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KMID : 0918520140140020191
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Journal of the Korean Society of Inherited Metabolic Disease
2014 Volume.14 No. 2 p.191 ~ p.194
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A Patient with Mitochondrial Myopathy who Experienced Diabetic Ketoacidosis with Auto-antibo
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Nam Soon-Young
Huh Rimm
Kwun Young-Hee
Lee Ji-Eun
Cho Sung-Yoon
Jin Dong-Kyu
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Abstract
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Mitochondrial myopathy results from a primary dysfunction of the respiratory chain and is frequently accompanied with endocrine manifestations. Among the endocrine manifestations of mitochondrial disease, diabetes mellitus is relatively common. Diabetes mellitus in the mitochondrial myopathy is usually insulin dependent due to the defect in insulin secretion resulted from mitochondrial dysfunction. But it is seldom manifested as diabetes ketoacidosis and doesn’t usually have an auto-antibody. We report a patient with mitochondrial myopathy who was diagnosed as having diabetes
mellitus by presenting as diabetes ketoacidosis and had both of the auto-glutamic acid decarboxylase (GAD) antibody and anti-insulin auto-antibody.
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KEYWORD
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Mitochondrial myopathies, Diabetic ketoacidosis, Diabetes mellitus, Autoimmune disease
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