Narrative review: the systemic capillary leak syndrome.

Narrative review: the systemic capillary leak syndrome.
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DOI:
10.7326/0003-4819-153-2-201007200-00005
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发表时间:
2010-07-20
影响因子:
39.2
通讯作者:
Greipp PR
Greipp PR
中科院分区:
医学1区
文献类型:
--
作者:
Druey KM;Greipp PR

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1960 年,贝亚德·克拉克森 (Bayard Clarkson) 医生描述了一位患有偶发性低血容量、低血压和水肿的患者。当大鼠全身给予急性发作期间的血浆时,会引发类似“休克”的综合征。以克拉克森博士命名的不寻常且神秘的“系统性毛细血管渗漏综合征”(SCLS) 病因不明,其特点是由于液体和大分子(高达 900 kDa)渗漏到组织中而导致短暂、严重、可逆的血液浓缩和低蛋白血症。自 1960 年以来,报道的 SCLS 病例不到 150 例,但非特异性的症状和体征以及高死亡率可能导致人们对这种疾病的认识不足。鉴于 SCLS 与其他“休克”综合征(包括脓毒症、过敏反应和血管性水肿)有很大重叠,临床医生应在出现不明原因水肿、血细胞比容升高和低血压的患者中考虑这一诊断。
In 1960, Dr. Bayard Clarkson described a patient experiencing sporadic episodes of hypovolemia, hypotension, and edema. Plasma during the acute attack induced a “shock”-like syndrome when given systemically in rats. The unusual and enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is of unknown etiology and is characterized by transient, severe, reversible hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules (up to 900 kDa) into tissues. Fewer than 150 cases of SCLS have been reported since 1960, but the nonspecific presenting symptoms and signs and high mortality rate may have resulted in under-recognition of this disorder. Given the substantial overlap of SCLS with other “shock” syndromes, including sepsis, anaphylaxis, and angioedema, clinicians should consider this diagnosis in patients with unexplained edema, increased hematocrit, and hypotension.
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