特发性系统性毛细血管渗漏综合征：一例报告
Idiopathic Systemic Capillary Leak Syndrome: A Case Report.
作者信息
Yardimci Bulent, Kazancioglu Rumeyza
机构信息
Department of Internal Medicine, Istanbul Florence Nightingale Hospital, Istanbul, Turkey.
Department of Nephrology, Bezmialem Vakif University, Faculty of Medicine, Istanbul, Turkey.
出版信息
Iran Red Crescent Med J. 2016 Feb 20;18(2):e29249. doi: 10.5812/ircmj.29249. eCollection 2016 Feb.
INTRODUCTION
Idiopathic systemic capillary leak syndrome (ISCLS) is rarely seen, and presents with recurrent episodes of hypotension, shock, hemoconcentration, and hypoproteinemia. The main pathology is the dysfunction of the vascular endothelium, and it is characterized by an increase of capillary permeability that is accompanied by the loss of intravascular fluid and protein.
CASE PRESENTATION
We present a 58-year-old female who presented with peripheral edema, leg pain, and syncope at the emergency department. Interestingly demyemilising neuropathy, which is a rare finding, ensued on day 4. She is still being treated using intravenous immunoglobulin therapy.
CONCLUSIONS
The early signs and symptoms of ISCLS may be subtle; therefore the diagnosis can easily be missed and prompt treatment of the syndrome may be postponed. Thus, the clinician must consider ISCLS in differential diagnosis in cases of hypotension, hemoconcentration, and hypoalbuminemia.
引言
特发性系统性毛细血管渗漏综合征（ISCLS）较为罕见，表现为反复出现的低血压、休克、血液浓缩和低蛋白血症。主要病理是血管内皮功能障碍，其特征是毛细血管通透性增加，伴有血管内液体和蛋白质丢失。
病例介绍
我们报告一名58岁女性，在急诊科表现为外周水肿、腿痛和晕厥。有趣的是，在第4天出现了罕见的脱髓鞘性神经病变。她仍在接受静脉注射免疫球蛋白治疗。
结论
ISCLS的早期体征和症状可能不明显；因此，诊断很容易被遗漏，该综合征的及时治疗可能会被推迟。因此，临床医生在低血压、血液浓缩和低白蛋白血症病例的鉴别诊断中必须考虑ISCLS。
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