Pedersen N A, Owen-Falkenberg A P, Lund C
Anaestesiologisk og intensiv afdeling Y., Københavns Amts Sygehus i Glostrup.
Ugeskr Laeger. 1993 May 24;155(21):1622-6.
The porphyrias are a group of disorders of haem metabolism. A knowledge of which anaesthetic can precipitate an acute attack of porphyria is important, since an accumulation of metabolites can result in life threatening symptoms, such as abdominal pain, vomiting, photophobia, neuropathy, bulbar paresis and respiratory failure. Treatment consists primarily of adequate calorie intake e.g. glucose, but is otherwise symptomatic. Anaesthetic drug recommendations are based both on animal experiments and patient experience, primarily case histories. An array of local anaesthetics, hypnotics, sedatives, neuroleptics, analgesics, muscle relaxants, inhalation anaesthetics and some antibiotics are reviewed. Patients with a history of porphyria should be in an optimal condition and maintain a high calorie intake perioperatively. The pre-operative fast should be a minimum and iv-glucose is advisable while fasting. There are anaesthetic agents that are safe for both regional and general anaesthesia.
卟啉症是一组血红素代谢紊乱疾病。了解哪种麻醉剂会诱发卟啉症急性发作很重要,因为代谢产物的积累会导致危及生命的症状,如腹痛、呕吐、畏光、神经病变、延髓麻痹和呼吸衰竭。治疗主要包括摄入足够的热量,如葡萄糖,但其他方面则是对症治疗。麻醉药物的建议基于动物实验和患者经验,主要是病例史。本文综述了一系列局部麻醉剂、催眠药、镇静剂、抗精神病药、镇痛药、肌肉松弛剂、吸入麻醉剂和一些抗生素。有卟啉症病史的患者应处于最佳状态,并在围手术期保持高热量摄入。术前禁食时间应尽量缩短,禁食期间建议静脉输注葡萄糖。有一些麻醉剂对区域麻醉和全身麻醉都是安全的。