{"id":68063,"date":"2013-01-23T10:17:16","date_gmt":"2013-01-23T16:17:16","guid":{"rendered":"https:\/\/www.erdheim-chester.org\/%e7%b5%84%e7%b9%94%e7%b4%b0%e8%83%9e%e7%96%be%e7%97%85%e7%99%bc%e7%97%85%e6%a9%9f%e5%88%b6%e5%92%8c%e6%b2%bb%e7%99%82%e4%b8%ad%e7%9a%84%e9%ab%94%e7%b4%b0%e8%83%9e%e9%81%ba%e5%82%b3%e6%94%b9%e8%ae%8a\/"},"modified":"2025-09-11T09:49:21","modified_gmt":"2025-09-11T14:49:21","slug":"%e7%b5%84%e7%b9%94%e7%b4%b0%e8%83%9e%e7%96%be%e7%97%85%e7%99%bc%e7%97%85%e6%a9%9f%e5%88%b6%e5%92%8c%e6%b2%bb%e7%99%82%e4%b8%ad%e7%9a%84%e9%ab%94%e7%b4%b0%e8%83%9e%e9%81%ba%e5%82%b3%e6%94%b9%e8%ae%8a","status":"publish","type":"post","link":"https:\/\/www.erdheim-chester.org\/zh-hant\/%e7%b5%84%e7%b9%94%e7%b4%b0%e8%83%9e%e7%96%be%e7%97%85%e7%99%bc%e7%97%85%e6%a9%9f%e5%88%b6%e5%92%8c%e6%b2%bb%e7%99%82%e4%b8%ad%e7%9a%84%e9%ab%94%e7%b4%b0%e8%83%9e%e9%81%ba%e5%82%b3%e6%94%b9%e8%ae%8a\/","title":{"rendered":"\u7d44\u7e54\u7d30\u80de\u75be\u75c5\u767c\u75c5\u6a5f\u5236\u548c\u6cbb\u7642\u4e2d\u7684\u9ad4\u7d30\u80de\u907a\u50b3\u6539\u8b8a"},"content":{"rendered":"[et_pb_section global_module=\"47408\"][\/et_pb_section]\n<h4><strong>\u6388\u4e88\u5e74\u4efd\uff1a<\/strong> 2013<br \/>\n<strong>\u91d1\u984d\uff1a <\/strong>50,000 \u7f8e\u5143<\/h4>\n<p>2013 \u5e74 9 \u6708\uff0c ECD \u806f\u76df\u5ba3\u4f48\u5411\u7d10\u7d04\u7d00\u5ff5\u65af\u9686-\u51f1\u7279\u7433\u764c\u75c7\u4e2d\u5fc3 \uff08MSKCC\uff09 \u7684 Omar Abdel-Wahab \u91ab\u5b78\u535a\u58eb\u3001David Hyman \u91ab\u5b78\u535a\u58eb\u548c Eli Diamond \u91ab\u5b78\u535a\u58eb\u63d0\u4f9b 50,000 \u7f8e\u5143\u7684 ECD \u8d08\u6b3e\uff0c\u7528\u65bc\u7814\u7a76\u300c\u904e\u654f\u6027\u75be\u75c5\u7684\u75c5\u539f\u9ad4\u548c\u6cbb\u7642\u4e2d\u7684\u9ad4\u7d30\u80de\u907a\u50b3\u6539\u8b8a\u300d\u3002\u6b63\u5982 <a href=\"http:\/\/www.mskcc.org\/pressroom\/50-000-research-funds-go-mskcc-study-erdheim-chester-disease\" target=\"_blank\" rel=\"noopener\">MSKCC \u65b0\u805e\u7a3f<\/a>\u4e2d\u6240\u8ff0\uff0c\u672c\u7814\u7a76\u7684\u76ee\u7684\u662f\uff1a <\/p>\n<ol>\n<li>\u8b58\u5225 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\u7a81\u8b8a\uff0c\u8207\u6cd5\u570b\u7684 Julien Haroche \u548c Jean-Francois Emile \u535a\u58eb\u5408\u4f5c\u9032\u884c\u4e86\u4e00\u9805\u5927\u578b ECD \u57fa\u56e0\u7d44\u7814\u7a76\u60a3\u8005\uff0c\u4e26\u7e7c\u7e8c\u5728 ECD \u4e2d\u9032\u884c\u751a\u81f3\u66f4\u5927\u898f\u6a21\u7684\u57fa\u56e0\u7d44\u5b78\u7814\u7a76\u3002\u8a72\u5c08\u6848\u90e8\u5206\u767c\u8868\u4e86\u591a\u7bc7\u671f\u520a\u6587\u7ae0\u3002   <\/p>\n<h3><strong>\u6700\u7d42\u5831\u544a<\/strong><\/h3>\n<ul>\n<li>Vemurafenib \u5728\u651c\u5e36 BRAFV600E \u7a81\u8b8a\u7684 ECD \u548c LCH \u60a3\u8005\u4e2d\u7684 II \u671f\u7814\u7a76\u5df2\u5b8c\u6210\u3002\u9019\u9805\u7814\u7a76\u662f\u5728\u7f85\u6c0f\u8d0a\u52a9\u7684\u4e00\u9805\u570b\u969b\u7814\u7a76\u7684\u80cc\u666f\u4e0b\u9032\u884c\u7684\uff0c\u7d50\u679c\u63d0\u4ea4\u7d66\u4e86\u300c\u65b0\u82f1\u683c\u862d\u91ab\u5b78\u96dc\u8a8c\u300d\uff0cHyman \u535a\u58eb\u662f\u7b2c\u4e00\u4f5c\u8005\u3002\u9019\u9805\u7814\u7a76\u7684\u7d50\u679c\u5c07\u5c0e\u81f4 FDA \u6279\u51c6 vemurafenib \u7528\u65bc\u7f8e\u570b\u5177\u6709 BRAFV600E \u7a81\u8b8a\u7684 ECD \u548c LCH \u60a3\u8005\u3002  <\/li>\n<li>\u6b64\u5916\uff0cMEK \u6291\u88fd\u5291 cobimetinib \u7528\u65bc\u7121 BRAFV600E \u7a81\u8b8a\u7684 ECD \u60a3\u8005\u7684 II \u671f\u81e8\u5e8a\u8a66\u9a57\u5df2\u958b\u555f\u3002\u8a72\u8a66\u9a57\u7531 Genentech \u8d0a\u52a9\uff0c\u81ea 2015 \u5e74\u6625\u5b63\u8d77\u958b\u59cb\u63a5\u53d7\u60a3\u8005\u62db\u52df\u3002 <\/li>\n<\/ul>\n<p>\u4f5c\u70ba vemurafenib \u81e8\u5e8a\u8a66\u9a57\u7684\u5fc5\u7136\u7d50\u679c\uff0c\u8207 MD \u5b89\u5fb7\u68ee\u764c\u75c7\u4e2d\u5fc3\u7684 Filip Janku \u535a\u58eb\u9032\u884c\u4e86\u4e00\u9805\u5408\u4f5c\u7814\u7a76\uff0c\u4ee5\u4e86\u89e3\u7121\u7d30\u80de\u7684\u6548\u7528<\/p>\n<ul>\n<li>DNA \u5206\u6790\u4ee5\u8b58\u5225\u548c\u8ddf\u8e64 ECD \u60a3\u8005\u7684 BRAFV600E 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\u7a81\u8b8a\uff09\u3002\u9019\u4ef6\u4f5c\u54c1\u767c\u8868\u5728\u300cBlood\u300d\u96dc\u8a8c\u4e0a\uff0c\u4e26\u767b\u4e0a\u4e86\u300cBlood\u300d\u7684\u5c01\u9762\u548c\u300cInside Blood\u300d\u793e\u8ad6\u3002  <\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u7d00\u5ff5\u65af\u9686-\u51f1\u7279\u7433\u764c\u75c7\u4e2d\u5fc3\u7372\u5f97 50,000 \u7f8e\u5143\u7684\u8d08\u6b3e\uff0c\u7528\u65bc\u7814\u7a76\u7d44\u7e54\u7d30\u80de\u75be\u75c5\u7684\u57fa\u56e0\u6539\u8b8a\u3002\u8a72\u7814\u7a76\u4fc3\u6210\u4e86 vemurafenib \u7684\u958b\u5275\u6027 II \u671f\u7814\u7a76\uff0c\u63ed\u793a\u4e86\u654f\u611f\u7684\u7a81\u8b8a\u8ffd\u8e64\u65b9\u6cd5\u4e26\u78ba\u5b9a\u4e86 Erdheim-Chester \u75c5\u60a3\u8005\u7684\u591a\u500b\u53ef\u9776\u5411\u57fa\u56e0\u7a81\u8b8a\u3002 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