一名69岁女性因室性心律失常确诊线粒体肌病

Ventricular arrhythmia revealing mitochondrial myopathy in a 69-year-old woman.

作者信息

Constans J, LeHérissier A, Coquet M, Mazat J P, Letellier T, Durandet P, Roudaut R, Gosse P, Conri C, Dallocchio M

机构信息

Service de Médecine Interne et Pathologie Vasculaire, Hôpital Saint-André, Bourdeaux, France.

出版信息

Eur Heart J. 1993 Aug;14(8):1137-9. doi: 10.1093/eurheartj/14.8.1137.

DOI:10.1093/eurheartj/14.8.1137

PMID:8104789

Abstract

We report a case of mitochondrial myopathy (MM), assessed by histological and biochemical studies. This illness was diagnosed in a 69-year-old patient with myocardiopathy revealed by ventricular arrhythmias. The originality of this case lies in the patient's age, the mode of onset and the biochemical features (i.e. normal mitochondrial enzymatic complexes but very low respiration when using glutamate as a substrate).

摘要

我们报告了一例经组织学和生化研究评估的线粒体肌病（MM）病例。该疾病在一名69岁患有心肌病的患者中被诊断出来，其心肌病由室性心律失常揭示。该病例的独特之处在于患者的年龄、发病方式以及生化特征（即线粒体酶复合物正常，但以谷氨酸作为底物时呼吸非常低）。