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儿童早衰症 – 译学馆
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儿童早衰症

Genetic Condition That Makes You Age Too Fast - The Aging Disease

Imagine if you aged incredibly fast,
想象一下如果你老得非常快
say for every year you aged 10 years.
比如 每年会老十岁
It sounds like something from a fairytale,
这听起来像邪恶的女巫给人下了魔咒
a magic spell put on someone by a wicked witch.
是在童话中才会发生的事
But in the real world something does indeed at least resemble this.
但在现实生活中确实有类似的案例
It’s known as Progeria syndrome,
被称为儿童早衰综合症
which is just one of what are called Progeroid syndromes.
这仅是所谓的早衰综合症中的一种
These are basically genetic disorders
其实 这类疾病是由基因紊乱导致
that seem to fast-forward the aging process.
可能导致衰老过程加快
No doubt you’ve heard of two fairly common diseases
你肯定听说过两个常见的例子
that fall into this category that affect older people,
它们属于早衰症中会影响老年人的一类疾病
and those are Alzheimer’s disease and Parkinson’s disease.
阿尔茨海默症和帕金森症
Progeria is different
儿童早衰症不同
as sadly it starts manifesting at a very young age,
因为不幸的是 它在患者年龄很小时发病
and so by the time children are aged just a few years
在患儿仅几岁时
they already resemble a much, much older person
已经看起来像是老得多的人
Let’s now look deeper into this.
现在来深入了解一下儿童早衰症
The Progeria Research Foundation tells us
根据早衰症研究基金会的说法
that the full name given to this disease is
该疾病的全名是
Hutchinson-Gilford Progeria Syndrome,
哈钦森-吉尔福德早衰综合症
named after the two people that discovered it
是以19世纪早期 发现并记录该病的
and wrote about it in the 19th century.
两人的名字来命名
The first part, Progeria, actually comes from Greek
第一个词 早衰 实际源自希腊语
and means premature ageing.
意为过早衰老
We are told that the basis of this disorder is a faulty gene,
已知这种发育紊乱的本质是基因缺陷
and scientists explain that
科学家们解释到
absence of the Lamin A/C protein makes the nucleus of cells unstable
纤层蛋白A/C的缺乏会导致细胞核不稳定
and has the effect of very speeded-up ageing.
并导致快速衰老
This doesn’t mean of course
显然这并不意味着
that the young people have the wisdom of the aged
年轻患者可以拥有老年人的智慧
or that they are old in any way in their minds.
抑或说他们具有老年人的心态
What does happen is the body ages,
实际发生的只有身体衰老
meaning these people as early as two years old
也就是说尽管他们只有两岁
may start to have a wrinkled appearance;
可能已经开始长皱纹
they lose their hair, have stiff joints,
患者会脱发 关节僵硬
have a high-pitched voice, a larger head, a thin nose,
嗓门尖 头部更大 鼻子干瘪
and unfortunately
而且不幸的是
may experience kinds of problems associated with older age
他们可能会患上各种老年疾病
including atherosclerosis and heart disease.
包括动脉粥样硬化 心脏病
They might also suffer many other diseases
他们同时可能遭受其它
that people don’t usually get until their autumn years.
正常人到中老年才会患的病
These differences in physicality don’t exactly mean
这些身体上的差别意味着
the children age and look as if they would have if they aged normally.
患儿的外貌不会像没患病那样随年龄正常变化
People with progeria often look quite alike.
通常 儿童早衰症患者外貌比较相似
According to various sources,
各种资料显示
it can affect any race and occurs in both males and females
儿童早衰症的发病率不受种族和性别的影响
across the globe at about the same frequency.
在全球范围内没有差别
You can usually tell if someone has Progeria at around 18 to 24 months.
通常早衰症患儿可以在18-24个月大时确诊
The first signs might be loss of all body and head hair,
早期症状可能有 全身的体毛以及头发脱落
as well as a small pinched nose,
鼻子又小又尖
the appearance of many veins visible through the skin,
皮肤下血管突出易见
the absence of growth – including
各方面发育停滞
what looks like a very small face but large head.
包括脸看起来小但是脑袋大
This won’t happen to many young people,
这种病不会发生在大多数年轻人身上
with Progeria it’s said to affect one in about four million people
据说早衰症发病率仅400万分之一
There is no cure as yet,
目前没有治愈方法
and most of the sufferers can only expect to make it into their teens
大多数患者都只能寄希望于活到青少年时期
or in very few cases perhaps the early twenties.
极少数能活到20岁左右
We are told the reason for their premature death
据悉患者过早死亡的原因
is almost always related to the heart having a stroke or having a heart attack.
大多与中风或心脏病发相关
These young kids may have some kind of heart surgery to extend their life,
这些患儿可以通过某种心脏手术来延长寿命
but no amount of medical procedures so far
但是到目前为止 没有任何医疗方案
have managed to prevent serious heart-associated problems for very long.
能有效且长久地防止严重心脏问题发生
But what about the people suffering from it,
那么此类患者该怎么办呢?
how do they get on?
他们如何应对?
One of the people who survived the longest
其中一位活得最久的患者
with this disorder became something of a celebrity.
因患此病 也算变成了一位名人
His name was Leon Botha
他就是莱昂·博塔
and he lived a full life until his death aged 26.
他过着充实的生活 直至26岁去世
This South African man was a DJ, a Hip-hop artist,
他来自南非 是一位DJ 嘻哈音乐艺术家
writer, photographer and a painter.
同时也是作家 摄影师和画家
You can see him spinning on YouTube,
他在油管上有很多视频
or look at some of his impressive drawings.
也有一些令人印象深刻的画作
The disease certainly didn’t hold him back in an artistic way.
早衰症并未耽误他的艺术创作
We should stress again that
我们需要再次强调
progeria affects the body, not the mental faculties.
早衰症只影响身体 不会影响智力
In the British media in 2015 it was announced
2015年 英国媒体宣布
that a progeria sufferer who had captured the hearts and minds of the public
人们密切的关注那位早衰症患者去世
had died at age just 17.
去世时年仅17岁
Her name was Hayley Okines and the press said
她的名字叫海莉·奥金尼斯
at the time of her death she had the body of a 104-year old.
据报道 她死时身体的年龄已有104岁
She had been told she might not make it past the age of 13,
她曾被告知不会活过13岁
but she fought on, only succumbing to pneumonia in the end.
但她坚持与疾病抗争 直到最后死于肺炎
During her life she did some amazing things,
她在短暂的一生中做了许多很棒的事情
including writing her own biography called “Old Before My Time.”
包括为自己写的自传《未老先衰》
She was also featured in two documentary films,
她也曾出演两部记录片
one called “ The Girl Who Is Older Than Her Mother” and “World’s Oldest Teenager.”
即《那个比她妈妈还老的女孩》和《世界上最老的青年》
Her father told the press that he was besotted with his little girl.
她父亲曾向记者表示最初没有意识到女儿得病
As she got older, though, he noticed that she wasn’t growing.
他注意到 随着女儿年纪的增长 身体却停止发育
At first he thought it might not be anything as he is a very short man.
最开始他不以为意 因为自己也很矮小
He then said that
随后他又提到
at age 13 months she was still wearing clothes for a 3-month old and
女儿在13个月大时仍穿着3个月大时的衣服
he and his wife knew something was wrong.
这时他和妻子才感觉到不对劲
She was then diagnosed with this unusual illness.
随后女儿被确诊患上这种罕见的疾病
It didn’t hold the girl back, though,
但疾病没有阻碍这个女孩
and she fulfilled a few dreams by travelling around the world
她在环游世界的时候还实现了几个梦想
swimming in the ocean with dolphins…
比如 在大海中与海豚一起游泳
and maybe the best of all, she got to meet her idol, Justin Bieber.
最棒的或许是 她见到了自己的偶像 贾斯汀·比伯
She also had some words with Prince Charles and Kylie Minogue.
还和查尔斯王子以及凯莉·米洛聊过天
Another well-known sufferer was called Sam Berns.
另一位有名的患者叫 萨姆·伯恩斯
You may have seen him when he did one of the TEDx Talks.
你可能看过他在TEDx上做的演讲
You can see quite clearly that this disease has no effect on how articulate he is.
你可以清楚的看到早衰症没有影响到他明确清晰的表达
He said if he wanted anyone to know anything about him,
他说自己最想要被人了解的事
it was this, “ I have a very happy life.”
就是“这辈子我活的很开心”
He went on to say,
他又提到
“ Even though there are many obstacles in my life,
“尽管我的生活中有许多挫折”
many of them created by Progeria
“而且大部分都是由早衰症引起的”
I don’t want people to feel bad for me.”
“但我不想让别人同情我”
His philosophy for a happy life,
他说自己对幸福生活的信仰
he said, is that he is ok with what he can’t do,
在于坦然接受自己做不到的事
because there are so many things that he can do.
因为他还有许多事情可以做到的
While he can’t run a long way,
尽管他跑不远
he only focuses on all the things he is able to do
但只要专注于他能做的事情就好了
Which are many.
而这样的事也很多
Like many of you,
和大多数人一样
he must understand his abilities and adapt his life to knowing his boundaries
他必须明确自己的能力 并调整生活去适应自己的界限
and then becoming the best at what he can do.
然后将力所能及之事做到最好
He also said he surrounds himself with people that are good for him
他还说身边都是待他好的人
and that is something he believes we all should do.
他也相信这种善良的举动是每个人该做的
They provide him with a positive influence
善良的人们带给他积极的影响
and he returns that back to them.
他也以同样的善意回报
His other main reason for his happiness he said
他说自己另一个幸福的重要秘诀是
was that he was always moving forward,
永远向前看
always planning the days ahead and making the best of those days.
提前做计划 让每一天都很充实
Keep focused, he said.
他说 一定要专注
“ I try not to lose energy feeling bad for myself, he said
“我尽量不浪费精力自怨自艾” 他说
“ because when I do, I get stuck in a paradox where there is no room for
“如果我自怨自艾 就没有精力感受幸福和其他美好
any happiness or any other emotion. ”
那将是个恶性循环”
He died aged 17.
他在17岁时去世
You might also have seen someone
你也许还听说过另一个患者
who has been called an Internet superstar,
她被称为互联网巨星
having garnered much attention on platforms such as YouTube.
在不同平台 比如油管 获得了非常多的关注
Her name is Adalia Rose
她就是 阿达利亚·罗斯
and as we speak just one clip on YouTube featuring
以她发布在油管上的一个视频为例
her has been watched almost seven million times.
该视频的点击率已达70万次
At the age of eleven in 2018
在2018年 她11岁时
she already had 170 million views on YouTube
她在油管上的视频已有170万的浏览量
while her Facebook account had 13 million likes.
同时她的脸书账号已获得1300万个赞
This girl, from Austin, Texas, said in one interview,
这个来自德州奥斯汀的女孩曾在采访中说到
“ I like to make videos for my YouTube fans, because, I love ‘em I guess.”
“我喜欢为油管粉丝制作视频 因为我觉得我爱他们”
Her parents say they try not to talk too much with her about Progeria
她父母说他们尽量避免跟她谈论儿童早衰症
only that they treat her like any other child.
只有这样他们才能把她当作正常孩子对待
She seems to live just like any other girl,
她看起来过着普通女孩的生活
with only the ageing process making her look different.
除了患病导致的加速衰老让她看起来不同
As we write this, one of her last videos posted to Facebook
在我们制作视频时 她又在油管上传了最新视频
Opens with the words,
开场白如下
“ If you have a disease, or a sickness, don’t be ashamed of it.
“不要因为你得病或不健全感到羞耻
Because look at my eye ( she shows an eye that Won’t open)
因为就算我眼睛这样(她指了指无法睁开的那只眼睛)
and I have no hair, and I just went outside.
我还没有头发 那我也会出去玩
I felt ashamed, but now I’m ok.”
我也曾难为情 但是现在不会”
She is perhaps the biggest viral star that talks openly
她可能是公开讨论残疾人状况的人里面
about a condition that has handicapped her in some way
最火爆的明星了
This disease is very uncommon as we said,
如前所述 这种疾病非常罕见
with the media saying right now about 500 children in the world have it.
据报道目前全世界大约有500个患儿
But will any of these kids be cured?
那这些患儿有治愈的可能吗?
What hope does science have of treating this disorder?
科学界存在治愈这种基因紊乱的希望吗?
You can actually go the Progeria Research Foundation website
你可去儿童早衰症研究基金会网站
and see the bios of many of the kids that live with this disease or have passed away.
看看这些正遭受疾病折磨 或已去世的患儿名单
Just looking at a map of where cases were found,
只单看地图上的案例分布
it seems that Progeria sufferers are spread all over the world quite evenly
儿童早衰症发病率似乎在世界范围概率相同
You can find Meghan from the USA,
你可以看到美国青少年患者梅根
a teenager that is an athlete, an author, a poet and a musician.
他同时是运动员 作家 诗人和音乐家
You’ve got 23-year old Sammy from Italy,
来自意大利 23岁的萨米
a budding actor and painter who’s been on TV in Italy many times.
一个新锐演员兼画家 曾多次出现在意大利电视节目上
Over in Australia there is Enzo,
以及澳大利亚的恩佐
whose mother says enjoys anything with wheels,
据他妈妈说 从玩马里奥赛车游戏到骑摩托车
from playing Mario Kart to riding motorcycles.
一切有轮子的东西他都喜欢
In Belgium a brother and sister both have progeria,
在比利时 有一对兄妹都得了儿童早衰症
Michiel who is 20 and his sister Amber who is 12.
20岁的迈克尔和他12岁的妹妹安布尔
They have a multi-lingual website
他们创建了一个多语种网站
which details their day-to-day lives and their ongoing treatment.
用来记录他们日常生活和持续性治疗过程
Yes we say treatment,
是的 治疗
because some of the people we have mentioned are taking part in trials.
因为我们提及的部分人 正在参与实验性治疗
Doctors hope one day to reverse this fast-ageing process.
医生们希望将来能逆转这种加速变老过程
We are told that there have been some success stories
据了解已经有一些成功的例子
when sufferers took the drug lonafarnib,
当患者服用了药物 洛那法尼
what’s called a farnesyltransferase inhibitor, during medical trials.
这种药在治疗中 被称为法尼基转移酶抑制剂
The drug was in the past used to treat people suffering from various kinds of cancer,
在过去被用于治疗各种癌症患者
But during trials Progeria sufferers experienced positive results.
但在试验中 早衰症患者服用该药后出现了治疗效果
these results improved bone structure
这包括改善患者骨质结构
better hearing, improved weight
改善听力 增长体重
and best of all the participant’s blood vessels became more flexible.
最重要的是患者的血管变得更加柔韧
The reason this is very important is that
血管柔韧之所以重要的原因是
stiffness of the arteries is one of the reasons for heart attack or stroke.
动脉僵硬是造成心脏病发和中风的原因之一
Twenty-eight young people from 16 countries, all with Progeria,
来自16个国家的28位患有早衰症的年轻人
take part in the trail that lasted two and a half years.
参与了这个为期两年半的实验性治疗
One of the doctors leading the trial said,
一位主持该试验的医生说到
“ When we started this clinical trial
“当我们开始这项临床实验的时候
we had no idea whether any aspect of Progeria would be reversible,
我们不确定早衰症的哪一方面可以被逆转
because no one had ever conducted a clinical treatment trial for Progeria before.”
因为从未有人做过儿童早衰症相关的临床试验”
His aim of course is to give people diagnosed
显然 他的目的是给确诊患者提供一个
with the condition the chance to live as long as anyone else
像普通人一样长寿的机会
This is not a cure, however,
然后 这并非治愈手段
and if you read journals featuring Progeria you can see that
如果读过儿童早衰症的相关专题报道你就会明白
scientists are still busy trying to stop this abnormal protein
科学家至今仍在努力的尝试
that advances ageing from doing its damage.
阻断这种导致快速衰老的异常蛋白带来的破坏
We are told that there is just no way to tell
据悉 目前还无法预测
if anyone will have this irregular mutation of genes
什么人会有这种基因突变
as it doesn’t seem to occur in families that have had any history of the condition.
这种疾病似乎并不会遗传
It just seems to happen, albeit as we have said, not often.
发病率不高 但是很随机
How do you think you’d deal with living with this condition?
如果你不幸患病 会如何生活下去?
Have you been watching the people we’ve featured, either online or on TV?
你在电视或网络上看到过我们采访的相关患者吗?
What do you think about them?
你如何看待他们?
Tell us in the comments.
在评论中告诉我们
Also be sure to check out our other show what is Stoneman
记得看我们另外一个视频 什么是“石头人”?
Thanks for watching,
谢谢观看
and as always,don’t forget to like, share and subscribe.
不要忘了点赞 分享以及订阅哦

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视频概述

儿童早衰症,是一种非常罕见的疾病,由基因突变导致的患者生长速度加快。患者一般只能活到7~20岁,并且大部分死于心血管类疾病,目前还没有有效的治疗手段。

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视频来源

https://www.youtube.com/watch?v=mI6fu4AiLv8

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