Malcić I, Barisić N, Pazanin L, Richter D, Jadro-Santel D, Senecić I, Jelić I, Hlavka V
Department of Pediatrics, University Hospital Centre, Zagreb.
Acta Med Croatica. 1993;47(1):47-50.
A 14-year old boy was admitted for signs of heart failure and scapuloperoneal muscle weakness. He fulfilled the clinical, functional and diagnostic criteria for dilated cardiomyopathy. There was also a moderate increase in pulmonary vascular resistance. The immunohistochemical examination of the heart muscle revealed a slightly positive phytohemagglutinin reaction and minimal IgM deposits without complement. The electron microscopy examination disclosed increased numbers of abnormal mitochondria disrupting the usual cell structure; the mitochondria were of various sizes with irregular and abnormal structure of the cristae. The scapuloperoneal spinal muscular atrophy was mild and diagnosed according to clinical and electromyographic findings. Light microscope examination of the skeletal muscle revealed hypotrophic fibers. This patient is presumed to have postinflammatory mitochondriopathy and is currently being managed on low-dose digitalis, diuretics and captopril.
一名14岁男孩因心力衰竭和肩胛腓骨肌无力症状入院。他符合扩张型心肌病的临床、功能和诊断标准。肺血管阻力也有中度升高。心肌的免疫组织化学检查显示植物血凝素反应略呈阳性，有少量无补体的IgM沉积。电子显微镜检查发现异常线粒体数量增加，破坏了正常的细胞结构；线粒体大小不一，嵴的结构不规则且异常。肩胛腓骨型脊髓性肌萎缩较轻，根据临床和肌电图检查结果确诊。骨骼肌的光镜检查显示肌纤维萎缩。该患者推测患有炎症后线粒体病，目前正在接受小剂量洋地黄、利尿剂和卡托普利治疗。
