the dietary period, and the results were correlated with the morphology of the cells. Feeding Vivonex to the patients led to an essential fatty acid deficiency, which was manifested in a 50% decrease in the linoleic acid content of the phosphatidylcholine of plasma and red blood cells at days 7 and 14 and in a 20% decrease in the linoleic acid content of red cell phosphatidylethanolamine at day 14. There was no significant alteration in the levels or composition of the other phospholipid classes and in the free cholesterol/phospholipid ratio. The decrease in the linoleic acid content of the erythrocytes was accompanied by a dramatic increase in the proportion of cells as echinocytes. We conclude that restricted linoleic acid availability in cystic fibrosis patients causes a change in red blood cell shape either directly by decreasing the linoleoylphosphatidylcholine content of the membrane or indirectly by affecting enzyme activity.
通过鼻胃管给三名患有胰腺外分泌功能不全的囊性纤维化患者喂食半合成饮食(Vivonex)14天以增加体重。膳食必需脂肪酸以红花油形式提供,其占总热量的1.3%。在饮食开始时以及饮食期的第7天和第14天,分析血浆和红细胞中脂质的含量和组成,并将结果与细胞形态相关联。给患者喂食Vivonex导致必需脂肪酸缺乏,这表现为在第7天和第14天血浆和红细胞磷脂酰胆碱中亚油酸含量降低50%,以及在第14天红细胞磷脂酰乙醇胺中亚油酸含量降低20%。其他磷脂类别的水平和组成以及游离胆固醇/磷脂比率没有显著变化。红细胞中亚油酸含量的降低伴随着棘形红细胞比例的急剧增加。我们得出结论,囊性纤维化患者亚油酸可利用性受限通过直接降低膜中亚油酰磷脂酰胆碱含量或间接影响酶活性导致红细胞形状改变。