Biomaterial Database

Anaesthesia and pyruvate dehydrogenase deficiency. 1983

S F Dierdorf, and W L McNiece

A ten-month-old infant with pyruvate dehydrogenase deficiency received anaesthesia on two occasions, once for a laparotomy and once for a tracheostomy. During both anaesthetics (different techniques) she developed an increase in arterial lactate levels and a metabolic acidosis. Pyruvate dehydrogenase deficiency results in the inability to metabolize pyruvate with resultant accumulation of pyruvate and lactate. Inhibition of gluconeogenesis, which may be produced by halothane and thiopentone, will also increase lactate levels. Other causes of increased lactate levels are hypocarbia and high carbohydrate intake. In this patient hypocarbia may have produced increased lactate levels and increased the metabolic acidosis. Recommendations include avoidance of halogenated anaesthetics, avoidance of lactate containing solutions, maintenance of normocarbia, and stress-free anaesthesia.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007773	Lactates	Salts or esters of LACTIC ACID containing the general formula CH3CHOHCOOR.
D008661	Metabolism, Inborn Errors	Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.	Inborn Errors of Metabolism,Metabolism Errors, Inborn,Error, Inborn Metabolism,Errors Metabolism, Inborn,Errors Metabolisms, Inborn,Errors, Inborn Metabolism,Inborn Errors Metabolism,Inborn Errors Metabolisms,Inborn Metabolism Error,Inborn Metabolism Errors,Metabolism Error, Inborn,Metabolism Inborn Error,Metabolism Inborn Errors,Metabolisms, Inborn Errors
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000138	Acidosis	A pathologic condition of acid accumulation or depletion of base in the body. The two main types are RESPIRATORY ACIDOSIS and metabolic acidosis, due to metabolic acid build up.	Metabolic Acidosis,Acidoses,Acidoses, Metabolic,Acidosis, Metabolic,Metabolic Acidoses
D000758	Anesthesia	A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures.
D000777	Anesthetics	Agents capable of inducing a total or partial loss of sensation, especially tactile sensation and pain. They may act to induce general ANESTHESIA, in which an unconscious state is achieved, or may act locally to induce numbness or lack of sensation at a targeted site.	Anesthetic,Anesthetic Agents,Anesthetic Drugs,Anesthetic Effect,Anesthetic Effects,Agents, Anesthetic,Drugs, Anesthetic,Effect, Anesthetic,Effects, Anesthetic
D015325	Pyruvate Dehydrogenase Complex Deficiency Disease	An inherited metabolic disorder caused by deficient enzyme activity in the PYRUVATE DEHYDROGENASE COMPLEX, resulting in deficiency of acetyl CoA and reduced synthesis of acetylcholine. Two clinical forms are recognized: neonatal and juvenile. The neonatal form is a relatively common cause of lactic acidosis in the first weeks of life and may also feature an erythematous rash. The juvenile form presents with lactic acidosis, alopecia, intermittent ATAXIA; SEIZURES; and an erythematous rash. (From J Inherit Metab Dis 1996;19(4):452-62) Autosomal recessive and X-linked forms are caused by mutations in the genes for the three different enzyme components of this multisubunit pyruvate dehydrogenase complex. One of the mutations at Xp22.2-p22.1 in the gene for the E1 alpha component of the complex leads to LEIGH DISEASE.	Ataxia with Lactic Acidosis, Type I,Lactic Acidosis with Ataxia, Type I,Ataxia with Lactic Acidosis,Ataxia with Lactic Acidosis I,Ataxia, Intermittent, with Abnormal Pyruvate Metabolism,Ataxia, Intermittent, with Pyruvate Dehydrogenase, or Decarboxylase, Deficiency,Intermittent Ataxia with Pyruvate Dehydrogenase Deficiency,Juvenile Pyruvate Dehydrogenase Complex Deficiency Disease,Neonatal Pyruvate Dehydrogenase Complex Deficiency Disease,PDH Deficiency,PDHC Deficiency,PDHC Deficiency Disease,Pyruvate Decarboxylase Deficiency,Pyruvate Dehydrogenase Complex Deficiency,Pyruvate Dehydrogenase Complex Deficiency Disease, Juvenile,Pyruvate Dehydrogenase Complex Deficiency Disease, Neonatal,Pyruvate Dehydrogenase Deficiency,Type I Ataxia with Lactic Acidosis,Deficiency, PDH,Deficiency, PDHC,Deficiency, Pyruvate Decarboxylase,Deficiency, Pyruvate Dehydrogenase
D019344	Lactic Acid	A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed)	Lactate,2-Hydroxypropanoic Acid,2-Hydroxypropionic Acid,Ammonium Lactate,D-Lactic Acid,L-Lactic Acid,Propanoic Acid, 2-Hydroxy-, (2R)-,Propanoic Acid, 2-Hydroxy-, (2S)-,Sarcolactic Acid,2 Hydroxypropanoic Acid,2 Hydroxypropionic Acid,D Lactic Acid,L Lactic Acid,Lactate, Ammonium