背景:低钾性周期性麻痹是一种慢性疾病,其特征是与急性低钾血症相关的虚弱的偶发性发作。攻击通常与特定的触发器相关联,例如运动后长时间休息或食用高碳水化合物膳食。最常见的是,这种情况是由常染色体显性钙通道突变引起的,并且患者通常具有已确定的低钾性周期性麻痹家族史。长期并发症包括进行性近端肌病的发展。口服氯化钾可用于急性发作的治疗,给予乙酰唑胺或二氯苯甲酰胺作为长期预防。肾脏科医师可以在识别和治疗先前未诊断的低钾性周期性瘫痪中发挥重要作用。
方法:我们总结了一名19岁的白人男子的病例,该男子到急诊科就诊,未确诊为低钾性周期性麻痹发作,谁报告了,在后续行动中,用二氯苯甲酰胺改善发作的严重程度和频率。
结论:这个案例证明了肾脏病学家可以发挥的关键作用,不仅在低钾性周期性麻痹的诊断中,而且在这种情况的持续管理中。由于发生肌病的风险,应建议患者定期随访其肾脏病小组进行评估。
BACKGROUND: Hypokalemic periodic paralysis is a chronic condition characterized by sporadic attacks of weakness associated with acute hypokalemia. Attacks are typically associated with specific triggers, such as prolonged rest following exercise or consumption of a high-carbohydrate meal. Most commonly, this condition is caused by an autosomal dominant calcium channel mutation, and patients typically have an established family medical history of hypokalemic periodic paralysis. Long-term complications include the development of progressive proximal myopathy. Oral potassium chloride may be considered for the treatment of an acute attack, with administration of acetazolamide or
dichlorphenamide as long-term prophylaxis. Nephrologists can play an important role in the recognition and treatment of previously undiagnosed hypokalemic periodic paralysis.
METHODS: We summarize the
case of a 19-year-old white man who presented to the emergency department with undiagnosed attacks of hypokalemic periodic paralysis, and who reported, at follow-up, improvement in the severity and frequency of attacks with
dichlorphenamide.
CONCLUSIONS: This
case demonstrates the crucial role nephrologists can play, not only in the diagnosis of hypokalemic periodic paralysis, but also in the ongoing management of this condition. Patients should be advised to regularly follow up with their nephrology team for evaluation due to the risk of developing myopathy.