{"id":67965,"date":"2017-01-23T12:24:21","date_gmt":"2017-01-23T18:24:21","guid":{"rendered":"https:\/\/www.erdheim-chester.org\/%e5%ae%9a%e7%be%a9-erdheim-chester-%e7%97%85%e7%9a%84%e8%b5%b7%e6%ba%90%e7%b4%b0%e8%83%9e\/"},"modified":"2025-09-11T09:46:10","modified_gmt":"2025-09-11T14:46:10","slug":"%e5%ae%9a%e7%be%a9-erdheim-chester-%e7%97%85%e7%9a%84%e8%b5%b7%e6%ba%90%e7%b4%b0%e8%83%9e","status":"publish","type":"post","link":"https:\/\/www.erdheim-chester.org\/zh-hant\/%e5%ae%9a%e7%be%a9-erdheim-chester-%e7%97%85%e7%9a%84%e8%b5%b7%e6%ba%90%e7%b4%b0%e8%83%9e\/","title":{"rendered":"\u5b9a\u7fa9 Erdheim-Chester \u75c5\u7684\u8d77\u6e90\u7d30\u80de"},"content":{"rendered":"[et_pb_section global_module=\"47423\"][\/et_pb_section]\n<h4><strong>\u7372\u734e\u5e74\u4efd\uff1a<\/strong> 2017<br \/>\n<strong>\u91d1\u984d\uff1a<\/strong> 50,000 \u7f8e\u5143<\/h4>\n<p>\u4f4d\u65bc\u7d10\u7d04\u7684\u7d00\u5ff5\u65af\u9686-\u51f1\u7279\u7433\u764c\u75c7\u4e2d\u5fc3\u7372\u5f97\u4e86 50,000 \u7f8e\u5143\u7684\u7814\u7a76\u8cc7\u52a9\u3002\u9996\u5e2d\u7814\u7a76\u54e1\uff0c\u91ab\u5b78\u535a\u58eb Benjamin H. Durham \u9818\u5c0e\u4e86\u7814\u7a76 \u5b9a\u7fa9 Erdheim-Chester \u75c5\u7684\u8d77\u6e90\u7d30\u80de\u3002\u5c07\u7814\u7a76 ECD \u60a3\u8005\u7684\u9aa8\u9ad3\u7d30\u80de\uff0c\u4ee5\u5617\u8a66\u78ba\u5b9a\u5c0e\u81f4 ECD \u7684\u7d30\u80de\u3002\u6709\u4e86\u9019\u4e9b\u8cc7\u8a0a\uff0c\u5c31\u6709\u53ef\u80fd\u5275\u5efa\u4e00\u500b\u6210\u529f\u7684 ECD \u6a21\u578b\u53ef\u7528\u65bc\u4f86\u81ea\u4e16\u754c\u5404\u5730\u7684\u79d1\u5b78\u5bb6\u7684\u672a\u4f86\u7814\u7a76\u3002\u672c\u7814\u7a76\u7684\u7b2c\u4e8c\u500b\u76ee\u6a19\u662f\u5c07 ECD \u7d30\u80de\u7684\u57fa\u56e0\u8b5c\u8207 ECD \u60a3\u8005\u4e2d\u5e38\u898b\u7684\u5176\u4ed6\u8840\u764c\u7d30\u80de\u9032\u884c\u6bd4\u8f03\u3002Durham \u535a\u58eb\u662f\u4e00\u540d\u8840\u6db2\u75c5\u7406\u5b78\u5bb6\u548c\u5206\u5b50\u907a\u50b3\u75c5\u7406\u5b78\u5bb6\uff0c\u4ed6\u4e4b\u524d\u5df2\u7d93\u78ba\u5b9a\u4e86\u8207 ECD \u76f8\u95dc\u7684\u5404\u7a2e\u57fa\u56e0\u7a81\u8b8a\u3002\u9019\u4e9b\u767c\u73fe\u70ba\u60a3\u8005\u63d0\u4f9b\u4e86\u5373\u6642\u7684\u6cbb\u7642\u9078\u64c7\u3002<\/p>\n<h3><strong>\u6700\u7d42\u5831\u544a<\/strong><\/h3>\n<p>\u901a\u904e\u5c0d\u8fc4\u4eca\u70ba\u6b62\u7528\u7d44\u7e54\u7d30\u80de\u816b\u7624\u6e2c\u5e8f\u7684\u6700\u5927\u5152\u7ae5\u548c\u6210\u4eba\u7fa4\u9ad4\uff08270 \u540d\u60a3\u8005\uff09\u9032\u884c\u5168\u9762\u7684\u57fa\u56e0\u7d44\u6e2c\u5e8f\u5206\u6790\uff0c\u5176\u4e2d\u6700\u5927\u7684\u4e9e\u7d44\u662f Erdheim-Chester \u75c5 \uff08ECD\uff09\uff0c\u73fe\u5df2\u5728 ECD \u548c\u5176\u4ed6\u7d44\u7e54\u7d30\u80de\u589e\u591a\u75c7\u4e2d\u767c\u73fe\u4e86\u5e7e\u7a2e\u65b0\u7684\u548c\u53ef\u9776\u5411\u7684\u6fc0\u9176\u907a\u50b3\u6539\u8b8a\u3002\u5927\u7d04 600 \u5e74\u524d\u5728\u9019\u4e9b\u75be\u75c5\u4e2d\u767c\u73fe BRAFV8Emutations \u5c0e\u81f4\u6211\u5011\u5c07\u9019\u4e9b\u75be\u75c5\u7406\u89e3\u70ba\u4e00\u7a2e\u764c\u75c7\uff0c\u672a\u4f86\u7684\u7814\u7a76\u5c0e\u81f4 FDA \u6279\u51c6 vemurafenib \u7528\u65bc BRAFV600E \u7a81\u8b8a ECD \u7684\u6210\u5e74\u60a3\u8005\uff08Hyman\uff0c D \u7b49\u4eba\uff0cNEJM 2015\uff09\u3002\u7136\u800c\uff0c\u76ee\u524d\u5c1a\u4e0d\u6e05\u695a\u54ea\u4e9b\u7d30\u80de\u985e\u578b\u5c0e\u81f4 ECD \u548c\u5176\u4ed6\u7d44\u7e54\u7d30\u80de\u589e\u591a\u75c7\u7684\u767c\u5c55 \uff08Mass E et al. Nature 2017\uff09\uff0c\u4e5f\u6c92\u6709\u5b9a\u7fa9\u9019\u7a2e\u75be\u75c5\u7684\u6240\u6709\u907a\u50b3\u6539\u8b8a\u3002\u5728\u9019\u88e1\uff0c\u6211\u5011\u78ba\u5b9a\u4e86\u4efb\u4f55\u75be\u75c5\u4e2d\u5fa9\u767c\u6027 CSF1R\u6fc0\u9176 \u7a81\u8b8a\u7684\u7b2c\u4e00\u500b\u4f8b\u5b50\uff0c\u4ee5\u53ca\u9a45\u52d5 ECD \u548c\u5176\u4ed6\u7d44\u7e54\u7d30\u80de\u589e\u591a\u75c7\u7684\u8a31\u591a\u5176\u4ed6\u65b0\u6fc0\u9176\u6539\u8b8a\u3002   <\/p>\n<p>CSF1R \u7a81\u8b8a\u7684\u767c\u73fe\u610f\u7fa9\u91cd\u5927\uff0c\u56e0\u70ba \uff081\uff09 CSF1R \u5b9a\u7fa9\u4e86\u55ae\u6838\u7d30\u80de\/\u5de8\u566c\u7d30\u80de\u8b5c\u7cfb\uff0c\u56e0\u6b64\u63ed\u793a\u4e86\u6e90\u81ea\u55ae\u6838\u7d30\u80de\/\u5de8\u566c\u7d30\u80de\u7684\u7d44\u7e54\u7d30\u80de\u816b\u7624\uff0c\u4ee5\u53ca \uff082\uff09 \u6709\u8a31\u591a CSF1R \u6291\u88fd\u5291\u6b63\u5728\u958b\u767c\u4e2d\uff08\u5176\u4e2d\u4e00\u7a2e\u5df2\u7d93\u88ab FDA \u6279\u51c6\u7528\u65bc\u5176\u4ed6\u75be\u75c5\uff09\u3002\u6211\u5011\u9084\u5c55\u793a\u4e86 RET \u91cd\u6392\u7d44\u7e54\u7d30\u80de\u589e\u591a\u75c7\u4e2d\u5c0d RET \u6291\u5236\u7684\u81e8\u5e8a\u53cd\u61c9\u7684\u7b2c\u4e00\u500b\u4f8b\u5b50\uff0c\u4ee5\u53ca CSF1R \u7a81\u8b8a\u7d30\u80de\u4e2d\u5c0d CSF1R \u6291\u5236\u7684\u81e8\u5e8a\u524d\u53cd\u61c9\u7684\u8b49\u64da\u3002\u9019\u4e9b\u6578\u64da\u5177\u6709\u9ad8\u5ea6\u7684\u81e8\u5e8a\u91cd\u8981\u6027\uff0c\u4e26\u4e14\u7531\u65bc\u5c0d\u764c\u75c7\u4e2d CSF1R \u6291\u5236\u3001RET \u6291\u5236\u548c ECD \u7b49\u7f55\u898b\u75be\u75c5\u7684\u7cbe\u6e96\u91ab\u5b78\u7684\u8208\u596e\uff0c\u9019\u4e9b\u6578\u64da\u5c07\u7acb\u5373\u5f15\u8d77\u4eba\u5011\u7684\u8208\u8da3\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\u8cc7\u52a9\uff0c\u7528\u65bc Benjamin H. Durham \u535a\u58eb\u5c0d Erdheim-Chester \u75c5\u7d30\u80de\u8d77\u6e90\u7684\u7814\u7a76\u3002<br \/>\n\u5168\u9762\u7684\u57fa\u56e0\u7d44\u7814\u7a76\u767c\u73fe\u4e86\u91cd\u8981\u7684\u57fa\u56e0\u7a81\u8b8a\uff0c\u5305\u62ec\u6709\u53f2\u4ee5\u4f86\u7b2c\u4e00\u500b CSF1R \u7a81\u8b8a\uff0c\u63ed\u793a\u4e86 ECD \u662f\u4e00\u7a2e\u6e90\u81ea\u55ae\u6838\u7d30\u80de\/\u5de8\u566c\u7d30\u80de\u7684\u75be\u75c5\uff0c\u4e26\u53ef\u80fd\u958b\u95e2\u65b0\u7684\u6cbb\u7642\u9014\u5f91\u3002<\/p>\n","protected":false},"author":20,"featured_media":92358,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_et_pb_use_builder":"","_et_pb_old_content":"","_et_gb_content_width":"","_wds_title":"","_wds_metadesc":"","_wds_focus-keywords":"","_wds_meta-robots-adv":"","_wds_meta-robots-noindex":false,"_wds_meta-robots-nofollow":false,"_wds_meta-robots-index":false,"_wds_meta-robots-follow":false,"_wds_autolinks-exclude":false,"_wds_canonical":"","_wds_redirect":"","_wds_opengraph":[],"_wds_twitter":[],"cybocfi_hide_featured_image":"","wds_primary_category":0,"footnotes":"","_links_to":"","_links_to_target":""},"categories":[604],"tags":[2099],"class_list":["post-67965","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-604","tag-2017-zh-hant"],"_links":{"self":[{"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/posts\/67965","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/users\/20"}],"replies":[{"embeddable":true,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/comments?post=67965"}],"version-history":[{"count":0,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/posts\/67965\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/media\/92358"}],"wp:attachment":[{"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/media?parent=67965"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/categories?post=67965"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.erdheim-chester.org\/zh-hant\/wp-json\/wp\/v2\/tags?post=67965"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}