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作者报告姐弟俩患心肌病并非进行性骨骼肌病病例。例1,女,5岁,因进行性乏力和运动耐受不能,于1977年6月入院。其父母系第二代表亲。体查:心底部可闻第三心音并奔马律及短全程柔和喷射性杂音。无肌萎缩。神经系统检查正常。胸片发现心脏轻度增大。心电图示右室传导延迟,肢体及V_2-V_6导联可见S-T段下移和T波低平。心导管检查示心内结构正常,左室舒张期压及肺动脉楔入压轻度增高,左室收缩增强并轻度肥厚,符合心肌病。柯萨奇A及B、流感A及B、腮腺炎等病毒抗体滴度正常。1983年行肌活检。肌电图正常。患儿呈临床良性经过。例2。系例1之弟,1978年剖腹产生。生后第3天开始出现黄疸,并有从左下肢开始
The authors report that siblings suffering from cardiomyopathy are not cases of skeletal myopathy. Example 1, female, 5 years old, admitted to hospital in June 1977 due to progressive fatigue and exercise tolerance. His parents are the second generation of relatives. Physical examination: the bottom of the heart can hear the third heart sound and run the horse and the short full soft jet sex murmur. No muscle atrophy. Nervous system examination is normal. Chest X-ray showed a slight increase in heart. Electrocardiogram showed right ventricular conduction delay, limb and V_2-V_6 lead shows S-T segment down and T wave low level. Cardiac catheterization showed normal cardiac structure, left ventricular diastolic pressure and pulmonary artery wedge pressure increased slightly, left ventricular systolic enhanced and mild hypertrophy, in line with cardiomyopathy. Coxsackie A and B, influenza A and B, mumps and other virus antibody titers normal. 1983 muscle biopsy. EMG normal. Children were clinically benign after. Example 2. Department of 1 brother, 1978 cesarean section. Jaundice starts on the third day after birth and starts from the lower left leg