CARDIOMYOPATHY OF GLYCOGEN-STORAGE-DISEASE TYPE-III

被引：20

作者：

CARVALHO, JS

MATTHEWS, EE

LEONARD, JV

DEANFIELD, J

机构：

[1] Department of Paediatric Cardiology, Hospitals for Sick Children, London, WC1N 3JH, Great Ormond Street

[2] Institute of Child Health, London, WC1N 1EH

来源：

HEART AND VESSELS | 1993年 / 8卷 / 03期

关键词：

CARDIOMYOPATHY; GLYCOGEN STORAGE DISEASE; ECHOCARDIOGRAPHY;

D O I：

10.1007/BF01744800

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

To identify the severity of cardiac involvement in glycogen storage disease type III (GSDIII), and its relation to skeletal muscle involvement and age, 23 patients were studied. The median age was 10 years. Echocardiography, electrocardiography, and creatine phosphokinase (CK) levels were used to assess cardiac and skeletal muscle involvement. Septal and left ventricular posterior wall measurements were compared with normal data. Shortening fraction was derived from left ventricular cavity dimensions. In some patients the echocardiogram resembled that of hypertrophic cardiomyopathy. Thirteen of 20 electrocardiograms (ECG) were abnormal. Eleven patients had septal and/or posterior wall thickness >95% confidence limits (CL). Despite this, cardiac symptoms were uncommon. The CK levels were not directly associated with cardiac abnormalities. Older patients (>20 years) had more abnormal measurements of posterior wall thickness than did younger ones (<20 years). This finding, albeit in a cross-sectional series, suggests progressive myocardial involvement with age despite the absence of symptoms.

引用

页码：155 / 159

页数：5

共 16 条

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