{"id":4004,"date":"2018-04-25T00:03:37","date_gmt":"2018-04-25T00:03:37","guid":{"rendered":"https:\/\/www.progeriaresearch.org\/?page_id=4004"},"modified":"2023-09-21T12:15:37","modified_gmt":"2023-09-21T16:15:37","slug":"first-ever-demonstrate-survival-benefit-in-progeria","status":"publish","type":"page","link":"https:\/\/www.progeriaresearch.org\/zh\/first-ever-demonstrate-survival-benefit-in-progeria\/","title":{"rendered":"\u9996\u4e2a\u7597\u6cd5\u8bc1\u5b9e\u5bf9\u65e9\u8870\u75c7\u60a3\u8005\u6709\u751f\u5b58\u76ca\u5904"},"content":{"rendered":"<p>2018 \u5e74 4 \u6708 24 \u65e5\uff1a\u300a\u7f8e\u56fd\u533b\u5b66\u4f1a\u6742\u5fd7\u300b\uff08JAMA\uff09\u53d1\u8868\u7684\u4e00\u9879\u65b0\u7814\u7a76\u62a5\u544a\u79f0\uff0c\u6cd5\u5c3c\u57fa\u8f6c\u79fb\u9176\u6291\u5236\u5242 (FTI) lonafarnib \u6709\u52a9\u4e8e\u5ef6\u957f\u65e9\u8870\u75c7\u513f\u7ae5\u7684\u751f\u5b58\u671f\u3002\u6ce2\u58eb\u987f\u513f\u7ae5\u533b\u9662\u548c\u5e03\u6717\u5927\u5b66\u7684\u4f5c\u8005\u8ffd\u8e2a\u4e86\u6765\u81ea\u516d\u5927\u6d32\u7684 250 \u591a\u540d\u513f\u7ae5\uff0c\u4ee5\u8bc1\u660e lonafarnib \u6cbb\u7597\u4e0e\u5ef6\u957f\u751f\u5b58\u671f\u4e4b\u95f4\u7684\u8054\u7cfb\u3002<\/p>\n<p><a href=\"https:\/\/jamanetwork.com\/journals\/jama\/article-abstract\/2679278?redirect=true&amp;appId=scweb\" target=\"_blank\" rel=\"noopener noreferrer\"><b>\u70b9\u51fb\u6b64\u5904\u9605\u8bfb\u7814\u7a76\u6458\u8981<\/b><\/a><\/p>\n<p><strong>\u5a92\u4f53\u62a5\u9053\uff1a\u00a0 <\/strong><\/p>\n<ul>\n<li><strong><a href=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2018\/04\/PRF-JAMA-Announcement_Press-Release-1.pdf\">\u65b0\u95fb\u7a3f<\/a><\/strong><\/li>\n<li><strong><a href=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2018\/04\/PRF-JAMA-Announcement-Infographic.pdf\">\u4fe1\u606f\u56fe\u8868<\/a><\/strong><strong>\u00a0<\/strong><\/li>\n<li><a href=\"https:\/\/www.dropbox.com\/sh\/uuq64xv0w0kfrcf\/AAACRWWU4ZKezFTksabaimzba?dl=0\" target=\"_blank\" rel=\"noopener noreferrer\"><strong>B-roll \u89c6\u9891<\/strong><\/a><\/li>\n<\/ul>\n<p><strong>\u7ed3\u679c\u00a0<\/strong><a href=\"https:\/\/www.progeriaresearch.org\/zh\/clinical-trials\/\"><strong>\u513f\u7ae5\u4e34\u5e8a\u8bd5\u9a8c<\/strong><\/a><strong>\u00a0\u65e9\u8870\u75c7\u5df2\u7ecf\u516c\u5e03\u5e76\u4e14\u662f\u5b98\u65b9\u6d88\u606f\uff01<\/strong>\u00a0 \u00a0\u8fd9\u9879\u7814\u7a76\u4f7f\u7528\u4e86\u4e00\u79cd\u6cd5\u5462\u57fa\u8f6c\u79fb\u9176\u6291\u5236\u5242 (FTI) lonafarnib\uff0c\u8be5\u6291\u5236\u5242\u6700\u521d\u5f00\u53d1\u7528\u4e8e\u6cbb\u7597\u764c\u75c7\uff0c\u652f\u6301\u5ef6\u957f\u65e9\u8870\u75c7\u513f\u7ae5\u7684\u5bff\u547d\u3002\u5728\u4e34\u5e8a\u8bd5\u9a8c\u4e2d\uff0c27 \u540d\u65e9\u8870\u75c7\u513f\u7ae5\u6bcf\u5929\u4e24\u6b21\u53e3\u670d lonafarnib \u4f5c\u4e3a\u5355\u4e00\u7597\u6cd5\u3002\u672c\u7814\u7a76\u7684\u5bf9\u7167\u7ec4\u7531\u4e0e\u63a5\u53d7\u6cbb\u7597\u7684\u60a3\u8005\u5e74\u9f84\u3001\u6027\u522b\u548c\u5c45\u4f4f\u5730\u76f8\u4f3c\u7684\u65e9\u8870\u75c7\u513f\u7ae5\u7ec4\u6210\uff0c\u4ed6\u4eec\u4e0d\u662f\u4e34\u5e8a\u8bd5\u9a8c\u7684\u4e00\u90e8\u5206\uff0c\u56e0\u6b64\u6ca1\u6709\u63a5\u53d7 lonafarnib \u6cbb\u7597\u3002\u7ed3\u679c\u8868\u660e\uff0c\u5728\u4e2d\u4f4d 2.2 \u5e74\u7684\u968f\u8bbf\u540e\uff0c\u5355\u72ec\u4f7f\u7528 lonafarnib \u6cbb\u7597\u4e0e\u672a\u63a5\u53d7\u6cbb\u7597\u76f8\u6bd4\uff0c\u6b7b\u4ea1\u7387\u663e\u8457\u964d\u4f4e\uff083.7% vs. 33.3%\uff09\u3002\u8be5\u7814\u7a76\u7684\u7ed3\u679c\u00a0<strong>\u7531\u65e9\u8870\u75c7\u7814\u7a76\u57fa\u91d1\u4f1a\u8d44\u52a9\u548c\u534f\u8c03<\/strong>\uff0c\u4e8e 2018 \u5e74 4 \u6708 24 \u65e5\u53d1\u5e03\u4e8e <em>\u7f8e\u56fd\u533b\u5b66\u4f1a\u6742\u5fd7\u3002<\/em><\/p>\n<p><em>Gordon \u7b49\u4eba\uff0cLonafarnib \u6cbb\u7597\u4e0e\u672a\u6cbb\u7597\u5bf9\u54c8\u94a6\u68ee-\u5409\u5c14\u798f\u5fb7\u65e9\u8870\u7efc\u5408\u5f81\u60a3\u8005\u6b7b\u4ea1\u7387\u7684\u5f71\u54cd<\/em>, <em>JAMA\uff0c2018 \u5e74 4 \u6708 24 \u65e5\u7b2c 319 \u5377\u7b2c 16 \u671f<\/em><strong>\u7ed3\u679c\u5e26\u6765\u65b0\u7684\u5e0c\u671b\u548c\u4e50\u89c2<br \/>\n<\/strong>\u8fd9\u4e9b\u5b69\u5b50\u88ab\u9001\u5f80\u6ce2\u58eb\u987f\u513f\u7ae5\u533b\u9662\u63a5\u53d7\u5168\u9762\u7684\u533b\u7597\u68c0\u67e5\u548c\u7814\u7a76\u836f\u7269\u6cbb\u7597\u3002\u6240\u6709\u5b69\u5b50\u90fd\u63a5\u53d7\u4e86\u7531\u9ed8\u514b\u516c\u53f8\u63d0\u4f9b\u7684\u53e3\u670d lonafarnib\uff0c\u8fd9\u662f\u4e00\u79cd\u5feb\u901f\u6291\u5236\u5242\u3002\u201c\u8fd9\u9879\u53d1\u8868\u5728 JAMA \u4e0a\u7684\u7814\u7a76\u8868\u660e\uff0c\u6211\u4eec\u53ef\u4ee5\u5f00\u59cb\u51cf\u7f13\u65e9\u8870\u75c7\u513f\u7ae5\u7684\u5feb\u901f\u8870\u8001\u8fc7\u7a0b\u3002\u8fd9\u4e9b\u7ed3\u679c\u4e3a\u65e9\u8870\u75c7\u793e\u533a\u5e26\u6765\u4e86\u65b0\u7684\u5e0c\u671b\u548c\u4e50\u89c2\uff0c\u201dPRF \u8054\u5408\u521b\u59cb\u4eba\u517c\u533b\u5b66\u603b\u76d1\u3001\u4e3b\u8981\u7814\u7a76\u4f5c\u8005 Leslie Gordon \u533b\u5b66\u535a\u58eb\u8bf4\u3002<\/p>\n<p>\u201c\u5728 PRF\uff0c\u6211\u4eec\u6b63\u5b5c\u5b5c\u4e0d\u5026\u5730\u4e3a\u60a3\u6709\u65e9\u8870\u75c7\u7684\u513f\u7ae5\u63d0\u4f9b\u65b0\u7684\u79d1\u5b66\u7a81\u7834\u8d44\u91d1\u3002\u8fd9\u9879\u7814\u7a76\u8868\u660e\uff0c\u4eca\u5929\u8fdb\u884c\u7684\u4e34\u5e8a\u8bd5\u9a8c\u662f\u6211\u4eec\u4e3a\u8fd9\u4e9b\u5b69\u5b50\u672a\u6765\u633d\u6551\u751f\u547d\u7684\u6700\u5927\u5e0c\u671b\u3002\u57fa\u4e8e lonafarnib \u8fd9\u9879\u7814\u7a76\u7684\u826f\u597d\u7ed3\u679c\uff0c\u6211\u4eec\u6bd4\u4ee5\u5f80\u4efb\u4f55\u65f6\u5019\u90fd\u611f\u5230\u66f4\u52a0\u7d27\u8feb\u3002\u4eca\u5929\u7684\u91cc\u7a0b\u7891\u6807\u5fd7\u7740\u8fd9\u4e9b\u5b69\u5b50\u53ca\u5176\u5bb6\u4eba\u7684\u65b0\u5f00\u59cb\u3002\u6bcf\u4e00\u5929\u3001\u6bcf\u4e00\u523b\u90fd\u5f88\u91cd\u8981\u3002PRF \u7684\u76ee\u6807\u662f\u627e\u5230\u6cbb\u7597\u65e9\u8870\u75c7\u7684\u65b9\u6cd5\uff0c\u8fd9\u9879\u7814\u7a76\u4f7f\u6211\u4eec\u5411\u8fd9\u4e00\u76ee\u6807\u53c8\u8fc8\u8fdb\u4e86\u4e00\u6b65\uff0c\u201dPRF \u603b\u88c1\u517c\u6267\u884c\u8463\u4e8b Meryl Fink \u8bf4\u9053<\/p>\n<p><strong>\u5a92\u4f53\u62a5\u9053<\/strong><\/p>\n<p>\u4ee5\u4e0b\u662f\u90e8\u5206\u65b0\u95fb\u62a5\u9053\uff1a<\/p>\n<ul>\n<li><strong>\u4eca\u65e5\u533b\u5b66\u9875\u9762<br \/>\n<\/strong><a href=\"https:\/\/www.medpagetoday.com\/blogs\/themethodsman\/72505\" target=\"_blank\" rel=\"noopener noreferrer\"><em>\u836f\u7269\u53ef\u4ee5\u4f7f\u65e9\u8870\u75c7\u53d8\u5f97\u53ef\u63a7 <\/em><\/a><\/li>\n<li><strong>\u6ce2\u58eb\u987f\u73af\u7403\u62a5<\/strong><br \/>\n<a href=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2018\/07\/The-Boston-Globe_A-pediatric-researcher-whose-son-died-of-progeria-sees-....pdf\" target=\"_blank\" rel=\"noopener noreferrer\"><em>\u513f\u5b50\u6b7b\u4e8e\u65e9\u8870\u75c7\u7684\u513f\u79d1\u7814\u7a76\u4eba\u5458\u770b\u5230\u4e86\u65b0\u7597\u6cd5\u7684\u5e0c\u671b<\/em> 2018 \u5e74 4 \u6708 24 \u65e5<\/a><\/li>\n<li><strong>\u5e03\u6717\u65b0\u95fb<\/strong><br \/>\n<a href=\"https:\/\/news.brown.edu\/articles\/2018\/04\/progeria\" target=\"_blank\" rel=\"noopener noreferrer\"><em>\u7814\u7a76\u8868\u660e\uff0c\u836f\u7269\u6cbb\u7597\u65e9\u8870\u75be\u75c5\u524d\u666f\u5149\u660e<\/em> 2018 \u5e74 4 \u6708 24 \u65e5<\/a><\/li>\n<\/ul>\n<p><em>\u201c\u8fd9\u4e2a\u6545\u4e8b\u5728\u5f88\u591a\u5c42\u9762\u4e0a\u90fd\u9f13\u821e\u4eba\u5fc3\u3002\u5b83\u6709\u53ef\u80fd\u6539\u53d8\u4e00\u7fa4\u975e\u5e38\u7279\u6b8a\u7684\u5b69\u5b50\u7684\u751f\u6d3b\u3002\u4f46\u5b83\u4e5f\u662f\u79d1\u5b66\u65b9\u6cd5\u7684\u5178\u8303\uff0c\u4e25\u8c28\u7684\u57fa\u7840\u79d1\u5b66\u548c\u73b0\u6709\u836f\u7269\u7684\u5de7\u5999\u5e94\u7528\u76f8\u7ed3\u5408\uff0c\u4ea7\u751f\u4e86\u771f\u6b63\u7684\u7a81\u7834\u6027\u6210\u679c\u3002\u201d<\/em><\/p>\n<p><em>\u2013 F. Perry Wilson \u533b\u5b66\u535a\u58eb\u3001MSCE\u3001\u8036\u9c81\u5927\u5b66\u533b\u5b66\u9662\u3001MedPage Today<\/em><\/p>\n<p><strong>\u6211\u4eec\u662f\u5982\u4f55\u8fce\u6765\u8fd9\u7f8e\u597d\u4e00\u5929\u7684\uff1f<\/strong><br \/>\n\u7ee7 <a href=\"https:\/\/www.progeriaresearch.org\/zh\/identification-of-gene-gives-hope-to-children-with-progeria\/\" target=\"_blank\" rel=\"noopener noreferrer\">2003 \u5e74\u53d1\u73b0\u57fa\u56e0\u7a81\u53d8<\/a> PRF \u8d44\u52a9\u7684\u7814\u7a76\u4eba\u5458\u53d1\u73b0\u5bfc\u81f4\u65e9\u8870\u75c7\u7684\u56e0\u7d20\u00a0<a href=\"https:\/\/www.progeriaresearch.org\/zh\/the-fti-drug\/\">\u5feb\u4f20\u53f7<\/a>\u00a0\u4f5c\u4e3a\u6f5c\u5728\u7684\u836f\u7269\u6cbb\u7597\u3002\u5bfc\u81f4\u65e9\u8870\u75c7\u7684\u7a81\u53d8\u4f1a\u5bfc\u81f4\u86cb\u767d\u8d28\u7684\u4ea7\u751f\u00a0<em>\u65e9\u8870\u7d20<\/em>\uff0c\u8fd9\u4f1a\u635f\u5bb3\u7ec6\u80de\u529f\u80fd\u3002\u65e9\u8001\u7d20\u5bf9\u4eba\u4f53\u7684\u6bd2\u6027\u4f5c\u7528\u90e8\u5206\u662f\u7531\u4e00\u79cd\u79f0\u4e3a\u201c\u6cd5\u5462\u57fa\u201d\u7684\u5206\u5b50\u5f15\u8d77\u7684\uff0c\u8fd9\u79cd\u5206\u5b50\u9644\u7740\u5728\u65e9\u8001\u7d20\u86cb\u767d\u4e0a\u5e76\u5e2e\u52a9\u5176\u635f\u5bb3\u4eba\u4f53\u7ec6\u80de\u3002FTI \u901a\u8fc7\u963b\u6b62\u6cd5\u5462\u57fa\u9644\u7740\u5728\u65e9\u8001\u7d20\u4e0a\u8d77\u4f5c\u7528\uff0c\u4ece\u800c\u51cf\u5c11\u65e9\u8001\u7d20\u9020\u6210\u7684\u5371\u5bb3\u3002<\/p>\n<p><strong>\u5982\u9700\u4e86\u89e3\u66f4\u591a\u7814\u7a76\u7ec6\u8282\uff0c\u00a0<a href=\"https:\/\/www.progeriaresearch.org\/wp-content\/uploads\/2018\/04\/PRF-JAMA-Announcement_Press-Release-1.pdf\">\u70b9\u51fb\u6b64\u5904\u67e5\u770b\u65b0\u95fb\u7a3f<\/a> <\/strong><\/p>\n<p><em>\u201c\u8bc1\u660e\u8fd9\u79cd\u7f55\u89c1\u81f4\u547d\u75be\u75c5\u5bf9\u8fd9\u4e00\u5c0f\u7fa4\u60a3\u513f\u7684\u6cbb\u7597\u6548\u679c\u662f\u4e00\u9879\u91cd\u5927\u6311\u6218\u3002\u56e0\u6b64\uff0c\u8fd9\u4e9b\u6700\u65b0\u53d1\u73b0\u4ee4\u6211\u7279\u522b\u9f13\u821e\u3002\u201d<\/em><\/p>\n<p><em>\u2013 \u7f8e\u56fd\u56fd\u7acb\u536b\u751f\u7814\u7a76\u9662\u9662\u957f\u5f17\u6717\u897f\u65af\u00b7\u67ef\u6797\u65af\u535a\u58eb<\/em><\/p>\n<p><strong>\u65e9\u8870\u75c7\u4e0e\u6b63\u5e38\u8870\u8001\u8fc7\u7a0b\u6709\u5173<\/strong><a href=\"https:\/\/web-beta.archive.org\/web\/20170128050324\/https:\/www.progeriaresearch.org\/whats_new_in_progeria_research.html#2\"><br \/>\n<\/a>\u7814\u7a76\u8868\u660e\uff0c\u5bfc\u81f4\u65e9\u8870\u75c7\u7684\u86cb\u767d\u8d28\u00a0<em>\u65e9\u8870\u7d20<\/em>\u00a0\u666e\u901a\u4eba\u7fa4\u4e2d\u4e5f\u4f1a\u4ea7\u751f\u8fd9\u79cd\u7269\u8d28\uff0c\u5e76\u4e14\u968f\u7740\u5e74\u9f84\u7684\u589e\u957f\u800c\u589e\u52a0\u3002\u7814\u7a76\u4eba\u5458\u8ba1\u5212\u7ee7\u7eed\u63a2\u7d22 FTI \u7684\u5f71\u54cd\uff0c\u8fd9\u53ef\u80fd\u6709\u52a9\u4e8e\u79d1\u5b66\u5bb6\u66f4\u591a\u5730\u4e86\u89e3\u5f71\u54cd\u6570\u767e\u4e07\u4eba\u7684\u5fc3\u8840\u7ba1\u75be\u75c5\u4ee5\u53ca\u5f71\u54cd\u6211\u4eec\u6240\u6709\u4eba\u7684\u6b63\u5e38\u8870\u8001\u8fc7\u7a0b\u3002<\/p>\n<p><strong>\u8c22\u8c22\u5927\u5bb6<\/strong>\u00a0<strong>\u6ca1\u6709\u4f60\u6211\u4eec\u5c31\u505a\u4e0d\u5230\uff01<\/strong><br \/>\n\u6211\u4eec\u53d6\u5f97\u8fd9\u4e9b\u7a81\u7834\u6027\u6210\u679c\u7684\u4e3b\u8981\u539f\u56e0\u4e4b\u4e00\u662f\u5f97\u76ca\u4e8e\u5927\u91cf\u652f\u6301\u8005\u63d0\u4f9b\u8d44\u91d1\u548c\u5176\u4ed6\u652f\u6301\uff0c\u5e2e\u52a9\u6211\u4eec\u5411\u6700\u7ec8\u76ee\u6807\u2014\u2014\u6cbb\u6108\u65e9\u8870\u75c7\u2014\u2014\u8fc8\u8fdb\u4e86\u4e00\u6b65\u3002<\/p>","protected":false},"excerpt":{"rendered":"<p>April 24, 2018: A new study published in The Journal of the American Medical Association (JAMA) reports that lonafarnib, a farnesyltransferase inhibitor (FTI), helped extend survival in children with Progeria. Authors from Boston Children\u2019s Hospital and Brown University tracked more than 250 children from six continents to demonstrate a link between lonafarnib treatment and extended [&hellip;]<\/p>","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_et_pb_use_builder":"","_et_pb_old_content":"","_et_gb_content_width":"","footnotes":"","_links_to":"","_links_to_target":""},"class_list":["post-4004","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v26.8 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>First Ever | The Progeria Research Foundation<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.progeriaresearch.org\/zh\/first-ever-demonstrate-survival-benefit-in-progeria\/\" \/>\n<meta property=\"og:locale\" content=\"zh_CN\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"First Ever | The Progeria Research Foundation\" \/>\n<meta property=\"og:description\" content=\"April 24, 2018: A new study published in The Journal of the American Medical Association (JAMA) reports that lonafarnib, a farnesyltransferase inhibitor (FTI), helped extend survival in children with Progeria. 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