易误诊为多发性肌炎的肌营养不良临床及病理分析

目的明确肢带型肌营养不良2B（LGMD2B）与多发性肌炎（PM）的鉴别诊断。方法分析LGMD2B临床表现、骨骼肌病理学特点及与PM的关系。结果LGMD2B临床表现为四肢近端肌无力，血清肌酸激酶显著增高，肌电图呈肌源性损害，与PM相似。组织化学染色：呈不同程度肌纤维变性、坏死、再生和炎性细胞浸润，与PM相似。免疫组织化学染色：抗-Dysferlin单克隆抗体染色，5例LGMD2B患者Dysferlin蛋白在肌膜上完全缺失，PM均表达正常。结论LGMD2B临床表现、骨骼肌病理学特点与PM相似，抗-Dysferlin单克隆抗体免疫组织化学染色是鉴别二者的关键。

The relationships between PAC-1,P-selectin and pulmonary hypertension in chronic obstructive pulmonary disease

Objective To clarify the differential diagnosis between limb-girdle muscular dystrophy type 2B （LGMD2B） and polymyositis （PM）. Methods Analyzed the character of clinical, histochemical and immunohistochemical pathologic with biopsied skeletal muscle in 5 patients with LGMD2B and the relationship with PM. Results Both LGMD2B and PM showed the muscle weakness in proximal limbs and elevated creatinkinase level and electromyagram showed myogenic damage. Histochemical stains： it was similar to PM, that was the muscle fibers degenerating, regenerating and necrotic and the infiltrating inflammatory cells in different extent in 5 patients. Immunohistochemical stains： anti-Dysferlin monoclonal antibody showed that Dysferlin was deficient on sarcolemma in LGMD2B, but it was normal in PM. Conclusions It is resemble on the clinical and histochemical pathologic characters in both LGMD2B and PM. It is key to antidiastole LGMD2B and PM by the stain of anti-Dysferlin monoclonal antibody.