12例患者均详细讯问了病史作了系统的体检,有关的血尿检验、肌电图等检查,临床确诊为进行性肌营养不良,肌组织活检病理组织学检查符合本病。早期(或轻度)肌原纤维病变的亚微结构多见于I带尤其是Z线处,主要是细肌丝的溶解。在几个有改变的I带之间的A带可结构如常,病变进一步发展则累及整个或若干肌节,在有改变的肌原纤维周围的肌原纤维也可 All 12 patients were questioned in detail about the medical history, physical examinations, the related hematuria test, EMG test, clinical diagnosis of progressive muscular dystrophy, and histopathological examination of muscle biopsy in line with this disease. The submicroscopic structures of early (or mild) myofibrillar lesions are more common in the I-band, especially in the Z-line, mainly in the dissolution of thin filaments. The A-band between several altered I-strands may be structurally normal, with further progression of the lesion involving the entire or several sarcomeres, myofibrils surrounding the altered myofibrils may also be