Association between Levocarnitine Treatment and the Change in Knee Extensor Strength in Patients Undergoing Hemodialysis: A Post-Hoc Analysis of the Osaka Dialysis Complication Study (ODCS)

Carnitine deficiency is prevalent in patients undergoing hemodialysis, and it could result in lowered muscle strength. So far, the effect of treatment with levocarnitine on lower limb muscle strength has not been well described. This observational study examined the association between treatment with levocarnitine with the change in knee extensor strength (KES) in hemodialysis patients. Eligible patients were selected from the participants enrolled in a prospective cohort study for whom muscle strength was measured annually. We identified 104 eligible patients for this analysis. During the one-year period between 2014 to 2015, 67 patients were treated with intravenous levocarnitine (1000 mg per shot, thrice weekly), whereas 37 patients were not. The change in KES was significantly higher (p = 0.01) in the carnitine group [0.02 (0.01–0.04) kgf/kg] as compared to the non-carnitine group [−0.02 (−0.04 to 0.01) kgf/kg]. Multivariable-adjusted regression analysis showed the positive association between the change in KES and the treatment with levocarnitine remained significant after adjustment for the baseline KES and other potential confounders. Thus, treatment with intravenous levocarnitine was independently and positively associated with the change in KES among hemodialysis patients. Further clinical trials are needed to provide more solid evidence.     

Levocarnitine and dialysis: a review.

Among the various metabolic abnormalities documented in dialysis patients are abnormalities related to the metabolism of fatty acids. Aberrant fatty-acid metabolism has been associated with the promotion of free-radical production, insulin resistance, and cellular apoptosis. These processes have been identified as important contributors to the morbidity experienced by dialysis patients. There is evidence that levocarnitine supplementation can modify the deleterious effects of defective fatty-acid metabolism. Patients receiving hemodialysis and, to a lesser degree, peritoneal dialysis have been shown to be carnitine deficient, as manifested by reduced levels of plasma free carnitine and an increase in the acyl:free carnitine ratio. Cardiac and skeletal muscles are particularly dependent on fatty-acid metabolism for the generation of energy. A number of clinical abnormalities have been correlated with a low plasma carnitine status in dialysis patients. Clinical trials have examined the efficacy of levocarnitine therapy in a number of conditions common in dialysis patients, including skeletal-muscle weakness and fatigue, cardiomyopathy, dialysis-related hypotension, hyperlipidemia, and anemia poorly responsive to recombinant human erythropoietin therapy (rHuEPO). This review examines the evidence for carnitine deficiency in patients requiring dialysis, and documents the results of relevant clinical trials of levocarnitine therapy in this population. Consensus recommendations by expert panels are summarized and contrasted with present guidelines for access to levocarnitine therapy by dialysis patients.     

Levocarnitine Supplementation Suppresses Lenvatinib-Related Sarcopenia in Hepatocellular Carcinoma Patients: Results of a Propensity Score Analysis

This study investigated the inhibitory effect of levocarnitine supplementation on sarcopenia progression in hepatocellular carcinoma (HCC) patients treated with lenvatinib. We evaluated the skeletal muscle index (SMI). After propensity score matching for age, sex, modified albumin-bilirubin grade, baseline presence of sarcopenia, and branched-chain amino acid administration, we selected 17 patients who received levocarnitine supplementation after starting lenvatinib therapy and 17 propensity-score-matched patients who did not receive levocarnitine. Sarcopenia was present in 76% of the patients at baseline. Changes in baseline SMI at 6 and 12 weeks of treatment were significantly suppressed in the group with levocarnitine supplementation compared with those without (p = 0.009 and p = 0.018, respectively). While there were no significant differences in serum free carnitine levels in cases without levocarnitine supplementation between baseline and after 6 weeks of treatment (p = 0.193), free carnitine levels were significantly higher after 6 weeks of treatment compared with baseline in cases with levocarnitine supplementation (p < 0.001). Baseline SMI and changes in baseline SMI after 6 weeks of treatment were significantly correlated with free carnitine levels (r = 0.359, p = 0.037; and r = 0.345, p = 0.045, respectively). Levocarnitine supplementation can suppress sarcopenia progression during lenvatinib therapy.     

Carnitine nutriture of dialysis patients

Hemodialysis patients often experience muscle weakness, cardiac arrhythmias, and hypertriglyceridemia, along with other conditions that may lead to atherosclerosis and coronary heart disease. A contributing factor in the etiology of the symptoms may be carnitine deficiency. Patients undergoing renal dialysis treatment are at risk for developing a carnitine deficiency. The small carnitine molecule can be easily lost into the dialysate. A diseased kidney may lead to a decrease in the endogenous supply of carnitine since the kidney is a major site of carnitine biosynthesis. The diet of dialysis patients may be limiting in preformed carnitine as well as in the precursors and micronutrients required for carnitine biosynthesis. Both oral and intravenous supplementation of L-carnitine have been shown to alleviate muscle weakness, reduce the incidence and severity of arrhythmias, and decrease plasma triglyceride levels, along with alleviating other complications noted in dialysis patients. Health care professionals must be aware of the possible benefits of providing carnitine supplementation for renal dialysis patients.     

Carnitine as an essential nutrient

Carnitine performs a critically important role in energy metabolism and is synthesized in the healthy adult predominantly in the liver and kidney. The typical well balanced American diet contains significant amounts of carnitine as well as the essential amino acids and micronutrients needed for carnitine biosynthesis. Thus carnitine is an infrequent problem in the healthy, well nourished adult population in the United States. However, carnitine can be a conditionally essential nutrient for several different types of individuals. Preterm infants require carnitine for life-sustaining metabolic processes but have a carnitine biosynthetic capability that is not fully developed. There is an increasing number of documented problems with carnitine metabolism in preterm infants not receiving an exogenous source of carnitine indicating that endogenous biosynthesis of carnitine is not adequate to meet the infant's need. Children with different forms of organic aciduria appear to have a greatly increased need for carnitine to function in the excretion of the accumulating organic acids. This need exceeds their dietary carnitine intake and carnitine biosynthetic capability. Renal patients treated with chronic hemodialysis appear to lose carnitine via the hemodialysis treatment, and this loss cannot be repleted simply by endogenous biosynthesis and dietary intake. Treatment with drugs such as valproic acid and metabolic stresses such as trauma, sepsis, organ failure, etc, can also result in a requirement for exogenous carnitine. Accurate assessment of the carnitine status of patients at risk for carnitine deficiency is fundamental to the identification of those patients who require carnitine as the result of altered metabolism.     

Dietary carnitine intake and carnitine status in endurance-trained males

Background: Carnitine is an integral component of fatty acid transfer into the mitochondria, and also buffers excess intramitochondrial acyl‐CoA. It has previously been suggested that athletes may be at risk of low carnitine status and could therefore benefit from carnitine supplementation. Objective: To report the habitual dietary carnitine intakes of endurance‐trained adult males, and to determine whether they are at risk of carnitine insufficiency by measuring plasma and urinary carnitine concentrations. Methods: Fourteen non‐vegetarian endurance‐trained males completed a seven‐day weighed food record and exercise logs to determine habitual dietary carnitine intake. Resting venous blood samples and 24‐hour urine collections were used to determine plasma carnitine concentration and urinary carnitine excretion. Results: The mean dietary carnitine intake was 64 (range 21–110) mg/day. Mean ± SD resting plasma total carnitine was 44 ± 7 µmol/L and acyl : free carnitine ratio was 0.28 ± 0.11, which were within normal ranges. Urinary carnitine excretion was 437 ± 236 µmol/day. There was no correlation between dietary carnitine intake or dietary macro‐ and micronutrients and plasma carnitine or urinary carnitine excretion. Conclusion: The results of the present study indicate there is no evidence that endurance‐trained males consuming a mixed diet are at risk of carnitine insufficiency.     

Depletion of heart and skeletal muscle carnitine in the normal rat by peritoneal dialysis

Serum carnitine values are markedly reduced during dialysis in patients with end-stage renal disease. In an effort to obtain more evidence for dialysis induced carnitine depletion in tissue an antimal model was developed. It was found that a relatively short term peritoneal dialysis treatment in the normal rat led to a 50% decrease in serum carnitine and a 50% reduction of carnitine in the heart and skeletal muscle. The concentration of carnitine in liver did not change. The results indicate that this model may be suitable for determining specific effects of carnitine depletion in heart and skeletal muscle as well as studying the effects of dialysis under various experimental conditions.     

Preliminary Safety and Efficacy of L‐carnitine Infusion for the Treatment of Vasopressor‐Dependent Septic Shock

Background: Sepsis is characterized by metabolic disturbances, and previous data suggest a relative carnitine deficiency may contribute to metabolic dysfunction. Studies regarding safety and patient‐centered efficacy of carnitine during septic shock are lacking. Methods: This was a double‐blind randomized control trial of levocarnitine (L‐carnitine) infusion vs normal saline for the treatment of vasopressor‐dependent septic shock. Patients meeting consensus definition for septic shock with a cumulative vasopressor index ≥3 and sequential organ failure assessment (SOFA) score ≥5 enrolled within 16 hours of the recognition of septic shock were eligible. The primary safety outcome was difference in serious adverse events (SAEs) per patient between groups. Efficacy outcomes included proportion of patients demonstrating a decrease in SOFA score of 2 or more points at 24 hours and short‐ and long‐term survival. Results: Of the 31 patients enrolled, 16 were in the L‐carnitine and 15 were in the placebo arm. There was no difference in SAEs between placebo and intervention (2.1 vs 1.8 SAEs per patient, P = .44). There was no difference in the proportion of patients achieving a decrease in SOFA score of 2 or more points at 24 hours between placebo and treatment (53% vs 44%, P = .59). Mortality was significantly lower at 28 days in the L‐carnitine group (4/16 vs 9/15, P = .048), with a nonsignificant improved survival at 1 year (P = .06). Conclusion: L‐carnitine infusion appears safe in vasopressor‐dependent septic shock. Preliminary efficacy data suggest potential benefit of L‐carnitine treatment, and further testing is indicated.     

血液透析对游离肉碱的清除及影响因素的研究

目的　研究维持性血液透析 (MHD)患者血浆游离肉碱 (FC)水平以及透析对FC清除的影响。方法　应用酶法检测MHD组血浆和透析液中FC水平及对照组尿中FC水平。结果　MHD患者透前血浆FC水平明显低于正常人 (P <0 .0 1)。透析后 ,血浆FC水平较透析前下降 70 %。每周MHD患者透析所致的FC丢失量远远超过对照组尿液排出量 (P <0 .0 1)。透析过程中 ,FC在第 1小时清除最多。透析中FC的清除量与血浆FC浓度呈正相关 (R =0 .5 1,P =0 .0 1)。应用不同透析器及其复用对FC的清除无显著差异。结论　透析中丢失是MHD患者肉碱缺乏的重要原因。应用不同透析器及其复用对FC清除无明显影响。     

CARNITINE AND DISEASES OF SKELETAL MUSCLE

Carnitine deficiency in man whether due to the lack of the enzyme carnitine palmityl transferase (CPT) or lack of the biosynthetic enzymes for carnitine can produce muscle weakness, rhabdomyolysis and myoglobinuria. These patients respond to high carbohydrate feeding with or without added carnitine to their diets.     

Carnitine Intake and Serum Levels Associate Positively with Postnatal Growth and Brain Size at Term in Very Preterm Infants

Carnitine has an essential role in energy metabolism with possible neuroprotective effects. Very preterm (VPT, <32 gestation weeks) infants may be predisposed to carnitine deficiency during hospitalization. We studied the associations of carnitine intake and serum carnitine levels with growth and brain size at term equivalent age (TEA) in VPT infants. This prospective cohort study included 35 VTP infants admitted to Kuopio University Hospital, Finland. Daily nutrient intakes were registered at postnatal weeks (W) 1 and 5, and serum carnitine levels were determined at W1, W5, and TEA. The primary outcomes were weight, length, and head circumference Z-score change from birth to TEA, as well as brain size at TEA in magnetic resonance imaging. Carnitine intake at W1 and W5, obtained from enteral milk, correlated positively with serum carnitine levels. Both carnitine intake and serum levels at W1, W5, and TEA showed a positive correlation with weight, length, and head circumference Z-score change and with brain size at TEA. In linear models, independent positive associations of carnitine intake and serum carnitine levels with length and head circumference Z-score change and brain size at TEA were seen. In VPT infants, sufficient carnitine intake during hospitalization is necessary since it is associated with better postnatal growth and larger brain size at term age.     

Nutritional and Hydration Status and Adherence to Dietary Recommendations in Dalmatian Dialysis Patients

Protein-energy wasting (PEW) is considered one of the major complications of chronic kidney disease (CKD), particularly in dialysis patients. Insufficient energy and protein intake, together with clinical complications, may contribute to the onset and severity of PEW. Therefore, the aim of the study was to analyze the differences in nutritional and hydration status and dietary intake among Dalmatian dialysis patients. Fifty-five hemodialysis (HD) and twenty peritoneal dialysis (PD) participants were included. For each study participant, data about body composition, anthropometric, laboratory, and clinical parameters were obtained. The Malnutrition Inflammation Score (MIS) and two separate 24-h dietary recalls were used to assess nutritional status and dietary intake. The Nutrient Adequacy Ratio (NAR) and Mean Adequacy Ratio (MAR) were calculated to compare actual dietary intake with recommended intake. Additionally, the estimated 10-year survival was calculated using the Charlson Comorbidity Index. The prevalence of malnutrition according to MIS was 47.3% in HD and 45% in PD participants. Significant differences in fat tissue parameters were found between HD and PD participants, whereas significant differences in hydration status and muscle mass parameters were not found. A significant difference in NAR between HD and PD participants was noticed for potassium and phosphorus intake, but not for MAR. MIS correlated negatively with anthropometric parameters, fat mass, visceral fat level and trunk fat mass, and iron and uric acid in HD participants, whereas no significant correlations were found in PD participants. The estimated 10-year survival correlated with several parameters of nutritional status in HD and PD participants, as well as nutrient intake in HD participants. These results indicate a high prevalence of malnutrition and inadequate dietary intake in the Dalmatian dialysis population which, furthermore, highlights the urgent need for individualized and structural nutritional support.     

左旋肉毒碱的代谢与营养效应

Carnitine is a water solule quaternary ammonium compound,which isa natural constituent of higher organisms,in particular of cells of animal origin.In humans,carnitine is synthesized in liver,brain and kidney starting from protein-bound lysine and methionine.Skeletal and heart muscle cannot synthesize carnitine.Therefore,these tissues are entirely dependent on carnitine uptake from the blood.In tissues and in physiological fluids carnitine is present in a free and an esterified form.The proportion of esterified carnitine may vary considerably with nutritional conditions,exercise and disease states.Tissue carnitine content depends on many factors: dietary carnitine,lysine,methionine and co-factor intake,carnitine synthesis (in uremia carnitine synthesis in the kidney is obviously reduced or absent),carnitine transport inside and outside tissues,and carnitine excretion.The transport of long-chain fatty acid esters to sites of beta-oxidation in the mitochondrial matrix requires L-carnitine.Besides,carnitine acts as a sink and allows a shift of the acyl pressure from the mitochondria to the cytoplasm.It has been suggested that carnitine is also important for the transport of the acyl groups (metabolic energy)from one cell to another cell and into the appropriate cellular compartment.Tissue carnitine content is much higher htan tissue CoA content and so acylcarnitines may also serve as storage for metabolic energy.By modulating the tissue content of acyl-CoA compounds which inhibit many enzyme activities (e.g.pyruvate dehydrogenase activity),carnitine may regulate many metabolic pathways.Carnitine system is located in the crossroads of intermediate metabolism and carnitine deficiency and supplementation may affect lipid,glucose and protein metabolism (and eventually nutrition) not only in primary,but also in secondary carnitine deficiency.Some positive effects of carnitine supplementation have been reported in experimental studies,in newborns,in patients treated with artificial nutrition (e.g. in acutely ill patients,in which carnitine excretion may be elevated),and in several disease states.It may be difficult to identify carnitine depleted patients which could benefit from carnitine Suplementation,because serum carnitine levels may be unrelated to tissue carnitine content.Therefore,a trial of L-carnitine may be considered,when insufficient intake or increased requirements are suspected.     

Iron in the diets of rural Honduran women and children

Iron deficiency, a major cause of nutritional anemia, is the most prevalent micronutrient deficiency in the world. One of the main causes of iron deficiency anemia in developing countries is poor availability of absorbable iron from the diet. This study investigates the level of iron intake, bio‐availability, and adequacy in the diets of women and children in the rural west of Honduras. We find that the dietary iron status of this population is very poor. Fifty‐seven percent of the children under one year of age and 23% of 1 to 2 year olds are likely to have inadequate intakes to prevent iron deficiency anemia. Almost all of the pregnant women and 33% of the non‐pregnant non‐lactating women are at risk of developing iron deficiency anemia. The corn based Honduran diet, owing to high phytate levels, has a low bio‐availabiiity for iron. Extremely low intakes of fruits and vegetables and of meats, coupled with significant intake of coffee by all age groups, further limits the availability of dietary iron in the Honduran population.     

The Role of Carnitine and Carnitine Supplementation During Exercise in Man and in Individuals with Special Needs

Carnitine is critical for normal skeletal muscle bioenergetics. Carnitine has a dual role as it is required for long-chain fatty acid oxidation, and also shuttles accumulated acyl groups out of the mitochondria. Muscle requires optimization of both of these metabolic processes during peak exercise performance. Theoretically, carnitine availability may become limiting for either fatty acid oxidation or the removal of acyl-CoAs during exercise. Despite the theoretical basis for carnitine supplementation in otherwise healthy persons to improve exercise performance, clinical data have not demonstrated consistent benefits of carnitine administration. Additionally, most of the anticipated metabolic effects of carnitine supplementation have not been observed in healthy persons. The failure to demonstrate clinical efficacy of carnitine may reflect the complex pharmacokinetics and pharmacodynamics of carnitine supplementation, the challenges of clinical trial design for performance endpoints, or the adequacy of endogenous carnitine content to meet even extreme metabolic demands in the healthy state.

In patients with end stage renal disease there is evidence of impaired cellular metabolism, the accumulation of metabolic intermediates and increased carnitine demands to support acylcarnitine production. Years of nutritional changes and dialysis therapy may also lower skeletal muscle carnitine content in these patients. Preliminary data have demonstrated beneficial effects of carnitine supplementation to improve muscle function and exercise capacity in these patients.

Peripheral arterial disease (PAD) is also associated with altered muscle metabolic function and endogenous acylcarnitine accumulation. Therapy with either carnitine or propionylcarnitine has been shown to increase claudication-limited exercise capacity in patients with PAD.

Further clinical research is needed to define the optimal use of carnitine and acylcarnitines as therapeutic modalities to improve exercise performance in disease states, and any potential benefit in healthy individuals.     

History of L-carnitine: implications for renal disease.

L-carnitine (LC) plays an essential metabolic role that consists in transferring the long chain fatty acids (LCFAs) through the mitochondrial barrier, thus allowing their energy-yielding oxidation. Other functions of LC are protection of membrane structures, stabilizing a physiologic coenzyme-A (CoA)-sulfate hydrate/acetyl-CoA ratio, and reduction of lactate production. On the other hand, numerous observations have stressed the carnitine ability of influencing, in several ways, the control mechanisms of the vital cell cycle. Much evidence suggests that apoptosis activated by palmitate or stearate addition to cultured cells is correlated with de novo ceramide synthesis. Investigations in vitro strongly support that LC is able to inhibit the death planned, most likely by preventing sphingomyelin breakdown and consequent ceramide synthesis; this effect seems to be specific for acidic sphingomyelinase. The reduction of ceramide generation and the increase in the serum levels of insulin-like growth factor (IGF)-1, could represent 2 important mechanisms underlying the observed antiapoptotic effects of acetyl-LC. Primary carnitine deficiency is an uncommon inherited disorder, related to functional anomalies in a specific organic cation/carnitine transporter (hOCTN2). These conditions have been classified as either systemic or myopathic. Secondary forms also are recognized. These are present in patients with renal tubular disorders, in which excretion of carnitine may be excessive, and in patients on hemodialysis. A lack of carnitine in hemodialysis patients is caused by insufficient carnitine synthesis and particularly by the loss through dialytic membranes, leading, in some patients, to carnitine depletion with a relative increase in esterified forms. Many studies have shown that LC supplementation leads to improvements in several complications seen in uremic patients, including cardiac complications, impaired exercise and functional capacities, muscle symptoms, increased symptomatic intradialytic hypotension, and erythropoietin-resistant anemia, normalizing the reduced carnitine palmitoyl transferase activity in red cells.     

串联质谱技术在高危儿原发性肉碱缺乏症的价值

目的 通过运用串联质谱技术检测2 493例高危儿患者的肉碱谱代谢水平,了解本区域高危儿原发性肉碱缺乏群体的发生情况,及其基因突变表达方式以及串联质谱技术的应用价值。方法 选取2013年1月-2016年4月本院住院及门诊疑似遗传代谢疾病高危患儿共2 493例,运用串联质谱技术筛查酰基肉碱谱水平,经基因测序确诊,初步了解本地区高危儿肉碱缺乏症发病情况,并进行随访治疗。结果 在2 493例高危儿的检测结果中,肉碱代谢障碍初筛阳性为55例,占2.21%;原发性肉碱缺乏症确诊例数为10例为0.4%。致病突变位点存在五种类型。结论 本区域高危儿群体原发性肉碱缺乏症所占遗传代谢疾病比率较高,基因突变类型与其他地区存在差别。在诊疗该疾病过程中,运用串联质谱技术对该群体进行早期筛查可以为临床提供有力依据和方向。     

Plasma level and transfer capacity of thiamin in patients undergoing long-term hemodialysis.

Water-soluble vitamins have a molecular size small enough to pass through the membrane of an artificial kidney. This fact has led to the suggestion that these vitamins be substituted in patients undergoing long-term hemodialysis. In sharp contrast to this general belief, our study has shown that the plasma thiamin levels in patients on long-term hemodialysis were not different from those found in normal subjects. It also remained unchanged before and after the dialysis, althoug thiamin was obviously removed in in vitro dialysis. Accordingly, dietary thiamin appears to be sufficient, making further supplementation unnecessary.     

Plasma and muscle carnitine in experimental uremia

The effect of uremia and dietary protein intake on carnitine levels in skeletal muscle and plasma of rats was evaluated. Male Sprague-Dawley rats weighing 160–180 g were made chronically uremic by partial ligation of left renal artery and contralateral nephrectomy. Rats were fed diets containing 8% or 18% casein ad libitum for 8 weeks and sham-operated pair-fed animals were used as controls. At the end of the experiment, all animals were fasted overnight and decapitated. Skeletal muscle and plasma were assayed for free, total acid-soluble and long-chain acylcarnitines. The concentration of total carnitines in skeletal muscle were similar in uremic and control rats fed either 8% or 18% casein diet. However, the muscle long chain acylcarnitines were higher in rats fed the 18% casein diet than those fed the 8% casein diet. Diets (8% or 18% casein) had no effect on the plasma carnitine level, although uremic rats had lower levels than the controls. This study has shown that kidney dysfunction may affect the plasma carnitines level but not the total skeletal muscle carnitines concentration.     

左卡尼汀和曲美他嗪联合治疗对维持性血液透析患者左心室重塑的影响

目的 通过对维持性血液透析(MHD)合并心血管疾病患者采用左卡尼汀和曲美他嗪联合治疗,评估其对MHD患者左心室重塑的影响.方法 选择透析龄≥3个月的MHD患者86例,已排除急性感染及其他活动性疾病,按照接受血液透析治疗的时间分为两组:患者治疗组(46例),每次透析结束后,静脉注射左卡尼汀1.0 g,3次/周,同时口服曲美他嗪20 mg,3次/d,疗程6个月;患者对照组(40例),不用左卡尼汀和曲美他嗪.分别检测治疗前后患者的血清游离脂肪酸(FFA)、游离肉碱(FC)和超声心动图以及血清高敏C反应蛋白(hs-CRP)、白细胞介素(IL).1β、IL-β、肿瘤坏死因子(TNF)-α、谷胱甘肽过氧化物酶(GSHPx)、超氧化物歧化酶(SOD)、丙二醛(MDA)等.另选择健康对照组40例.结果 治疗前,患者治疗组和患者对照组血清FFA、hs-CRP、IL-1β、IL-6、TNF-αβ、MDA水平均显著高于健康对照组(P<0.05或<0.01),FC、GSHPx、SOD水平均显著低于健康对照组(P<0.05或<0.01);治疗6个月后与治疗前比较,患者治疗组FFA、hs-CRP、IL-1β、IL-6、TNF-α、MDA水平均显著下降(P<0.05或<0.01),FC、GSHPx、SOD水平均显著升高(P<0.05或<0.01);治疗6个月后与治疗前比较,患者治疗组的左房内径、左室舒张末内径、室间隔厚度、左室后壁厚度、左室心肌质量指数均显著下降(P<0.05),左室射血分数显著升高(P<0.05);患者对照组以上各项指标治疗前后均无明显变化,治疗6个月后患者治疗组与患者对照组以上各项指标比较差异均有统计学意义(P<0.05或<0.01).结论 联合应用左卡尼汀和曲美他嗪可明显纠正MHD患者的高FFA血症和肉碱缺乏症以及微炎性反应和氧化应激状态,从而改善患者的左心室重塑.