左旋肉碱缺乏的维持性血液透析患者临床特征及氨基酸谱代谢特点研究

Clinical characteristics and metabolic characteristics of amino acid profile of maintenance hemodialysis patients with L-carnitine deficiency

目的探究左旋肉碱(简称肉碱)缺乏的维持性血液透析(MHD)患者临床特征及氨基酸谱的代谢特点。方法选择2021年7月至2022年2月在同济大学附属第十人民医院血液净化中心行MHD治疗的患者227例,测定其肉碱并按游离肉碱水平是否小于20μmol/L分为肉碱缺乏组106例和肉碱非缺乏组121例,探究左旋肉碱缺乏的MHD患者的临床特征;随机选取91例患者分别纳入肉碱缺乏组50例和肉碱非缺乏组41例,测定氨基酸谱,探究肉碱缺乏的MHD患者氨基酸谱代谢特点。结果MHD患者肉碱缺乏症发生率为46.70%,肉碱缺乏组患者的年龄、透析龄、血钠、血氯、脑钠肽显著高于肉碱非缺乏组,差异有统计学意义(P<0.05);总蛋白、白蛋白、丙氨酸氨基转移酶、尿素氮、肌酐、血钾、血磷显著低于左旋肉碱非缺乏组,差异有统计学意义(P<0.05)。肉碱水平与患者年龄、透析龄、β_(2)-微球蛋白、血氯、脑钠肽呈负相关(P<0.05);与总蛋白、白蛋白、丙氨酸氨基转移酶、尿素氮、肌酐、尿酸、血钾、血磷呈正相关(P<0.05)。年龄、透析龄、血氯是肉碱缺乏的影响因素(P<0.05)。肉碱缺乏组与肉碱非缺乏组相比,人体必需氨基酸中的苏氨酸、缬氨酸、亮氨酸水平显著升高,色氨酸、异亮氨酸水平显著降低,非必需氨基酸中的天冬氨酸水平显著降低,差异有统计学意义(P<0.05)。肉碱水平与亮氨酸(r=-0.258,P=0.013)、缬氨酸(r=-0.295,P=0.005)、苏氨酸(r=-0.291,P=0.005)、精氨酸(r=-0.226,P=0.031)呈负相关,与色氨酸(r=0.224,P=0.033)、天冬氨酸(r=0.296,P=0.004)呈正相关。结论肉碱缺乏的MHD患者蛋白水平更低,电解质紊乱更明显,尿素氮、肌酐水平更低。肉碱缺乏的MHD患者存在明显的氨基酸代谢谱异常。

Objective To explore the clinical characteristics and metabolic characteristics of amino acid profile of maintenance hemodialysis(MHD)patients with L-carnitine deficiency.Methods Totally 227 patients who were treated with MHD in the blood purification center of the Tenth People's Hospital Affiliated to Tongji University from July 2021 to February 2022 were selected,and L-carnitine was measured in all participants.The patients were divided into 106 of carnitine deficiency group and 121 of carnitine non-deficiency group,according to whether the level of free L-carnitine was less than 20μmol/L to explore the clinical characteristics.91 patients were randomly selected including 50 cases of carnitine deficiency group and 41 cases of carnitine non-deficiency group to determine profiling of blood amino,to explore the metabolic characteristics of amino acid profile of MHD patients with L-carnitine deficiency.Results The incidence rate of L-carnitine deficiency in MHD patients was 46.70%.The age,time of hemodialysis,blood sodium,blood chlorine and brain natriuretic peptide in carnitine deficiency group were significantly higher than those in carnitine non-deficiency group(P<0.05);Total protein,albumin,alanine aminotransferase,urea,creatinine,blood potassium and blood phosphorus were significantly lower than those in carnitine non-deficiency group(P<0.05).Carnitine level was negatively related to patients'age,time of hemodialysis β_(2)-microglobulin,blood chlorine and brain natriuretic peptide(P<0.05);it was positively correlated with total protein,albumin,alanine aminotransferase,urea,creatinine,uric acid,blood potassium and blood phosphorus(P<0.05).The age,time of hemodialysis and blood chlorine were influencing factors of carnitine deficiency(P<0.05).Compared with carnitine non-deficiency group,the levels of threonine,valine and leucine,the essential amino acids of human body,in carnitine deficiency group increased significantly,and the levels of tryptophan and isoleucine decreased significantly(P<0.05);the level of aspartic acid,in unessential amino acids,decreased significantly,and the difference was statistically significant(P<0.05).Carnitine level was negatively correlated with leucine(r=-0.258,P=0.013),valine(r=-0.295,P=0.005),threonine(r=-0.291,P=0.005),and arginine(r=-0.226,P=0.031),and positively correlated with tryptophan(r=0.224,P=0.033)and aspartic acid(r=0.296,P=0.004).Conclusion The level of protein in MHD patients with Lcarnitine deficiency is lower,the electrolyte disorder is more obvious,and the levels of urea and creatinine are lower.MHD patients with L-carnitine deficiency have obvious metabolic abnormality of amino acid profile.