title: Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship?
abstract: 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.
authors.affiliation: Department of Anaesthesiology, Herlev Hospital, Denmark.
authors.forename: P
authors.initials: P
authors.lastname: Porsborg
authors.forename: G
authors.initials: G
authors.lastname: Astrup
authors.forename: D
authors.initials: D
authors.lastname: Bendixen
authors.forename: A M
authors.initials: AM
authors.lastname: Lund
authors.forename: H
authors.initials: H
authors.lastname: Ording
mesh_terms: 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*