Khoss A E, Steger H, Legenstein E, Proll E, Salzer-Muhar U, Schlemmer M, Balzar E, Wimmer M
Universitäts-Kinderklinik, Wien.
Wien Klin Wochenschr. 1989 Jan 6;101(1):17-20.
Six out of 14 chronically hemodialysed children with significantly decreased serum carnitine levels were substituted with L-carnitine (15-30 mg/kg/day-Biocarn) up to normal and above normal carnitine levels. None of these patients were digitalised. During the time of investigation plasma carnitine levels were investigated monthly and, simultaneously, three echocardiographic parameters in M-mode were quantitatively und qualitatively determined: shortening fraction (SF-%), ratio of left ventricular pre-ejection/ejection time (LVPT/LVET) and velocity of circumferential fibre shortening (Vcf). Carnitine substitution produced measurable changes in echocardiographic parameters, and a significant quantitative improvement in left ventricular function and performance: after 6 months of carnitine substitution a 24% improvement was seen, after 18 months a 44% mean improvement. No side effects of carnitine were observed; the compatibility was good. Beside these positive effects all patients reported decreased dialysis-associated spasms and polyneuropathic symptoms and increased somatic ability.
14名长期接受血液透析且血清肉碱水平显著降低的儿童中，有6名接受了L-肉碱（15 - 30毫克/千克/天 - 生物肉碱）替代治疗，直至肉碱水平恢复正常及高于正常水平。这些患者均未进行洋地黄化治疗。在研究期间，每月检测血浆肉碱水平，同时，以M型超声心动图定量和定性测定三个参数：缩短分数（SF-%）、左心室射血前期/射血时间比值（LVPT/LVET）和圆周纤维缩短速度（Vcf）。肉碱替代治疗使超声心动图参数发生了可测量的变化，并使左心室功能和性能有显著的定量改善：肉碱替代治疗6个月后改善了24%，18个月后平均改善了44%。未观察到肉碱的副作用；耐受性良好。除了这些积极效果外，所有患者均报告透析相关痉挛和多神经病变症状减轻，身体能力增强。
