Department of Internal Medicine, University Hospital Bern, 3010 Bern, Switzerland.
Eur J Intern Med. 2006 Aug;17(5):363-5. doi: 10.1016/j.ejim.2005.12.011.
The systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a rare disorder characterized by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. A 35-year-old previously healthy woman was admitted with rapidly developing hypovolemic shock syndrome, rhabdomyolysis, and diffuse edema. Laboratory analysis revealed a severe hemoconcentration, renal insufficiency, and paraproteinemia. After exclusion of infection, allergy, burning or drug-induced conditions, the clinical presentation was consistent with the diagnosis of SCLS. Though this is a rare entity, the substantial morbidity and mortality associated with it necessitate the physician's awareness in order to provide timely therapy. This report is meant to enhance awareness of SCLS.
全身性毛细血管渗漏综合征（SCLS），也称为克拉克森病，是一种罕见的疾病，其特征是阵发性毛细血管通透性增加，导致血浆液体从血管内转移到间质空间。一名 35 岁的既往健康女性因迅速发展的低血容量性休克综合征、横纹肌溶解症和弥漫性水肿入院。实验室分析显示严重的血液浓缩、肾功能不全和副蛋白血症。排除感染、过敏、烧伤或药物诱导的情况后，临床表现与 SCLS 的诊断一致。尽管这是一种罕见的疾病，但与之相关的高发病率和死亡率需要医生意识到，以便及时提供治疗。本报告旨在提高对 SCLS 的认识。
