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目的探讨线粒体肌病并发呼吸衰竭的发病机制和临床特点。方法报告1例以呼吸衰竭为首发表现的成年线粒体肌病患者的临床资料,并复习相关文献。结果患者临床表现为胸闷;血清肌酶谱检查:肌酸激酶(CK)208U/L,乳酸脱氢酶(LDH)546U/L,天冬氨酸氨基转移酶(AST)50U/L;血气分析提示Ⅱ型呼吸衰竭,肺功能存在限制性通气功能障碍,肌电图提示肌源性损害。肌肉病理发现组织中散在典型的不整红边纤维,电镜下见线粒体内存在晶格状包涵体。结论线粒体肌病可以单纯累及呼吸肌,因通气功能障碍而导致呼吸衰竭。成年人可以慢性呼吸衰竭为线粒体肌病的首发表现。
Objective To investigate the pathogenesis and clinical features of mitochondrial myopathies complicated with respiratory failure. Methods One case of adult mitochondrial myopathy with respiratory failure was reported, and the related literature was reviewed. Results The clinical manifestations of the patients were chest tightness. Serum muscle enzymes: 208U / L creatine kinase (CK), 546U / L lactate dehydrogenase (LDH) and 50U / L aspartate aminotransferase (AST) Type II respiratory failure, pulmonary function limitations of ventilatory dysfunction, myoelectricity prompted myogenic damage. Muscle pathology found in the organization scattered in the typical irregular red edge fibers, mitochondria seen in electron microscopy lattice-like inclusions. Conclusions Mitochondrial myopathy can affect the respiratory muscles, causing respiratory failure due to ventilatory dysfunction. Adults with chronic respiratory failure can be the first manifestation of mitochondrial myopathy.