Systemic capillary leak syndrome

Systemic capillary leak syndrome
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DOI:
10.2169/internalmedicine.41.953
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发表时间:
2002-11-01
期刊:
影响因子:
1.2
通讯作者:
Suzuki, H
Suzuki, H
中科院分区:
医学4区
文献类型:
--
作者:
Chihara, R;Nakamoto, H;Suzuki, H

文献摘要

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系统性毛细血管渗漏综合征(SCLS)的特征是由于全身毛细血管渗漏增加而引起的复发性低血容量性休克。一名46岁男性因反复发作的全身性水肿伴低血容量性休克而入院。血液实验室数据显示严重的低蛋白血症伴小单克隆IgG-kappa蛋白。这些结果有力地提示了scs的诊断。尝试了类固醇脉冲治疗方案;然而,血管内超载伴随着最初的外渗液体的补充导致急性肺水肿。重症监护和仔细监测液体量在scs是必需的。
Systemic capillary leak syndrome (SCLS) is characterized by recurrent hypovolemic shock attributable to increased systemic capillary leakage. A 46-year-old man was admitted to our hospital because of recurrent episodes of generalized edema with hypovolemic shock. Blood laboratory data revealed severe hypoproteinemia with a small monoclonal IgG-kappa protein. These findings suggested strongly the diagnosis of SCLS. A regimen with steroid pulse therapy was tried; however, intravascular overloading accompanied by the recruitment of the initially extravasated fluids resulted in acute pulmonary edema. Intensive care and careful monitoring of fluid volume are required in SCLS.