{"id":46,"date":"2017-12-01T19:00:40","date_gmt":"2017-12-01T11:00:40","guid":{"rendered":"http:\/\/u2.cfoh.org.tw\/?page_id=46"},"modified":"2024-11-25T14:58:30","modified_gmt":"2024-11-25T06:58:30","slug":"mcad","status":"publish","type":"page","link":"https:\/\/www.cfoh.org.tw\/index.php\/item\/mcad\/","title":{"rendered":"\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7 (MCAD)"},"content":{"rendered":"<h2 style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u4f55\u8b02\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7?<\/span><\/strong><\/h2>\n<div class=\"artical\" style=\"text-align: justify;\">\n<p style=\"padding-left: 40px;\">\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7\u662f\u8102\u80aa\u9178\u4ee3\u8b1d\u7570\u5e38\u7684\u7f55\u898b\u75be\u75c5\uff0c\u5c6c\u65bc\u9ad4\u67d3\u8272\u9ad4\u96b1\u6027\u907a\u50b3\u75be\u75c5\u3002\u6211\u5011\u8eab\u9ad4\u901a\u5e38\u4ee5\u78b3\u6c34\u5316\u5408\u7269\u70ba\u6211\u5011\u7684\u4e3b\u8981\u80fd\u91cf\u4f86\u6e90\uff0c\u800c\u4ee5\u8102\u80aa\u70ba\u5132\u5b58\u80fd\u91cf\u65b9\u5f0f\u3002\u7576\u5728\u6211\u5011\u7684\u8eab\u9ad4\u88e1\u7684\u78b3\u6c34\u5316\u5408\u7269\u548c\u7cd6\u90fd\u4e0d\u5920\u7528\u6642\uff0c\u6211\u5011\u6703\u5206\u89e3\u8102\u80aa\u8f49\u63db\u70ba\u80fd\u91cf\u3002\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176(Medium chain acyl coenzyme A dehydrogenase)\u662f\u5206\u89e3\u8102\u80aa\u4ee3\u8b1d\u904e\u7a0b\u5176\u4e2d\u4e00\u500b\u9175\u7d20\u3002\u82e5\u9ad4\u5167\u7f3a\u5c11\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\uff0c\u5c07\u4f7f\u5f97\u8102\u80aa\u4ee3\u8b1d\u7121\u6cd5\u9032\u884c\u9806\u5229\uff0c\u56e0\u800c\u5c0e\u81f4\u90e8\u5206\u5206\u89e3\u7684\u8102\u80aa\u5806\u7a4d\u5728\u9ad4\u5167\u7522\u751f\u6bd2\u6027\uff0c\u5c0d\u5927\u8166\u548c\u795e\u7d93\u7cfb\u7d71\u9020\u6210\u50b7\u5bb3\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7\u4e4b\u75c7\u72c0?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u4e2d\u93c8\u8102\u80aa\u9178\u53bb\u6c2b\u9175\u7d20\u7f3a\u4e4f\u75c7(MCAD deficiency)\u7684\u75c5\u60a3\u901a\u5e38\u6703\u5728\u51fa\u751f\u5f8c\u7684\u524d\u5169\u5e74\u51fa\u73fe\u81e8\u5e8a\u75c7\u72c0\uff0c\u53ef\u80fd\u6703\u767c\u751f\u9032\u98df\u4e0d\u826f\u6240\u5f15\u767c\u7684\u5614\u5410\u3001\u809d\u81df\u816b\u5927\u3001\u4f4e\u8840\u916e\u6027\u4f4e\u8840\u7cd6\u3001\u610f\u8b58\u6a21\u7cca\u3001\u660f\u8ff7\u53ca\u62bd\u6410\u3002\u5728\u6025\u6027\u671f\uff0c\u8840\u4e2d\u7684\u6c28\u3001\u5c3f\u9178\u3001\u809d\u81df\u8f49\u80fa(liver transaminase)\u4ee5\u53ca\u808c\u9178\u78f7\u9178\u6fc0\u9176 (creatine phosphokinase)\u5e38\u5e38\u6703\u4e0a\u5347\uff1b\u809d\u81df\u5207\u7247\u53ef\u80fd\u6703\u770b\u5230\u5fae\u5c0f\u6c23\u6ce1\u72c0\u7684\u8102\u80aa\u6d78\u6f64\u3002\u4f46\u6709\u4e00\u4e9b\u7f3a\u4e4f\u6b64\u9175\u7d20\u7684\u75c5\u4eba\u76f4\u5230\u9752\u6625\u671f\u6216\u6210\u4eba\u671f\u624d\u6709\u81e8\u5e8a\u8868\u73fe\uff0c\u751a\u81f3\u4e5f\u6709\u4e00\u4e9b\u60a3\u8005\u5b8c\u5168\u6c92\u6709\u75c7\u72c0\u3002\u9019\u500b\u75be\u75c5\u53ef\u80fd\u88ab\u8aa4\u8a3a\u70ba\u96f7\u6c0f\u75c7\u5019\u7fa4\uff0c\u53e6\u5916\uff0c\u56e0\u70ba\u6b64\u75be\u75c5\u670925\uff05\u7684\u75c5\u4f8b\u5728\u7b2c\u4e00\u6b21\u767c\u4f5c\u6642\u6b7b\u4ea1\uff0c\u6240\u4ee5\u4e5f\u5e38\u5e38\u88ab\u8aa4\u8a3a\u70ba\u5b30\u5152\u731d\u6b7b\u75c7\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7\u767c\u751f\u7387\u70ba\u4f55?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u76ee\u524d\u570b\u5167\u767c\u751f\u7387\u4ecd\u5728\u8a55\u4f30\u4e2d\u3002\u6b64\u75c7\u76db\u884c\u65bc\u767d\u7a2e\u4eba\uff0c\u5c24\u5176\u662f\u5317\u6b50\u4eba\u7684\u5f8c\u88d4\uff0c\u5927\u81f4\u4e0a\u767c\u751f\u7387\u4f30\u8a08\u7d04\u70ba\u4e94\u5343\u81f3\u4e00\u842c\u4e03\u5343\u5206\u4e4b\u4e00\u3002\u800c\u7f8e\u570b\u7d71\u8a08\u7d04\u70ba\u4e00\u842c\u4e94\u5343\u5206\u4e4b\u4e00\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u65b0\u751f\u5152\u7be9\u6aa2\u901a\u77e5\u7570\u5e38(\u7591\u967d\u6027)\u4e4b\u8655\u7406?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u65b0\u751f\u5152\u7be9\u6aa2\u65b9\u6cd5\u662f\u6e2c\u5b9a\u6ffe\u7d19\u8840\u7247\u6aa2\u9ad4\u4e2d\u8f9b\u91af\u8089\u9e7c(C8)\uff0c\u5df1\u91af\u8089\u9e7c(C6)\uff0c\u7678\u91af\u8089\u9e7c(C10)\u7684\u542b\u91cf\uff0c\u53caC8\/C10\u6bd4\u503c\u3002\u7576\u8840\u7247\u542b\u91cf\u9ad8\u65bc\u6b63\u5e38\u503c\uff0c\u63a5\u7372\u901a\u77e5\u8acb\u5118\u901f\u914d\u5408\u91ab\u7642\u4eba\u54e1\u6307\u793a\u56de\u8a3a\uff0c\u5982\u5b30\u5152\u6709\u7279\u6b8a\u75c7\u72c0\u7522\u751f\u7acb\u5373\u8207\u65b0\u751f\u5152\u7be9\u6aa2\u4e2d\u5fc3\u6216\u7576\u5730\u6240\u5c6c\u8f49\u4ecb\u91ab\u9662\u806f\u7d61\uff0c\u5c0b\u6c42\u6b63\u78ba\u91ab\u7642\u652f\u63f4\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u5982\u4f55\u78ba\u8a8d\u8a3a\u65b7\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7?<\/span><\/strong><\/h3>\n<ul>\n<li>\u5728\u6c92\u6709\u767c\u75c5\u6642\uff0c\u4e32\u806f\u8cea\u8b5c\u5100\u7684\u8840\u6e05\u91af\u57fa\u8089\u9e7c\u5206\u6790\uff0c\u5728\u75c5\u4eba\u4ecd\u6703\u770b\u5230C8\uff0cC8:1\u548cC10:1\u6aa2\u67e5\u503c\u5347\u9ad8\uff0c\u56e0\u6b64\u4efb\u4f55\u6642\u5019\u61f7\u7591\u6b64\u75c5\u6642\uff0c\u90fd\u61c9\u8a72\u8981\u9032\u884c\u4e32\u806f\u8cea\u8b5c\u5100\u7684\u8840\u6e05\u91af\u57fa\u8089\u9e7c\u5206\u6790\u3002\u5728\u6025\u6027\u671f\u6642\uff0c\u60a3\u8005\u6703\u51fa\u73feC6\uff0cC8\u548cC10\u96d9\u7fa7\u57fa\u9178\u5c3f\u75c7 (dicaboxylic aciduria)\uff0c\u7136\u800c\u9019\u6a23\u7684\u767c\u73fe\u5e38\u5e38\u6703\u88ab\u5ffd\u7565\uff0c\u56e0\u70ba\u96d9\u7fa7\u57fa\u9178\u5c3f\u75c7\u4e5f\u53ef\u80fd\u7531\u5176\u4ed6\u539f\u56e0\u6240\u7522\u751f\u3002<\/li>\n<li>\u5c3f\u6db2\u4e2d\u6709hexanoylglycine\u548csuberylglycine\u51fa\u73fe\uff0c\u5247\u53ef\u4ee5\u52a0\u5f37\u8a3a\u65b7\u7684\u53ef\u9760\u6027\u3002<\/li>\n<li>\u6e2c\u5b9a\u7e96\u7dad\u6bcd\u7d30\u80de\u9175\u7d20\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7684\u6d3b\u6027\u3002<\/li>\n<\/ul>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u5982\u4f55\u6cbb\u7642\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7?<\/span><\/strong><\/h3>\n<ul>\n<li>\u6025\u6027\u671f\u7684\u8655\u7406\u4e3b\u8981\u662f\u6cbb\u7642\u4f4e\u8840\u7cd6\u72c0\u6cc1<\/li>\n<li>\u9577\u671f\u7684\u6cbb\u7642\u4e3b\u8981\u662f\u98f2\u98df\u65b9\u9762\u61c9\u5c11\u91cf\u591a\u9910\uff0c\u907f\u514d\u9577\u6642\u9593\u7a7a\u8179\u8207\u98e2\u9913\uff0c\u8981\u5728\u5c31\u5be2\u524d\u63d0\u4f9b\u78b3\u6c34\u5316\u5408\u7269\u9ede\u5fc3\u3002\u9664\u5916\u9700\u7a4d\u6975\u6cbb\u7642\u611f\u67d3\u6216\u80c3\u8178\u708e\u7b49\u7a81\u767c\u72c0\u6cc1\u3002<\/li>\n<li>\u53e3\u670d\u8089\u9e7c(100mg\/kg\/day)\u53ef\u4ee5\u77ef\u6b63\u8089\u9e7c\u7f3a\u4e4f\u72c0\u6cc1\uff0c\u4e5f\u53ef\u4ee5\u589e\u52a0\u6709\u6bd2\u4e2d\u9593\u7522\u7269\u7684\u6392\u51fa(\u6bd4\u5982\u4e00\u4e9b\u8089\u9e7c\u916f)\u3002<\/li>\n<\/ul>\n<\/div>\n<div class=\"artical\">\n<h3 style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7\u7684\u9810\u5f8c\u5982\u4f55?<\/span><\/strong><\/h3>\n<p style=\"text-align: justify; padding-left: 40px;\">\u767c\u5c55\u9072\u7de9\uff0c\u884c\u70ba\u554f\u984c\u548c\u5176\u4ed6\u6162\u6027\u4e2d\u6a1e\u795e\u7d93\u7cfb\u7d71\u7684\u969c\u7919\u5728\u4e00\u958b\u59cb\u767c\u75c5\u6642\uff0c\u4e26\u975e\u4e0d\u5e38\u898b\u3002\u5047\u5982\u9019\u6a23\u7684\u50b7\u5bb3\u6c92\u6709\u767c\u751f\uff0c\u5247\u6700\u7d42\u7684\u9810\u5f8c\u662f\u76f8\u7576\u597d\u7684\u3002.<\/p>\n<h3 style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7<\/span><\/strong><strong><span style=\"font-size: 14pt;\">\u7684\u7be9\u6aa2\u65b9\u6cd5<\/span><\/strong><\/h3>\n<ul>\n<li style=\"text-align: justify;\">\u6aa2\u9a57\u65b9\u5f0f\uff1a\u751f\u5316\u6aa2\u9a57\uff0c\u4f7f\u7528\u542b\u7a69\u5b9a\u540c\u4f4d\u7d20\u6a19\u8a18\u7684\u5167\u90e8\u6a19\u6e96\u54c1\uff08Internal Standard\uff09\u6eb6\u5291\u8403\u53d6\u4e7e\u71e5\u8840\u9ede\u4e2d\u7684\u8102\u80aa\u9178\uff0c\u518d\u4ee5\u4e32\u806f\u8cea\u8b5c\u5100\uff08MS\/MS\uff09\u9032\u884c\u5206\u6790\u3002\uff08111.3-\u81f3\u4eca\uff09<\/li>\n<li style=\"text-align: justify;\">\u6aa2\u9a57\u8a66\u5291\uff1aNeoBase<sup>TM<\/sup>\u00a02 Non-derivatized MSMS kit\uff08PerkinElmer\uff09<\/li>\n<\/ul>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>\u4f55\u8b02\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7? \u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7\u662f\u8102\u80aa\u9178\u4ee3\u8b1d\u7570\u5e38\u7684\u7f55\u898b\u75be\u75c5\uff0c\u5c6c\u65bc\u9ad4\u67d3\u8272\u9ad4\u96b1\u6027\u907a\u50b3\u75be\u75c5\u3002 &#8230; <a title=\"\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7 (MCAD)\" class=\"read-more\" href=\"https:\/\/www.cfoh.org.tw\/index.php\/item\/mcad\/\" aria-label=\"\u95b1\u8b80\u3008\u4e2d\u93c8\u91af\u8f14\u9176A\u53bb\u6c2b\u9176\u7f3a\u4e4f\u75c7 (MCAD)\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-K","_links":{"self":[{"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/46"}],"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=46"}],"version-history":[{"count":7,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/46\/revisions"}],"predecessor-version":[{"id":2629,"href":"https:\/\/www.cfoh.org.tw\/index.php\/wp-json\/wp\/v2\/pages\/46\/revisions\/2629"}],"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=46"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}