Inborn errors of metabolism:
Indications for: REVONTO
Management of the fulminant hypermetabolism of skeletal muscle characteristic of malignant hyperthermia (MH). Prevention of malignant hyperthermia in susceptible patients during pre-op or sometimes post-op.
Adults and Children:
Treatment: Give by rapid IV push at a minimum of 1mg/kg, continue until symptoms subside or max cumulative dose of 10mg/kg has been reached; if physiologic and metabolic abnormalities reappear, repeat regimen. Prevention: Start approx. 75 mins prior to anesthesia, give 2.5mg/kg by IV infusion over 1hr; may give additional doses during anesthesia or prolonged surgery.
Institute supportive measures (eg, discontinue MH-triggering agents, increase oxygen requirements, manage metabolic acidosis, cooling if necessary, monitor urinary output and electrolyte imbalance). Monitor for skeletal muscle weakness, difficulty swallowing and choking. Increased risk of hepatic injury (esp. in females or patients >35yrs of age); monitor hepatic function frequently (eg, SGOT, SGPT). Avoid extravasation. Elderly. Pregnancy (Cat.C). Nursing mothers: not recommended.
Muscle relaxant (skeletal).
Avoid MH-triggering agents. Concomitant calcium channel blockers (eg, verapamil): not recommended. Caution with concomitant tranquilizers. May potentiate vecuronium-induced neuromuscular block. Protein binding reduced by warfarin or clofibrate; and increased by tolbutamide.
Loss of grip strength, weakness in the legs, drowsiness, dizziness, thrombophlebitis, tissue necrosis secondary to extravasation, inj site reactions (eg, pain, erythema, swelling); rare: pulmonary edema, urticaria, erythema.