title: Anesthetic considerations in patients with mitochondrial defects.
abstract: Mitochondrial disease, once thought to be a rare clinical entity, is now recognized as an important cause of a wide range of neurologic, cardiac, muscle, and endocrine disorders . The incidence of disorders of the respiratory chain alone is estimated to be about 1 per 4-5000 live births, similar to that of more well-known neurologic diseases . High-energy requiring tissues are uniquely dependent on the energy delivered by mitochondria and therefore have the lowest threshold for displaying symptoms of mitochondrial disease. Thus, mitochondrial dysfunction most commonly affects function of the central nervous system, the heart and the muscular system . Mutations in mitochondrial proteins cause striking clinical features in those tissues types, including encephalopathies, seizures, cerebellar ataxias, cardiomyopathies, myopathies, as well as gastrointestinal and hepatic disease. Our knowledge of the contribution of mitochondria in causing disease or influencing aging is expanding rapidly . As diagnosis and treatment improve for children with mitochondrial diseases, it has become increasingly common for them to undergo surgeries for their long-term care. In addition, often a muscle biopsy or other tests needing anesthesia are required for diagnosis. Mitochondrial disease represents probably hundreds of different defects, both genetic and environmental in origin, and is thus difficult to characterize. The specter of possible delayed complications in patients caused by inhibition of metabolism by anesthetics, by remaining in a biochemically stressed state such as fasting/catabolism, or by prolonged exposure to pain is a constant worry to physicians caring for these patients. Here, we review the considerations when caring for a patient with mitochondrial disease.
authors.affiliation: Department of Pediatric Anesthesiology, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Julie
authors.initials: J
authors.lastname: Niezgoda
authors.forename: Phil G
authors.initials: PG
authors.lastname: Morgan
mesh_terms: D000758:Anesthesia; D000777:Anesthetics / Q000009:adverse effects; D002648:Child; D006801:Humans; D008928:Mitochondria / Q000187:drug effects / Q000378:metabolism / Q000502:physiology; D028361:Mitochondrial Diseases / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*; D010347:Patient Care Planning; D019990:Perioperative Care
chemical_list: D000777:Anesthetics
keywords: anesthetics, inhaled agents; anesthetics, intravenous agents; general anesthesia; mitochondrial disorders; muscle disorders; neurologic disease