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据外媒报道，全球最长寿的早衰症患者，意大利生物学家——萨米·巴索（Sammy Basso）于当地时间10月5日去世，年仅28岁。
可能很多人对萨米·巴索这个名字比较陌生，但相信你一定对这个英文演讲视频有点印象。
那是十年前。萨米·巴索登上TED演讲的舞台，向观众介绍自己的病症，并大声向命运发起宣言：I have a very happy life。
早衰症患者的寿命鲜少超过20岁，萨米·巴索已经创造了奇迹。在他短暂的一生中，他不仅以无比乐观的精神面对自身的疾病，还积极参与实验，推动了世界首款延缓早衰症的药物问世。
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01 世界上最长寿的早衰症患者
Sammy Basso, who was the longest living survivor of the rare genetic disease progeria, has died at the age of 28, the Italian Progeria Association said on Sunday.
意大利早衰症协会周日表示，世界上最长寿的早衰症患者萨米·巴索逝世，年仅28岁。
Born in 1995 in Schio, in the northern Italian region of Veneto, Basso was diagnosed with progeria at the age of two. In 2005, he and his parents founded the Italian Progeria Association.
巴索于1995年出生在意大利北部威尼托大区的斯基奥，2岁时确诊患有早衰症。2005年，他和父母共同成立了意大利早衰症协会。
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He became famous through the National Geographic documentary "Sammy's Journey," which recounts his journey along Route 66 in the United States, from Chicago to Los Angeles, with his parents and one of his best friends, Riccardo.
他因国家地理频道的纪录片《萨米的旅程》而成名，该片讲述了他与父母和最好的朋友之一里卡尔多一起，沿着美国66号公路从芝加哥到洛杉矶的旅程。
"Today our light, our guide, has gone out. Thank you Sammy for making us part of this wonderful life," the association wrote on its Instagram page.
“今天，我们的光芒，我们的指引，熄灭了。感谢萨米，让我们成为这段美好人生的一部分。”该协会在其Instagram页面上写道。
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Progeria, also known as Hutchinson–Gilford syndrome (HGPS), causes people to age rapidly, leading them to appear older than they are, with a reduced quality of life and a life expectancy of only 13.5 years without treatment, the association's website said.It affects one in every eight million people born, and has a worldwide incidence of one in every 20 million.早衰症，又称哈钦森-吉尔福德氏综合征，是一种罕见的遗传性疾病。患上这种病的人，身体会加速老化，看起来比实际年龄要老得多，生活质量也大大降低。如果不进行治疗，患者的平均寿命仅有13.5岁。相关协会的网站显示，这种病每八百万个新生儿中就有一例，全球的发病率为每二千万分之一。There are only 130 recognised cases of classic progeria worldwide, of which four are in Italy.However, the Italian Progeria Association estimated there could be as many as 350 cases as they can be difficult to trace especially in developing countries.全球范围内被确诊的早衰症病例仅有130例，其中4例在意大利。然而，意大利早衰症协会估计，实际病例数可能高达350例，因为特别是在发展中国家，这些病例很难被追踪到。 “命运以痛吻我，我愿报之以歌。”萨米·巴索并未屈服于命运的安排，从来没有放弃过对人生的希望和追求。2018 年，他毕业于帕多瓦大学，获得自然科学学位，并发表了一篇关于 HGPS 小鼠基因编辑方法的论文。同年晚些时候，由于他对残疾问题的深入研究以及与意大利政府的合作，他被授予意大利共和国勋章骑士勋章。2021 年，他又获得了分子生物学第二学位，深入研究治疗早衰症的方法。“In a way, I should thank progeria,” he once said onstage, in his eloquent and Italian-inflected English. “Progeria does not prevent me to have a happy life. Maybe without progeria, I wouldn’t have understood that biology and science is my path.”他曾在台上用流利的带点意大利口音的英语说道：“从某种意义上来说，我应该感谢早衰症。早衰症并没有阻止我拥有快乐的生活。也许如果没有早衰症，我就不会明白生物学和科学是我该走的道路。”The fact that the science of progeria has advanced so much is in part due to Basso himself. He volunteered for the clinical trial of what would become the first approved drug for the disease, which helps prevent the buildup of toxic protein, slowing the progression of symptoms and extending some patients’ lives. 早衰症的科学研究之所以取得如此大的进展，部分归功于巴索本人。他自愿参加了首个获批的早衰症药物的临床试验。这种药物有助于防止有毒蛋白质的积聚，从而减缓症状进展，延长一些患者的生命。![]()
"I like my life as it is, because it's my life. I have my friends, my parents, my family. These are the most important things," he said in a 2016 interview with the Catholic publication Aleteia. "Progeria is a small part of my life, because it only affects the body."他在2016年接受天主教杂志《Aleteia》采访时说：“我喜欢现在的生活，因为它是我的生活。我有我的朋友、我的父母、我的家人。这些才是最重要的。” “早衰症只是我生活的一小部分，因为它只影响身体。”Those who knew him say he was not only committed to the cause, but also funny and kind, a brilliant conversationalist, the life of a party, and someone who extolled the kind of joie-de-vivre that comes from knowing all too well that every second counts.认识他的人说，他不仅对事业充满热情，而且是一个幽默风趣、心地善良的人。他不仅是聚会中的焦点，还是一个出色的谈话者。他总是宣扬一种“活在当下”的生活态度，这源于他对生命的深刻理解——每一秒都弥足珍贵。正如史铁生在《病隙碎笔》里写得那样：“人不是苟活苟死的物类，不是以过程的漫长为自豪，而是以过程的精彩、尊贵和独具爱愿为骄傲的。”萨米·巴索的一生虽然短暂，但他找到了自己愿意奋斗终生的科学事业，并为其他挣扎在病痛下的人们点燃了希望的火苗。这是非常了不起的一生！progeria / prəʊˈdʒɪərɪə / n.早衰症；早衰，早老biology / baɪˈɒlədʒi / n.生物学
clinical / ˈklɪnɪk(ə)l / adj.诊所的，医务室的；临床的
joie-de-vivre 法语词组，翻译成中文可以是“生活乐趣”、“活在当下”
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