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镰状细胞性贫血的病因

来源:可可英语 编辑:kelly   可可英语APP下载 |  可可官方微信:ikekenet

Imagine for a minute that your blood cells are shaped like sickles.

想象一下,你的血细胞形似镰刀。
But what exactly causes our friendly little red blood cells to turn against us?
但究竟是什么导致了我们友善的小红细胞转而去对抗我们呢?
Sickle Cell Anemia is a blood disorder, where instead of supple soft-frisbee red blood cells that live for 120 days,
镰状细胞贫血症是一种血液疾病,这种红细胞不像柔软的飞盘状红细胞那样可以存活120天,
your body forms stiff pointy sickle-shaped cells that only live for 10 to 20 days.
你的身体会产生坚硬的镰状细胞,并且这些细胞只能存活10到20天。
RBCs need to be soft and pliable to squeeze through your blood vessels safely and efficiently,
红细胞必须体态柔韧,才能安全有效地挤入血管。
so when they're pointy they can get stuck in vessels.
所以如果它们很尖,就会卡在血管里。
When this happens in the chest, abdomen or joints it can cause pain, if they get stuck in the hands or feet you can get swelling,
如果卡在胸部、腹部或关节上,就会引起疼痛,如果卡在手上或脚上,就会引起肿胀,
and of course if they get stuck in organs they can cause infections or if they get caught in the eye, vision problems.
当然,如果卡在器官里,可能还会引起感染,或者如果卡在眼睛里,则会造成视力问题。
And if that wasn't bad enough, the brittle cells can break apart, delaying oxygen delivery and causing fatigue! This all sounds terrible.
如果这还不够糟糕,这些脆性细胞会分裂,延迟氧气输送,造成疲劳!这听起来很可怕。
It was first described in 1910, in a dental student named Walter Clement Noel who went to a doctor in Chicago complaining of pain,
1910年,这种疾病得到了首次描述,一位名叫Walter Clement Noel的牙科学生,去芝加哥看医生,主诉有疼痛感,
but this student wasn't from Chicago -- he was from Grenada.
但这个学生不是芝加哥人,他来自格林纳达。
Sickle cell anemia predominantly affects approximately 1 in 400 African American births.
镰状细胞贫血症在非裔美国人中的比率约为1/400。
It was a disease known in Africa for five thousand years, but it hadn't been described in Western Medicine before 100 years ago.
这种疾病在非洲已有五千年的历史,但在100年前还未有西医对其做过任何描述。
The doctor in Chicago, who's name was James B. Herrick was the first to describe it in a paper
这个名叫James B. Herrick的芝加哥医生,首次在一篇论文中描述了它。
and over the next century many doctors have attempted to uncover how this debilitating disorder works, and why it mainly affects those of African descent.
而在随后一个世纪里,许多医生试图揭示这种疾病的作用机理,以及为何它主要对非裔人士产生影响。
In the 1930s, a D.C.-based doctor named William Cardozo was one of the first black doctors to conduct research into this disease
20世纪30年代,一位名叫威廉·卡多佐的华盛顿医生成为了首位对这种疾病进行研究的黑人医生,
and he did so with a grant from Alpha Phi Alpha, the first black Fraternal organization.
他的研究获得了第一家黑人互助慈善组织Alpha Phi Alpha的资助。
Cardozo felt that research into sickle cell anemia had "reached an impasse" and that no one had yet found the CAUSE of this horrible disease.
卡多佐认为镰状细胞贫血症的研究已经“陷入僵局”,迄今还没有人可以找到这种可怕疾病的发病原因。
IF someone could provide a "new stimulus" to get the research going, he wrote, maybe we could discover more.
他写道,如果有人能够提供一个“新的刺激”来进行研究,也许我们可以发现其中更多的奥秘。
His research, published in 1937, helped medicine realize sickle cell anemia was inherited.
他的研究在1937年发表,医学界从此认识到镰状细胞贫血症具有遗传性。
Thanks to his research we now knew sickle cell came from us -- and he helped explain why it mainly affected people of African descent.
他的研究使得我们知道镰状细胞来自我们自身——他的研究也有助于解释为什么这种疾病主要影响非裔人口。
While he was working on that, other doctors started to realize this disease was based on oxygen content.
在他对此病进行研究时,其他医生开始意识到这种疾病与血液中的氧含量相关。
In fact, Québécois researchers in 1930 Montreal took a seven year old black girl and restricted blood flow to her finger with a rubber band
事实上,1930年,蒙特利尔魁北克-科伊斯的研究人员在一个七岁的黑人女孩身上做了试验,即用橡皮筋限制血液流入她的手指
-- no, this probably wouldn't pass an ethics board today -- In doing so, they found sickle cells formed in the low oxygen environment of her fingertip.
——不,如果放到今天,这在伦理委员会哪儿可能不会通过——由此,他们发现镰状细胞在她指尖的低氧环境下形成。
Thus, they learned that with a bit of hypoxia, or lack of oxygen, the cells would collapse into all manner of weird pointy shapes,
因此,他们了解到,在缺氧的情况下,细胞会崩塌成各种奇怪的尖状,
nearly instantly returning to normal when exposed to air.
当暴露在空气中时,它们几乎立刻恢复正常。
Their methods were questionable, to say the least, but the results did shape future research.
他们的研究方法尚可质疑,但至少可以说,其结果确实对未来的研究产生了影响。
In the mid-20th century doctors discovered that a single amino acid was responsible for the hemoglobin on the red blood cells that cause the sickling.
20世纪中期,医生们发现了一个单独的氨基酸可以影响红细胞的血红蛋白,导致此病发生。
But now that we understand the disorder enough to try and solve it but unfortunately,
但是现在,我们已经对这种疾病有了一定的了解,并尝试解决,但不幸的是,
we're at another moment when we may need more "new stimulus" for SCD research.
我们又卡壳了,我们可能还需要更多“新的刺激”才能继续针对SCD的研究。
According to a Clinical Investigation from 2007, there are many possible treatments,
2007年以来的临床调查显示,此病的治疗方法很多,
including a bone marrow transplant, but there's still no cure.
其中还包括骨髓移植,但彻底治愈的方法仍未找到。
One option is medicine that helps fetuses develop more hemoglobin, but it CAN make SCD worse.
一种方法是在胎儿期施用药物,产生更多的血红蛋白,但它可能会使SCD恶化。
Maybe in the future an option could be genetic manipulation of the system that helps our bodies create blood, using genome engineering basically, gene therapy.
也许在将来,我们可以操纵帮助身体造血的基因系统,基本上讲,使用基因工程,实现基因治疗。
But for now, sickle cell anemia is just out there, a life-threatening thorn in many people's sides.
但现在,镰状细胞性贫血就在那里,是许多人身上的一根利刺。

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Cardozo was one of many doctors of many races in the hunt for the causes of sickle cell anemia.

不同种族的众多医生都在寻找镰状细胞贫血症的病因,卡多佐只是其中之一。
He's notable because in the 1930s doctors that looked like him were often not welcome in the annals of medicine.
他之所以引人注目,是因为在20世纪30年代,像他这样的医生,在医学界不受欢迎。
That HE was able to make a discovery for a disorder that affected so many people that also looked like him, is a pretty big deal,
他能发现一种影响许多看起来和他一样的人的疾病,真是相当了不起,
so we're happy to recognize the part he played in this story for Black History Month.
所以在黑人历史月里,我们很高兴看到,他在这个故事中扮演了至关重要的角色。
For more on red blood, watch Jules' video about what bone marrow actually does!
更多关于红色血液的内容,请看朱勒的视频节目,那里他在讲述骨髓的用途!
And please subscribe for more seeker.
敬请订阅。
Whenever I think of red blood cells, I think of those soft frisbees that I used to throw at my brothers.
每当我想到红细胞,我就会想到过去自己常和兄弟们玩的软飞盘。
Maybe that's why I think of RBCs as so darn friendly! Thanks for watching.
也许这就是为什么我认为红细胞们如此友好!谢谢收看。

重点单词   查看全部解释    
recognize ['rekəgnaiz]

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vt. 认出,认可,承认,意识到,表示感激

 
approximately [ə'prɔksimitli]

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adv. 近似地,大约

 
understand [.ʌndə'stænd]

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vt. 理解,懂,听说,获悉,将 ... 理解为,认为<

 
questionable ['kwestʃənəbəl]

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adj. 可疑的,可置疑的

 
genetic [dʒi'netik]

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adj. 基因的,遗传的,起源的

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therapy ['θerəpi]

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n. 疗法,治疗

 
ethics ['eθiks]

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n. 道德规范

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rubber ['rʌbə]

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n. 橡胶,橡皮,橡胶制品
adj. 橡胶的

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abdomen ['æbdəmen]

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n. 腹部

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