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杜氏进行性肌营养不良患儿的收缩期时间间期特征

Systolic time interval characteristics in children with Duchenne's progressive muscular dystrophy.

作者信息

Sanyal S K, Tierney R C, Rao P S, Pitner S E, George S L, Givins D R

出版信息

Pediatrics. 1982 Dec;70(6):958-64.

PMID:7145553
Abstract

Systolic time interval (STI) characteristics of 17 boys with Duchenne's muscular dystrophy (DMD) were compared with those of 80 normal boys who served as control subjects. The heart rate decreased linearly with age in normal control subjects (r = -.47, P less than .01). By contrast, heart rate was significantly higher in patients with DMD (P less than .001) and tended to increase further with age. Each STI variable for normal control subjects increased significantly with age (P less than or equal to .01); QII, left ventricular ejection time (LVET), and pre-ejection period (PEP), in addition, decreased with increasing heart rate (P less than or equal to .05). In dystrophic patients QII and LVET decreased with increasing heart rate (P less than .001) but were not influenced by age. None of the other STI values in dystrophic patients was significantly influenced by either age or heart rate. Mean QII, LVET, and QI were shorter and PEP, isometric contraction time (ICT), and PEP/LVET ratio were longer (P less than .001) for DMD patients than for normal control subjects. In 13/17 patients, QII and LVET were below the 95% confidence interval of the normal mean, whereas PEP, ICT, and PEP/LVET exceeded the upper limits of normal in 8, 9 and 11 patients, respectively. For dystrophic patients, the difference (delta) between the observed values and those predicted from regression equations for normal control subjects was lower for QII, LVET, and QI (P less than .01) but higher for PEP (P less than .04), ICT, and PEP/LVET ratio (P less than .001). delta QII and delta LVET increased with age (P = .001 and .032, respectively). Duchenne's muscular dystrophy is thus documented to be associated with substantial alterations in STI characteristics that suggest a compromise of global left ventricular performance. Some of these abnormalities increase with age, probably reflecting the progressive cardiomyopathy characteristics of this disease.

摘要

将17例杜氏肌营养不良症(DMD)男孩的收缩期时间间期(STI)特征与80例正常男孩(作为对照对象)的特征进行了比较。正常对照对象的心率随年龄呈线性下降(r = -0.47,P<0.01)。相比之下,DMD患者的心率显著更高(P<0.001),并且有随年龄进一步增加的趋势。正常对照对象的每个STI变量均随年龄显著增加(P≤0.01);此外,QII、左心室射血时间(LVET)和射血前期(PEP)随心率增加而降低(P≤0.05)。在营养不良患者中,QII和LVET随心率增加而降低(P<0.001),但不受年龄影响。营养不良患者的其他STI值均未受到年龄或心率的显著影响。与正常对照对象相比,DMD患者的平均QII、LVET和QI较短,而PEP、等长收缩时间(ICT)和PEP/LVET比值较长(P<0.001)。在17例患者中的13例中,QII和LVET低于正常均值的95%置信区间,而PEP、ICT和PEP/LVET分别在8例、9例和11例患者中超过正常上限。对于营养不良患者,QII、LVET和QI的观察值与根据正常对照对象回归方程预测的值之间的差异(δ)较低(P<0.01),但PEP(P<0.04)、ICT和PEP/LVET比值的差异较高(P<0.001)。δQII和δLVET随年龄增加(P分别为0.001和0.032)。因此,已证明杜氏肌营养不良症与STI特征的实质性改变有关,这表明左心室整体功能受损。其中一些异常随年龄增加,可能反映了该疾病的进行性心肌病特征。

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