Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Korean J Anesthesiol. 2012 Jan;62(1):83-6. doi: 10.4097/kjae.2012.62.1.83. Epub 2012 Jan 25.
Porphyrias are a group of diseases characterized by an enzyme deficiency in the heme biosynthesis pathway, resulting in accumulation of precursor molecules in the tissue. Some porphyric patients develop progressive liver disease that requires liver transplantation. This case report describes special anesthetic challenges, including careful selection of drugs and the use of special filters that can exclude harmful wavelengths of ultraviolet, in a patient with porphyria who underwent living donor liver transplantation. Understanding the patient's status and disease process, and avoiding triggering factors of porphyria attacks, are important for successful liver transplantation anesthesia in patients with porphyria.
卟啉症是一组疾病，其特征为血红素生物合成途径中的酶缺乏，导致组织中前体分子积累。一些卟啉症患者会发展为需要进行肝移植的进行性肝病。本病例报告描述了特殊的麻醉挑战，包括仔细选择药物和使用特殊的过滤器来排除有害的紫外线波长，在接受活体供肝移植的卟啉症患者中。了解患者的状况和疾病过程，并避免卟啉症发作的诱因，对于卟啉症患者的肝移植麻醉成功至关重要。
