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KMID : 0356920080550050629
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Korean Journal of Anesthesiology
2008 Volume.55 No. 5 p.629 ~ p.633
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Anesthetic experience in a pediatric patient with pyruvate dehydrogenase complex (PDHC) deficiency - A case report -
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Ro Ji-Young
Kim Eun-Ju
Lee Ji-Hyang
Lee Sang-Gon
Ban Jong-Seouk
Min Byung-Woo
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Abstract
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Mitochondrial dysfunction represents a biochemically and clinically diverse group of conditions that can affect any organs with high energy requirement such as brain and muscle being particularly vulnerable. Pyruvate dehydrogenase complex (PDHC) deficiency is one type of mitochondrial dysfuntion that is anesthetically associated with lactic acidosis, muscle hypotonia, malignant hyperthermia, and postoperative respiratory failure. We report a case of general anesthetic management during ventriculoperitoneal shunt in a pediatric patient with PDHC deficiency and its possible considerations.
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KEYWORD
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lactic acidosis, mitochondrial dysfunction, pyruvate dehydrogenase complex (PDHC) deficiency, respiratory failure
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