儿科
词汇介绍
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解析
hereditary 英 /həˈredɪtri/ 美 /həˈredɪteri/
释 义 n. 遗传类
adj. 遗传的;世袭的;世代相传的
短 语 hereditary tremor 遗传性震颤
hereditary enzymopathy 遗传性酶病
hereditary coproporphyria 遗传性粪卟啉病
例 句 The susceptibility may be familial and hereditary. 易感性具有家族性和遗传性。
spherocytosis 英 /'sfiərəsi'təusis/ 美 /'sfiərəsi'təusis/
释 义 n. [医] 球形红细胞症
例 句 The sinusoids are packed with RBC's in this case of hereditary spherocytosis. The osmotic fragilityof spherocytes is increased, because the RBC's have decreased surface area per unit volume. 遗传性球形红细胞增多症中,窦状隙被RBC塞满,球形红细胞的渗透性脆性增加,因为RBC每单位体积的表面积减少。
概述
红细胞密度、异质性和变形性作为遗传性球形红细胞增多症临床严重程度的标志物
发表时间:2019-05-30
影响因子:7.6
作者: Huisjes Rick
期刊:Haematologica
Hereditary spherocytosis originates from defective anchoring of the cytoskeletal network to the transmembrane protein complexes of the red blood cell. Red cells in hereditary spherocytosis are characterized by membrane instability, reduced deformability and a large heterogeneity in disease severity among patients. To unravel this variability in disease severity, we analyzed blood samples from 21 spherocytosis patients with defects in ankyrin, band 3, α-spectrin and β-spectrin using red cell indices, eosin-5-maleimide binding, microscopy and the osmotic fragility test, Percoll density gradients, vesiculation and ektacytometry to assess cell membrane stability, cellular density and deformability. Reticulocyte counts, CD71 abundance, band 4.1 a:b ratio, and HbA1c were used as markers of red blood cell turnover. We observed that patients with moderate/severe spherocytosis have short-living erythrocytes of low density and abnormally high intercellular heterogeneity.
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