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Korean Journal of Anesthesiology 2008;55(5):629-633.
DOI: https://doi.org/10.4097/kjae.2008.55.5.629   
Anesthetic experience in a pediatric patient with pyruvate dehydrogenase complex (PDHC) deficiency: A case report.
Jiyung Ro, Eun Ju Kim, Ji Hyang Lee, Sang Gon Lee, Jong Suk Ban, Byung Woo Min
Department of Anesthesiology and Pain Medicine, Daegu Fatima Hospital, Daegu, Korea. kej710418@hanmail.net
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.
Key Words: lactic acidosis; mitochondrial dysfunction; pyruvate dehydrogenase complex (PDHC) deficiency; respiratory failure


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