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Sickle-cell anaemia镰状细胞贫血

Sickle-cell disease is an inherited blood disorder in which the red blood cells change shape in a characteristic way.镰状细胞病是一种遗传性血液紊乱,其中红血细胞的变化形成的一个特点。

Haemoglobin is a special protein in red blood cells that carries oxygen around the bloodstream.血红蛋白是一种特殊的蛋白在红血球携带氧气周围的血液。 In sickle-cell disease, the haemoglobin is abnormal and this causes a change in the shape of the red blood cells.在镰状细胞病,血红蛋白是不正常的,这造成的改变形状的红血细胞。

A continuous supply of oxygen is essential for life.连续供应的氧气是必不可少的生活。 Oxygen is carried from the lungs to all parts of the body by the blood.In sickle-cell disease if the oxygen content in the haemoglobin is low, the haemoglobin sticks together to form long rods inside the red blood cells making these cells rigid and sickle shaped (hence the name).氧气是通过从肺部所有身体部分由blood.in镰状细胞病,如果氧含量,在血色素偏低,血红蛋白枝在一起,形成长期棒内的红血细胞,使这些细胞的僵化和镰刀形(由此得名) 。 Red blood cells are normally a rounder shape, rather like a donut.红血细胞,通常一圆形状,而不是像一个甜甜圈。

The sickled red cells:该sickled红细胞:

  • Have a weaker structure and a much shorter life than the normal four months for red blood cells.有一个较弱的结构和更短的生命比正常四个月红血细胞。 This leads to Anaemia.·这导致anaemia.â
  • Because of their shape, sickled cells clump together, especially in the smallest blood vessels, the blood becomes more viscous (sticky).因为他们的形状, sickled细胞丛一起,特别是在最小的血管,血液变得更加粘稠(粘) 。 The result is slowing of the flow, even greater loss of oxygen from the haemoglobin, further sickling and, in extreme cases, complete blockage of small blood vessels.结果是减缓流动,甚至更大的损失,氧从血红蛋白,进一步sickling和,在极端情况下,完成淤塞的小血管。 Such blockage is always serious, as it deprives tissues of their oxygen, glucose fuel and other nutrients and leads to local death of tissue and severe pain.·这种淤塞,始终是严重的,因为它剥夺了他们的组织中的氧气,葡萄糖,燃料及其他营养物质,并导致当地死亡的组织和严重pain.â
  • After going through a series of cycles of sickling and de-sickling during their passage around the circulation, red blood cells containing a high concentration of sickled haemoglobin finally become permanently sickled.经历了一系列的周期sickling和德- sickling期间,他们通过围绕流通,红血细胞含有高浓度的sickled血红蛋白终于成为永久sickled 。 This is the result of changes in the membrane of the red cell.这是结果的变化,在膜的红细胞。