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目的探讨假肥大型进行性肌营养不良症的临床病理特征,提高对本病的认识。方法回顾性分析进行性肌营养不良症患儿20例的临床资料,包括病史、临床症状、CT,MRI检查结果、病理结果。结果早期镜下见肌纤维粗细不等,见有不同程度的肥大,肿胀,变圆形,部分肌核不在边缘,内移至胞核内,肌纤维间有不同程度的脂肪细胞及纤维组织浸润,少许肌纤维伴有萎缩及变性。结论目前尚未找到一种可逆转本病病程的特效疗法。故应强调采取有效的措施降低本病在人群中的遗传负荷,减少本病的发生。
Objective To investigate the clinical and pathological features of duchenal muscular dystrophy and to improve the understanding of the disease. Methods The clinical data of 20 patients with progressive muscular dystrophy were analyzed retrospectively, including their medical history, clinical symptoms, CT and MRI findings and pathological findings. Results In the early stage, we found muscle fibers ranging in thickness, see varying degrees of hypertrophy, swelling, turning round, part of the muscle nucleus is not at the edge, within the nucleus, there are varying degrees of muscle fibers and fibrous tissue infiltration, a little Muscle fibers accompanied by atrophy and degeneration. Conclusions At present, no specific therapy has been found to reverse the course of this disease. It should be emphasized that effective measures to reduce the genetic burden of the disease in the crowd and reduce the incidence of the disease.