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Fresh frozen plasma (FFP) and cryoprecipitate, often called “cryo” for short, are transfused to patients who have abnormal or low levels of blood clotting proteins, such as in hemophilia.

新鲜冷冻浆及冷(常简称为冷)可用来输因子异常或缺乏的患者(比如,友病患者)

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Osmosis-液肿

And this deficiency is called hemophilia A (or classic hemophilia).

这种缺乏症也称作病A(甲型病)或者经典病。

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双语版 TED-Ed 演讲精选

Alexei, the heir to the throne, had a life-threatening blood disease called hemophilia.

阿列克谢是王位继承人,他得了,这是一种可能会致死液疾病

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双语版 TED-Ed 演讲精选

Today, we know that the doctors had prescribed aspirin, a drug that worsens hemophilia.

现今,我们知道那些医生开处方是阿司匹林。这种药会让恶化

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Osmosis-液肿

Hemophilia usually refers to inherited deficiencies of coagulation factors, which can be either quantitative or qualitative.

通常是指遗传性缺乏症,可以是数量或者活性上缺陷。

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Osmosis-液肿

Finally, it's best for individuals with hemophilia to avoid contact sports and medicines that promote bleeding, like aspirin.

最后对于患者来说,最好避免接触性运动,不用促进药物,如阿司匹林。

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Osmosis-液肿

An insufficient concentration or decreased activity of any coagulation factor can cause hemophilia, except factor XII deficiency which is asymptomatic.

任何凝浓度不够或者活性降低都会引起一个例外是缺乏XII话,不会有相关症状出现。

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世界奇趣图谱

The youngest of five children, he was born with hemophilia (when blood doesn’t clot).

他是五个孩中最小一个, 生来就患有(当液不凝固时)。

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影视剧中医学知识

He needs the fluid drained, but because of his hemophilia, I could kill him trying to save him.

他需要排出液体,但由于他患有我可以为了救他而杀了他。

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Osmosis-液肿

Treatment for hemophilia A and B are done with injections of the missing or nonfunctional clotting factor.

治疗A和B方法通常是注射那些缺失或失活

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Osmosis-液肿

For hemophilia A, desmopressin, also called DDAVP (1-deamino-8-D-arginine vasopressin) is helpful for patients with mild, quantitative factor VIII deficiency.

对于A,去氨加压素(DDAVP)有助于治疗轻症VIII数量缺乏。

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Osmosis-液肿

Believe it or not, this is a somewhat simplified version of the coagulation cascade; but, it contains all of the key parts needed to understand hemophilia.

总之,这就是简化版级联反应,已经包含了理解病理所需关键部分

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Osmosis-液肿

Also, vitamin K deficiency can cause hemophilia since vitamin K is needed by the liver to synthesize and release factors II, VII, IX, and X.

并且维生素K缺乏症也可引起为维生素K参与肝脏合成及释放II、VII、IX和X。

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TED-Ed(视频版)

Disorders that are associated with mutations of X chromosome genes, like color blindness, or hemophilia, are often less severe in individuals with two X chromosomes.

与 X 染色体基突变相关疾病,如色盲或在具有两条 X 染色体个体中通常不太严重。

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Osmosis-液肿

To confirm hemophilia A or B, tests to look at specific factor activities, and mutation testing of the genes encoding them, can be done.

为确诊A或B,可以检测特定活性或进行基诊断。

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Osmosis-液肿

Now, a mimic of hemophilia A is von Willebrand disease, which is an inherited problem with primary hemostasis caused by a deficiency of von Willebrand factor.

还有一种类A,即管性病,是一种遗传性初级止障碍,由管性(vWF)缺乏引起。

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Osmosis-液肿

Women with one mutated gene copy have a remaining healthy copy, so they don't get hemophilia unless X-chromosome inactivation turns off the normal copy in the majority of cells.

携带一个突变基女性还有一个健康拷贝,所以她们一般不会患,除非X染色体失活使得大多数细胞中正常拷贝沉默。

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Crash Course 解剖生理学篇

In the family of disorders known as hemophilia, a patient can usually complete the first two steps of hemostasis just fine, but they can't make an effective fibrin clot.

在被称为疾病家族中病人通常可以很好地完成止前两个步骤,但是他们不能制造有效纤维蛋白凝块。

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Osmosis-液肿

Another common deficiency is deficiency of factor IX, called hemophilia B (which used to be called Christmas disease, named after the first patient who had it, not the holiday).

另一种常见缺陷是IX缺乏症,称作B(乙型病),以前也称作Christmas病,是以第一个被报道病患命名,而不是指圣诞节。

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Osmosis-液肿

Signs and symptoms hemophilia A and B are nearly clinically identical, which makes sense since factors VIIIa and IXa work together in the coagulation cascade to activate factor X.

A和B体征和症状在临床上几乎相同,也说得通,VIIIa和IXa在凝级联反应中,共同作用以激活X。

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Osmosis-液肿

Diagnosis of hemophilia A and B usually starts with lab tests including a platelet count which is usually normal, a prothrombin (PT) time, and a partial thromboplastic (PTT) time.

A和B诊断通常是进行实验检测,包括小板计数(一般正常)、凝酶原时间(PT),以及部分凝活酶时间(PTT)。

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