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特发性毛细血管渗漏综合征1例罕见病例

A Rare Case of Idiopathic Capillary Leak Syndrome.

作者信息

Rao Suman, Nasser Omar, Sunkara Akhila, Singhal Rishi, Manta Dragos

机构信息

Internal Medicine, State University of New York Upstate Medical University, Syracuse, USA.

Critical Care, State University of New York Upstate Medical University, Syracuse, USA.

出版信息

Cureus. 2021 Apr 8;13(4):e14370. doi: 10.7759/cureus.14370.

Abstract

Idiopathic capillary leak syndrome (ICLS) occurs as a result of vascular membrane instability, which results in the leakage of several proteins from the vascular compartment to the interstitial spaces. It is an extremely rare disorder, with around 260 cases documented thus far. We present a case of a 35-year-old male with a past medical history of asthma and gastroesophageal reflux disease who initially presented to our hospital for the treatment of chronic demyelinating inflammatory neuropathy requiring plasmapheresis and steroid therapy. After removal of his vascular catheter, he experienced sudden onset of dyspnea, hypotension, and respiratory distress. His lab work showed a hemoglobin of 21.3 g/dL and a hematocrit of 62.6%. Protein electrophoresis showed a mildly decreased albumin at 3.28 g/dL. These findings were consistent with ICLS. He required management with colloids and systemic steroids. The difficult diagnosis of ICLS is due to its overlap with several medical emergencies, such as sepsis and anaphylaxis. Further studies are required to study the role of steroids and colloids in the management of ICLS.

摘要

特发性毛细血管渗漏综合征(ICLS)是由血管膜不稳定引起的,这导致多种蛋白质从血管腔渗漏到间质间隙。它是一种极其罕见的疾病,迄今为止记录在案的病例约有260例。我们报告一例35岁男性病例,该患者有哮喘和胃食管反流病病史,最初因需要进行血浆置换和类固醇治疗的慢性脱髓鞘性炎性神经病变而到我院就诊。拔除血管导管后,他突然出现呼吸困难、低血压和呼吸窘迫。他的实验室检查显示血红蛋白为21.3 g/dL,血细胞比容为62.6%。蛋白电泳显示白蛋白轻度降低,为3.28 g/dL。这些发现与ICLS一致。他需要用胶体和全身性类固醇进行治疗。ICLS难以诊断是因为它与几种医疗急症重叠,如败血症和过敏反应。需要进一步研究以探讨类固醇和胶体在ICLS治疗中的作用。

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