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考虑单克隆丙种球蛋白病合并休克时的系统性毛细血管渗漏综合征。

Consider systemic capillary leak syndrome in monoclonal gammopathy with shock.

作者信息

Bouchlarhem Amine, Lamzouri Oussama, El Aidouni Ghizlane, Haddar Leila, Mimouni Hamza, Bkiyar Houssam, Housni Brahim

机构信息

Department of Anesthesiology and Intensive Care Unit, Mohammed VI University Hospital, Mohammed I University, Oujda, Morocco.

Faculty of Medicine and Pharmacy, Mohammed I University, Oujda, Morocco.

出版信息

Ann Med Surg (Lond). 2021 Nov 2;72:103013. doi: 10.1016/j.amsu.2021.103013. eCollection 2021 Dec.

DOI:10.1016/j.amsu.2021.103013
PMID:34824836
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8604768/
Abstract

INTRODUCTION AND IMPORTANCE

The cappilary leacking syndrome is a very rare disease that can be idiopathic (clarkson syndrome) or secondary to other pathologys.

CASE PRESENTATION

We report a case of 37-year-old women who was admitted in the emergency room for a hemodynamic shock of neither cardiac nor septic cause, and the patient wasn't presenting any bleeding. The investigations showed that the diagnosis was a Clarkson syndrome crisis and the patient was having supportive treatment containing fluid therapy, vasoactive drugs, and ECMO. And died after 48h of hospitalization.

CLINICAL DISCUSSION

the cappilary leacking syndrome is a very fatal affection, its physiopathologis remains unknown. It evoluate by crisis made by hypotension and anasarca, in severe cases it is presented as fatal hypovolemic schock. Biological investigations show hemoconcentration associated with hypoalbunemia which is pathognomonic of the disease. The treatment is essentially based on crisis treatment support by fluid therapy, vasoactives drugs, some practicien report the use of theophilyn for prevention but without any proven efficiency.

CONCLUSION

For all this reasons we are in the obligation of investing in fundamental studies to better understand this fatal disease.

摘要

引言与重要性

毛细血管渗漏综合征是一种非常罕见的疾病,可为特发性(克拉克森综合征)或继发于其他病理状况。

病例介绍

我们报告一例37岁女性,因非心源性或感染性原因导致的血流动力学休克入住急诊室,且患者无出血表现。检查显示诊断为克拉克森综合征危象,患者接受了包括液体治疗、血管活性药物和体外膜肺氧合(ECMO)的支持治疗。住院48小时后死亡。

临床讨论

毛细血管渗漏综合征是一种非常致命的病症,其病理生理机制尚不清楚。它以低血压和全身性水肿引发的危象形式发展,严重时表现为致命的低血容量性休克。实验室检查显示血液浓缩伴低白蛋白血症,这是该疾病的特征性表现。治疗主要基于通过液体治疗、血管活性药物对危象进行支持治疗,一些医生报告使用氨茶碱进行预防,但尚无任何经证实的疗效。

结论

基于所有这些原因,我们有义务投入基础研究以更好地了解这种致命疾病。

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