Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease).
Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease).
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DOI：
10.12659/ajcr.906514
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发表时间：
2018-02-16
期刊：
影响因子：
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通讯作者：
Druey KM
中科院分区：
文献类型：
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作者：
Bøe OW;Sveen K;Børset M;Druey KM
病人：女性，49岁最终诊断：全身性毛细血管渗漏综合征（SCLS）症状：低血压药物治疗：-临床操作：无专长：变态反应学罕见疾病全身性毛细血管渗漏综合征（SCLS）（克拉克森病）是一种病因不明的罕见疾病，其特征是短暂性低血压发作，液体微血管渗漏进入外周组织，导致水肿。80-90%的SCLS患者伴有单克隆丙种球蛋白病。尽管体外转化研究表明血管内皮屏障功能障碍与SCLS的病因有关，但临床病例中的病因和疾病相关性仍不清楚。我们报告一例49岁女性SCLS，最初表现为上呼吸道感染，并发四肢水肿和筋膜室综合征，需要进行筋膜切开术。通过酶联免疫测定法（ELISA）测量细胞表面硫酸乙酰肝素蛋白聚糖（syndecan-1，CD 138）的血清水平，这是一种内皮表面糖萼（ESG）损伤的指标，峰值高达500 ng/mL（参考范围，50-100 ng/mL），并在疾病缓解时标准化。该病例报告支持微血管内皮损伤在急性SCLS发病机制中起作用的观点。该病例还表明，监测血清syndecan-1（CD 138）水平可用于监测SCLS发作的进展和消退。
Patient: Female, 49 Final Diagnosis: Systemic capillary leak syndrome (SCLS) Symptoms: Hypotension Medication: — Clinical Procedure: None Specialty: Allergology Rare disease Systemic capillary leak syndrome (SCLS) (Clarkson’s disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80–90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown. We report a case of SCLS in a 49-year-old woman who initially presented with an upper respiratory tract infection, which was complicated by edema and compartment syndromes in the extremities that required fasciotomies. Serum levels of the cell surface heparan sulfate proteoglycan, syndecan-1 (CD138), a measure of endothelial surface glycocalyx (ESG) damage, were measured by enzyme-linked immunoassay (ELISA), peaked at up to 500 ng/mL (reference range, 50–100 ng/mL) and normalized on disease remission. This case report supports the view that damage to the microvascular endothelium, has a role in the pathogenesis of acute SCLS. This case also indicated that monitoring serum levels of syndecan-1 (CD138) might be used to monitor the progression and resolution of episodes of SCLS.