{"id":42,"date":"2017-12-01T13:14:55","date_gmt":"2017-12-01T05:14:55","guid":{"rendered":"http:\/\/u2.cfoh.org.tw\/?page_id=42"},"modified":"2024-11-25T14:39:41","modified_gmt":"2024-11-25T06:39:41","slug":"msud","status":"publish","type":"page","link":"https:\/\/www.cfoh.org.tw\/index.php\/item\/msud\/","title":{"rendered":"\u6953\u6f3f\u5c3f\u75c7 (MSUD)"},"content":{"rendered":"<h2 style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u4f55\u8b02\u6953\u6f3f\u5c3f\u75c7?<\/span><\/strong><\/h2>\n<div class=\"artical\" style=\"text-align: justify;\">\n<p style=\"padding-left: 40px;\">\u6953\u7cd6\u5c3f\u75c7\u662f\u7279\u6b8a\u652f\u93c8\u80fa\u57fa\u9178\u4ee3\u8b1d\u7570\u5e38\u7684\u7f55\u898b\u75be\u75c5\uff0c\u5c6c\u65bc\u9ad4\u67d3\u8272\u9ad4\u96b1\u6027\u907a\u50b3\u75be\u75c5\u3002\u662f\u56e0\u9ad4\u5167\u7f3a\u5c11\u652f\u93c8\u7532\u578b\u916e\u9178\u812b\u6c2b\u9176(Branched-Chain \u03b1-Keto Acid Dehydrogenase)\uff0c\u4f7f\u5f97\u652f\u93c8\u80fa\u57fa\u9178\uff08\u7e88\u80fa\u9178\u3001\u767d\u80fa\u9178\u3001\u7570\u767d\u80fa\u9178\uff09\u7684\u4ee3\u8b1d\u7121\u6cd5\u9032\u884c\u9806\u5229\uff0c\u56e0\u800c\u9019\u4e09\u500b\u652f\u93c8\u80fa\u57fa\u9178\u5806\u7a4d\u5728\u9ad4\u5167\u7522\u751f\u6bd2\u6027\uff0c\u5c0d\u8166\u7d30\u80de\u9020\u6210\u50b7\u5bb3\uff0c\u7531\u65bc\u60a3\u8005\u7684\u9ad4\u6db2\u548c\u5c3f\u6db2\u6703\u6709\u6953\u6a39\u7cd6\u6f3f\u7684\u751c\u5473\u56e0\u800c\u547d\u540d\u70ba\u6953\u6f3f\u5c3f\u75c7\uff08Maple Syrup Urine Disease\uff0c\u7c21\u7a31MSUD\uff09\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u6953\u6f3f\u5c3f\u75c7\u4e4b\u75c7\u72c0?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u901a\u5e38\u7f79\u60a3\u5178\u578b\u6b64\u75c7\u7684\u5b30\u5152\uff0c\u5728\u958b\u59cb\u9935\u98df\u5f8c\u6578\u5929\u81f37\u5929\u5167\uff0c\u6703\u9010\u6f38\u51fa\u73fe\u5614\u5410\u3001\u55dc\u7761\u3001\u98df\u617e\u6e1b\u4f4e\u3001\u547c\u5438\u6025\u4fc3\u3001\u9ec3\u75b8\u3001\u62bd\u6410\u7b49\u73fe\u8c61\uff0c\u8eab\u4e0a\u6563\u767c\u7126\u7cd6\u9ad4\u5473\u6216\u5c3f\u5473\uff0c\u56b4\u91cd\u8005\u6703\u610f\u8b58\u4e0d\u6e05\u3001\u660f\u8ff7\u751a\u81f3\u6b7b\u4ea1\u3002<\/p>\n<\/div>\n<div class=\"artical\" style=\"text-align: justify;\">\n<h3><strong><span style=\"font-size: 14pt;\">\u6953\u6f3f\u5c3f\u75c7\u767c\u751f\u7387\u70ba\u4f55?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u76ee\u524d\u570b\u5167\u767c\u751f\u7387\u4e0d\u660e\uff0c\u81e8\u5e8a\u4e0a\u767c\u73fe\u539f\u4f4f\u6c11\u75c5\u60a3\u7d04\u4f54\u534a\u6578\u4ee5\u4e0a\u3002\u6b50\u6d32\u767d\u4eba\u767c\u751f\u7387\u7d04\u5341\u4e8c\u842c\u5206\u4e4b\u4e00\uff0c\u7f8e\u570b\u7d04\u4e8c\u5341\u4e94\u842c\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\u652f\u93c8\u80fa\u57fa\u9178\uff08\u7e88\u80fa\u9178\u3001\u767d\u80fa\u9178\u3001\u7570\u767d\u80fa\u9178\uff09\u7684\u542b\u91cf\uff0c\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\u6953\u6f3f\u5c3f\u75c7?<\/span><\/strong><\/h3>\n<p style=\"padding-left: 40px;\">\u8a3a\u65b7\u4e0a\u85c9\u7531\u5206\u6790\u8840\u6db2\u4e2d\u7e88\u6c28\u9178\u3001\u767d\u80fa\u9178\u3001\u7570\u767d\u80fa\u57fa\u9178\u53ca\u7570\u767d\u80fa\u57fa\u9178\u7684\u7570\u69cb\u7269\uff08Alloisoleucine\uff09\u7684\u6fc3\u5ea6\uff0c\u53ef\u767c\u73fe\u5178\u578b\u75c5\u60a3\u7684\u7684\u6578\u503c\u5f88\u9ad8\uff0c\u5c3f\u6db2\u4e2d\u76f8\u95dc\u80fa\u57fa\u9178\u884d\u751f\u7684\u7570\u5e38\u6709\u6a5f\u9178\u542b\u91cf\uff0c\u6216\u76f4\u63a5\u6e2c\u5b9a\u8868\u76ae\u7d30\u80de\u6216\u6dcb\u5df4\u7403\u4e2d\u652f\u93c8\u7532\u578b\u916e\u9178\u812b\u6c2b\u9176(Branched-Chain \u03b1-Keto Acid Dehydrogenase)\u7684\u6d3b\u6027\u3002<\/p>\n<\/div>\n<div class=\"artical\">\n<h3 style=\"text-align: justify;\"><strong><span style=\"font-size: 14pt;\">\u5982\u4f55\u6cbb\u7642\u6953\u6f3f\u5c3f\u75c7?<\/span><\/strong><\/h3>\n<p style=\"text-align: justify; padding-left: 40px;\">\u6953\u6f3f\u5c3f\u75c7\u6cbb\u7642\u5148\u53e3\u670d\u5927\u91cf\u7684\u7dad\u751f\u7d20B1\uff0c\u89c0\u5bdf\u75c5\u60a3\u8840\u6db2\u4e2d\u7e88\u6c28\u9178\u3001\u767d\u80fa\u9178\u3001\u7570\u767d\u80fa\u57fa\u9178\u662f\u5426\u6709\u53cd\u61c9\u800c\u964d\u4f4e\uff0c\u518d\u4f9d\u964d\u4f4e\u4e4b\u60c5\u5f62\u4f86\u914d\u5408\u4f4e\u652f\u93c8\u80fa\u57fa\u9178\u98f2\u98df\u6cbb\u7642\u3002\u5982\u7dca\u6025\u75c7\u72c0\u7522\u751f\uff0c\u6025\u6027\u6551\u6cbb\u5148\u6c42\u7a69\u5b9a\u60a3\u8005\u4e4b\u751f\u547d\u5fb5\u8c61\u70ba\u4e3b\uff0c\u518d\u61c9\u7528\u8840\u6db2\u900f\u6790\u6216\u8179\u819c\u900f\u6790\u79fb\u9664\u9ad4\u5167\u5806\u7a4d\u7684\u6709\u6bd2\u7269\u8cea\u3002\u5728\u9577\u671f\u6cbb\u7642\u65b9\u9762\uff0c\u5247\u662f\u5229\u7528\u7279\u6b8a\u5976\u7c89\u9069\u7576\u7ba1\u5236\u75c5\u60a3\u5c0d\u9019\u4e09\u7a2e\u652f\u93c8\u80fa\u57fa\u9178\u7684\u651d\u53d6\uff0c\u4e0d\u904e\u652f\u93c8\u80fa\u57fa\u9178\u662f\u5fc5\u9700\u80fa\u57fa\u9178\uff0c\u8eab\u9ad4\u7121\u6cd5\u81ea\u884c\u88fd\u9020\uff0c\u5fc5\u9808\u9760\u5916\u754c\u9069\u91cf\u7684\u88dc\u5145\uff0c\u6240\u4ee5\u8981\u8abf\u6574\u4e00\u822c\u5b30\u5152\u5976\u7c89\u8207\u7279\u6b8a\u5976\u7c89\u7684\u6bd4\u4f8b\uff0c\u4ee5\u517c\u9867\u5fc5\u9700\u80fa\u57fa\u9178\u7684\u4f86\u6e90\u6070\u8db3\u4ee5\u4f9b\u61c9\u75c5\u7ae5\u751f\u9577\uff0c\u4f46\u53c8\u4e0d\u80fd\u904e\u9ad8\u800c\u9020\u6210\u6bd2\u6027\uff1b\u56e0\u6b64\uff0c\u75c5\u7ae5\u8981\u7d93\u5e38\u62bd\u8840\u4ee5\u5075\u6e2c\u8840\u6db2\u4e2d\u80fa\u57fa \u9178\u7684\u6fc3\u5ea6\uff0c\u4ee5\u907f\u514d\u56e0\u9ad4\u5167\u6709\u6a5f\u9178\u5806\u7a4d\u9020\u6210\u8166\u7d30\u80de\u4e4b\u640d\u5bb3\u3002<\/p>\n<h3><strong><span style=\"font-size: 14pt;\">\u6953\u6f3f\u5c3f\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\u80fa\u57fa\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>\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\u6953\u6f3f\u5c3f\u75c7? \u6953\u7cd6\u5c3f\u75c7\u662f\u7279\u6b8a\u652f\u93c8\u80fa\u57fa\u9178\u4ee3\u8b1d\u7570\u5e38\u7684\u7f55\u898b\u75be\u75c5\uff0c\u5c6c\u65bc\u9ad4\u67d3\u8272\u9ad4\u96b1\u6027\u907a\u50b3\u75be\u75c5\u3002\u662f\u56e0\u9ad4\u5167\u7f3a\u5c11\u652f\u93c8\u7532\u578b\u916e\u9178 &#8230; 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