肉碱缺乏对透析患者相关并发症发生机制的研究

Research on the mechanism of complications related to dialysis patients with carnitine deficiency

肉碱作为游离脂肪酸进出线粒体的中间转运体,其在人体脂肪酸的β氧化和能量代谢中发挥重要作用。肉碱缺乏可因阻碍线粒体能量代谢而危及生命,肉碱缺乏的原因主要包括原发性合成不足及继发性丢失过多,透析治疗患者是肉碱继发性缺乏较为常见的人群。肉碱缺乏的可能后果包括:细胞内游离脂肪酸积累产生毒性效应,透析过程中出现低血压及心脏病变;继发线粒体内某些关键酶代谢紊乱及改变细胞膜的稳定性,使红细胞变形性及渗透脆性增加进而加重贫血;骨骼肌氧供不足,肌肉含量下降导致患者出现肌病、肌无力、透析期间肌肉痉挛。本文概括了肉碱的生成、代谢途径、生物学功能及缺乏时对机体的影响,阐述了肉碱缺乏时透析患者并发心功能障碍、血流动力学不稳定、贫血、肌肉痉挛的发生机制,为肉碱治疗透析相关并发症提供参考。

Carnitine,as an intermediate transporter of free fatty acids in and out of mitochondria,plays a vital role in the β oxidation and energy metabolism of fatty acids in the human body.Carnitine deficiency can be life-threatening by hindering mitochondrial energy metabolism,and the main causes of carnitine deficiency include primary insufficiency and secondary excessive losses,and patients receiving dialysis treatment are the more common people with secondary carnitine deficiency.The possible consequences of carnitine deficiency include:toxic effects of intracellular free fatty acid accumulation,hypotension and cardiac changes during dialysis,secondary metabolic disorders of some key enzymes in mitochondria,and altering the stability of cell membranes,increasing red blood cell deformability and osmotic fragility and aggravating anemia,and hypothermia in patients with insufficient oxygen supply to skeletal muscles,resulting in myopathy,muscle weakness,and muscle cramps during dialysis.This article summarizes the production and metabolic pathways,biological functions,and effects of carnitine deficiency on the body,and expounds the pathway mechanism of carnitine deficiency in dialysis patients with kidney disease complicated by cardiac dysfunction,hemodynamic instability,anemia,muscle spasm,etc.,so as to provide a reference for the treatment of complications related to dialysis patients with carnitine deficiency.