表现为感染性休克的特发性系统性毛细血管渗漏综合征：一例报告。
Idiopathic systemic capillary leak syndrome presenting as septic shock: A case report.
作者信息
Raith Eamon P, Ihle Joshua F, Jamieson Jennifer, Kalff Anna, Bosco Julian
机构信息
Department of Intensive Care Medicine, The Royal Adelaide Hospital, Port Road, Adelaide, SA 5000, Australia; School of Public Health and Preventative Medicine, Monash University, Melbourne, VIC, Australia; Discipline of Acute Care Medicine, School of Medicine, The University of Adelaide, Adelaide, SA, Australia.
School of Public Health and Preventative Medicine, Monash University, Melbourne, VIC, Australia; Department of Intensive Care and Hyperbaric Medicine, The Alfred Hospital, Commercial Road, Prahran, Melbourne, VIC 3181, Australia.
出版信息
Heart Lung. 2018 Jul-Aug;47(4):425-428. doi: 10.1016/j.hrtlng.2018.04.008. Epub 2018 May 18.
BACKGROUND
Idiopathic capillary leak syndrome (Clarkson's Disease) is a rare angiopathy with a heterogenous phenotype that may present as distributive shock refractory to resuscitative management.
OBJECTIVE
We report a case of idiopathic systemic capillary leak syndrome presenting as septic shock.
METHODS
Structured case report and review of the literature.
RESULTS
A 27-year old man admitted to our institution with coryzal symptoms rapidly deteriorated with presumed sepsis, leading to intensive care unit admission. Following further deterioration, Idiopathic systemic capillary leak syndrome was considered and intravenous immunoglobulin administered, resulting in rapid improvement in the patient's clinical status.
CONCLUSIONS
Idiopathic systemic capillary leak syndrome is a rare and potentially life-threatening angiopathy that may present as, and should be considered in, refractory distributive shock. Administration of intravenous immunglobulin resulted in rapid recovery in this patient, and has been associated with positive outcomes in previous cases.
背景
特发性毛细血管渗漏综合征（克拉克森病）是一种罕见的血管病，具有异质性表型，可表现为对复苏治疗无效的分布性休克。
目的
我们报告一例表现为脓毒性休克的特发性全身性毛细血管渗漏综合征病例。
方法
结构化病例报告及文献回顾。
结果
一名27岁男性因感冒症状入院，很快因疑似脓毒症而病情恶化，被收入重症监护病房。病情进一步恶化后，考虑为特发性全身性毛细血管渗漏综合征，并给予静脉注射免疫球蛋白，患者临床状况迅速改善。
结论
特发性全身性毛细血管渗漏综合征是一种罕见且可能危及生命的血管病，可表现为难治性分布性休克，对此应予以考虑。静脉注射免疫球蛋白使该患者迅速康复，在之前的病例中也与良好预后相关。
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