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Plasma PTX3 levels in sickle cell disease patients, during vaso occlusion and acute chest syndrome (data from Saudi population)

机译:在血管闭塞和急性胸腔综合征期间,镰状细胞病患者的血浆PTX3水平(来自沙特人群的数据)

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Background: Sickle cell disease (SCD) is a chronic, incurable hereditary disease. The vaso-occlusive crisis (VOC) is the most frequently occurring acute complication in sickle cell patients and accounts for the majority of SCD-related hospital admissions. Another major complication is the potentially fatal acute chest syndrome (ACS). The prototypic long pentraxin-3 (PTX3), an acute phase protein and a key component of innate immunity, is linked to ischemia-induced inflammation, a condition incriminated in SCD complications. Aim: To investigate the expression of PTX3 in stable SCD and VOC patients and to assess its relation to the development and progression of ACS. Subjects and methods: We conducted this study on 160 patients with confirmed SCD (20 stable SCD and 140 in VOC), and 10 healthy age- and sex-matched controls. Patients were diagnosed as SCD by highperformance liquid chromatography. PTX3 levels were assessed using enzyme-linked immunosorbant assay. Results: In the stable state, all 20 SCD patients had PTX3 levels (range = 0.9-2.1 ng/ml; median = 1.1) comparable to those of healthy controls (range = 0.8-2.0 ng/ml; median = 1.0) (P > 0.05). During the VOC, plasma PTX3 significantly increased (range = 8.7-37.2 ng/ml; median = 22.3) (P < 0.01). Out of 140 VOC patients, 15 (10.7%) developed ACS and four required mechanical ventilation, of which two died. The median plasma level of PTX3 (22.3 ng/ml) was set as a cut-off value to stratify patients into low- and high-PTX3 expressers. Of the 140 VOC patients, 43 (30.7%) had PTX3 levels >22.3 ng/ml, of these, 13 patients developed ACS (13/43; 30.2%); of the remaining 97 patients who had PTX3 ≤22.3 ng/ml, only two patients (2/97; 2.1%) progressed to ACS, with a further increment in PTX3 in all of them. PTX3 levels were correlated with length of hospital stay in VOC patients and markers of lung injury in ACS patients. Conclusion: PTX3 levels were higher in SCD patients in VOC, being associated with longer hospital stay. Higher initial PTX3 concentrations were related to the development of ACS with a further increase in PTX3 levels observed upon progression to ACS. Thus, PTX3 could be used as a subjective method to predict occurrence and severity of SCD acute complications.
机译:背景:镰状细胞病(SCD)是一种慢性,无法治愈的遗传性疾病。血管闭塞性危机(VOC)是镰状细胞患者中最常见的急性并发症,占SCD相关医院入院的大多数。另一个主要并发症是可能致命的急性胸综合症(ACS)。原型长五聚体蛋白3(PTX3)是一种急性期蛋白,是先天免疫的关键组成部分,与缺血诱导的炎症有关,后者是SCD并发症的病因。目的:研究PTX3在稳定的SCD和VOC患者中的表达,并评估其与ACS发生和发展的关系。受试者和方法:我们对160例确诊为SCD的患者(20例稳定的SCD和VOC中的140例)以及10例年龄和性别匹配的健康对照者进行了这项研究。通过高效液相色谱法将患者诊断为SCD。使用酶联免疫吸附测定法评估PTX3水平。结果:在稳定状态下,所有20名SCD患者的PTX3水平(范围= 0.9-2.1 ng / ml;中位数= 1.1)与健康对照组(范围= 0.8-2.0 ng / ml;中位数= 1.0)相当(P > 0.05)。在VOC期间,血浆PTX3显着增加(范围= 8.7-37.2 ng / ml;中位数= 22.3)(P <0.01)。在140例VOC患者中,有15例(10.7%)患了ACS,其中4例需要机械通气,其中2例死亡。将PTX3的血浆中值(22.3 ng / ml)设置为临界值,以将患者分为低和高PTX3表达体。在140例VOC患者中,有43例(30.7%)PTX3水平> 22.3 ng / ml,其中13例发展为ACS(13/43; 30.2%)。在其余97名PTX3≤22.3ng / ml的患者中,只有2名患者(2/97; 2.1%)进展为ACS,所有患者中PTX3进一步升高。 PTX3水平与VOC患者的住院时间和ACS患者的肺损伤指标相关。结论:VOC SCD患者中PTX3水平较高,与住院时间长有关。较高的初始PTX3浓度与ACS的发展有关,在进展为ACS时观察到PTX3的水平进一步升高。因此,PTX3可作为主观方法来预测SCD急性并发症的发生和严重程度。

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