Proulx F, Lacroix J, Qureshi I A, Nadeau D, Gauthier M, Lambert M
Department of Paediatrics, Sainte-Justine Hospital, Montreal, Quebec, Canada.
Eur J Pediatr. 1997 Nov;156(11):864-9. doi: 10.1007/s004310050732.
In order to characterize the role of carnitine during metabolic stress, we prospectively determined carnitine profiles in plasma and urine on admission, days 2, 5, 10 and 15, among 28 critically ill children free of any known conditions associated with secondary carnitine deficiency. More than 25% of plasma and 50% of urinary carnitine measurements were abnormal; 96% (27/28) of patients displayed on at least one occasion an abnormal [< -2 SD or > +2 SD] carnitine value in plasma. Three children had extremely low [< 10 micromol/l] free carnitine (FC) levels in plasma. Plasma esterified and FC levels on admission were not related to the risk of mortality [PRISM score], to muscle lysis [CK values], and to the caloric intake. Levels of FC and esterified carnitine in plasma were unrelated to those measured in urine.
Abnormal plasma and urine carnitine measurements are frequently found in critically ill children; the biological significance of these perturbations remains unclear. Caution must be exercised before concluding that an abnormal carnitine value is indicative of an underlying hereditary metabolic disorder in this population.
为了明确肉碱在代谢应激中的作用,我们前瞻性地测定了28名无任何已知继发性肉碱缺乏相关疾病的危重症儿童入院时、第2天、第5天、第10天和第15天血浆和尿液中的肉碱谱。超过25%的血浆肉碱测定值和50%的尿液肉碱测定值异常;96%(27/28)的患者至少有一次血浆肉碱值异常[< -2标准差或> +2标准差]。三名儿童血浆中游离肉碱(FC)水平极低[< 10微摩尔/升]。入院时血浆酯化肉碱和FC水平与死亡风险[PRISM评分]、肌肉溶解[肌酸激酶值]及热量摄入无关。血浆中FC和酯化肉碱水平与尿液中测定的水平无关。
危重症儿童中经常发现血浆和尿液肉碱测定异常;这些紊乱的生物学意义尚不清楚。在得出异常肉碱值表明该人群存在潜在遗传性代谢紊乱的结论之前,必须谨慎。