Senior PG Registrar, Department of Endocrinology and Metabolism, Christian Medical College, Vellore, India.
Associate Professor, Department of Endocrinology and Metabolism, Christian Medical College, Vellore, India.
Trop Doct. 2021 Apr;51(2):246-248. doi: 10.1177/0049475520979299. Epub 2020 Dec 22.
Idiopathic systemic capillary leak syndrome (ISCLS) is a potentially fatal disorder characterised by 'attacks' of varying intensity of hypovolemic shock in association with haemoconcentration and hypoalbuminaemia. It is a disease of exclusion, and the severity of attacks may mimic sepsis at presentation. We report a case of a lady with recurrent attacks of ISCLS with at least two life-threatening episodes, having been treated elsewhere as a case of steroid insufficiency. The diagnosis is often challenging, and treatment of an acute episode involves the judicious use of fluids and vasopressors, as required. Prophylaxis to prevent further attacks is of varied success.
特发性全身性毛细血管渗漏综合征（ISCLS）是一种潜在致命的疾病，其特征是在血液浓缩和低白蛋白血症的情况下出现不同强度的低血容量性休克“发作”。它是一种排他性疾病，发作的严重程度在出现时可能类似于败血症。我们报告了一例患有反复发作的 ISCLS 的女性患者，至少有两次危及生命的发作，曾在其他地方被误诊为类固醇功能不全。该诊断通常具有挑战性，急性发作的治疗需要合理使用液体和血管加压素。预防进一步发作的预防措施效果不一。
