A case report of Clarkson's disease: If you don't know it, you'll miss it
A case report of Clarkson's disease: If you don't know it, you'll miss it
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DOI：
10.1016/j.ejim.2005.12.011
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发表时间：
2006-08-01
影响因子：
8
通讯作者：
Mueller, Beatrice U.
中科院分区：
文献类型：
--
作者：
Bonadies, Nicolas;Baud, Patricia;Mueller, Beatrice U.
系统性毛细血管渗漏综合征（SCLS），也被称为克拉克森病，是一种罕见的疾病，其特征是阵发性毛细血管高通透性，血浆从血管内转移到间隙。一名35岁的健康女性因快速发展的低血容量性休克综合征、横纹肌溶解和弥漫性水肿入院。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。在排除感染、过敏、灼烧或药物引起的情况后，临床表现符合scs的诊断。虽然这是一个罕见的实体，大量的发病率和死亡率与它相关，需要医生的意识，以便提供及时的治疗。本报告旨在提高对scs的认识。(C) 2006年欧洲内科联合会。Elsevier b.v.版权所有。
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. (C) 2006 European Federation of Internal Medicine. Published by Elsevier B.V All rights reserved.