Mayer G, Graf H, Legenstein E, Linhart L, Auer B, Lohninger A
2nd Department of Internal Medicine, University of Vienna, Austria.
Nephron. 1989;52(4):295-9. doi: 10.1159/000185666.
Patients on chronic hemodialysis with hyperlipidemia were found to respond either with decreased levels (responders) or with a further increase of the plasma triglyceride levels (nonresponders) to a carnitine substitution therapy. The aim of the present study was to find possible predictors to distinguish between responders and nonresponders prior to the initiation of therapy. Since it is suggested that erythrocytes are involved in carnitine transport to tissues, it was of interest to determine plasma and erythrocyte carnitine concentrations in the hemodialyzed patients before and during carnitine substitution therapy and to compare the results with those of healthy controls. Before therapy, comparatively lower plasma levels of both free and total carnitine, but higher portions of short-chain acylcarnitine on total carnitine were found in all patients. In erythrocytes, the nonresponders showed significantly higher total carnitine levels, compared to responders and controls. After the start of carnitine substitution, the increase of total plasma carnitine during the substitution period corresponded with the carnitine dose administered in responders, in nonresponders the highest carnitine values were found in the second week when the lower carnitine dose was administered. The changes of the plasma short-chain acylcarnitine levels with time were very similar to those of plasma triglycerides. All patients showed a time-delayed accumulation of carnitine in erythrocytes and, interestingly, markedly higher concentrations in the second week when the lower carnitine dose was administered. The results of the present study demonstrate that the erythrocyte carnitine content is a reliable predictor to distinguish between responders and nonresponders prior to the start of a carnitine substitution therapy.
接受慢性血液透析且患有高脂血症的患者，在接受肉碱替代疗法后，血浆甘油三酯水平要么降低（反应者），要么进一步升高（无反应者）。本研究的目的是在治疗开始前找到区分反应者和无反应者的可能预测指标。由于有研究表明红细胞参与肉碱向组织的转运，因此测定血液透析患者在肉碱替代治疗前和治疗期间的血浆和红细胞肉碱浓度，并将结果与健康对照组进行比较具有重要意义。治疗前，所有患者的游离和总肉碱血浆水平相对较低，但短链酰基肉碱占总肉碱的比例较高。在红细胞中，无反应者的总肉碱水平显著高于反应者和对照组。开始肉碱替代治疗后，反应者在替代期间血浆总肉碱的增加与给予的肉碱剂量相对应，而无反应者在给予较低肉碱剂量的第二周发现了最高的肉碱值。血浆短链酰基肉碱水平随时间的变化与血浆甘油三酯的变化非常相似。所有患者的红细胞中肉碱都有延迟积累，有趣的是，在给予较低肉碱剂量的第二周，红细胞中的肉碱浓度明显更高。本研究结果表明，红细胞肉碱含量是在肉碱替代治疗开始前区分反应者和无反应者的可靠预测指标。
