{"id":417,"date":"2018-08-31T15:16:34","date_gmt":"2018-08-31T07:16:34","guid":{"rendered":"http:\/\/u2.cfoh.org.tw\/?page_id=417"},"modified":"2024-11-25T15:22:12","modified_gmt":"2024-11-25T07:22:12","slug":"ald","status":"publish","type":"page","link":"https:\/\/www.cfoh.org.tw\/index.php\/item\/ald\/","title":{"rendered":"\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7 (ALD)"},"content":{"rendered":"<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><span style=\"font-size: 14pt;\"><strong>\u4ec0\u9ebc\u662f\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\uff1f<\/strong><\/span><\/p>\n<p style=\"padding-left: 40px; text-align: justify;\">\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u4e3b\u8981\u662f\u7531\u65bc\u4f4d\u65bcX\u67d3\u8272\u9ad4\u4e0a\u4e4b<em>ABCD<\/em>1\uff08ATP-binding cassette, sub-family D\uff09\u57fa\u56e0\u767c\u751f\u7a81\u8b8a\uff0c\u9020\u6210\u7d30\u80de\u5167\u904e\u6c27\u5316\u5c0f\u9ad4\uff08peroxisome\uff09\u7121\u6cd5\u4ee3\u8b1d\u6975\u9577\u93c8\u98fd\u548c\u6027\u8102\u80aa\u9178\uff08very long-chain fatty acids\uff0c\u7c21\u7a31 VLCFA\uff0c\u5982C24\u53caC26\u78b3\u93c8\u8102\u80aa\u9178\uff09\uff0c\u5c0e\u81f4VLCFA\u5927\u91cf\u5806\u7a4d\u5728\u5927\u8166\u767d\u8cea\u548c\u814e\u4e0a\u817a\u76ae\u8cea\uff0c\u4fb5\u5bb3\u8166\u795e\u7d93\u7cfb\u7d71\u7684\u9ad3\u9798\u8cea\uff0c\u59a8\u7919\u795e\u7d93\u50b3\u5c0e\u529f\u80fd\uff0c\u4f7f\u5f97\u4e2d\u6a1e\u795e\u7d93\u767c\u5c55\u9072\u6eef\u9000\u5316\u3002<\/p>\n<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u60a3\u8005\u75c7\u72c0<\/span><\/strong><\/p>\n<p style=\"padding-left: 40px;\">\u6b64\u75be\u75c5\u53ef\u80fd\u5728\u4e0d\u540c\u5e74\u9f61\u767c\u75c5\uff0c\u81e8\u5e8a\u8868\u5fb5\u4ea6\u76f8\u7576\u591a\u6a23\u5316\u3002\u4f9d\u5176\u767c\u75c5\u5e74\u9f61\u53ca\u81e8\u5e8a\u75c7\u72c0\uff0c\u5927\u81f4\u53ef\u5206\u6210\u6578\u7a2e\u985e\u578b\uff1a<\/p>\n<ul style=\"text-align: justify;\">\n<li><strong>\u814e\u4e0a\u817a\u9ad3\u9798\u795e\u7d93\u75c5\u8b8a\u578b\uff08<\/strong>Adrenomyeloneuropathy\uff0c\u7c21\u7a31AMN\uff09<br \/>\n\u7d04\u4f54\u6240\u6709 ALD \u500b\u6848\u768440-45%\uff0c\u597d\u767c\u65bc20-40\u6b72\uff0c\u4e3b\u8981\u4fb5\u72af\u810a\u9ad3\u8207\u5468\u908a\u795e\u7d93\uff0c\u6545\u500b\u6848\u6703\u6709\u817f\u90e8\u50f5\u786c\u3001\u5168\u8eab\u7121\u529b\uff0c\u7121\u6cd5\u63a7\u5236\u62ec\u7d04\u808c\u7b49\u73fe\u8c61\u3002\u7d0410%\u81f320%\u7684\u500b\u6848\uff0c\u6703\u56e0\u8166\u90e8\u7684\u9000\u5316\uff0c\u6709\u56b4\u91cd\u7684\u8a8d\u77e5\u3001\u884c\u70ba\u53ca\u8a9e\u8a00\u969c\u7919\uff0c\u4e26\u60e1\u5316\u81f3\u5b8c\u5168\u5931\u80fd\u53ca\u55aa\u5931\u751f\u547d\u3002<\/li>\n<li><strong>\u8166\u767d\u8cea\u5316\u578b\uff08<\/strong>Cerebral ALD\uff09\n<ul style=\"text-align: justify; list-style-type: square;\">\n<li>\u5152\u7ae5\u5927\u8166\u578b\uff08Childhood Cerebral ALD\uff0c\u7c21\u7a31\u70baCCALD\uff09\uff1a\u7d04\u4f54\u6240\u6709ALD \u60a3\u8005\u768435%\uff0c\u7d04\u57283\u81f310\u6b72\u6642\u767c\u75c5\uff0c\u6703\u6709\u56b4\u91cd\u7684\u795e\u7d93\u75c7\u72c0\uff0c\u521d\u671f\u8868\u73fe\u51fa\u5b78\u7fd2\u7570\u5e38\u3001\u6ce8\u610f\u529b\u7f3a\u640d\u3001\u8996\u89ba\u53ca\u807d\u529b\u969c\u7919\uff0c\u723e\u5f8c\u53ef\u80fd\u51fa\u73fe\u8907\u8996\u53ca\u7672\u7647\u7684\u60c5\u6cc1\uff0c\u901a\u5e38\u65bc\u767c\u75c5\u5f8c2-3\u5e74\u5167\u75c5\u60c5\u6703\u8fc5\u901f\u60e1\u5316\uff0c\u9010\u6f38\u55aa\u5931\u795e\u7d93\u81ea\u4e3b\u53ca\u904b\u52d5\u80fd\u529b\uff0c\u751a\u81f3\u5371\u53ca\u751f\u547d\u3002<\/li>\n<li>\u9752\u5c11\u5e74\u5927\u8166\u578b\uff1a\u7d04\u4f54\u6240\u6709ALD \u60a3\u8005\u76844%-7%\uff0c\u7d04\u572810\u81f320\u6b72\u6642\u767c\u75c5\uff0c\u75c7\u72c0\u8207\u5152\u7ae5\u5927\u8166\u578b\u76f8\u4f3c\u3002<\/li>\n<li>\u6210\u4eba\u5927\u8166\u578b\uff1a\u7d04\u4f54\u6240\u6709ALD \u60a3\u8005\u76842%-5%\uff0c\u7d04\u572821\u6b72\u5f8c\u767c\u75c5\uff0c\u75c7\u72c0\u8207\u5152\u7ae5\u5927\u8166\u578b\u76f8\u4f3c\u3002<\/li>\n<\/ul>\n<\/li>\n<li><strong>\u611b\u8fea\u751f\u6c0f\u75c5\uff08<\/strong>Addison Disease\uff09<\/li>\n<li><strong>\u814e\u4e0a\u817a\u529f\u80fd\u7f3a\u5931<\/strong>\uff0c\u6700\u5e38\u898b\u5fb5\u72c0\u70ba\u75b2\u52de\uff0c\u808c\u8089\u7121\u529b\uff0c\u98df\u617e\u4e0d\u632f\uff0c\u9ad4\u91cd\u6e1b\u8f15\uff0c\u8179\u75db\u548c\u4e0d\u660e\u539f\u56e0\u7684\u5614\u5410\u3002<\/li>\n<li><strong>\u5973\u6027\u7570\u5408\u5b50\u75c5\u5fb5\u57fa\u56e0\u578b<\/strong>\uff08Female Symptomatic Heterozygote\uff09\uff1a\u7d04\u591a\u65bc50%\u5973\u6027\u5e36\u56e0\u8005\u53ef\u80fd\u6703\u572840-50\u6b72\u5de6\u53f3\u958b\u59cb\u51fa\u73fe\u8f03\u70ba\u8f15\u5fae\u7684AMN-like\u75c7\u72c0\u3002<\/li>\n<\/ul>\n<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u767c\u751f\u7387\u53ca\u907a\u50b3\u6a21\u5f0f<\/span><\/strong><\/p>\n<p style=\"padding-left: 40px;\">\u6839\u64da\u7d10\u7d04\uff0c\u52a0\u5dde\u53ca\u5eb7\u4e43\u72c4\u514b\u5dde\u4e4b\u5831\u5c0e\uff0c\u7537\u6027\u500b\u6848\u7684\u767c\u751f\u7387\u7d04\u70ba1:14000-1:35000\uff0c\u52a0\u4e0a\u5973\u6027\u7570\u5408\u5b50\u57fa\u56e0\u578b\u7684\u767c\u751f\u7387\u7d04\u70ba1:7000-1:17000\u3002<\/p>\n<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u65b0\u751f\u5152\u7be9\u6aa2\u901a\u77e5\u7570\u5e38(\u7591\u967d\u6027)\u4e4b\u8655\u7406?<\/span><\/strong><\/p>\n<p style=\"padding-left: 40px;\">\u65b0\u751f\u5152\u7be9\u6aa2\u53ef\u4ee5\u65e9\u671f\u5075\u6e2c\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\uff0c\u7be9\u6aa2\u65b9\u6cd5\u70ba\u6e2c\u91cf\u9ad4\u5167\u6975\u9577\u93c8\u8102\u80aa\u9178\u4e4b\u6fc3\u5ea6\u3002\u7576\u6975\u9577\u93c8\u8102\u80aa\u9178\u6fc3\u5ea6\u9ad8\u65bc\u6b63\u5e38\u503c\uff0c\u800c\u63a5\u7372\u63a1\u96c6\u9662\u6240\u901a\u77e5\u6642\uff0c\u8acb\u5118\u901f\u914d\u5408\u91ab\u7642\u4eba\u54e1\u6307\u793a\u9032\u884c\u8907\u6aa2\u3002\u82e5\u65b0\u751f\u5152\u6709\u76f8\u95dc\u81e8\u5e8a\u75c7\u72c0\u6642\uff0c\u8acb\u7acb\u5373\u8207\u65b0\u751f\u5152\u7be9\u6aa2\u4e2d\u5fc3\u6216\u7576\u5730\u6240\u5c6c\u8f49\u4ecb\u91ab\u9662\u91ab\u5e2b\u806f\u7d61\uff0c\u5c0b\u6c42\u53ca\u6642\u7684\u91ab\u7642\u5354\u52a9\u3002<\/p>\n<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u78ba\u8a3a\u8a55\u4f30\u65b9\u5f0f<\/span><\/strong><\/p>\n<ul style=\"text-align: justify;\">\n<li>\u81e8\u5e8a\u75c7\u72c0\u8a55\u4f30<\/li>\n<li>\u5bb6\u65cf\u53f2\u53ca\u5bb6\u65cf\u5206\u6790<\/li>\n<li><em>ABCD1<\/em>\u57fa\u56e0\u5206\u6790<\/li>\n<li>\u8166\u90e8\u96fb\u8166\u65b7\u5c64<\/li>\n<\/ul>\n<p class=\"ti_0 p_ul\" style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u6cbb\u7642\u65b9\u5f0f<\/span><\/strong><\/p>\n<ul>\n<li style=\"text-align: justify;\">\u7f85\u502b\u4f50\u7684\u6cb9\uff08Lorenzo&#8217;s Oil\uff09\uff1a\u542b\u6709\u4e0d\u98fd\u548c\u8102\u80aa\u9178\uff0c\u53ef\u4ee5\u6e1b\u4f4e\u500b\u6848\u9ad4\u5167\u5408\u6210VLCFA\uff0c\u5c0d\u65bc\u5c1a\u672a\u51fa\u73fe\u795e\u7d93\u75c7\u72c0\u7684\u500b\u6848\uff0c\u53ef\u4f7f\u75c5\u7a0b\u7684\u767c\u5c55\u901f\u5ea6\u6e1b\u7de9\uff0c\u70ba\u76ee\u524d\u666e\u904d\u63a1\u7528\u7684\u6cbb\u7642\u65b9\u5f0f\uff0c\u5c0d\u65bc\u5df2\u7d93\u7522\u751f\u7684\u795e\u7d93\u75c5\u8b8a\u53ca\u6210\u5e74\u60a3\u8005\u4e0d\u5177\u6cbb\u7642\u6548\u679c\u3002<\/li>\n<li style=\"text-align: justify;\">\u985e\u56fa\u9187\u88dc\u5145\u6cbb\u7642\uff1a\u5c0d\u65bc\u50c5\u6709\u814e\u4e0a\u817a\u529f\u80fd\u7f3a\u5931\u800c\u5c1a\u672a\u51fa\u73fe\u795e\u7d93\u75c7\u72c0\u7684\u500b\u6848\uff0c\u53ef\u4ee5\u670d\u7528\u985e\u56fa\u9187\u6539\u5584\u75c5\u60c5\uff0c\u5c0d\u65bc\u6709\u660e\u986f\u795e\u7d93\u75c7\u72c0\u7684\u60a3\u8005\u5247\u5e6b\u52a9\u4e0d\u5927\u3002<\/li>\n<li style=\"text-align: justify;\">\u9aa8\u9ad3\u79fb\u690d\uff1a\u5c0d\u65bc\u75be\u75c5\u65e9\u671f\u5c1a\u672a\u51fa\u73fe\u56b4\u91cd\u795e\u7d93\u6027\u75c7\u72c0\u7684\u500b\u6848\uff0c\u7d93\u8a55\u4f30\u5f8c\u53ef\u63a1\u7528\u6b64\u65b9\u6cd5\u9032\u884c\u6cbb\u7642\uff0c\u4f46\u5c0d\u65bc\u5df2\u6709\u660e\u986f\u795e\u7d93\u75c7\u72c0\u7684\u500b\u6848\uff0c\u4e26\u4e0d\u5efa\u8b70\u63a1\u7528\u9aa8\u9ad3\u79fb\u690d\uff1b\u6b64\u6cbb\u7642\u65b9\u6cd5\u9700\u8003\u616e\u624b\u8853\u5f8c\u53ef\u80fd\u6703\u7522\u751f\u6392\u65a5\u53cd\u61c9\u3002<\/li>\n<\/ul>\n<p class=\"ti_0 p_ul\"><strong><span style=\"font-size: 14pt;\">\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u7684\u7be9\u6aa2\u65b9\u6cd5<\/span><\/strong><\/p>\n<ul>\n<li>\u6aa2\u9a57\u65b9\u5f0f\uff1a\u751f\u5316\u6aa2\u9a57\uff0c\u6db2\u76f8\u5c64\u6790\u4e32\u806f\u8cea\u8b5c\u5100\u6cd5\uff08LC-MS\uff09\u3002\uff08107.17-\u81f3\u4eca\uff09<\/li>\n<li>\u6aa2\u9a57\u8a66\u5291\uff1aLDTs<\/li>\n<\/ul>\n","protected":false},"excerpt":{"rendered":"<p>\u4ec0\u9ebc\u662f\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\uff1f \u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7\u4e3b\u8981\u662f\u7531\u65bc\u4f4d\u65bcX\u67d3\u8272\u9ad4\u4e0a\u4e4bABCD1\uff08ATP-binding c &#8230; <a title=\"\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7 (ALD)\" class=\"read-more\" href=\"https:\/\/www.cfoh.org.tw\/index.php\/item\/ald\/\" aria-label=\"\u95b1\u8b80\u3008\u814e\u4e0a\u817a\u8166\u767d\u8cea\u5931\u990a\u75c7 (ALD)\u3009\u5168\u6587\">\u95b1\u8b80\u5168\u6587<\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"parent":781,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"jetpack_post_was_ever_published":false,"footnotes":""},"jetpack_sharing_enabled":true,"jetpack_shortlink":"https:\/\/wp.me\/P9uzrk-6J","_links":{"self":[{"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/417"}],"collection":[{"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/comments?post=417"}],"version-history":[{"count":5,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/417\/revisions"}],"predecessor-version":[{"id":2649,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/417\/revisions\/2649"}],"up":[{"embeddable":true,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/781"}],"wp:attachment":[{"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/media?parent=417"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}