Mees D E, Frederickson E L
South Med J. 1975 Jan;68(1):29-32. doi: 10.1097/00007611-197501000-00009.
A simplified classification of the porphyrias is given which is thought to be advantageous to the anesthesiologist in determining those patients who are predisposed to acute attacks. These acute attacks may be precipitated by the administration of barbiturates, but may also be spontaneous. The current theory for the precipitation of the acute attack is described, with the probable mechanism being a decrease in uroporphyrinogen synthetase levels and the resultant interference in heme production. Increased formation of cytochrome P-450 with barbiturates also produces increased levels of delta aminolevulinic acid, which may be a cause of the acute attack. The significance in anesthesia and suggested means of anesthetic management are discussed.
本文给出了卟啉病的简化分类，认为这对麻醉医生确定哪些患者易发生急性发作具有优势。这些急性发作可能由巴比妥类药物诱发，但也可能是自发的。文中描述了急性发作的当前诱发理论，其可能机制是尿卟啉原合成酶水平降低以及由此对血红素生成的干扰。巴比妥类药物使细胞色素P - 450的生成增加，也会使δ-氨基乙酰丙酸水平升高，这可能是急性发作的一个原因。文中讨论了其在麻醉中的意义以及建议的麻醉管理方法。
