Porsborg P, Astrup G, Bendixen D, Lund A M, Ording H
Department of Anaesthesiology, Herlev Hospital, Denmark.
Anaesthesia. 1996 Sep;51(9):863-5. doi: 10.1111/j.1365-2044.1996.tb12619.x.
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.
我们描述了一名患有成骨不全症的患者，在使用巴比妥类药物、芬太尼、泮库溴铵和氧化亚氮进行麻醉期间，出现了心动过速、代谢性和呼吸性酸中毒（pH 7.14，PCO2 8.4 kPa，碱剩余 -8.5 mmol/L）以及高达40摄氏度的高热。怀疑为恶性高热，并对患者进行了相应治疗。两年后，该患者的恶性高热体外挛缩试验结果完全正常。我们得出结论，成骨不全症患者的高代谢是由除恶性高热之外的未知机制引起的。
