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Anesthetic considerations in patients with mitochondrial defects
Author(s) -
Niezgoda Julie,
Morgan Phil G.
Publication year - 2013
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/pan.12158
Subject(s) - medicine , mitochondrial disease , mitochondrion , disease , respiratory chain , mitochondrial myopathy , mitochondrial encephalomyopathy , bioinformatics , mitochondrial dna , pathology , physiology , genetics , gene , biology
Summary 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 [1][Falk MJ, 2010], [2][Muravchick S, 2006], [3][Falk MJ, 2010]. 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 [4][Schaefer AM, 2004], [5][Zaragoza MV, 2011]. 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 [1][Falk MJ, 2010], [3][Falk MJ, 2010], [4][Schaefer AM, 2004]. 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 [4][Schaefer AM, 2004], [5][Zaragoza MV, 2011]. 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.