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两名先天性无纤维蛋白原血症患者的血浆黏度模式和红细胞聚集。

Plasma viscosity pattern and erythrocyte aggregation in two patients with congenital afibrinogenemia.

机构信息

Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties.

Department of Psychology, Educational Science and Human Movement, Università degli Studi di Palermo, Palermo, Italy.

出版信息

Blood Coagul Fibrinolysis. 2020 Jul;31(5):330-332. doi: 10.1097/MBC.0000000000000906.

Abstract

: In this case report, we examine the behavior of plasma viscosity, explored at high and low shear rates, and erythrocyte aggregation in two patients with congenital afibrinogenemia, a clinical disorder firstly described in 1920 and that has an estimated incidence of 1 : 1-200 0000. The two hemorheological parameters examined by us showed a marked decrease in both patients, in one of whom erythrocyte aggregation was even undetectable. Keeping in mind that spontaneous thrombosis (venous and arterial) has been often described in congenital afibrinogenemia, it can be hypothesized that the decrease in plasma viscosity and erythrocyte aggregation might cause a reduction of the endothelial synthesis and release of nitric oxide through the fall of the wall shear stress.

摘要

在本病例报告中,我们检查了两名先天性无纤维蛋白原血症患者的血浆粘度(高剪切率和低剪切率下)和红细胞聚集行为。先天性无纤维蛋白原血症是一种临床疾病,于 1920 年首次描述,其发病率估计为 1:10000000-20000000。我们检查的两个血液流变学参数在两名患者中均明显降低,其中一名患者的红细胞聚集甚至无法检测到。鉴于先天性无纤维蛋白原血症常伴有自发性血栓形成(静脉和动脉),可以假设血浆粘度和红细胞聚集的降低可能通过降低壁切应力导致内皮合成和释放一氧化氮减少。

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