Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship?
We describe a patient with osteogenesis imperfecta who developed tachycardia, metabolic and respiratory acidosis (pH 7.14, PCO2 8.4 kPa, BE -8.5 mmol.l-1) and hyperthermia up to 40 degrees C during anaesthesia with barbiturates, fentanyl, pancuronium, and nitrous oxide. Malignant hyperthermia was suspected and the patient treated accordingly. Two years later the in-vitro contracture test for malignant hyperthermia was completely normal. We conclude that hypermetabolism in patients with osteogenesis imperfecta is due to unknown mechanisms other than malignant hyperthermia.
Porsborg
P
P
Department of Anaesthesiology, Herlev Hospital, Denmark.
Astrup
G
G
Bendixen
D
D
Lund
A M
AM
Ording
H
H
D000771:Anesthesia, Intravenous; D002648:Child; D003937:Diagnosis, Differential; D005334:Fever / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007431:Intraoperative Complications / Q000175:diagnosis*; D008297:Male; D008305:Malignant Hyperthermia / Q000175:diagnosis*; D010013:Osteogenesis Imperfecta / Q000145:classification / Q000150:complications*