Health encyclopaedia - Alphabetical Topic List健康百科全书-按字母顺序排列的主题清单
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Haemophilia血友病
Haemophilia is a genetic condition where the clotting factor in the blood is either partly or completely missing, leading to a life-long tendency to excessive bleeding with very slow clotting of the blood.血友病是一种遗传条件,如凝血因子在血液中或者是部分或完全缺失,导致了生命的长期趋势,出血过多与非常缓慢,凝血的血。 It affects females as carriers and males who inherit the condition.它影响女性为载体和男性谁继承的条件。
Blood clotting involves a complicated process occurring in stages.凝血涉及一个复杂的过程中出现的阶段。 The sequence is called a cascade and it involves a series of chemical factors described as factor I to factor XIII.序列是所谓的级联和它涉及到一系列的化学因素形容为因子I因素十三。 There is no factor VI.是没有的因素六。 These factors are proteins, and there is a gene that sets out the chemical structure of each one.这些因素是蛋白质,是有基因列明的化学结构的每一个。 The genes for two of the factors, VIII and IX, are on the X (sex) chromosome.该基因的两个因素,第八和第九,是对X (性别)的染色体。
The blood-clotting cascade ends in the formation of a substance that converts a blood protein called prothrombin into the protein thrombin, which actually forms the substance of the blood clot.血凝血级联结束在形成的一种物质转换血液蛋白所谓的凝血酶原纳入蛋白凝血酶,这实际上构成了实质的血块。 Thrombin tangles up a lot of small cell fragments in the blood, called platelets, to form a mass that can effectively seal off small bleeding blood vessels.凝血酶缠结了很多小细胞碎片在血液中,所谓的血小板,形成群众能够有效地封锁小血管出血。
A gene mutation is a change in the gene code.一基因突变是一个变化,在基因代码。 This change is likely to change the structure of the protein that the gene causes to be made.这一变化可能会改变结构的蛋白质,该基因的原因了。 There are different kinds of mutations, some having a more serious effect on the structure of the protein than do others.有不同种基因突变,有些更严重影响结构的蛋白质比别人做的。 If a male person has a significant mutation in the gene for factor VIII or factor IX, he will have haemophilia.如果一个男性的人有一个显着的突变基因因子VIII或因子IX ,他将有血友病。
Haemophilia A occurs in about 1 baby in 5,000 live births.血友病一发生在约1婴儿在5000个活产。 Haemophilia B is much less common, occurring in about 1 in 30,000.血友病B是常见的少得多,发生在约1在30000 。 Haemophilia can be mild, moderate or severe, depending on the amount of functioning factor VIII or IX the mutated gene is able to produce.血友病可轻度,中度或严重的,视金额的运作因素,第八或第九突变基因是能够生产。 About 6,000 people are affected with haemophilia in the UK.约6000人受影响,与血友病,在英国。









