Burdge G C, Goodale A J, Hill C M, Halford P J, Lambert E J, Postle A D, Rolles C J
Department of Child Health, Southampton General Hospital.
Br J Nutr. 1994 Jun;71(6):959-64. doi: 10.1079/bjn19940199.
Impaired digestion of dietary fat is an almost universal feature of cystic fibrosis (CF) which results in low concentrations of essential fatty acids in plasma lipids. We have evaluated the effect of a high-lipid diet and pancreatic enzyme supplementation, using enteric-coated microsphere preparations, on plasma lipid concentrations in paediatric CF patients. Absorption of dietary lipid was comparable between control and CF subjects. This resulted in plasma cholesterol, triacylglycerol, total phosphatidylcholine and individual phosphatidylcholine molecular species concentrations in CF patients which were in the same range as those in controls. Normal values for these variables were also found in patients with clinically detectable liver disease. These results show that present dietary management of CF patients supports normal plasma lipid concentrations.
膳食脂肪消化功能受损是囊性纤维化（CF）几乎普遍存在的特征，这会导致血浆脂质中必需脂肪酸浓度降低。我们评估了高脂饮食和使用肠溶微球制剂补充胰酶对儿科CF患者血浆脂质浓度的影响。对照组和CF受试者之间膳食脂质的吸收情况相当。这使得CF患者的血浆胆固醇、三酰甘油、总磷脂酰胆碱和各个磷脂酰胆碱分子种类的浓度与对照组处于同一范围。在有临床可检测到的肝病患者中也发现了这些变量的正常值。这些结果表明，目前对CF患者的饮食管理可维持正常的血浆脂质浓度。
