Bitman J, Hamosh M, Wood D L, Freed L M, Hamosh P
Milk Secretion and Mastitis Laboratory, US Department of Agriculture, Beltsville, Maryland 20705.
Pediatrics. 1987 Dec;80(6):927-32.
Milk lipids from six mothers with cystic fibrosis were compared with milk lipids from six mothers without cystic fibrosis. Mean neutral lipids (in g/dL) were colostrum, 2.4; transitional, 2.9; mature milk, 2.9, suggesting that milk fat content was sufficient to support energy needs of the infant. Lipid class composition was normal, 98% being present as triglyceride. Cholesterol and cholesteryl ester concentrations were similar in the milk of both groups of lactating women. Phospholipid class distribution of cystic fibrosis milk was different from that in control milk. Approximately 85% of the lipids were present in six major fatty acids from 12:0 to 18:2 in both groups, indicating that the profile of most of the lipid was similar. The linoleic acid content of cystic fibrosis milk was decreased significantly to 75% of normal values. Other polyunsaturated fatty acids were elevated in cystic fibrosis milk. Concentrations of 16:2, 18:3, and several longer chain polyunsaturated fatty acids were twice that in the milk of the control women, suggesting increased delta 6-desaturation. Elevated proportions of several other polyunsaturated fatty acid metabolites suggested increased chain elongation to C20 and C22 polyunsaturated fatty acids. These differences in fatty acid patterns in cystic fibrosis milk were similar to differences found in blood lipid patterns of children with cystic fibrosis. These results suggest abnormal polyunsaturated fatty acid nutrition or metabolism in cystic fibrosis.
将六位患有囊性纤维化的母亲的乳汁脂质与六位未患囊性纤维化的母亲的乳汁脂质进行了比较。中性脂质的平均含量（以克/分升计）分别为：初乳2.4；过渡乳2.9；成熟乳2.9，这表明乳脂肪含量足以满足婴儿的能量需求。脂质类组成正常，98%以甘油三酯形式存在。两组哺乳期妇女乳汁中的胆固醇和胆固醇酯浓度相似。囊性纤维化乳汁的磷脂类分布与对照乳汁不同。两组中约85%的脂质存在于12:0至18:2的六种主要脂肪酸中，这表明大多数脂质的分布相似。囊性纤维化乳汁中的亚油酸含量显著降低至正常值的75%。其他多不饱和脂肪酸在囊性纤维化乳汁中升高。16:2、18:3和几种长链多不饱和脂肪酸的浓度是对照妇女乳汁中的两倍，这表明δ6-去饱和作用增强。几种其他多不饱和脂肪酸代谢产物比例升高表明链延长至C20和C22多不饱和脂肪酸的过程增强。囊性纤维化乳汁中脂肪酸模式的这些差异与囊性纤维化儿童血脂模式中的差异相似。这些结果表明囊性纤维化患者存在多不饱和脂肪酸营养或代谢异常。
