血友病
Overview
血友病 is a bleeding disorder caused by a lack of blood clotting factor. 这种紊乱可分为轻度、中度或重度，取决于凝血因子的水平. More than 15,000 people in the United States have hemophilia.
The condition is usually inherited from a parent with a genetic mutation. 然而, 在大约三分之一的患者中, 没有家族病史，这种疾病是由一种新的基因突变引起的. 虽然大多数患有血友病的儿童在婴儿期或儿童期有出血的迹象, some people with mild hemophilia may not have 症状 until they're adults.
血友病的类型
最常见的血友病, 是由于缺乏凝血因子VIII引起的, 叫做A型血友病, 或者是典型的血友病. The second most common type is caused by a lack of clotting factor IX, 被称为B型血友病, 也被称为圣诞病, 以斯蒂芬·圣诞节命名, the first person diagnosed with the factor IX deficiency.
血友病 A and B occur almost always in males. 第三个, 非常罕见的血友病, 有时被称为血友病C, 是由于缺乏凝血因子XI引起的吗, 男性和女性都有.
血友病的原因
血友病A和B是由因子VIII或因子IX基因的基因突变引起的. Mutations are abnormal changes that occur in a gene. The gene for factor VIII and factor IX is located on the X chromosome, making Hemophila A and B X-linked disorders. 像这样, 异常因子VIII或因子IX基因的一个拷贝导致男性血友病, 而女性则处于携带者状态.
When there is a family history of hemophilia, 母亲通常是一个携带者，她的一半儿子和她的一些男性亲属会有血友病. If there is no family history of hemophilia, 这种疾病是由于在受影响的个体或个体的母亲中发生了新的突变, 谁的载体状态可能被忽视了.
我们治疗血友病的方法
As a federally designated hemophilia 治疗 center, UCSF provides the full range of serv冰s – including diagnosis, 治疗, counseling and education – to patients with all types of hemophilia.
我们相信要治疗整个人. 我们强调倾听患者的意见，并制定个性化的护理计划，以解决出血性疾病患者生活的所有医疗和情感方面的问题.
奖 & 识别
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是全国顶尖的十大赌博平台排行榜之一
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血友病定点治疗中心(疾病预防控制中心)
迹象 & 症状
血友病最常见的症状是出血，特别是出血进入关节和肌肉. 当血友病患者受伤时, he does not bleed faster than a person without hemophilia, 但是止血需要更长的时间. Bleeding also may start again several days after an injury or surgery.
Small cuts or surface bruises usually are not a problem, 但深度损伤可能导致出血发作，除非及时治疗，否则可能导致永久性残疾.
血友病的其他症状包括容易擦伤、长时间流鼻血或吐血.
血友病的症状和严重程度
血友病可发生在轻度, 中度和重度, 根据病人的症状和血液中凝血因子的水平或数量.
- 轻微的血友病. The person has 6 percent to 49 percent of the normal factor level, and usually bleeds only after serious injury, 创伤或手术. The first episode of bleeding may not occur until adulthood.
- 温和的血友病. 这个人有正常因子水平的1%到5%，受伤后出血发作, 重大创伤或手术. He also may experience occasional bleeding without obvious cause, 称为自发性出血发作.
- 血友病严重. 这个人的因子水平低于1%，并且在受伤或手术后出血, and may have frequent spontaneous bleeding episodes into the joints and muscles.
A person's severity of hemophilia does not change over time, because factor level is determined by genetics.
诊断
Accurate diagnosis is essential for the optimal management of hemophilia. 血友病检测应在经验丰富的专门凝血实验室进行. 不经常进行这些专门检测的实验室可能无法准确诊断.
Most people with hemophilia are diagnosed at an early age. 然而, 那些患有轻度血友病的人可能要到成年后才会被诊断出来，因为他们经历了创伤或手术引起的出血.
血友病的诊断是通过血液检查来确定是否缺少凝血因子或凝血因子水平过低, 哪些是造成问题的原因. 如果你有血友病家族史, 让你的十大赌博平台排行榜知道你的亲属缺少凝血因子是很重要的. 你可能会错过同样的一个.
如果你知道自己是血友病携带者, the testing for hemophilia in your newborn usually occurs soon after birth. 这些测试可以在脐带或新生儿静脉抽取的血液上进行. 你可能会被建议推迟一些程序, 比如割礼, until after you learn whether your child has hemophilia.
Some families with a history of hemophilia may want to request prenatal testing, 或者产前检查. 这项测试可以在怀孕早期进行, allowing your family to make informed decisions and preparations. 如果需要的话，加州大学旧金山分校有遗传咨询师可以帮助你进行产前检测.
If you are pregnant and think you could be a carrier, 或者如果你的孩子被诊断出患有血友病，并期待着另一个孩子, 告诉你的产科十大赌博平台排行榜是很重要的.
There are three ways to determine if you are a carrier:
- 家谱. If you have a son with hemophilia and have another son, 哥哥, 父亲, 叔叔, 堂兄或祖父患有这种疾病, 那么你就是一个载体. 不需要额外的测试.
- 凝血因子. If the clotting factor level in your blood is below 50 percent of normal, you are probably a carrier and have mild hemophilia. If the clotting factor level is above 50 percent, 你仍然可能是携带者, since other conditions can elevate the factor level. 其他测试可能是必要的.
- DNA测试. DNA测试可以在你的儿子或其他亲属身上寻找导致血友病的突变, 并与你的DNA进行比较.
治疗
我们的医疗团队可以帮助您选择最适合您的治疗方案. We rely on your input to help develop a plan of care that will keep you healthy, active and able to live successfully with the challenges of hemophilia.
The present goal of hemophilia therapy is to raise factor levels, 减少出血发作的频率和严重程度，预防出血并发症. 血友病 治疗 uses clotting factor concentrate, 也称为factor," which is a dried powder form of the clotting factor. It is mixed with water to form a liquid before it is given.
一些凝血因子产品, 称为血浆衍生因子产物, 是由捐献的人类血浆制成的吗. Others, called recombinant factor products, are made in a laboratory.
凝血因子浓缩液是在你受伤或出现出血迹象后不久注射到你的静脉中. 当给定凝血因子时, 它会立即在血液中循环，这样身体就可以利用它形成血块. Once the blood clot is established and the bleeding has stopped, 身体开始重新吸收渗漏到组织和关节的血液.
如果你没有得到及时治疗, 多余的血液会聚集在关节或软组织中，引起疼痛和肿胀，需要更长的时间才能消失. 随着时间的推移，反复的关节出血会导致严重的关节损伤和关节炎. Early 治疗 will minimize the risk of joint damage.
如果你有轻微的血友病, you may only need to be treated episodically, 这是, 每次你出血的时候. 如果你的血友病是中度或重度，你的血友病可能需要更频繁的治疗.
回家治疗
All factor 治疗s are infused or injected intravenously into a vein. 起初, you will be treated at a hemophilia 治疗 center, 你的十大赌博平台排行榜办公室或急诊室. Later, you may be taught how to administer the factor at home.
无论你是否在家治疗, you should always have factor concentrate at home, since you may need to take it to the emergency room when you need a 治疗. If the decision is made to infuse factor to treat your bleeding episode, the most important thing you can do is to administer it as soon as possible.
After you receive factor 治疗 for a joint bleed, 休息, 冰, compression and elevation (or "RICE") are required. 您还可以从支持设备中受益, such as crutches following a bleed into the knee or ankle, or a sling following a bleed into a muscle or joint in the arm. 根据出血部位的不同，出血后几天你可能要限制活动.
非处方药物
对乙酰氨基酚被推荐为血友病患者的安全止痛药. 仔细按照说明服用，并确保只服用推荐量的药物.
Never take any product with aspirin, or acetylsalicylic acid, in it. 阿司匹林会妨碍凝血. 许多常见的家庭疗法, 比如苏打水, 含有阿司匹林, so read labels very carefully before you take any medication.
Nonsteroidal anti-inflammatory drugs such as Advil, Aleve和Motrin, also may interfere with clotting; these should be used only if you have discussed their side effects with your doctor.
If you have any questions about what is or is not safe for you to take, talk to your doctor or hemophilia medical staff.
实验治疗
基因疗法和长效凝血因子浓缩物等治疗方法的研究正在进行中. 加州大学旧金山分校血友病治疗中心经常十大赌博靠谱网络平台涉及新疗法的临床试验.
加州大学旧金山分校健康 medical specialists have reviewed this information. 它仅用于教育目的，并不打算取代您的十大赌博平台排行榜或其他医疗保健提供者的建议. 我们鼓励您与您的供应商讨论您可能遇到的任何问题或疑虑.
