Korean J Anesthesiol.  2008 Nov;55(5):629-633. 10.4097/kjae.2008.55.5.629.

Anesthetic experience in a pediatric patient with pyruvate dehydrogenase complex (PDHC) deficiency: A case report

Affiliations
  • 1Department of Anesthesiology and Pain Medicine, Daegu Fatima Hospital, Daegu, Korea. kej710418@hanmail.net

Abstract

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.

Keyword

lactic acidosis; mitochondrial dysfunction; pyruvate dehydrogenase complex (PDHC) deficiency; respiratory failure

MeSH Terms

Acidosis, Lactic
Brain
Humans
Malignant Hyperthermia
Muscle Hypotonia
Muscles
Pyruvate Dehydrogenase Complex
Pyruvic Acid
Respiratory Insufficiency
Ventriculoperitoneal Shunt
Pyruvate Dehydrogenase Complex
Pyruvic Acid
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