论文部分内容阅读
遗传性球形红细胞增多症(Hereditary Spherocytosis,HS)是一种常染色体显性遗传性红细胞膜缺陷性疾病。血中球形红细胞明显增多和红细胞渗透脆性增加是HS的主要特征。据统计,美国的HS的发病率为22/10万。在我国,HS也不少见。在先天性溶血性贫血中,其发病率最高。近几年,在HS的发病机理、诊断和治疗等方面的研究有了较大进展。本文就此作一简单的文献复习。正常红细胞膜结构正常人类红细胞膜主要由两大部分所组成,即双脂层和双脂层周围的大量蛋白质。二者共同组成了红细胞膜的双脂层结构,而后者
Hereditary spherocytosis (Hereditary Spherocytosis, HS) is an autosomal dominant inherited erythrocyte membrane disease. Significant increase in blood globular erythrocytes and erythrocyte infiltration fragility is the main feature of HS. According to statistics, the incidence of HS in the United States is 22 / 100,000. In our country, HS is not uncommon. In congenital hemolytic anemia, the highest incidence. In recent years, great progress has been made in the research on the pathogenesis, diagnosis and treatment of HS. This article for a simple review of the literature. Normal erythrocyte membrane structure Normal human erythrocyte membrane is mainly composed of two parts, namely, a large number of double-lipid layer and lipid layer around the protein. The two together form the double lipid layer structure of the erythrocyte membrane, while the latter