Lloyd-Still J D, Bohan T, Hughes S, Wessel H U
Department of Pediatrics, Children's Memorial Hospital, Northwestern University, Chicago, Illinois.
Acta Paediatr Scand. 1990 Apr;79(4):427-30. doi: 10.1111/j.1651-2227.1990.tb11488.x.
Carnitine metabolites (total, free, short and long chain) were analyzed in cord blood of cystic fibrosis (n = 5), non-CF siblings (n = 7), and controls (n = 8). Total acylcarnitine (short and long chain combined) was significantly lower (less than 0.001) in CF compared to both control groups. Total and free carnitine showed no significant differences between the three groups. These findings are compatible with disturbed fatty acid metabolism in utero and may be related to the increased energy expenditure characteristic of CF infants.
对囊性纤维化患者（n = 5）、非囊性纤维化患者的兄弟姐妹（n = 7）以及对照组（n = 8）的脐带血中的肉碱代谢物（总量、游离、短链和长链）进行了分析。与两个对照组相比，囊性纤维化患者的总酰基肉碱（短链和长链合并）显著降低（小于0.001）。三组之间的总肉碱和游离肉碱无显著差异。这些发现与子宫内脂肪酸代谢紊乱相符，可能与囊性纤维化婴儿能量消耗增加的特征有关。
