急性间歇性卟啉病所致顽固性低钠血症的临诊应对  

Refractory hyponatremia caused by acute intermittent porphyria

作　　者：李青阳 任毅[1] 侯敬天[1] 刘云峰[1] 王彦[1] 杨静[1] Li Qingyang;Ren Yi;Hou Jingtian;Liu Yunfeng;Wang Yan;Yang Jing(Department of Endocrinology,First Hospital of Shanxi Medical University,Taiyuan 030001,China)

机构地区：[1]山西医科大学第一医院内分泌科,太原030001

出　　处：《中华内分泌代谢杂志》2022年第9期815-818,共4页Chinese Journal of Endocrinology and Metabolism

基　　金：山西省重点研发计划项目(201903D321048、201903D321127);山西省卫生计生委课题(2017043)。

摘　　要：低钠血症是急性间歇性卟啉病(acute intermittent porphyria,AIP)急性发作期常见的实验室异常,可引起癫痫、昏迷等不良事件的发生,危及患者的生命安全。目前,临床上主要采用糖负荷治疗以控制AIP的急性发作。但葡萄糖的应用又可引起低钠血症的加重。在缓解AIP症状的同时又能有效纠正低钠血症成为治疗的难点。本科收治1例3次就诊于本院的AIP病例,其顽固性低钠血症在短期应用小剂量托伐普坦后得以纠正。本文对其临床资料进行回顾性分析,以积累诊治经验。Hyponatremia is common in the attack of acute intermittent porphyria(AIP),which can cause epilepsy,coma and other adverse events and endanger the life of patients.Carbohydrate loading therapy is applied to control the attack of AIP in the clinic.But the application of glucose can exacerbate hyponatremia.It is difficult for clinicians to effectively correct hyponatremia while treating AIP with glucose.We reported a case of AIP whose refractory hyponatremia was corrected with short-term low-dose tolvaptan to improve knowledge in management.

关 键 词：急性间歇性卟啉病 低钠血症 抗利尿激素分泌异常综合征 托伐普坦 

分 类 号：R589.8[医药卫生—内分泌]