Medical Residents, Department of Internal Medicine, Ascension Saint Francis Hospital, 355 Ridge Ave, Evanston, IL, 60202, USA.
Pulmonary and Critical Care Attending, Department of Critical Care and Pulmonology, Ascension Saint Francis Hospital, Evanston, IL, USA.
CEN Case Rep. 2024 Feb;13(1):59-65. doi: 10.1007/s13730-023-00801-x. Epub 2023 Jun 5.
Calciphylaxis, also known as Calcific uremic arteriolopathy (CUA), is a serious disorder that presents with skin necrosis due to calcification of dermal and subcutaneous adipose tissue capillaries and arterioles. The condition occurs primarily in patients with end-stage renal disease (ESRD) on dialysis, and it carries high morbidity and mortality, primarily due to sepsis, with an estimated six-month survival of approximately 50%. Although there are no high-quality studies to guide the optimal treatment approach for patients with calciphylaxis, many retrospective studies and case series support treatment with sodium thiosulfate (STS). Despite the frequent use of STS as an off-label treatment, data regarding its safety and efficacy are limited. STS has generally been considered a safe drug with mild side effects. However, severe metabolic acidosis associated with STS is a rare and life-threatening complication of STS treatment and is often unpredictable. Herein, we report a 64-year-old female with ESRD on peritoneal dialysis (PD) who presented with a profound high anion gap metabolic acidosis and severe hyperkalemia while on STS treatment for CUA. No other etiology for her severe metabolic acidosis other than STS was identified. ESRD patients receiving STS should be monitored closely for this side effect. Dose reduction, increasing the duration of infusion, or even discontinuing STS treatment should be considered if severe metabolic acidosis develops.
钙化防御,也称为钙化尿毒症性小动脉病(CUA),是一种严重的疾病,由于皮肤和皮下脂肪组织毛细血管和小动脉的钙化导致皮肤坏死。这种情况主要发生在透析的终末期肾病(ESRD)患者中,发病率和死亡率都很高,主要是由于败血症,估计六个月的生存率约为 50%。虽然没有高质量的研究来指导钙化防御患者的最佳治疗方法,但许多回顾性研究和病例系列支持使用硫代硫酸钠(STS)治疗。尽管 STS 经常被用作标签外治疗,但关于其安全性和疗效的数据有限。STS 通常被认为是一种安全的药物,副作用轻微。然而,STS 治疗相关的严重代谢性酸中毒是 STS 治疗的一种罕见且危及生命的并发症,通常是不可预测的。在此,我们报告了一例 64 岁女性,患有 ESRD,正在接受腹膜透析(PD)治疗,在接受 STS 治疗 CUA 时出现严重阴离子间隙代谢性酸中毒和严重高钾血症。除 STS 外,没有发现其他导致其严重代谢性酸中毒的病因。接受 STS 治疗的 ESRD 患者应密切监测这种副作用。如果出现严重代谢性酸中毒,应考虑减少剂量、增加输注时间甚至停止 STS 治疗。