SummaryPatients with Duchenne and Becker muscular dystrophy suffer from a progressive deterioration in muscle secondary to a defect in the dystrophin gene. As such, they are susceptible to perioperative respiratory, cardiac and other complications, such as rhabdomyolysis. Inhalational anesthetic agents have been implicated as a cause of acute rhabdomyolysis that can resemble malignant hyperthermia (MH). This article reviews perioperative 'MH-like' reactions reported in muscular dystrophy patients and groups them into three categories according to clinical presentation. The etiology and underlying pathophysiological process responsible for these reactions is discussed and recommendations are proposed for the safe anesthetic management of these patients.
To compare the dose-response of remifentanil for tracheal intubation in infants and children, 32 healthy full-term infants and 32 children were anesthetized with 10 mug/kg glycopyrrolate and 4.0 mg/kg propofol and administered 1 of 4 doses of remifentanil (1.25, 1.50, 1.75, or 2.00 microg/kg) to facilitate tracheal intubation. We determined the effective doses of remifentanil in 50% (ED50) and 98% (ED98) of patients by using logistic regression analysis. We found that logistic regression curves were similar for infants and children (P = 0.38). ED50 and ED98 values for remifentanil were 1.70 +/- 0.1 microg/kg and 2.88 +/- 0.5 microg/kg, respectively. In a second double-blind study, 24 infants were anesthetized with propofol and randomized to receive either 3.0 microg/kg remifentanil or 2.0 mg/kg succinylcholine to facilitate tracheal intubation. The duration of apnea, tracheal intubating conditions and hemodynamic changes were determined. We found that the duration of apnea and intubating conditions after propofol/remifentanil were similar to those after propofol/succinylcholine. Bradycardia, hypotension, and chest wall rigidity did not occur. We conclude that the dose-response of remifentanil for tracheal intubation is similar in infants and children. Propofol/remifentanil provides clinically acceptable intubating conditions, stable hemodynamics, and a duration of apnea comparable to that with propofol/succinylcholine in infants.
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