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特发性全身性毛细血管渗漏综合征(克拉克森病)表现为反复发作的低血容量性休克。

Idiopathic systemic capillary leak syndrome (Clarkson's disease) presenting with recurrent hypovolemic shock.

机构信息

MBChB (Hons) MRes, Clinical Research Fellow in Rheumatology Northumbria Speciailist Emergency Care Hospital, Cramlington, NE23 6NZ.

Advanced Critical Care Practitioner, Northumbria Speciailist Emergency Care Hospital, Cramlington, NE23 6NZ.

出版信息

Acute Med. 2021;20(1):74-77.

PMID:33749696
Abstract

A 49-year old male with a past medical history of myocardial infarction and compartment syndromes requiring fasciotomies presented on five occasions with hypovolemic shock. We describe his admissions and presumptive diagnoses which required large volumes of intravenous fluids, admission to intensive care for vasopressors and renal replacement therapy. The presentations were always precipitated by a prodrome of fatigue and pre-syncopal episodes. On his last admission, a diagnosis of Idiopathic systemic capillary leak syndrome (ISCLS), also known as Clarkson's Disease, was reached. He is currently receiving high dose intravenous immunoglobulins on a monthly basis.

摘要

一位 49 岁的男性,既往有心肌梗死和筋膜切开术治疗的间隔综合征病史,曾五次因低血容量性休克就诊。我们描述了他的住院情况和推测诊断,这些情况需要大量静脉输液、入住重症监护病房使用升压药和肾脏替代治疗。这些发作总是由疲劳和晕厥前发作的前驱症状引发。在他最后一次住院时,诊断为特发性全身毛细血管渗漏综合征(ISCLS),也称为克拉克森病。他目前每月接受高剂量静脉注射免疫球蛋白治疗。

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Idiopathic systemic capillary leak syndrome (Clarkson's disease) presenting with recurrent hypovolemic shock.特发性全身性毛细血管渗漏综合征(克拉克森病)表现为反复发作的低血容量性休克。
Acute Med. 2021;20(1):74-77.
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A case report of systemic capillary leak syndrome (Clarkson's disease).全身性毛细血管渗漏综合征(克拉克森病)病例报告。
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引用本文的文献

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Systemic capillary leak syndrome.全身性毛细血管渗漏综合征。
Nat Rev Dis Primers. 2024 Nov 14;10(1):86. doi: 10.1038/s41572-024-00571-5.
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Idiopathic systemic capillary leak syndrome: a case report.特发性全身性毛细血管渗漏综合征:一例报告。
BMC Nephrol. 2023 Mar 25;24(1):72. doi: 10.1186/s12882-023-03122-4.