Castro Tavares J, Maciel L
Department of Anaesthesia, Hospital de S. João, Porto, Portugal.
Can J Anaesth. 1989 Mar;36(2):209-11. doi: 10.1007/BF03011446.
Although familial amyloid polyneuropathy of the Portuguese type (FAP-PT) was first described in 1952, there is little in the medical literature detailing the anaesthetic management of such patients. FAP-PT is a disease with multiple clinical manifestations which include disturbances of sensibility, progressive paresis starting in the lower extremities, autonomic dysfunction, cardiac conduction disturbances, gastro-intestinal disorders, nephrotic syndrome, sexual and sphincter disorders, extreme emotionalism and apprehension. Several intermingling problems have to be considered in the anaesthetic management of each individual case. In our patient a sinus dysrhythmia resolved after isoflurane and this seems to be a good choice for general anaesthesia in patients with FAP-PT, if they are in an early stage of heart involvement.
尽管葡萄牙型家族性淀粉样多神经病（FAP-PT）于1952年首次被描述，但医学文献中鲜有详细阐述此类患者麻醉管理的内容。FAP-PT是一种具有多种临床表现的疾病，包括感觉障碍、始于下肢的进行性麻痹、自主神经功能障碍、心脏传导紊乱、胃肠疾病、肾病综合征、性功能和括约肌功能障碍、极度情绪波动和焦虑。在每个病例的麻醉管理中都必须考虑几个相互交织的问题。在我们的患者中，异氟烷麻醉后窦性心律失常得到缓解，对于处于心脏受累早期的FAP-PT患者，这似乎是全身麻醉的一个不错选择。
