大剂量静脉注射免疫球蛋白：治疗特发性系统性毛细血管渗漏综合征的一种有前景的方法。
High-dose intravenous immunoglobulins: a promising therapeutic approach for idiopathic systemic capillary leak syndrome.
作者信息
Zipponi Manuel, Eugster Roland, Birrenbach Tanja
机构信息
Department of General Internal Medicine, Inselspital Bern, University Hospital Bern, Bern, Switzerland.
出版信息
BMJ Case Rep. 2011 May 3;2011:bcr1220103599. doi: 10.1136/bcr.12.2010.3599.
Abstract
The systemic capillary leak syndrome (SCLS), also known as Clarkson's disease, is a life-threatening disorder of unknown cause. It is characterised by recurrent acute episodes of hypotension, weight gain and generalised oedema with haemoconcentration and hypoproteinaemia caused by paroxysmal capillary hyperpermeability with a shift of plasma fluid from the intravascular to the interstitial space. We report the case of a 40-year-old woman with chronic SCLS treated with high-dose intravenous immunoglobulins, after a prophylactic therapy with theophylline and terbutaline was poorly tolerated and failed to decrease the frequency and severity of the attacks sufficiently.
摘要
系统性毛细血管渗漏综合征（SCLS），也称为克拉克森病，是一种病因不明的危及生命的疾病。其特征为反复发作的急性低血压、体重增加和全身性水肿，并伴有血液浓缩和低蛋白血症，这是由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间隙引起的。我们报告了一例40岁慢性SCLS女性患者的病例，该患者在接受茶碱和特布他林预防性治疗后耐受性差且未能充分降低发作频率和严重程度，随后接受了大剂量静脉注射免疫球蛋白治疗。
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