Blanloeil Y
Service d'Anesthésie et Réanimation Chirurgicale, Hôpital G. et R. Laennec, Nantes.
Ann Fr Anesth Reanim. 1994;13(4):485-9. doi: 10.1016/s0750-7658(05)80678-1.
Propofol may be used without any restriction in all cases of asymptomatic hepatic porphyria, as a bolus injection for induction and maintenance of anaesthesia and as a continuous infusion to produce anaesthesia of short and medium duration. If in doubt as to the absolute safety of propofol in case of symptomatic hepatic porphyria, preference has to be given to neuroleptanalgesia, benzodiazepines and muscle relaxants. If propofol is to be used, treatment with haematin should be readily available. In all cases, it is essential to determine porphyrins and their precursors prior to and after surgery. Urine collection should strictly adhere to the recommended procedure: sterile conditions, protected from light and high temperature. Any abnormal reaction after the administration of a drug, especially propofol, should be notified to and discussed with the National Porphyria Centre (Centre Français des Porphyries). In case of symptomatic hepatic porphyria the use of propofol should always be notified to the National and to the European Porphyria Centres (Orphan Europe).
丙泊酚可在所有无症状性肝卟啉症病例中无任何限制地使用，作为单次推注用于诱导和维持麻醉，以及作为持续输注以产生短期和中期麻醉。如果对丙泊酚在症状性肝卟啉症情况下的绝对安全性存在疑问，则应优先选择神经安定镇痛、苯二氮䓬类药物和肌肉松弛剂。如果要使用丙泊酚，应随时准备好使用血红素进行治疗。在所有情况下，术前和术后测定卟啉及其前体至关重要。尿液收集应严格遵循推荐程序：无菌条件，避光和高温。给药后出现的任何异常反应，尤其是丙泊酚给药后，应通知法国国家卟啉病中心并与其讨论。对于症状性肝卟啉症病例，使用丙泊酚应始终通知法国国家和欧洲卟啉病中心（欧洲孤儿病组织）。
