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克拉克森病 1 例报告:不识庐山真面目,只缘身在此山中。

A case report of Clarkson's disease: If you don't know it, you'll miss it.

机构信息

Department of Internal Medicine, University Hospital Bern, 3010 Bern, Switzerland.

出版信息

Eur J Intern Med. 2006 Aug;17(5):363-5. doi: 10.1016/j.ejim.2005.12.011.

Abstract

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.

摘要

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。

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