葡萄糖-6-磷酸脱氢酶缺乏症患者在二巯基磺酸钠治疗肝豆状核变性期间发生溶血：两例首次报告
Haemolysis during sodium dimercaptosulphonate therapy for Wilson's disease in G6PD-deficient patients: First report of two cases.
作者信息
Lai S, Huang Y Q, Liu A Q, Wu H W
机构信息
Department of Pharmacy, the First Affiliated Hospital of Guangdong Pharmaceutical University, Guangzhou, China.
Department of Clinical Pharmacy, Guangdong Pharmaceutical University, Guangzhou, China.
出版信息
J Clin Pharm Ther. 2017 Dec;42(6):783-785. doi: 10.1111/jcpt.12576. Epub 2017 Jun 20.
WHAT IS KNOWN AND OBJECTIVE
Wilson's disease (WD) is an inherited disorder in which defective biliary excretion of copper leads to its accumulation. Sodium dimercaptosulphonate (DMPS) is used as the primary therapy in China.
CASE DESCRIPTION
We report two cases, with WD and G6PD deficiency, who developed haemolysis on treatment with DMPS, without any other known risk. After withdrawal of DMPS and administration of dexamethasone and packed red blood cells, the patients recovered.
WHAT IS NEW AND CONCLUSION
Clinicians should keep in mind haemolysis as a potentially life-threatening side effect of DMPS in patients with G6PD.
已知信息与目的
威尔逊病（WD）是一种遗传性疾病，其铜的胆汁排泄存在缺陷，导致铜蓄积。在中国，二巯基丙磺酸钠（DMPS）被用作主要治疗药物。
病例描述
我们报告了两例患有WD和葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症的患者，他们在接受DMPS治疗时发生了溶血，且无其他已知风险因素。停用DMPS并给予地塞米松和浓缩红细胞后，患者康复。
新发现与结论
临床医生应牢记，溶血是DMPS在G6PD缺乏症患者中可能危及生命的副作用。
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