Matsumura Tsuyoshi, Saito Toshio, Fujimura Harutoshi, Shinno Susumu
Department of Neurology, National Hospital Organization Toneyama National Hospital, Toneyama 5-1-1, Toyonaka, Osaka 560-8552, Japan.
Brain Dev. 2007 Sep;29(8):496-501. doi: 10.1016/j.braindev.2007.01.009. Epub 2007 Mar 21.
Brain natriuretic peptide (BNP) and left ventricular ejection fraction (LVEF) are standard indexes for cardiac function. However, they can not reveal myocardial damage directly and they often remain normal even in advanced cardiomyopathy in immobilized patients. Myocardial markers such as MB type of creatine kinase (CK-MB), heart-type fatty acid binding protein (H-FABP) and cardiac troponins are expected to evaluate active myocardial degeneration. However, their availabilities in these patients have not been examined yet.
Participants were 129 patients with dystrophinopathies; 100 Duchenne muscular dystrophy (DMD), 25 Becker muscular dystrophy (BMD) and 4 DMD/BMD carriers. Various serological cardiac indexes, including CK-MB, H-FABP, cardiac troponin I (cTnI), BNP and LVEF were measured and statistical analysis was done.
CK-MB and H-FABP was highly associated with creatine kinase (CK). On the contrary, cTnI, BNP and LVEF were independent from CK. In DMD, relatively high cTnI values were observed in patients with motor ability of rowing wheelchair and in their second decade. BNP and LVEF was strongly correlated. However, cTnI was independent from LVEF and only weak correlation could be detected between cTnI and BNP.
cTnI had been proven to be expressed in myocardium exclusively. Our results also certified that cTnI can assess cardiac degeneration independently from skeletal muscle degeneration and is practical index even in myopathic patients. Our findings also suggested that cardiac degeneration was preceded to functional impairment in many cases. It indicated that cTnI enable us to detect early stage of cardiac degeneration and initiate intervention at proper stage.
脑钠肽(BNP)和左心室射血分数(LVEF)是心功能的标准指标。然而,它们不能直接揭示心肌损伤,即使在固定不动患者的晚期心肌病中,它们通常仍保持正常。诸如肌酸激酶MB型(CK-MB)、心脏型脂肪酸结合蛋白(H-FABP)和心肌肌钙蛋白等心肌标志物有望评估活动性心肌变性。然而,尚未对这些患者中它们的可用性进行研究。
参与者为129例肌营养不良症患者;100例杜氏肌营养不良症(DMD)、25例贝克肌营养不良症(BMD)和4例DMD/BMD携带者。测量了包括CK-MB、H-FABP、心肌肌钙蛋白I(cTnI)、BNP和LVEF在内的各种血清心脏指标,并进行了统计分析。
CK-MB和H-FABP与肌酸激酶(CK)高度相关。相反,cTnI、BNP和LVEF与CK无关。在DMD患者中,在有划轮椅运动能力的患者及其第二个十年中观察到相对较高的cTnI值。BNP和LVEF密切相关。然而,cTnI与LVEF无关,cTnI与BNP之间仅检测到弱相关性。
已证实cTnI仅在心肌中表达。我们的结果还证明,cTnI可以独立于骨骼肌变性评估心脏变性,即使在肌病患者中也是实用指标。我们的研究结果还表明,在许多情况下心脏变性先于功能损害。这表明cTnI使我们能够检测心脏变性的早期阶段并在适当阶段开始干预。