Ii C, Tanaka N, Okamura H
Department of Obstetrics and Gynecology, Kumamoto University School of Medicine.
Nihon Rinsho. 1995 Jun;53(6):1498-502.
Although the morbidity of porphyria is rare, the surgical and anesthetic managements of patients with porphyria should be prudent, for various stresses including surgery and anesthesia may cause occurrence or exacerbation of this disease, occasionally resulting in the mortal course. Several drugs such as barbiturate, diazepam, pentazocine, and pancuronium, which can be used during anesthesia or after operation, reportedly exacerbate the disease. Furthermore, the acute exacerbation of porphyria may be misdiagnosed as acute abdomen, ileus, acute appendicitis, cholelithiasis, urolithiasis, or ectopic pregnancy. The managements of patients with acute porphyria during anesthesia and after surgery are discussed along with the introduction of our case report. Since there is no definitive treatment of porphyria, the most important thing is to understand the disease and to prevent the acute exacerbation of the disease. When patients are suspected of porphyria or possible porphyria, careful management is required during anesthesia and after operation with selecting secure drugs against the disease.
尽管卟啉病的发病率很低，但对于卟啉病患者的手术和麻醉管理仍应谨慎，因为包括手术和麻醉在内的各种应激可能会导致该疾病的发生或加重，偶尔会导致致命的病程。据报道，一些可在麻醉期间或术后使用的药物，如巴比妥酸盐、地西泮、喷他佐辛和泮库溴铵，会使病情加重。此外，卟啉病的急性发作可能会被误诊为急腹症、肠梗阻、急性阑尾炎、胆石症、尿路结石或异位妊娠。本文结合我们的病例报告，讨论了急性卟啉病患者在麻醉期间和术后的管理。由于卟啉病尚无确切的治疗方法，最重要的是了解该疾病并预防其急性发作。当患者被怀疑患有卟啉病或可能患有卟啉病时，在麻醉期间和术后需要谨慎管理，选择对该疾病安全的药物。
