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可使皮肤脱落的罕见疾病

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

Hey there! Welcome to Life Noggin! There are a number of rare conditions and diseases that people across the world deal with on a daily basis.

大家好!欢迎来到大脑洞开的生命奇想!在世界各地,每天都有人应对着一些罕见的疾病。
While they may not be super visible to us because of their low frequencies, it's important to know and learn about them.
因为他们的病例很少,所以我们可能不会注意到他们,但是知道和了解这些疾病是很重要的。
One such condition, or should I say groups of conditions, is peeling skin syndrome.
有一种情况,或者说有一类情况,叫做脱皮综合征。
Rather than being just a single thing, peeling skin syndrome is more of a general term for a group of rare inherited skin disorders
脱皮综合症不是一种单一的病症,它是一组罕见的遗传性皮肤病的的统称,
that are known for spontaneous and continual peeling of the skin, or exfoliation, that's oftentimes rather painless.
其特征是自发性和持续性的皮肤脱皮,经常是无痛的。
This is because the outermost layer of the epidermis is separated from the other layers below it.
这是因为表皮的最外层与它下面的其他层是分离的。
Blistering, reddening of the skin, and itching can also occur.
水泡、皮肤变红、发痒也会出现。
These symptoms are typically seen around birth or early childhood, though there have been cases of them showing up later in life.
这些症状通常在出生时或幼儿时出现,但是也有年龄更大时才出现症状的病例。
And when I said cases were rare, I mean like "under one hundred recorded cases in medical literature" type of rare.
当我说这种病例很罕见时,我是说少到“医学资料记载不超过100例”的那种罕见。
That said, since symptoms are often mild and similar to other skin conditions, cases could simply be underdiagnosed.
话虽如此,因为它的症状通常轻微,而且与其他皮肤问题很相似,所以它可能不会被确诊。
Like with most things, there can be a decent amount of variability with peeling skin syndrome from one case to another.
像大多数事物一样,不同的脱皮综合征病例可出现相当大的可变性。
A case report that was published back in 2012 even found significant variability in the cases of two siblings with acral peeling skin syndrome, a subdivision of peeling skin syndrome.
2012年发表的一项报告甚至发现,一对患肢端脱皮综合征的姐妹出现了很大的差异,肢端脱皮综合征是脱皮综合征的一种。
They also found that the peeling of their skin was worsened by occlusion and sweating.
他们也发现,阻塞和出汗也会加重他们皮肤的脱落。
Acral peeling skin syndrome is when the skin peeling is most prominent on the hands and feet.
皮肤脱皮最突出的部位是手和脚时出现的情况就是肢端脱皮综合征。
It's thought that this is because these areas are more frequently exposed to moisture and the friction from people moving around.
人们认为这是因为这些区域更频繁地暴露在湿气和处在人们移动带来的摩擦中。
That said, it can still occur on other parts of the body.
话虽如此,它也可出现在身体的其他部位。
Acral peeling skin syndrome can be caused by mutations in the TGM5 gene, with CSTA gene mutations also linked to the condition.
肢端脱皮综合征可由TGM5基因突变引起,CSTA基因突变也与此有关。
It's really all about genetic inheritance. Specifically, acral peeling skin syndrome is inherited in an autosomal recessive manner.
这完全是基因遗传的问题。具体来说,肢端脱皮综合征属于常染色体隐性遗传。
This means that to be affected, you have to have a mutation in both copies of the responsible gene in each cell.
这意味着要受到影响,个体必须在每个细胞的负责基因的两个拷贝中都有突变。

可使皮肤脱落的罕见疾病.png

Basically, you have to get a mutated copy of the gene from both of your parents.

就是说,你必须从父母那里分别得到一个突变的基因拷贝,
The normal gene is dominant, so if you get one normal gene and one mutated one,
正常基因是显性的,所以如果你有一个正常基因和一个突变基因,
you'll end up being a carrier — somebody who has a mutated gene but likely shows no symptoms.
你最终会成为一个携带者——一个有突变基因但可能没有症状的人。
When 2 carriers of an autosomal recessive condition get together and have children, each of the kids only have a 25% chance of being affected.
当2个常染色体隐性遗传基因携带者结婚生子后,每个孩子都有25%的几率出现该病。
However, they have a 50% chance of also being a carrier themselves,
但是,他们有50%的几率成为携带者,
While there is currently no cure for peeling skin syndrome, consistent treatments may offer some relief.
尽管现在还不能治愈肢端脱皮综合征,但是持续的治疗可以带来一些缓解。
These efforts are mainly aimed at treating the symptoms of each individual on a per case basis.
这些治疗主要是根据具体情况来治疗每个个体的症状。
These treatments include applying skin softening ointments like petroleum jelly or Vaseline to affected areas, especially at times when the skin is moist, like after a bath.
这些治疗包括,在发病区域使用皮肤软化软膏,如凡士林,尤其是在皮肤湿润的时候,比如洗澡后。
Corticosteroids or systemic retinoids haven't been shown to be effective and may cause serious side effects or adverse reactions.
皮质类固醇或系统性维甲酸还未证明有效,而且可能导致严重的副作用。
So while there might not be a cure yet, hopefully we can continue to work toward better treatments and eventually a cure itself.
尽管现在没有治愈方法,希望我们可以继续朝着更好的治疗努力,最终找到治愈方法。
Until then, we can be kind and keep on spreading information so that more of us know about the lives of others.
到那时之前,我们可以做个好人,持续散播信息,这样更多的人就能知道其他人的生活。
Are there any other conditions you want us to talk about next? Something you're really curious about?
有没有其他的疾病你想让我们接下来讨论?你非常好奇的事物?
Let me know now in the comments below! Curious to know how alien hand syndrome affects your body?
请在下方的评论区告诉我们!想知道异己手综合症是如何影响你的身体的吗?
Check out this video! Sometimes the hand will do the opposite of what you want it to do;
看看这个视频!有时这只手会做你想让它做的相反的事情;
like closing the fridge door after you just opened it, or trying to take off your clothes after you just put them on.
比如在你打开冰箱门之后马上又关上它,或者在你穿上衣服后想脱掉衣服。
As always my name is blocko, this has been life noggin, don't forget to keep on thinking!
我是宝高,这里是脑洞大开的生命奇想。不要忘记思考!

重点单词   查看全部解释    
decent ['di:snt]

想一想再看

adj. 体面的,正派的,得体的,相当好的

联想记忆
friction ['frikʃən]

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n. 摩擦,摩擦力,分歧

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mutation [mju:'teiʃən]

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n. 变化,转变,母音变化

 
rare [rɛə]

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adj. 稀罕的,稀薄的,罕见的,珍贵的
ad

 
visible ['vizəbl]

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adj. 可见的,看得见的
n. 可见物

 
continual [kən'tinjuəl]

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adj. 不断的,频繁的

 
layer ['leiə]

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n. 层
vi. 分层
vt. 将某

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separated ['sepəreitid]

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adj. 分居;分开的;不在一起生活的 v. 分开;隔开

 
recessive [ri'sesiv]

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adj. 后退的,逆行的,隐性遗传的 n. [遗传学]隐

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spontaneous [spɔn'teiniəs]

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adj. 自发的,自然产生的

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