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[血清肉碱在扩张型心肌病病程及预后中的临床意义]

[Clinical significance of serum carnitine in the course and prognosis of dilated cardiomyopathy].

作者信息

Conte A, Hess O M, Maire R, Gautschi K, Brogli S, Knaus U, Krayenbühl H P

出版信息

Z Kardiol. 1987 Jan;76(1):15-24.

PMID:3564613
Abstract

Serum carnitine is an essential cofactor for the transport of free fatty acids into the mitochondria. We determined the free and the total serum carnitine in 99 healthy blood donors and 58 patients with different forms of heart muscle disease. Thirty patients had dilated (DCM), 10 hypertrophic (HCM) and 8 alcoholic (ACM) cardiomyopathy and 10 patients had congestive heart failure of different etiology than cardiomyopathy (CHF). Free and total serum carnitine were determined by an enzymatic-spectrophotometric assay according to Pearson. Mean values for free and total serum carnitine were as follows: 47 and 74 mumol/l in controls (C; blood donors), 74 (P less than 0.01 vs. C) and 83 mumol/l in DCM, 66 (P less than 0.01 vs. C) and 89 mumol/l in HCM, 85 (P less than 0.01 vs. C) and 104 mumol/l (P less than 0.05 vs. C) in ACM and 86 (P less than 0.01 vs. C) and 129 mumol/l (P less than 0.01 vs. C) in CHF. Ten patients died during the mean observation time of 13 months, 8 patients with DCM and 2 with CHF; 9 of these 10 patients had initially a markedly increased serum carnitine. Patients with DCM were divided into two groups with normal (n = 15; 25-68 mumol/l) and increased (n = 15; greater than 68 mumol/l) free serum carnitine. Patients with increased serum carnitine showed a significantly higher mortality rate (47%) than patients with normal serum carnitine. It is concluded that free and total serum carnitine are elevated in patients with congestive heart failure, dilated and hypertrophic cardiomyopathy. The etiology of this carnitine metabolism disturbance is unclear but it is probably due to a secondary phenomenon in patients with congestive heart failure or primary myocardial hypertrophy. An increased serum carnitine is a poor prognostic sign in patients with dilated cardiomyopathy.

摘要

血清肉碱是游离脂肪酸转运进入线粒体所必需的辅助因子。我们测定了99名健康献血者以及58例患有不同类型心肌病患者的游离血清肉碱和总血清肉碱水平。其中30例为扩张型心肌病(DCM),10例为肥厚型心肌病(HCM),8例为酒精性心肌病(ACM),另有10例为病因与心肌病不同的充血性心力衰竭(CHF)。游离血清肉碱和总血清肉碱采用Pearson酶促分光光度法进行测定。游离血清肉碱和总血清肉碱的平均值如下:对照组(C;献血者)分别为47和74μmol/L,DCM组为74(与C组相比P<0.01)和83μmol/L,HCM组为66(与C组相比P<0.01)和89μmol/L,ACM组为85(与C组相比P<0.01)和104μmol/L(与C组相比P<0.05),CHF组为86(与C组相比P<0.01)和129μmol/L(与C组相比P<0.01)。在平均13个月的观察期内,有10例患者死亡,其中8例为DCM患者,2例为CHF患者;这10例患者中有9例最初血清肉碱水平显著升高。DCM患者被分为游离血清肉碱正常组(n = 15;25 - 68μmol/L)和升高组(n = 15;>68μmol/L)。血清肉碱升高的患者死亡率(47%)显著高于血清肉碱正常的患者。研究得出结论,充血性心力衰竭、扩张型心肌病和肥厚型心肌病患者的游离血清肉碱和总血清肉碱水平升高。这种肉碱代谢紊乱的病因尚不清楚,但可能是充血性心力衰竭患者的继发现象或原发性心肌肥厚所致。血清肉碱升高是扩张型心肌病患者预后不良的标志。

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