Anesthesia for a child with complex I respiratory chain enzyme deficiency.
The mitochondrial myopathies are a rare group of conditions affecting the respiratory chain and oxidative phosphorylation. The anesthetic management of a 6-year-old girl with complex I respiratory chain deficiency requiring surgery for a fractured hip is presented and discussed. Potential problems were masseter spasm, tendency to develop lactate acidosis, and malignant hyperthermia susceptibility. These problems were avoided by the use of a laryngeal mask airway, allowing the patient to spontaneously ventilate; caudal analgesia; and maintenance of anesthesia with a proprofol infusion.
Cheam
E W
EW
Department of Anaesthesia and Intensive Care, Chinese University of Hong Kong, Prince of Wales Hospital, People's Republic of China.
Critchley
L A
LA
D000758:Anesthesia / Q000379:methods*; D002648:Child; D042967:Electron Transport Complex I; D005260:Female; D006801:Humans; D008305:Malignant Hyperthermia / Q000209:etiology; D017240:Mitochondrial Myopathies / Q000503:physiopathology*; D009247:NADH, NADPH Oxidoreductases / Q000172:deficiency*; D015742:Propofol / Q000494:pharmacology
D009247:NADH, NADPH Oxidoreductases; D042967:Electron Transport Complex I; D015742:Propofol