Gordon K E, Ludman M D, Finley G A
Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada.
Pediatr Neurol. 1995 Apr;12(3):250-1. doi: 10.1016/0887-8994(95)00007-3.
Fabry disease is an X-linked disorder characterized in childhood by angiokeratoma, corneal opacities, and pain. At age 7 years our patient began experiencing an intermittent intense "burning" sensation within his feet and hands (acroparesthesias). Treatment with aspirin, acetaminophen, acetominophen with codeine, and phenytoin was unsuccessful. Carbamazepine and phenytoin reduced the frequency and duration of painful crises to 3-4 times annually. A treatment plan was developed consisting of a low-dose morphine infusion with increasing dosage until pain was relieved. Over the subsequent 28 months, we have had experienced treating 7 crises with morphine given as 0.06 mg/kg IV push, followed by a continuous infusion of 0.02 mg/kg/hr with amitriptyline 0.25 mg/kg at bedtime. Pain control is immediate, with the infusion gradually tapered after 24 hours.
法布里病是一种X连锁疾病，在儿童期以血管角质瘤、角膜混浊和疼痛为特征。我们的患者在7岁时开始在手脚部位间歇性地出现强烈的“灼烧”感（肢端感觉异常）。使用阿司匹林、对乙酰氨基酚、含可待因的对乙酰氨基酚以及苯妥英钠进行治疗均未成功。卡马西平和苯妥英钠将疼痛发作的频率和持续时间降低至每年3 - 4次。制定了一个治疗方案，包括低剂量吗啡输注，剂量逐渐增加直至疼痛缓解。在随后的28个月里，我们使用0.06 mg/kg静脉推注吗啡，随后以0.02 mg/kg/小时持续输注，并在睡前给予0.25 mg/kg阿米替林，共治疗了7次疼痛发作。疼痛得到了即时控制，输注在24小时后逐渐减量。
