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Mendelian Inheritance in Man,OMIM\uff09\u4ecd\u5bf9\u8fd9\u4e9b\u8868\u578b\u8d4b\u4e88\u4e0d\u540c\u7f16\u53f7\u30021990\u5e74\uff0c\u8f7b\u91cd\u4e0d\u540c\u75be\u75c5\u7684\u81f4\u75c5\u57fa\u56e0\u5b9a\u4f4d\u4e8e5\u53f7\u67d3\u8272\u4f53\u957f\u81c2\u540c\u4e00\u533a\u57df\uff0c\u6700\u7ec8\u88ab\u786e\u8ba4\u4e3a\u540c\u4e00\u75be\u75c5\u3002\u6839\u636e\u8d77\u75c5\u5e74\u9f84\u53ca\u80fd\u8fbe\u5230\u7684\u6700\u5927\u8fd0\u52a8\u91cc\u7a0b\u7891\u5c06SMA\u5206\u4e3a1\u578b\u30012\u578b\u548c3\u578b<sup>\uff3b14\uff3d<\/sup>\u3002\u6b64\u540e\u53c8\u5c061\u30012\u30013\u578b\u7ec6\u5206\u4e9a\u578b\uff0c\u5e76\u6dfb\u52a00\u578b\u548c4\u578b\u3002SMN2\u62f7\u8d1d\u6570\u662f\u516c\u8ba4\u7684\u5f71\u54cd\u75be\u75c5\u4e25\u91cd\u7a0b\u5ea6\u548c\u8fdb\u5c55\u901f\u5ea6\u7684\u4e3b\u8981\u56e0\u7d20\uff08\u88682\uff09\u3002<\/p>\n<p>SMA\u56fd\u9645\u5206\u578b\u53d7\u5230\u5e7f\u6cdb\u8ba4\u53ef\uff0c\u4f46\u5728\u6d89\u53ca\u9752\u5c11\u5e74\u548c\u6210\u4eba\u7684\u5206\u578b\u4e0a\u5374\u8f83\u4e3a\u6a21\u7cca\uff0c\u672a\u80fd\u5c06\u75c5\u60c5\u4e25\u91cd\u7a0b\u5ea6\u548c\u529f\u80fd\u72b6\u6001\u4e0d\u540c\u7684\u60a3\u8005\u5206\u5f00\u3002\u5982\u6309\u76ee\u524d\u7684\u5206\u578b\u5b9a\u4e49\uff0cSMA-3b\u578b\u60a3\u8005\uff0c\u65e2\u5305\u62ec10\u5c81\u524d\u5c31\u4e27\u5931\u884c\u8d70\u80fd\u529b\uff0c\u540e\u671f\u51fa\u73b0\u4e25\u91cd\u810a\u67f1\u4fa7\u51f8\u7684\u60a3\u8005\uff0c\u53c8\u5305\u62ec10\u4f59\u5c81\u8d77\u75c5\uff0c40\uff5e50\u5c81\u540e\u4ecd\u53ef\u884c\u8d70\uff0c\u751f\u5b58\u671f\u548c\u751f\u6d3b\u8d28\u91cf\u63a5\u8fd1\u6b63\u5e38\u7684\u60a3\u8005\u3002\u8fd9\u4e9b\u75c5\u60c5\u8f7b\u91cd\u8fe5\u5f02\u7684\u60a3\u8005\u5206\u5728\u540c\u4e00\u5206\u578b\u4e2d\uff0c\u4e0d\u5229\u4e8e\u5e94\u7528\u4e34\u5e8a\u5206\u578b\u7ed9\u4e88\u4e0d\u540c\u75c5\u60c5\u60a3\u8005\u7cbe\u7ec6\u8bca\u6cbb\u3002\u672c\u6587\u5efa\u8bae\u5c06SMA\u56fd\u9645\u5206\u578b\u4e2d\u76843\u578b\u8fdb\u884c\u5fc5\u8981\u8bf4\u660e\u548c\u5c11\u8bb8\u8c03\u6574\uff0c\u5e76\u589e\u52a0SMA-3c\u578b\uff0c\u4ee5\u671f\u66f4\u597d\u5730\u7528\u4e8e\u9752\u5c11\u5e74\u6210\u4eba\u60a3\u8005\uff0c\u5177\u4f53\u89c1\u88683\u3002\u65b0\u7684\u5206\u578b\u8c03\u6574\u4ecd\u9700\u5728\u4e34\u5e8a\u5b9e\u8df5\u4e2d\u5f97\u5230\u68c0\u9a8c\u548c\u4f18\u5316\u3002\u6b64\u5916\uff0c\u5df2\u9646\u7eed\u62a5\u9053\u643a\u5e26SMN1\u57fa\u56e0\u5b8c\u5168\u5931\u80fd\u7a81\u53d8\u7684\u6781\u5c11\u6570\u4eba\u53ef\u7ec8\u751f\u65e0\u4e34\u5e8a\u8868\u73b0\uff08\u53ef\u80fd\u6709\u4e34\u5e8a\u4e0b\u8868\u73b0\uff0c\u5982\u808c\u7535\u56fe\u63d0\u793a\u795e\u7ecf\u6e90\u6027\u635f\u5bb3\uff09\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aSMA\u8d77\u75c5\u5e74\u9f84\u4e0e\u75c5\u60c5\u8fdb\u5c55\u5dee\u5f02\u5927\uff0c\u5efa\u8bae\u5728\u65e2\u5f80\u56fd\u9645\u516c\u8ba4\u7684SMA\u5206\u578b\u57fa\u7840\u4e0a\uff0c\u9488\u5bf9\u9752\u5c11\u5e74\u53ca\u6210\u4ebaSMA\u60a3\u8005\u7ed9\u51fa\u66f4\u4e3a\u6e05\u6670\u7684\u5206\u578b\u6807\u51c6\uff08\u2161\u7ea7\u63a8\u8350\uff0cD\u7ea7\u8bc1\u636e\uff09\u3002\u4fee\u8ba2\u7248\u65b0\u5206\u578b\u6709\u5f85\u5728\u4eca\u540e\u4e34\u5e8a\u5b9e\u8df5\u4e2d\u4e0d\u65ad\u68c0\u9a8c\u3002<\/p>\n<p>3<\/p>\n<p>\u8bca\u65ad\u4e0e\u9274\u522b\u8bca\u65ad<\/p>\n<p>3.1&nbsp;&nbsp;\u8bca\u65ad<\/p>\n<p>\u75c5\u53f2\u7279\u70b9\uff1a\u9752\u5c11\u5e74\u3001\u6210\u4ebaSMA\u60a3\u8005\u8868\u73b0\u4e3a\u9690\u533f\u8d77\u75c5\u3001\u7f13\u6162\u8fdb\u5c55\u7684\u80a2\u4f53\u65e0\u529b\u840e\u7f29\uff0c\u4ece\u4e0b\u80a2\u8fd1\u7aef\u8d77\u59cb\uff0c\u4ee5\u8e72\u8d77\u3001\u4e0a\u697c\u80fd\u529b\u4e0b\u964d\u4e3a\u521d\u59cb\u8868\u73b0\uff0c\u540e\u5411\u4e0a\u80a2\u8fd1\u7aef\u3001\u4e0a\u4e0b\u80a2\u8fdc\u7aef\u3001\u8eaf\u5e72\u808c\u7fa4\u3001\u547c\u5438\u808c\u53ca\u7403\u90e8\u808c\u8089\u8fdb\u5c55\u3002\u75c5\u7a0b\u5e38\u8fbe\u6570\u5e74\uff0c\u751a\u81f3\u6570\u5341\u5e74\u3002\u90e8\u5206\u60a3\u8005\u53ef\u5408\u5e76\u8089\u8df3\u611f\uff0c\u65e9\u671f\u591a\u65e0\u541e\u54bd\u56f0\u96be\u3001\u8a00\u8bed\u4e0d\u6e05\u7b49\u75c7\u72b6\uff0c\u53ef\u4f34\u8f7b\u5ea6\u810a\u67f1\u4fa7\u5f2f\u53ca\u5173\u8282\u631b\u7f29\u3002\u9752\u5c11\u5e74\u3001\u6210\u4ebaSMA\u60a3\u8005\u751f\u957f\u53d1\u80b2\u65b9\u9762\uff0c\u867d\u8fd0\u52a8\u53d1\u80b2\u7565\u843d\u540e\uff0c\u4f46\u57fa\u672c\u53ef\u83b7\u5f97\u5168\u90e8\u8fd0\u52a8\u91cc\u7a0b\u7891\u3002\u667a\u80fd\u6b63\u5e38\uff0c\u65e0\u611f\u89c9\u5f02\u5e38\u3001\u5c3f\u4fbf\u969c\u788d\u3002\u5c11\u90e8\u5206\u60a3\u8005\u5bb6\u5ead\u6709\u5e38\u67d3\u8272\u4f53\u9690\u6027\u9057\u4f20\u5bb6\u65cf\u53f2\u3002<\/p>\n<p>\u4f53\u683c\u68c0\u67e5\uff1a\u667a\u80fd\u6b63\u5e38\u3002\u5178\u578b\u4f53\u5f81\u4e3a\u80a2\u4f53\u8fd1\u7aef\uff08\u5305\u62ec\u80a2\u5e26\u808c\uff09\u4e3a\u91cd\uff0c\u53cc\u4fa7\u5bf9\u79f0\u7684\u56db\u80a2\u808c\u529b\u4e0b\u964d\u4f34\u808c\u8089\u840e\u7f29\uff0c\u8171\u53cd\u5c04\u51cf\u4f4e\u6216\u6d88\u5931\u3001\u75c5\u7406\u5f81\u9634\u6027\u3002\u90e8\u5206\u665a\u671f\u60a3\u8005\u53ef\u51fa\u73b0\u5f2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kinase\uff0cCK\uff09\u6b63\u5e38\u6216\u8f7b\u5ea6\u5347\u9ad8\uff0c\u901a\u5e38\u5728\u6b63\u5e38\u4e0a\u96503\u500d\u4ee5\u5185\uff0c\u9752\u58ee\u5e74\u75be\u75c5\u8fdb\u5c55\u8f83\u5feb\u6216\u5408\u5e76\u808c\u8089\u62bd\u6410\u65f6\uff0c\u53ef\u8fbe\u6b63\u5e38\u4e0a\u96505\u500d\u4ee5\u4e0a\u3002\u7535\u751f\u7406\u68c0\u67e5\u63d0\u793a\u5e7f\u6cdb\u795e\u7ecf\u6e90\u6027\u635f\u5bb3\uff0c\u795e\u7ecf\u4f20\u5bfc\u901f\u5ea6\u901a\u5e38\u6b63\u5e38\uff0c\u590d\u5408\u808c\u8089\u52a8\u4f5c\u7535\u4f4d\uff08compound muscle action potential\uff0c CMAP\uff09\u6ce2\u5e45\u660e\u663e\u51cf\u4f4e\u3002\u90e8\u5206\u60a3\u8005\u808c\u7535\u56fe\u53ef\u89c1\u5c11\u91cf\u81ea\u53d1\u7535\u4f4d\u3002\u5982\u60a3\u8005\u5904\u4e8e\u75c5\u7a0b\u665a\u671f\uff0c\u7535\u751f\u7406\u68c0\u67e5\u5728\u533a\u5206\u8fd0\u52a8\u795e\u7ecf\u8f74\u7d22\u3001\u9ad3\u9798\u53d7\u7d2f\u6216\u795e\u7ecf\u6e90\u6027\u3001\u808c\u6e90\u6027\u635f\u5bb3\u65f6\u53ef\u80fd\u5b58\u5728\u56f0\u96be\u3002\u51ed\u501f\u51c6\u786e\u7684\u7535\u751f\u7406\u68c0\u67e5\u7ed3\u679c\uff0c\u53ef\u4e34\u5e8a\u786e\u5b9a\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u7efc\u5408\u5f81\uff0c\u810a\u9ad3\u524d\u89d2\u6216\u8fd0\u52a8\u8f74\u7d22\u53d7\u7d2f\u3002<\/p>\n<p>\u57fa\u56e0\u68c0\u6d4b\uff1aSMA\u662f\u5e38\u67d3\u8272\u4f53\u9690\u6027\u9057\u4f20\u75be\u75c5\uff0c\u786e\u8bca\u9700\u8fdb\u884c\u57fa\u56e0\u68c0\u6d4b\u3002\u5bf9\u4e8e\u4e34\u5e8a\u7591\u8bcaSMA\u60a3\u8005\uff0c\u4e00\u822c\u5148\u91c7\u7528\u591a\u91cd\u8fde\u63a5\u63a2\u9488\u6269\u589e\u6280\u672f\uff08multiplex ligation-dependent probe amplification\uff0cMLPA\uff09\u68c0\u6d4bSMN1\u548cSMN2\u57fa\u56e0\u62f7\u8d1d\u6570\uff0c\u82e5\u786e\u8ba4SMN1\u57fa\u56e0\u62f7\u8d1d\u6570\u4e3a0\uff0c\u5219\u8bca\u65ad\u660e\u786e\u3002\u5982\u62f7\u8d1d\u6570\u4e3a1\uff0c\u800c\u4e34\u5e8a\u4ecd\u9ad8\u5ea6\u6000\u7591SMA\uff0c\u5219\u53ef\u5b8c\u5584\u957f\u7247\u6bb5\u805a\u5408\u9176\u94fe\u5f0f\u53cd\u5e94\uff08polymerase chain reaction\uff0cPCR\uff09\u3001\u5de2\u6c0fPCR\u6216RT\u514b\u9686\u6d4b\u5e8f\uff0c\u67e5\u627eSMN1\u6709\u65e0\u81f4\u75c5\u6027\u5fae\u5c0f\u7a81\u53d8\u300290%\uff5e95%\u7684SMA\u60a3\u8005\u7531SMN1\u57fa\u56e0\u5916\u663e\u5b507\u7eaf\u5408\u7f3a\u5931\u6240\u81f4\uff0c\u5269\u4f59\u60a3\u8005\u5219\u7531SMN1\u57fa\u56e0\u6742\u5408\u7f3a\u5931\u5408\u5e76\u70b9\u7a81\u53d8\u6240\u81f4\uff0c\u5177\u4f53\u57fa\u56e0\u8bca\u65ad\u6d41\u7a0b\u53ef\u53c2\u8003\u300a\u810a\u9ad3\u6027\u808c\u840e\u7f29\u75c7\u9057\u4f20\u5b66\u8bca\u65ad\u4e13\u5bb6\u5171\u8bc6\u300b<sup>\uff3b8\uff3d<\/sup>\u3002<\/p>\n<p>\u8bca\u65ad\u6d41\u7a0b\u5c0f\u7ed3\uff1a\u6162\u6027\u5bf9\u79f0\u6027\uff0c\u4ee5\u8fd1\u7aef\u4e3a\u4e3b\u7684\u80a2\u4f53\u65e0\u529b\u840e\u7f29\uff0c\u67e5\u4f53\u8171\u53cd\u5c04\u51cf\u9000\u3001\u75c5\u7406\u5f81\u9634\u6027\uff0c\u672a\u89c1\u611f\u89c9\u5f02\u5e38\u3002\u7535\u751f\u7406\u68c0\u67e5\u8fdb\u4e00\u6b65\u5b9a\u4f4d\u81f3\u810a\u9ad3\u524d\u89d2\u6216\u8fd0\u52a8\u8f74\u7d22\u53d7\u7d2f\u3002\u6700\u7ec8\u901a\u8fc7\u57fa\u56e0\u68c0\u6d4b\u786e\u8bcaSMA\u3002<\/p>\n<p>3.2&nbsp;&nbsp;\u9274\u522b\u8bca\u65ad<\/p>\n<p>\u82e5\u57fa\u56e0\u68c0\u6d4b\u6392\u96645q-SMA\u6216\u65e0\u6cd5\u786e\u8bca5q-SMA\uff0c\u5219\u9700\u8003\u8651\u5176\u4ed6\u5bfc\u81f4\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u7efc\u5408\u5f81\u7684\u53ef\u80fd\u75c5\u56e0\u3002\u5728\u8fdb\u4e00\u6b65\u786e\u8ba4\u75c5\u53f2\u3001\u4f53\u5f81\u7684\u57fa\u7840\u4e0a\uff0c\u53ef\u5b8c\u5584\u808c\u8089MR\u3001\u8170\u690e\u7a7f\u523a\u8111\u810a\u6db2\u68c0\u9a8c\u3001\u808c\u8089\u6d3b\u68c0\u3001\u795e\u7ecf\u6d3b\u68c0\uff0c\u4ee5\u53ca\u66f4\u5e7f\u6cdb\u7684\u57fa\u56e0\u68c0\u6d4b\u7b49\uff0c\u5e2e\u52a9\u786e\u8bca\u3002\u5bf9\u4e8e\u4e34\u5e8a\u8bc4\u4f30\uff0c\u7279\u522b\u662f\u7535\u751f\u7406\u68c0\u67e5\u660e\u786e\u5b9a\u4f4d\u4e8e\u810a\u9ad3\u524d\u89d2\u6216\u8fd0\u52a8\u8f74\u7d22\u53d7\u7d2f\u7684\u5176\u4ed6\u7d2f\u53ca\u9752\u5c11\u5e74\u3001\u6210\u5e74\u671f\u7684\u9057\u4f20\u6027\u75be\u75c5\uff0c\u4e3b\u8981\u9274\u522b\u8bca\u65ad\u5982\u4e0b\u3002<\/p>\n<p>\u808c\u840e\u7f29\u4fa7\u7d22\u786c\u5316\uff1a\u901a\u5e38\u4e2d\u5e74\u540e\u8d77\u75c5\uff0c\u75c5\u60c5\u8fdb\u5c55\u5feb\uff0c\u9664\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u5916\uff0c\u7d2f\u53ca\u4e0a\u8fd0\u52a8\u795e\u7ecf\u5143\uff0c\u51fa\u73b0\u9525\u4f53\u675f\u5f81\u3002\u591a\u4e3a\u6563\u53d1\u578b\uff0c\u5c11\u90e8\u5206\u4e3a\u5bb6\u65cf\u6027\uff08SOD1\u3001FUS\u7b49\u57fa\u56e0\u7a81\u53d8\u5bfc\u81f4\uff09\u3002<\/p>\n<p>\u810a\u9ad3\u5ef6\u9ad3\u6027\u808c\u840e\u7f29\u75c7\uff08Kennedy\u75c5\uff09\uff1a\u7537\u6027\u60a3\u75c5\uff0c\u5e3840\u5c81\u5de6\u53f3\u8d77\u75c5\uff0c\u8fdb\u5c55\u7f13\u6162\u3002\u9664\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u53d7\u7d2f\u5916\uff0c\u53ef\u51fa\u73b0\u611f\u89c9\u795e\u7ecf\u4f20\u5bfc\u5f02\u5e38\uff0c\u8fd8\u53ef\u51fa\u73b0\u96c4\u6fc0\u7d20\u529f\u80fd\u4e0d\u8db3\u7b49\u5176\u4ed6\u8868\u73b0\u3002\u53ef\u6709X\u8fde\u9501\u9690\u6027\u9057\u4f20\u5bb6\u65cf\u53f2\u3002\u7531AR\u57fa\u56e0CAG\u91cd\u590d\u5e8f\u5217\u589e\u591a\u6240\u81f4\u3002<\/p>\n<p>\u975e5q-SMA\uff1a\u4e34\u5e8a\u8868\u73b0\u548c\u7535\u751f\u7406\u68c0\u67e5\u7ed3\u679c\u5747\u4e0eSMA\u76f8\u4f3c\uff0c\u80a2\u4f53\u8fdc\u7aef\u65e0\u529b\u840e\u7f29\u5e38\u66f4\u660e\u663e\u3002\u7531DYNC1H1\u3001CHCHD10\u7b49\u57fa\u56e0\u7f3a\u9677\u6240\u81f4\u3002\u9274\u522b\u8bca\u65ad\u56f0\u96be\uff0c\u53ea\u80fd\u901a\u8fc7\u57fa\u56e0\u68c0\u6d4b\u6700\u7ec8\u786e\u8bca\u3002<\/p>\n<p>SMA\u53e0\u52a0\u7efc\u5408\u5f81\uff1a\u5728SMA\u5178\u578b\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u53d7\u7d2f\u8868\u73b0\u5916\uff0c\u5408\u5e76\u5176\u4ed6\u4e34\u5e8a\u8868\u73b0\uff0c\u5982\u4e25\u91cd\u5173\u8282\u631b\u7f29\u3001\u5c0f\u8111\u840e\u7f29\u3001\u808c\u9635\u631b\u766b\u75eb\u7b49\u3002\u7531VRK1\u3001ASAH1\u7b49\u57fa\u56e0\u7f3a\u9677\u6240\u81f4\u3002\u6839\u636e\u4e34\u5e8a\u7279\u70b9\u63d0\u793a\uff0c\u901a\u8fc7\u57fa\u56e0\u68c0\u6d4b\u786e\u8bca\u3002<\/p>\n<p>\u8fdc\u7aef\u578b\u9057\u4f20\u6027\u8fd0\u52a8\u795e\u7ecf\u75c5\uff1a\u672c\u75c5\u4e3b\u8981\u8868\u73b0\u4e3a\u5468\u56f4\u795e\u7ecf\u8fd0\u52a8\u8f74\u7d22\u53d7\u635f\uff0c\u4ee5\u80a2\u4f53\u8fdc\u7aef\u65e0\u529b\u840e\u7f29\u660e\u663e\u4e3a\u4e3b\u3002\u4f46\u4e34\u5e8a\u9274\u522b\u4e0a\u96be\u4ee5\u4e0e\u524d\u89d2\u8fd0\u52a8\u795e\u7ecf\u5143\u53d7\u7d2f\u5b8c\u5168\u5206\u5f00\u3002\u7531SIGMAR1\u3001DCTN1\u7b49\u57fa\u56e0\u7f3a\u9677\u6240\u81f4\uff0c\u9700\u57fa\u56e0\u68c0\u6d4b\u6700\u7ec8\u786e\u8bca\u3002<\/p>\n<p>\u9057\u4f20\u6027\u8fd0\u52a8\u611f\u89c9\u5468\u56f4\u795e\u7ecf\u75c5\uff08\u8153\u9aa8\u808c\u840e\u7f29\u75c7\uff092\u578b\uff1a\u672c\u75c5\u7d2f\u53ca\u5468\u56f4\u795e\u7ecf\uff0c\u4ee5\u8f74\u7d22\u53d7\u7d2f\u4e3a\u4e3b\uff0c\u8fd0\u52a8\u795e\u7ecf\u3001\u611f\u89c9\u795e\u7ecf\u5747\u53d7\u7d2f\u3002\u867d\u7136\u60a3\u8005\u53ef\u65e0\u611f\u89c9\u75c7\u72b6\uff0c\u4f46\u7535\u751f\u7406\u68c0\u67e5\u63d0\u793a\u8fd0\u52a8\u795e\u7ecf\u8f74\u7d22\u548c\u611f\u89c9\u795e\u7ecf\u8f74\u7d22\u5747\u53d7\u635f\u3002\u80a2\u4f53\u65e0\u529b\u840e\u7f29\u901a\u5e38\u4ee5\u8fdc\u7aef\u4e3a\u91cd\u3002\u7531MFN2\u3001HSPB1\u7b49\u57fa\u56e0\u7f3a\u9677\u5bfc\u81f4\uff0c\u9700\u57fa\u56e0\u68c0\u6d4b\u786e\u8bca\u3002<\/p>\n<p>\u80a2\u5e26\u578b\u808c\u8425\u517b\u4e0d\u826f\uff1a\u901a\u5e38\u9752\u5c11\u5e74\u81f3\u6210\u5e74\u671f\u8d77\u75c5\uff0c\u8868\u73b0\u4e3a\u80a2\u5e26\u808c\u53ca\u80a2\u4f53\u8fd1\u7aef\u808c\u8089\u65e0\u529b\u840e\u7f29\uff0c\u5448\u7f13\u6162\u8fdb\u5c55\u3002\u808c\u7535\u56fe\u63d0\u793a\u808c\u6e90\u6027\u635f\u5bb3\uff0c\u8bb8\u591a\u7c7b\u578b\u8840\u808c\u9178\u6fc0\u9176\u663e\u8457\u589e\u9ad8\u3002\u7531CAPN3\u3001DYSF\u7b49\u57fa\u56e0\u7f3a\u9677\u6240\u81f4\u3002\u57fa\u56e0\u68c0\u6d4b\u3001\u808c\u8089\u6d3b\u68c0\u5206\u5b50\u75c5\u7406\u53ef\u5e2e\u52a9\u786e\u8bca\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u4e34\u5e8a\u8bca\u65ad\u4e0b\u8fd0\u52a8\u795e\u7ecf\u5143\u7efc\u5408\u5f81\uff0c\u7b26\u5408\u4e0b\u80a2\u8fd1\u7aef\u8d77\u75c5\u7279\u70b9\uff0c\u9700\u8003\u8651SMA\u3002\u91c7\u7528\u9776\u5411\u7a81\u53d8\u5206\u6790\u8fdb\u884c\u5206\u5b50\u9057\u4f20\u5b66\u8bca\u65ad\uff0c\u82e5\u786e\u8ba4SMN1\u57fa\u56e07\u53f7\u5916\u663e\u5b50\u7eaf\u5408\u7f3a\u5931\u53ef\u786e\u8bcaSMA\u3002\u82e5SMN1\u57fa\u56e0\u6742\u5408\u7f3a\u5931\uff0c\u5219\u9700\u8fdb\u4e00\u6b65\u884c\u7279\u6b8aSanger\u6d4b\u5e8f\uff0c\u660e\u786e\u672a\u7f3a\u5931SMN1\u57fa\u56e0\u662f\u5426\u5b58\u5728\u81f4\u75c5\u6027\u5fae\u5c0f\u7a81\u53d8\uff08\u2160\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>4<\/p>\n<p>\u836f\u7269\u6cbb\u7597<\/p>\n<p>4.1&nbsp;&nbsp;\u75be\u75c5\u4fee\u6b63\u6cbb\u7597\u836f\u7269<\/p>\n<p>\u9488\u5bf9SMA\u57fa\u56e0\u7f3a\u9677\u53ca\u75c5\u7406\u751f\u7406\u673a\u5236\uff0c\u901a\u8fc7\u4e0d\u540c\u624b\u6bb5\u63d0\u5347SMN\u86cb\u767d\u6c34\u5e73\uff0c\u4ece\u800c\u6539\u53d8\u75be\u75c5\u53d1\u5c55\u8fdb\u7a0b\u7684\u6cbb\u7597\u836f\u7269\uff0c\u79f0\u4e3a\u75be\u75c5\u4fee\u6b63\u6cbb\u7597\uff08disease modifying treatment\uff0cDMT\uff09\u836f\u7269\u3002\u76ee\u524dSMA\u5df2\u67093\u79cdDMT\u836f\u7269\u5728\u5168\u7403\u8303\u56f4\u5185\u4e0a\u5e02\u3002<\/p>\n<p>4.1.1&nbsp;&nbsp;\u8bfa\u897f\u90a3\u751f<\/p>\n<p>\u8bfa\u897f\u90a3\u751f\u662f\u4e00\u79cd\u53cd\u4e49\u5be1\u6838\u82f7\u9178\u836f\u7269\uff0c\u9700\u9798\u5185\u6ce8\u5c04\u3002\u957f\u5ea6\u4e3a18\u6838\u82f7\u9178\uff0c\u4e0eSMN2\u57fa\u56e0\u524d\u4f53mRNA\u4e0a\u5185\u542b\u5b507\u7684ISS-N1\u533a\u6bb5\u4e92\u8865\uff0c\u53ef\u4e0e\u526a\u5207\u8c03\u63a7\u56e0\u5b50hnRNP A1\u7ade\u4e89\u7ed3\u5408\u8be5\u533a\u6bb5\uff0c\u524a\u5f31hnRNP A1\u7684\u4f5c\u7528\uff0c\u4f7f\u66f4\u591aSMN2\u57fa\u56e0\u5916\u663e\u5b507\u526a\u5207\u8fdb\u5165\u6210\u719fmRNA\uff0c\u4ece\u800c\u8f6c\u5f55\u4ea7\u751f\u5305\u542b7\u53f7\u5916\u663e\u5b50\u7684\u5168\u957fmRNA\u3002\u5728\u7ffb\u8bd1\u9636\u6bb5\uff0c\u5168\u957fmRNA\u751f\u6210\u6b63\u5e38SMN\u86cb\u767d\u53d1\u6325\u751f\u7406\u4f5c\u7528\u3002\u7ecf\u8fc7\u5927\u91cf\u4e34\u5e8a\u524d\u7814\u7a76\u521d\u6b65\u8bc1\u5b9e\u5176\u6709\u6548\u6027\u3001\u5b89\u5168\u6027\u540e\uff0c\u5728\u4e34\u5e8a\u8bd5\u9a8c\u9636\u6bb5\uff0c\u4e3b\u8981\u7684\u2162\u671f\u4e34\u5e8a\u8bd5\u9a8c\u8bc1\u636e\u5982\u4e0b\u3002CHERISH\/SHINE\uff08NCT02292537\uff09\uff1a\u4e00\u9879\u56fd\u9645\u591a\u4e2d\u5fc3\uff0c\u968f\u673a\u3001\u53cc\u76f2\u3001\u5047\u64cd\u4f5c\u5bf9\u7167\u7814\u7a76\uff0c\u5171\u7eb3\u5165126\u4f8b2\u578b\u548c3\u578bSMA\u60a3\u8005\u3002\u6cbb\u759715\u4e2a\u6708\u540e\uff0c\u8bfa\u897f\u90a3\u751f\u7ec4Hammersmith\u8fd0\u52a8\u529f\u80fd\u91cf\u8868\u6269\u5c55\u7248\uff08Hammersmith functional motor scale expanded\uff0cHFMSE\uff09\u8bc4\u5206\u8f83\u57fa\u7ebf\u5e73\u5747\u63d0\u9ad83.9\u5206\uff0c\u5bf9\u7167\u7ec4\u5e73\u5747\u4e0b\u964d1.0\u5206\uff08P=0.000 000 1\uff09\u3002\u8bfa\u897f\u90a3\u751f\u7ec4\u4e0a\u80a2\u6a21\u5757\u6d4b\u8bd5\u4fee\u8ba2\u7248\uff08revised upper limb module\uff0cRULM\uff09\u8bc4\u5206\u8f83\u57fa\u7ebf\u5e73\u5747\u589e\u52a04.2\u5206\u3002\u6269\u5c55\u671fSHINE\uff08the stroke hyperglycemia insulin network effort\uff09\u7814\u7a76\u663e\u793a\uff0c\u6301\u7eed\u63a5\u53d7\u8bfa\u897f\u90a3\u751f\u6cbb\u7597\u8fd0\u52a8\u529f\u80fd\u8fdb\u4e00\u6b65\u6539\u5584\u3002\u5b89\u5168\u6027\u65b9\u9762\uff0c\u8bfa\u897f\u90a3\u751f\u7ec4\u4e0e\u5bf9\u7167\u7ec4\u7684\u6574\u4f53\u4e0d\u826f\u4e8b\u4ef6\u53d1\u751f\u7387\u76f8\u4f3c\uff0c\u8bfa\u897f\u90a3\u751f\u7ec4\u5371\u6025\u4e0d\u826f\u4e8b\u4ef6\u66f4\u5c11<sup>\uff3b16\uff3d<\/sup>\u3002ENDEAR\/ SHINE\uff08NCT02193074\uff09\uff1a\u4e00\u9879\u56fd\u9645\u591a\u4e2d\u5fc3\u3001\u968f\u673a\u3001\u53cc\u76f2\u3001\u5047\u64cd\u4f5c\u5bf9\u7167\u7814\u7a76\uff0c\u5171\u7eb3\u5165121\u4f8b1\u578bSMA\u60a3\u8005\u3002\u6cbb\u759713\u4e2a\u6708\u540e\uff0c\u8bfa\u897f\u90a3\u751f\u7ec4\u60a3\u8005\u7684\u65e0\u4e8b\u4ef6\u751f\u5b58\u7387\u4e3a61%\uff0c\u5bf9\u7167\u7ec4\u4e3a32%\uff08P=0.005\uff09\uff1b\u8bfa\u897f\u90a3\u751f\u7ec4\u603b\u6b7b\u4ea1\u7387\u8f83\u5bf9\u7167\u7ec4\u964d\u4f4e63%\uff1b\u8bfa\u897f\u90a3\u751f\u7ec4\u8fbe\u5230Hammersmith\u5a74\u513f\u795e\u7ecf\u7cfb\u7edf\u8bc4\u4f30\u7b2c\u4e8c\u90e8\u5206\uff08Hammersmith neurolo-gical examination section-2, HINE-2)\u8fd0\u52a8\u91cc\u7a0b\u7891\u5e94\u7b54\u7684\u60a3\u8005\u6bd4\u4f8b\u4e3a51%\uff0c\u5bf9\u7167\u7ec4\u4e3a0\uff08P\uff1c0.0001\uff09\u3002\u8bfa\u897f\u90a3\u751f\u7ec4\u8d39\u57ce\u513f\u7ae5\u533b\u9662\u5a74\u513f\u795e\u7ecf\u808c\u8089\u75be\u75c5\u6d4b\u8bd5\uff08children&#8217;s hospital of Philadelphia infant test of neuromu-scular disoeders\uff0c CHOP-INTEND\uff09\u8bc4\u5206\u63d0\u9ad8\u22654\u5206\u7684\u60a3\u8005\u6bd4\u4f8b\u4e3a71%\uff0c\u5bf9\u7167\u7ec4\u4e3a3%\uff08P\uff1c0.001\uff09\u3002\u6269\u5c55\u671fSHINE \u7814\u7a76\u663e\u793a\uff0c\u8bfa\u897f\u90a3\u751f\u957f\u671f\u6cbb\u7597\u6301\u7eed\u63d0\u5347\u8fd0\u52a8\u80fd\u529b\u3002\u5b89\u5168\u6027\u65b9\u9762\uff0c\u8bfa\u897f\u90a3\u751f\u6cbb\u7597\u4e25\u91cd\u4e0d\u826f\u4e8b\u4ef6\u3001\u5371\u6025\u4e0d\u826f\u4e8b\u4ef6\uff0c\u4ee5\u53ca\u56e0\u4e0d\u826f\u4e8b\u4ef6\u800c\u505c\u836f\u7684\u6bd4\u4f8b\u5747\u4f4e\u4e8e\u5bf9\u7167\u7ec4<sup>\uff3b17\uff3d<\/sup>\u3002\u4e0a\u5e02\u540e\u771f\u5b9e\u4e16\u754c\u7814\u7a76\u65b9\u9762\uff0c\u4e00\u9879\u5fb7\u56fd\u524d\u77bb\u6027\u3001\u591a\u4e2d\u5fc3\u89c2\u5bdf\u6027\u961f\u5217\u7814\u7a76\u5171\u7eb3\u5165173\u4f8b\u63a5\u53d7\u8bfa\u897f\u90a3\u751f\u6cbb\u7597\u81f3\u5c116\u4e2a\u6708\u7684\u9752\u5c11\u5e74\u3001\u6210\u4ebaSMA\u60a3\u8005\uff08\u5e74\u9f84\u8303\u56f416\uff5e65\u5c81\uff09\uff0c\u968f\u8bbf14\u6708\u7684\u7ed3\u679c\u663e\u793a\uff0c\u8bfa\u897f\u90a3\u751f\u663e\u8457\u63d0\u9ad8\u60a3\u8005\u7684HFMSE\u8bc4\u5206\uff08\u8f83\u57fa\u7ebf\u589e\u52a03.12\u5206\uff0c95% CI\uff1a2.06\uff5e4.19\uff0cP\uff1c0.0001\uff09\u30016\u5206\u949f\u6b65\u884c\u8bd5\u9a8c\uff086 minutes walk test\uff0c6MWT\uff09\uff08\u8f83\u57fa\u7ebf\u589e\u52a046 m\uff0c95% CI\uff1a25.4\uff5e66.6\uff0cP\uff1c0.0001\uff09\u548cRULM\u8bc4\u5206\uff08\u8f83\u57fa\u7ebf\u589e\u52a01.09\u5206\uff0c95% CI\uff1a0.62\uff5e1.55\uff0cP\uff1c0.0001\uff09\u3002\u968f\u8bbf\u671f\u5185\u672a\u62a5\u544a\u4e25\u91cd\u4e0d\u826f\u4e8b\u4ef6<sup>\uff3b18\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u591a\u9879\u771f\u5b9e\u4e16\u754c\u7814\u7a76\u7ed3\u679c\u5df2\u53d1\u8868\u3002Gavriilaki\u7b49<sup>\uff3b19\uff3d<\/sup>\u53d1\u8868\u7cfb\u7edf\u56de\u987e\u548c\u835f\u8403\u5206\u6790\uff0c\u5171\u7eb3\u51657\u4e2a\u75c5\u4f8b\u7cfb\u5217\u548c5\u4e2a\u961f\u5217\u7814\u7a76\u4e2d\u7684428\u4f8bSMA\u60a3\u8005\uff0c\u663e\u793a\u8bfa\u897f\u90a3\u751f\u80fd\u663e\u8457\u6539\u5584HFMSE\u8bc4\u5206\uff08\u6807\u51c6\u5316\u5e73\u5747\u5dee0.17\uff0c95% CI\uff1a0.01\uff5e0.33\uff09\u548cRULM\u8bc4\u5206\uff08\u6807\u51c6\u5316\u5e73\u5747\u5dee0.22\uff0c95% CI\uff1a0.06\uff5e0.38\uff09\uff0c43.3% HFMSE\u548c38.9% RULM\u7684\u8bc4\u5206\u6539\u5584\u8fbe\u5230\u4e34\u5e8a\u610f\u4e49\u3002\u5bf9\u4e8e\u80b2\u9f84\u671f\u60a3\u8005\uff0c\u8bfa\u897f\u90a3\u751f\u5bf9\u4e8e\u751f\u6b96\u7684\u5f71\u54cd\u5c1a\u4e0d\u660e\u786e\u3002\u52a8\u7269\u5b9e\u9a8c\u672a\u53d1\u73b0\u660e\u663e\u751f\u6b96\u6bd2\u6027\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u8bfa\u897f\u90a3\u751f\u7684\u591a\u4e2a\u968f\u673a\u5bf9\u7167\u4e34\u5e8a\u8bd5\u9a8c\u53ca\u771f\u5b9e\u4e16\u754c\u7814\u7a76\u7ed3\u679c\u3001\u835f\u8403\u5206\u6790\uff0c\u8bc1\u5b9e\u5176\u7528\u4e8eSMA\u6cbb\u7597\u7684\u6709\u6548\u6027\u548c\u5b89\u5168\u6027\uff08\u2160\u7ea7\u63a8\u8350\uff0cA\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>4.1.2&nbsp;&nbsp;\u5229\u53f8\u6251\u5170<\/p>\n<p>\u5229\u53f8\u6251\u5170\u662f\u4e00\u79cd\u53e3\u670d\u5c0f\u5206\u5b50\u836f\u7269\uff0c\u540c\u6837\u9488\u5bf9SMN2\u57fa\u56e0\u524d\u4f53mRNA\u7684\u526a\u5207\u8c03\u8282\u3002\u5176\u4e0eSMN2\u57fa\u56e0\u524d\u4f53mRNA\u5916\u663e\u5b507\u4e0a\u7684\u526a\u5207\u589e\u5f3a\u5b502\uff08ESE2\uff09\u53ca\u5185\u542b\u5b507\u76845&#8217;\u526a\u5207\u4f4d\u70b9\uff085&#8242;-ss\uff09\u4e09\u7ef4\u7ed3\u6784\u76f8\u7ed3\u5408\uff0c\u63d0\u9ad8U1 snRNP\u7684\u8bc6\u522b\u548c\u7ed3\u5408\u80fd\u529b\uff0cU1 snRNP\u4e0e\u524d\u4f53mRNA\u7ed3\u5408\u540e\uff0c\u4f7f\u5f97\u66f4\u591a\u5916\u663e\u5b507\u5f97\u4ee5\u526a\u5207\u8fdb\u5165\u6210\u719fmRNA\uff0c\u751f\u6210\u5168\u957fmRNA\uff0c\u8fdb\u4e00\u6b65\u7ffb\u8bd1\u751f\u6210\u6b63\u5e38SMN\u86cb\u767d\u53d1\u6325\u751f\u7406\u4f5c\u7528\u3002\u5b8c\u6210\u4e34\u5e8a\u524d\u7814\u7a76\u540e\uff0c\u8fdb\u5165\u4e34\u5e8a\u8bd5\u9a8c\u9636\u6bb5\u3002\u5176\u4e2dSUNFISH\u7814\u7a76\uff08NCT02908685\uff09<sup>\uff3b20\uff3d<\/sup>\u4e3a\u2162\u671f\u56fd\u9645\u591a\u4e2d\u5fc3\uff08\u5305\u62ec\u4e2d\u56fd\uff09\u53cc\u76f2\u3001\u968f\u673a\u3001\u5b89\u6170\u5242\u5bf9\u7167\u7814\u7a76\uff0c\u5206\u4e3a\u5242\u91cf\u63a2\u7d22\u548c\u7597\u6548\u9a8c\u8bc12\u4e2a\u90e8\u5206\u3002\u5171\u7eb3\u5165180\u4f8b2\uff5e25\u5c81\u76842\u578b\u4e0e\u4e0d\u80fd\u884c\u8d70\u76843\u578b\u60a3\u8005\u3002\u4ee52:1\u7684\u6bd4\u4f8b\u968f\u673a\u5206\u914d\u5230\u6cbb\u7597\u7ec4\u4e0e\u5b89\u6170\u5242\u7ec4\u3002\u5728\u7b2c12\u4e2a\u6708\uff0c\u4e0e\u63a5\u53d7\u5b89\u6170\u5242\u6cbb\u7597\u7684\u60a3\u8005\u76f8\u6bd4\uff0c\u63a5\u53d7\u5229\u53f8\u6251\u5170\u6cbb\u7597\u7684\u60a3\u8005\u8fd0\u52a8\u529f\u80fd\u6d4b\u8bd5\uff08motor function measure 32,MFM32)\u603b\u5206\u8f83\u57fa\u7ebf\u6539\u5584\u66f4\u591a\uff0c\u6cbb\u7597\u7ec4\u4e0e\u5b89\u6170\u5242\u7ec4\u7684\u5dee\u5f02\u4e3a1.55\uff0895% CI\uff1a0.30\uff5e2.81\uff0cP=0.016\uff09\u3002\u5b89\u5168\u6027\u65b9\u9762\uff0c\u65e0\u5bfc\u81f4\u9000\u51fa\u3001\u5242\u91cf\u8c03\u6574\u6216\u4e2d\u65ad\u6cbb\u7597\u7684\u4e0d\u826f\u4e8b\u4ef6\u30022020\u5e74\u83b7\u6279\u4e0a\u5e02\uff0c\u771f\u5b9e\u4e16\u754c\u7814\u7a76\u6570\u636e\u4ecd\u5728\u79ef\u7d2f\u4e2d\u3002\u5bf9\u4e8e\u80b2\u9f84\u671f\u60a3\u8005\uff0c\u9700\u6309\u8bf4\u660e\u4e66\u8981\u6c42\u8c03\u6574\u7528\u836f\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u5229\u53f8\u6251\u5170\u62e5\u6709\u5e74\u9f84\u8de8\u5ea6\u5927\u53ca\u4e0d\u540c\u75be\u75c5\u72b6\u6001\u60a3\u8005\u7684\u9ad8\u8d28\u91cf\u968f\u673a\u5bf9\u7167\u4e34\u5e8a\u8bd5\u9a8c\u7ed3\u679c\uff0c\u4e14\u5176\u4e2d\u5305\u542b\u4e2d\u56fdSMA\u60a3\u8005\u6570\u636e\uff0c\u521d\u6b65\u8bc1\u5b9e\u4e86\u6709\u6548\u6027\u548c\u5b89\u5168\u6027\uff08\u2160\u7ea7\u63a8\u8350\uff0cB\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>4.1.3&nbsp;&nbsp;Zolgensma\uff08OAV-101\uff09<\/p>\n<p>Zolgensma\uff08OAV-101\uff09\u662f\u4e00\u79cd\u57fa\u56e0\u66ff\u4ee3\u6cbb\u7597\u836f\u7269\u3002\u7531\u91cd\u7ec4\u817a\u76f8\u5173\u75c5\u6bd29\u578b\uff08adeno-associated virus-9\uff0cAAV9\uff09\u8f7d\u4f53\u8fd0\u8f93\uff0c\u5e76\u6539\u9020\u5176\u57fa\u56e0\u7ec4\u88c5\u8f7dSMN1\u57fa\u56e0\u7f16\u7801DNA\u5e8f\u5217\u53ca\u914d\u5957\u542f\u52a8\u5b50\u3001\u589e\u5f3a\u5b50\u3001\u5185\u542b\u5b50\u3001polyA\u5c3e\u7b49\u3002\u75c5\u6bd2\u8f7d\u4f53\u8fdb\u5165\u8fd0\u52a8\u795e\u7ecf\u5143\u540e\uff0c\u5bfc\u5165\u7684\u5916\u6e90\u57fa\u56e0\u7ec4\u72ec\u7acb\u5b58\u5728\uff0c\u4e0d\u6574\u5408\u5165\u4eba\u4f53\u57fa\u56e0\u7ec4\uff0c\u5e76\u5f00\u59cb\u6301\u7eed\u8f6c\u5f55\u7ffb\u8bd1\u4ea7\u751fSMN\u86cb\u767d\uff0c\u53d1\u6325\u76f8\u5e94\u751f\u7406\u4f5c\u7528\u3002\u8be5\u57fa\u56e0\u66ff\u4ee3\u6cbb\u7597\u836f\u7269\u5728\u7f8e\u56fd\u5b8c\u6210\u4e34\u5e8a\u524d\u7814\u7a76\u548c\u4e34\u5e8a\u8bd5\u9a8c\uff0c2019\u5e74\u6279\u51c6\u7528\u4e8e2\u5c81\u4ee5\u4e0b\u7684SMA\u60a3\u513f\uff0c\u76ee\u524d\u5c1a\u672a\u5728\u4e2d\u56fd\u83b7\u6279\u4e0a\u5e02\u3002\u5176\u6700\u65b0\u9798\u5185\u6ce8\u5c04\u5168\u7403\u591a\u4e2d\u5fc3\u4e34\u5e8a\u8bd5\u9a8c\u5df2\u5728\u4e2d\u56fd\u5927\u9646\u540c\u6b65\u5f00\u5c55\u3002<\/p>\n<p>4.2&nbsp;&nbsp;\u5176\u4ed6\u836f\u7269<\/p>\n<p>\u9664\u4e86\u4e0a\u8ff0\u9488\u5bf9\u57fa\u56e0\u7f3a\u9677\u3001\u9776\u5411SMN\u86cb\u767d\u63d0\u5347\u7684DMT\u836f\u7269\uff0c\u8fd8\u6709\u9488\u5bf9SMA\u5176\u4ed6\u75c5\u7406\u751f\u7406\u8fc7\u7a0b\u7684\u836f\u7269\u5728\u4e0d\u540c\u7814\u53d1\u9636\u6bb5\uff0c\u5982\u9499\u79bb\u5b50\u7a33\u5b9a\u5242\uff08reldesemtiv\uff09\u3001myostatin\u5355\u514b\u9686\u6297\u4f53\uff08SRK-015\uff09\u7b49\u3002\u4e00\u4e9b\u5df2\u4e0a\u5e02\u7684\u836f\u7269\uff0c\u5982\u6c99\u4e01\u80fa\u9187\u3001\u4e19\u620a\u9178\u94a0\uff0c\u4e5f\u6709\u5c11\u91cf\u8d85\u8bf4\u660e\u4e66\u5e94\u7528\u3002<\/p>\n<p>4.3&nbsp;&nbsp;\u8054\u5408\u7528\u836f<\/p>\n<p>\u4e0d\u540cDMT\u836f\u7269\u7684\u8054\u5408\u5e94\u7528\u6216\u5e8f\u8d2f\u5e94\u7528\u5df2\u6709\u5c11\u91cf\u60a3\u8005\u5728\u771f\u5b9e\u4e16\u754c\u4e2d\u8fdb\u884c\u5c1d\u8bd5\uff0c\u4f46\u5c1a\u7f3a\u4e4f\u4e34\u5e8a\u7814\u7a76\u8bc1\u636e\u8bf4\u660e\u5176\u7597\u6548\u548c\u5b89\u5168\u6027\u3002\u6b64\u5916\uff0c\u6709\u4e34\u5e8a\u524d\u7814\u7a76\u53d1\u73b0DMT\u836f\u7269\u4e0e\u5176\u4ed6\u836f\u7269\u8054\u7528\u53ef\u80fd\u63d0\u5347\u6cbb\u7597\u6548\u679c<sup>\uff3b24\uff3d<\/sup>\uff0c\u6709\u5f85\u4e34\u5e8a\u7814\u7a76\u8fdb\u4e00\u6b65\u89c2\u5bdf\u5b89\u5168\u6027\u3001\u6709\u6548\u6027\u5e76\u8bbe\u5b9a\u89c4\u8303\u4f7f\u7528\u65b9\u6cd5\u3002<\/p>\n<p>5<\/p>\n<p>\u9057\u4f20\u54a8\u8be2\u53ca\u4ea7\u524d\u8bca\u65ad<\/p>\n<p>SMA\u4f5c\u4e3a\u5e38\u67d3\u8272\u4f53\u9690\u6027\u9057\u4f20\u75be\u75c5\uff0c\u7edd\u5927\u591a\u6570\u60c5\u51b5\u4e0b\uff0c\u60a3\u8005\u7236\u6bcd\u5747\u4e3a\u643a\u5e26\u8005\uff0c\u4ece\u800c\u5bfc\u81f41\/4\u6982\u7387\u751f\u80b2SMA\u60a3\u513f\u3002\u643a\u5e26\u8005\u7ed3\u5a5a\u751f\u80b2\u65f6\u5747\u9700\u9057\u4f20\u54a8\u8be2\uff0c\u5fc5\u8981\u65f6\u4ea7\u524d\u8bca\u65ad\u3002\u5efa\u8baeSMA\u60a3\u8005\u7236\u6bcd\u5728\u751f\u6b96\u9057\u4f20\u79d1\u8fdb\u884c\u9057\u4f20\u54a8\u8be2\uff0c\u6307\u5bfc\u518d\u6b21\u751f\u80b2\u4ea7\u524d\u8bca\u65ad\u3002<\/p>\n<p>\u8f7b\u578bSMA\u60a3\u8005\u5982\u51c6\u5907\u751f\u80b2\uff0c\u540e\u4ee3\u60a3\u75c5\u98ce\u9669\u4e3b\u8981\u7531\u5176\u914d\u5076SMN1\u57fa\u56e0\u60c5\u51b5\u6240\u51b3\u5b9a\uff0c\u5e94\u5bf9\u5176\u914d\u5076\u8fdb\u884c\u643a\u5e26\u8005\u68c0\u6d4b\uff0c\u82e5\u5176\u914d\u5076\u4ea6\u4e3a\u643a\u5e26\u8005\uff0c\u5219\u5e94\u8fdb\u884c\u4ea7\u524d\u8bca\u65ad<sup>\uff3b21-22\uff3d<\/sup>\u3002\u76f8\u5173\u68c0\u6d4b\u53ca\u6240\u9700\u91c7\u53d6\u63aa\u65bd\uff0c\u5efa\u8bae\u751f\u6b96\u9057\u4f20\u79d1\u5c31\u8bca\u3002\u6b64\u5916\uff0c\u60a3\u8005\u540e\u4ee3\u5fc5\u7136\u643a\u5e26SMN1\u81f4\u75c5\u7a81\u53d8\uff0c\u5728\u751f\u80b2\u65f6\u4e5f\u5e94\u8fdb\u884c\u914d\u5076\u643a\u5e26\u8005\u68c0\u6d4b\uff0c\u5fc5\u8981\u65f6\u9700\u884c\u4ea7\u524d\u8bca\u65ad\u3002<\/p>\n<p>\u5bf9\u4e8e\u65e0SMA\u5bb6\u65cf\u53f2\u7684\u4e00\u822c\u592b\u5987\uff0c\u82e5\u6709\u8bc4\u4f30\u751f\u80b2SMA\u60a3\u513f\u98ce\u9669\u7684\u610f\u613f\uff0c\u4e5f\u53ef\u8fdb\u884c\u5b55\u524d\u7b5b\u67e5\u3002\u5efa\u8bae\u751f\u6b96\u9057\u4f20\u79d1\u5c31\u8bca\uff0c\u8be6\u7ec6\u9057\u4f20\u54a8\u8be2\u540e\uff0c\u5b89\u6392\u7b5b\u67e5\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aSMA\u60a3\u8005\u5b55\u80b2\u540e\u4ee3\u65f6\uff0c\u5e94\u5bf9\u5176\u914d\u5076\u8fdb\u884c\u643a\u5e26\u8005\u68c0\u6d4b\uff0c\u82e5\u5176\u914d\u5076\u4ea6\u4e3a\u643a\u5e26\u8005\uff0c\u5219\u5e94\u8fdb\u884c\u4ea7\u524d\u8bca\u65ad\uff08\u2160\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002\u82e5\u592b\u5987\u53cc\u65b9\u5747\u643a\u5e26SMN1\u57fa\u56e0\u7a81\u53d8\uff0c\u9700\u8fdb\u884c\u4ea7\u524d\u8bca\u65ad\uff08\u2160\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>\uff08\u672c\u90e8\u5206\u6267\u7b14\u4eba\uff1a\u6234\u6bc5&nbsp; &nbsp;\u6768\u6d35\u54f2\uff09<\/p>\n<p>6<\/p>\n<p>\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406<\/p>\n<p>SMA\u60a3\u8005\u5728\u8fd0\u52a8\u529f\u80fd\u9010\u6e10\u4e0b\u964d\u9020\u6210\u8eab\u4f53\u6b8b\u75be\u540e\uff0c\u4f1a\u9010\u6e10\u51fa\u73b0\u810a\u67f1\u4fa7\u51f8\u3001\u5173\u8282\u631b\u7f29\u3001\u80f8\u5ed3\u53d8\u5f62\u3001\u547c\u5438\u529f\u80fd\u4e0d\u5168\u3001\u9aa8\u8d28\u758f\u677e\u3001\u5f20\u53e3\u53d7\u9650\u3001\u541e\u54bd\u56f0\u96be\u3001\u80c3\u98df\u7ba1\u53cd\u6d41\u3001\u8425\u517b\u4e0d\u826f\/\u8425\u517b\u8fc7\u5269\u7b49\u6d89\u53ca\u591a\u7cfb\u7edf\u7684\u635f\u5bb3\u75c7\u72b6\uff0c\u56e0\u800c\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406\u5bf9SMA\u60a3\u8005\u81f3\u5173\u91cd\u8981\u3002\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406\u5df2\u8bc1\u5b9e\u80fd\u591f\u5ef6\u957f\u60a3\u8005\u5bff\u547d\u3001\u63d0\u9ad8\u60a3\u8005\u751f\u6d3b\u8d28\u91cf<sup>\uff3b11-13\uff0c23\uff3d<\/sup>\u3002\u7ed3\u5408\u76ee\u524d\u56fd\u5185\u5916\u6587\u732e\u53ca\u56fd\u5185\u591a\u5bb6\u533b\u7597\u673a\u6784\u7684\u7ecf\u9a8c\uff0c\u6211\u4eec\u63a8\u8350\u6210\u7acbSMA\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406\u56e2\u961f\uff08multidisciplinary 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scale\uff0cALSFRS\uff09\u7b49\u8fdb\u884c\u8fd0\u52a8\u529f\u80fd\u7684\u91cf\u5316\u8bc4\u4f30,\u8be6\u89c1\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406-\u5eb7\u590d\u79d1\u90e8\u5206(\u672c\u520a\u540e\u7eed\u62a5\u9053\uff09\u3002\u5728\u6b64\u57fa\u7840\u4e0a\u4e3a\u60a3\u8005\u8bbe\u5b9a\u7b26\u5408\u5b9e\u9645\u7684\u4e2a\u4f53\u5316\u6cbb\u7597\u76ee\u6807\uff0c\u5e76\u505a\u597d\u957f\u671f\u968f\u8bbf\u89c2\u5bdf\u3002\u4e0e\u60a3\u8005\uff08\u53ca\u5176\u5bb6\u5c5e\uff09\u5145\u5206\u6c9f\u901a\uff0c\u5e2e\u52a9\u60a3\u8005\uff08\u53ca\u5176\u5bb6\u5c5e\uff09\u6811\u7acb\u6b63\u786e\u7684\u6cbb\u7597\u9884\u671f\u3002<\/p>\n<p>6.1.1.2&nbsp;&nbsp;\u810a\u67f1\u4fa7\u51f8\u4e0e\u9798\u5185\u6ce8\u5c04\u65b9\u5f0f&nbsp; SMA\u60a3\u8005\u968f\u7740\u5e74\u9f84\u589e\u957f\u3001\u75c5\u7a0b\u5ef6\u957f\uff0c\u5e38\u4f1a\u51fa\u73b0\u4e0d\u540c\u7a0b\u5ea6\u7684\u810a\u67f1\u4fa7\u51f8\u3002\u9488\u5bf9\u4e0d\u540c\u60c5\u51b5\u60a3\u8005\u9700\u91c7\u7528\u4e0d\u540c\u9798\u5185\u6ce8\u5c04\u65b9\u5f0f\u3002<\/p>\n<p>\uff081\uff09\u5bf9\u4e8e\u65e0\u810a\u67f1\u4fa7\u51f8\u6216\u4ec5\u6709\u8f7b\u5ea6\u810a\u67f1\u4fa7\u51f8\u7684SMA\u60a3\u8005\u6765\u8bf4\uff0c\u60a3\u8005\u5e8f\u8d2f\u9798\u5185\u6ce8\u5c04\u6cbb\u7597\u7684\u96be\u5ea6\u8f83\u4f4e\uff0c\u53ef\u4ee5\u8fdb\u884c\u5e38\u89c4\u9798\u5185\u6ce8\u5c04\u7ed9\u836f\u3002<\/p>\n<p>\uff082\uff09\u5bf9\u4e8e\u5408\u5e76\u91cd\u5ea6\u810a\u67f1\u4fa7\u51f8\u7684\u60a3\u8005\uff0c\u5219\u7531\u9798\u5185\u6ce8\u5c04\u7ecf\u9a8c\u4e30\u5bcc\u7684\u533b\u5e08\u5728\u8d85\u58f0\u5f15\u5bfc\u3001CT\u5f15\u5bfc\u6216X\u7ebfC\u81c2\u5f15\u5bfc\u4e0b\u8fdb\u884c\u8170\u690e\u6216\u9888\u690e\u7a7f\u523a\u53ca\u9798\u5185\u6ce8\u5c04\u7ed9\u836f\u3002\u5e76\u4e0e\u60a3\u8005\u53ca\u5176\u5bb6\u5c5e\u5145\u5206\u8ba8\u8bba\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u610f\u613f\u4e0e\u624b\u672f\u6307\u5f81\u3002<\/p>\n<p>\uff083\uff09\u5bf9\u4e8e\u5177\u6709\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u6307\u5f81\u548c\u610f\u613f\u7684\u60a3\u8005\uff0c\u82e5\u9aa8\u79d1\u3001\u9ebb\u9189\u79d1\u4e13\u79d1\u533b\u5e08\u5168\u9762\u8bc4\u4f30\u540e\u8003\u8651\u60a3\u8005\u53ef\u4ee5\u8010\u53d7\u624b\u672f\uff0c\u518d\u4e0e\u60a3\u8005\uff08\u53ca\u5176\u5bb6\u5c5e\uff09\u8fdb\u884c\u5145\u5206\u6c9f\u901a\uff0c\u7ed3\u5408\u60a3\u8005\u81ea\u8eab\u60c5\u51b5\uff0c\u9009\u62e9\u5148\u884c\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u3001\u518d\u542f\u52a8DMT\uff0c\u6216\u5148\u542f\u52a8DMT\u3001\u62e9\u671f\u884c\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u3002\u503c\u5f97\u6ce8\u610f\u7684\u662f\uff0c\u56e0\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u53ef\u80fd\u5bfc\u81f4\u540e\u671f\u9798\u5185\u6ce8\u5c04\u96be\u5ea6\u589e\u5927\uff0c\u63a8\u8350\u5728\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u4e2d\uff0c\u6839\u636e\u540e\u7eed\u9798\u5185\u6ce8\u5c04\u6cbb\u7597\u7684\u9700\u8981\u9884\u8bbe\u7a7f\u523a\u8def\u5f84\uff0c\u5728\u5bf9\u5e94\u7684\u690e\u677f\u95f4\u9699\u94b3\u9664\u90e8\u5206\u9aa8\u7ec4\u7ec7\uff0c\u9002\u5f53\u6269\u5927\u7a7f\u523a\u95f4\u9699\u3002\u6b64\u5916\uff0c\u82e5\u60a3\u8005\u7684\u8425\u517b\u72b6\u51b5\u6216\u547c\u5438\u529f\u80fd\u8f83\u5dee\uff0c\u7ecf\u8fc7\u9ebb\u9189\u79d1\u3001\u9aa8\u79d1\u8bc4\u4f30\u6682\u65f6\u65e0\u6cd5\u8010\u53d7\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\uff0c\u53ef\u8003\u8651\u5148\u8fdb\u884cDMT\u836f\u7269\u6cbb\u7597\u548c\u7efc\u5408\u7ba1\u7406\uff0c\u5f85\u60a3\u8005\u76f8\u5173\u60c5\u51b5\u6709\u6240\u6539\u5584\u540e\uff0c\u518d\u542f\u52a8\u9aa8\u79d1\u77eb\u5f62\u624b\u672f\u7684\u8bc4\u4f30\u5de5\u4f5c\u3002\u8be6\u89c1\u591a\u5b66\u79d1\u7efc\u5408\u7ba1\u7406-\u9aa8\u79d1\u90e8\u5206(\u672c\u520a\u540e\u7eed\u62a5\u9053\uff09\u3002<\/p>\n<p>\uff084\uff09\u5bf9\u4e8e\u5408\u5e76\u91cd\u5ea6\u810a\u67f1\u4fa7\u5f2f\u3001\u8170\u690e\u7a7f\u523a\u56f0\u96be\u7684\u60a3\u8005\uff0c\u6709\u7ecf\u9a8c\u7684\u4e2d\u5fc3\u53ef\u8003\u8651\u5b9e\u65bd\u8d85\u58f0\u5b9e\u65f6\u5f15\u5bfc\u4e0b\u9888\u690e\u7a7f\u523a\u7ed9\u836f\uff0c\u6216\u5728\u5f81\u5f97\u60a3\u8005\u53ca\u5176\u5bb6\u5c5e\u5145\u5206\u77e5\u60c5\u540c\u610f\u7684\u60c5\u51b5\u4e0b\uff0c\u5c1d\u8bd5\u901a\u8fc7\u5916\u79d1\u624b\u672f\u8fdb\u884c\u86db\u7f51\u819c\u4e0b\u8154\u7f6e\u7ba1\uff0c\u7528\u4e8e\u540e\u7eed\u7528\u836f\u3002\u6b64\u5916\uff0c\u4e5f\u53ef\u5efa\u8bae\u53e3\u670d\u836f\u7269\u6cbb\u7597\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u6839\u636e\u8fd0\u52a8\u529f\u80fd\u7684\u91cf\u5316\u5f97\u5206\u53ca\u7efc\u5408\u8bc4\u4f30\u7ed3\u679c\u534f\u52a9\u60a3\u8005\uff08\u53ca\u5176\u5bb6\u5c5e\uff09\u786e\u7acb\u5408\u7406\u7684\u6cbb\u7597\u9884\u671f\uff08\u2160\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002\u5145\u5206\u8003\u8651\u60a3\u8005\u810a\u67f1\u4fa7\u51f8\u3001\u8425\u517b\u72b6\u51b5\u7b49\u591a\u65b9\u9762\u56e0\u7d20\uff0c\u5728\u4e0e\u60a3\u8005\u5145\u5206\u6c9f\u901a\u7684\u57fa\u7840\u4e0a\uff0c\u5171\u540c\u786e\u5b9a\u4e2a\u4f53\u5316\u7684\u9798\u5185\u6ce8\u5c04\u65b9\u5f0f\uff08\u2160\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>6.1.2&nbsp;&nbsp;\u6cbb\u7597\u540e\u7684\u795e\u7ecf\u7cfb\u7edf\u8fd0\u52a8\u529f\u80fd\u8bc4\u4f30<\/p>\n<p>\u6cbb\u7597\u540e\u8fd0\u52a8\u529f\u80fd\u8bc4\u4f30\u9664\u8fd0\u52a8\u529f\u80fd\u91cf\u8868\u5916\uff0c\u8fd8\u5305\u62ec\u795e\u7ecf\u79d1\u533b\u5e08\u5b8c\u6210\u7684\u795e\u7ecf\u7cfb\u7edf\u5ba2\u89c2\u67e5\u4f53\u3001\u795e\u7ecf\u7535\u751f\u7406\u8bc4\u4f30\u4e24\u4e2a\u65b9\u9762\u3002\u6b64\u5916\uff0c\u53ef\u7a7f\u6234\u8bbe\u5907\u3001\u8fdc\u7a0b\u4ea4\u4e92\u7b49\u65b0\u5174\u8bc4\u4f30\u65b9\u5f0f\u4e5f\u5728\u79ef\u6781\u63a2\u7d22\u4e2d\u3002\u5b9a\u671f\u5bf9\u60a3\u8005\u6cbb\u7597\u540e\u7684\u8fd0\u52a8\u529f\u80fd\u8fdb\u884c\u8bc4\u4ef7\uff0c\u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\u795e\u7ecf\u7535\u751f\u7406\u68c0\u67e5\u53ef\u4ee5\u91cf\u5316\u8bc4\u4f30\u8fd0\u52a8\u5355\u4f4d\u7684\u529f\u80fd\u53ca\u6570\u91cf\u53d8\u5316\u3002\u5728\u56fd\u5916\u7684\u591a\u9879\u7814\u7a76\u4e2d\uff0cCMAP\u6ce2\u5e45\u3001\u8fd0\u52a8\u5355\u4f4d\u8ba1\u6570(motor unit number estimation,MUNE)\u7b49\u7535\u751f\u7406\u6307\u6807\u65e0\u8bba\u662f\u5728\u9488\u5bf9\u9752\u5c11\u5e74SMA\u60a3\u8005\u53ca\u6210\u4ebaSMA\u60a3\u8005\u7684\u81ea\u7136\u53f2\u7814\u7a76\uff0c\u8fd8\u662f\u5728DMT\u540e\u7684\u7597\u6548\u8bc4\u4f30\u65b9\u9762\uff0c\u5747\u6709\u6240\u5e94\u7528\u3002\u4f46\u9650\u4e8e\u7535\u751f\u7406\u68c0\u67e5\u6240\u9700\u6761\u4ef6\u548c\u4e13\u4e1a\u8981\u6c42\uff0c\u5e76\u4e0d\u8981\u6c42\u6240\u6709\u533b\u9662\u7edf\u4e00\u5f00\u5c55\uff0c\u4ec5\u63a8\u8350\u6709\u6761\u4ef6\u7684\u533b\u9662\u6839\u636e\u5b9e\u9645\u60c5\u51b5\u548c\u4e34\u5e8a\u79d1\u7814\u9700\u6c42\u5f00\u5c55\u68c0\u67e5\u3002<\/p>\n<p>\uff081\uff09\u8fd0\u52a8\u795e\u7ecf\u4f20\u5bfc\u6d4b\u5b9a\uff08motor conduction velocity\uff0cMCV\uff09\u3002\u5728MCV\u68c0\u67e5\u4e2d\uff0cSMA\u60a3\u8005\u4e3b\u8981\u5173\u6ce8\u7684\u6307\u6807\u662fCMAP\u3002\u56e0\u4e3aSMA\u7684\u75c5\u7406\u751f\u7406\u673a\u5236\u662f\u4ee5\u8fd0\u52a8\u795e\u7ecf\u5143\u51cb\u4ea1\u7ee7\u53d1\u8fd0\u52a8\u8f74\u7d22\u635f\u5bb3\uff0c\u800c\u8fd0\u52a8\u795e\u7ecf\u4f20\u5bfc\u6d4b\u5b9a\u4e2d\u7684\u5176\u4ed6\u6307\u6807\uff0c\u5982\u8fdc\u7aef\u8fd0\u52a8\u6f5c\u4f0f\u671f\u53ca\u795e\u7ecf\u4f20\u5bfc\u901f\u5ea6\u4e3b\u8981\u53cd\u6620\u9ad3\u9798\u529f\u80fd\u3002CMAP\u6ce2\u5e45\u5927\u5c0f\u4ee3\u8868\u523a\u6fc0\u7279\u5b9a\u795e\u7ecf\u6fc0\u6d3b\u7684\u8fd0\u52a8\u5355\u4f4d\u53ca\u76f8\u5e94\u808c\u7ea4\u7ef4\u7684\u603b\u6570\u76ee\u3002\u5927\u91cf\u7684\u7814\u7a76\u5c06CMAP\u6ce2\u5e45\u4f5c\u4e3a\u6b21\u8981\u9884\u540e\u6307\u6807\u3002\u5728\u56fd\u5916\u7684\u4e00\u9879\u9488\u5bf9\u513f\u7ae5SMA\u60a3\u8005\u7684\u8bfa\u897f\u90a3\u751f\u6cbb\u7597\u7814\u7a76\u4e2d\u89c2\u5bdf\u5230\uff0c\u968f\u7740\u60a3\u513f\u8fd0\u52a8\u529f\u80fd\u7684\u6301\u7eed\u6539\u5584\u53ca\u75c5\u60c5\u8d8b\u4e8e\u7a33\u5b9a\uff0cCMAP\u6ce2\u5e45\u4e5f\u76f8\u5bf9\u7a33\u5b9a<sup>\uff3b25\uff3d<\/sup>\u3002<\/p>\n<p>\uff082\uff09 MUNE\u3002MUNE\u662f\u4ece\u5b8f\u89c2\u7684\u89d2\u5ea6\uff0c\u5b9a\u91cf\u8bc4\u4ef7\u67d0\u795e\u7ecf\u6240\u652f\u914d\u7684\u4e00\u5757\u9aa8\u9abc\u808c\u6216\u808c\u7fa4\u603b\u7684\u3001\u6709\u529f\u80fd\u7684\u8fd0\u52a8\u5355\u4f4d\u6570\u91cf\uff0c\u5177\u6709\u65e0\u521b\u6027\u7684\u4f18\u70b9\uff0c\u76f8\u8f83\u4e8eCMAP\u66f4\u52a0\u654f\u611f\uff0c\u66f4\u9002\u7528\u4e8e\u8fd0\u52a8\u5355\u4f4d\u6570\u91cf\u7684\u5b9a\u671f\u8bc4\u4f30\u3002\u5c31\u68c0\u6d4b\u7684\u4e0d\u9002\u611f\u548c\u4fbf\u5229\u6027\u800c\u8a00\uff0c\u60a3\u8005\u5bf9\u8fd0\u52a8\u5355\u4f4d\u6570\u91cf\u6307\u6570\uff08motorunit number index\uff0cMUNIX\uff09\u7684\u8010\u53d7\u6027\u53ef\u80fd\u66f4\u597d\u3002\u73b0\u9636\u6bb5\u5c1a\u9700\u66f4\u591a\u7684\u4e34\u5e8a\u7814\u7a76\u5e2e\u52a9\u660e\u786eMUNE\u5728SMA\u60a3\u8005\u7597\u6548\u8bc4\u4f30\u4e2d\u7684\u4ef7\u503c\uff0c\u5c24\u5176\u662f\u5982\u4f55\u6839\u636e\u60a3\u8005\u7684\u75c5\u7a0b\u53ca\u75c5\u60c5\u4e25\u91cd\u7a0b\u5ea6\u6765\u8bbe\u8ba1\u4e2a\u6027\u5316\u7684\u68c0\u67e5\u7b56\u7565\uff0c\u4ee5\u671f\u8fbe\u5230\u7cbe\u51c6\u8bc4\u4f30\u7684\u6548\u80fd\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u901a\u8fc7\u795e\u7ecf\u7cfb\u7edf\u5ba2\u89c2\u67e5\u4f53\u53ca\u795e\u7ecf\u7535\u751f\u7406\u68c0\u67e5\u5bf9\u6cbb\u7597\u524d\u540e\u7684\u8fd0\u52a8\u529f\u80fd\u53d8\u5316\u8fdb\u884c\u8bc4\u4f30\uff0c\u6709\u52a9\u4e8e\u4e86\u89e3\u60a3\u8005\u75c5\u60c5\u53d8\u5316\uff08\u2161\u7ea7\u63a8\u8350\uff0cB\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>\uff08\u672c\u90e8\u5206\u6267\u7b14\u4eba\uff1a\u6234\u6bc5&nbsp; \u6768\u6d35\u54f2\uff09<\/p>\n<p>6.2&nbsp;&nbsp;\u5f71\u50cf\u79d1\u8bca\u7597\u7ba1\u7406<\/p>\n<p>SMA\u53d1\u75c5\u7387\u4f4e\uff0c\u7f3a\u4e4f\u7279\u5f81\u6027\u5f71\u50cf\u5b66\u5f81\u8c61\uff0c\u5f71\u50cf\u5b66\u76f8\u5173\u7814\u7a76\u7684\u6587\u732e\u8f83\u5c11\u3002\u6839\u636eSMA\u60a3\u8005\u7684\u4e34\u5e8a\u8868\u73b0\u53ca\u53d7\u7d2f\u90e8\u4f4d\uff0c\u5f71\u50cf\u5b66\u4e3b\u8981\u7528\u4e8e\u8bc4\u4f30\u75be\u75c5\u7684\u4e25\u91cd\u7a0b\u5ea6\u3001\u968f\u8bbf\u75c5\u7a0b\u8fdb\u5c55\u548c\u53ca\u65f6\u53d1\u73b0\u5e76\u53d1\u75c7\u3002\u5efa\u8bae\u6839\u636e\u4e34\u5e8a\u9700\u6c42\u9009\u62e9\u5408\u9002\u7684\u68c0\u67e5\u65b9\u6cd5\u3002<\/p>\n<p>6.2.1&nbsp;&nbsp;X\u7ebf\u68c0\u67e5<\/p>\n<p>SMA\u60a3\u8005\u5bb9\u6613\u51fa\u73b0\u810a\u67f1\u4fa7\u51f8\u53ca\u540e\u51f8\uff0c X\u7ebf\u68c0\u67e5\u662f\u8bc4\u4f30\u9aa8\u9abc\u7cfb\u7edf\u7684\u9996\u9009\u68c0\u67e5\u65b9\u6cd5\u3002\u5168\u810a\u67f1X\u7ebf\u53ef\u8bc4\u4f30\u662f\u5426\u5b58\u5728\u810a\u67f1\u4fa7\u51f8\u6216\u540e\u51f8\uff0c\u6709\u65e0\u5e76\u53d1\u7684\u690e\u4f53\u53d1\u80b2\u7578\u5f62\uff0c\u6d4b\u91cfCobb\u89d2\u4ee5\u8bc4\u4f30\u4fa7\u540e\u51f8\u7684\u4e25\u91cd\u7a0b\u5ea6\u53ca\u8fdb\u5c55\u901f\u5ea6\u3002\u9664\u62cd\u6444\u5168\u810a\u67f1\u6b63\u4fa7\u4f4dX\u7ebf\u7247\u5916\uff0c\u5efa\u8bae\u540c\u65f6\u884c\u524d\u5c48\u3001\u540e\u4f38\u53ca\u5de6\u53f3\u4fa7\u5c48\u4f4d\u4ee5\u5168\u9762\u8bc4\u4f30\u810a\u67f1\u6d3b\u52a8\u5ea6<sup>\uff3b12\uff3d<\/sup>\u3002SMA \u60a3\u8005\u5e38\u5408\u5e76\u9aa8\u8d28\u758f\u677e\u3002\u8170\u690e\u548c\u80a1\u9aa8\u9888\u7684\u53cc\u80fdX\u5c04\u7ebf\u5438\u6536\u6cd5\uff08dual energy X-ray absorptiometry\uff0cDEXA\uff09\u4ef7\u683c\u4f4e\u5ec9\uff0c\u8f90\u5c04\u5242\u91cf\u4f4e\uff0c\u53ef\u51c6\u786e\u6d4b\u91cf\u9aa8\u5bc6\u5ea6\uff0c\u53cd\u6620\u9aa8\u91cf\u51cf\u5c11\u7684\u7a0b\u5ea6\u3002\u867d\u7136\u7f3a\u4e4f\u513f\u7ae5\u4e0d\u540c\u5e74\u9f84\u6bb5\u7684\u9aa8\u5bc6\u5ea6\u6807\u51c6\u6570\u636e\uff0c\u4f46DEXA\u5bf9SMA\u9752\u5c11\u5e74\u3001\u6210\u4eba\u60a3\u8005\u7684\u968f\u8bbf\u89c2\u5bdf\u6709\u4e00\u5b9a\u7684\u610f\u4e49\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aSMA\u60a3\u8005\u5e94\u89c4\u5f8b\u968f\u8bbf\uff0c\u6bcf\u5e74\u5e38\u89c4\u884c\u4e00\u6b21\u5168\u810a\u67f1 X \u7ebf\u53ca DEXA \u68c0\u67e5\uff08\u2161\u7ea7\u63a8\u8350\uff0cC\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>6.2.2&nbsp;&nbsp;CT<\/p>\n<p>CT\u626b\u63cf\u7528\u4e8e\u5bf9\u5168\u810a\u67f1\u690e\u4f53\u53ca\u9644\u4ef6\u9aa8\u8d28\u7ed3\u6784\u7684\u8fdb\u4e00\u6b65\u89c2\u5bdf\uff0c\u591a\u5e73\u9762\u91cd\u5efa\u53ca\u5bb9\u79ef\u6210\u50cf\u80fd\u5168\u9762\u663e\u793a\u810a\u67f1\u7684\u4e09\u7ef4\u7ed3\u6784\u3002\u5bf9\u4e8e\u5408\u5e76\u91cd\u5ea6\u810a\u67f1\u4fa7\u51f8\u7684\u60a3\u8005\uff0c\u53ef\u7531\u9798\u5185\u6ce8\u5c04\u7ecf\u9a8c\u4e30\u5bcc\u7684\u533b\u5e08\u5728 CT\u5f15\u5bfc\u4e0b\u8fdb\u884c\u8170\u690e\u6216\u9888\u690e\u7a7f\u523a\u53ca\u9798\u5185\u6ce8\u5c04\u7ed9\u836f\u3002\u5728SMA\u60a3\u8005\u53d1\u751f\u5916\u4f24\u65f6\uff0cCT\u4e0d\u4ec5\u80fd\u660e\u786e\u662f\u5426\u5b58\u5728\u9aa8\u6298\uff0c\u4e5f\u53ef\u6e05\u6670\u663e\u793a\u9aa8\u6298\u65ad\u7aef\u4e4b\u95f4\u7684\u76f8\u4e92\u5173\u7cfb\u3002CT\u7684\u7f3a\u70b9\u662f\u8f6f\u7ec4\u7ec7\u5206\u8fa8\u7387\u4f4e\uff0c\u4e14\u5b58\u5728\u8f90\u5c04\uff0c\u6240\u4ee5\u8f83\u5c11\u7528\u4e8e\u8bc4\u4f30\u808c\u8089\u7684\u840e\u7f29\u7a0b\u5ea6\u3002<\/p>\n<p>SMA\u53ef\u7d2f\u53ca\u547c\u5438\u808c\uff0c\u5e38\u56e0\u547c\u5438\u529f\u80fd\u969c\u788d\u800c\u5e76\u53d1\u80ba\u90e8\u611f\u67d3\uff0c\u80f8\u90e8CT\u53ef\u7528\u4e8e\u8bc4\u4f30\u60a3\u8005\u6709\u65e0\u5408\u5e76\u80ba\u90e8\u611f\u67d3\u548c\u80ba\u4e0d\u5f20\u7b49\u5e76\u53d1\u75c7\u3002SMA \u4e5f\u6613\u53d1\u751f\u4e0d\u540c\u7a0b\u5ea6\u7684\u80ba\u529f\u80fd\u635f\u5bb3\uff0c\u5982\u53ef\u51fa\u73b0\u7528\u529b\u80ba\u6d3b\u91cf\uff08forced vital capacity\uff0c FVC\uff09\u30011\u79d2\u7528\u529b\u547c\u6c14\u5bb9\u79ef\uff08forced expiratory volume in the first second\uff0cFEV1\uff09\u3001\u547c\u6c14\u6d41\u91cf\u5cf0\u503c\uff08peak expiratory flow\uff0cPEF\uff09\u4e0b\u964d<sup>\uff3b26\uff3d<\/sup>\u3002\u5bf9\u4e8e\u4e00\u4e9b\u4e0d\u80fd\u8010\u53d7\u6216\u8005\u65e0\u6cd5\u5145\u5206\u914d\u5408\u5e38\u89c4\u80ba\u529f\u80fd\u68c0\u67e5\u7684\u60a3\u8005\uff0c\u884c\u57fa\u4e8eCT\u7684\u5438\u6c14\u672b\u4e0e\u547c\u6c14\u672b\u53cc\u671f\u76f8\u80ba\u5bb9\u79ef\u5b9a\u91cf\u6d4b\u91cf\u53ca\u5e73\u5747CT\u503c\u6d4b\u91cf\uff0c\u53ef\u4ee5\u53cd\u6620\u60a3\u8005\u7684\u80ba\u901a\u6c14\u529f\u80fd\u72b6\u6001\uff0c\u4f5c\u4e3a\u5e38\u89c4\u80ba\u529f\u80fd\u68c0\u67e5\u7684\u8865\u5145\uff0c\u6709\u52a9\u4e8e\u76f8\u5173SMA\u60a3\u8005\u7684\u591a\u5b66\u79d1\u4e2a\u4f53\u5316\u7ba1\u7406\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aSMA\u60a3\u8005\u53ef\u884c\u5168\u810a\u67f1CT\u626b\u63cf\u4e0e\u91cd\u5efa\uff0c\u5728CT\u5f15\u5bfc\u4e0b\u5b9e\u65bd\u9798\u5185\u6ce8\u5c04\u53ca\u5229\u7528CT\u8bc4\u4f30\u80ba\u529f\u80fd\uff08\u2161\u7ea7\u63a8\u8350\uff0cD\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>6.2.3&nbsp;&nbsp;MRI<\/p>\n<p>MRI\u5177\u6709\u65e0\u8f90\u5c04\u3001\u8f6f\u7ec4\u7ec7\u5206\u8fa8\u7387\u9ad8\u53ca\u591a\u65b9\u4f4d\u591a\u53c2\u6570\u6210\u50cf\u7684\u4f18\u52bf\uff0c\u5bf9\u808c\u8089\u75c5\u53d8\u7684\u663e\u793a\u4f18\u4e8e\u5176\u4ed6\u5f71\u50cf\u5b66\u68c0\u67e5\u65b9\u6cd5\u3002SMA \u60a3\u8005\u8868\u73b0\u4e3a\u4ee5\u4e0b\u80a2\u8fd1\u7aef\u8d77\u59cb\u3001\u7f13\u6162\u8fdb\u5c55\u7684\u56db\u80a2\u65e0\u529b\u840e\u7f29\u3002\u8bc4\u4f30\u4e0b\u80a2\u808c\u8089\u7684\u840e\u7f29\u7a0b\u5ea6\u53ef\u884c\u76f8\u5e94\u90e8\u4f4d\u7684MRI\u3002\u68c0\u67e5\u65f6\u5c3d\u91cf\u4f7f\u7528\u5927\u6210\u50cf\u91ce\uff08field of view\uff0c FOV\uff09\uff0c\u540c\u65f6\u8bc4\u4f30\u53cc\u4fa7\u80a2\u4f53\u53d7\u7d2f\u60c5\u51b5\u3002\u5e38\u7528\u8f74\u4f4d\u548c\u51a0\u72b6\u4f4d\u6210\u50cf\uff0c\u68c0\u67e5\u5e8f\u5217\u5305\u62ec\uff1aT<sub>1<\/sub>WI \u80fd\u6e05\u6670\u663e\u793a\u808c\u8089\u7684\u89e3\u5256\u7ed3\u6784\uff0c\u8bc4\u4f30\u808c\u8089\u662f\u5426\u5b58\u5728\u8102\u80aa\u6d78\u6da6\u53ca\u8102\u80aa\u6d78\u6da6\u7684\u4e25\u91cd\u7a0b\u5ea6\uff1b\u77ed\u65f6\u95f4\u53cd\u8f6c\u6062\u590d\uff08short time inversion recovery\uff0cSTIR\uff09\u76f8\u6216\u8102\u80aa\u6291\u5236\u7684 T<sub>2<\/sub>WI \u7528\u4e8e\u663e\u793a\u808c\u8089\u662f\u5426\u5b58\u5728\u6c34\u80bf<sup>\uff3b27\uff3d<\/sup>\u3002\u57fa\u4e8e\u6c34\u8102\u5316\u5b66\u4f4d\u79fb\uff08DIXON \u6cd5\uff09\u7684 T<sub>2<\/sub>WI\u4e00\u6b21\u91c7\u96c6\u80fd\u540c\u65f6\u83b7\u5f97\u6c34\u8102\u5206\u79bb\u56fe\u50cf\uff0c\u540c\u65f6\u8bc4\u4ef7\u808c\u8089\u7684\u8102\u80aa\u6d78\u6da6\u548c\u6c34\u80bf\u3002\u5728\u808c\u8089\u6d3b\u68c0\u524d\u884c MRI \u68c0\u67e5\u6709\u52a9\u4e8e\u9009\u62e9\u6d3b\u68c0\u90e8\u4f4d\uff0c\u964d\u4f4e\u6d3b\u68c0\u7684\u5047\u9634\u6027\u3002<\/p>\n<p>\u5927\u591a\u6570\u795e\u7ecf\u808c\u8089\u75be\u75c5\u7684\u65e9\u671f\u9636\u6bb5\uff0c\u808c\u8089\u5728 STIR \u76f8\u6216\u8102\u80aa\u6291\u5236\u7684 T<sub>2<\/sub>WI\u5448\u8f7b\u4e2d\u5ea6\u9ad8\u4fe1\u53f7\uff0c\u63d0\u793a\u6c34\u80bf\u3002SMA\u60a3\u8005\u662f\u5426\u6709\u808c\u8089\u6c34\u80bf\u7684\u5f71\u50cf\u8868\u73b0\u5c1a\u4e0d\u660e\u786e\u3002SMA\u60a3\u8005\u7684\u4e3b\u8981MRI\u8868\u73b0\u4e3a\u808c\u8089\u5185\u8102\u80aa\u6d78\u6da6\u548c\u808c\u8089\u840e\u7f29\u3002\u8102\u80aa\u6d78\u6da6\u5728T<sub>1<\/sub>WI\u53caT<sub>2<\/sub>WI\u5747\u5448\u9ad8\u4fe1\u53f7\uff0c\u538b\u8102\u5e8f\u5217\u4e3a\u4f4e\u4fe1\u53f7\uff0c\u4e0e\u76ae\u4e0b\u8102\u80aa\u7684\u4fe1\u53f7\u7279\u70b9\u7c7b\u4f3c\u3002\u4e2a\u6848\u62a5\u9053<sup>\uff3b28\uff3d<\/sup>SMA\u60a3\u8005\u5927\u817f\u808c\u8089\u91cd\u5ea6\u840e\u7f29\u65f6\uff0c\u957f\u6536\u808c\u53d7\u7d2f\u4ecd\u8f83\u8f7b\uff0c\u5448\u73b0\u201c\u8c41\u514d\u201d\u8868\u73b0\uff0c\u8be5\u808c\u8089\u53d7\u7d2f\u6a21\u5f0f\u53ef\u80fd\u6709\u52a9\u4e8e\u4e0e\u5176\u4ed6\u795e\u7ecf\u808c\u8089\u75be\u75c5\u9274\u522b\u8bca\u65ad\u3002<\/p>\n<p>\u5bf9\u808c\u8089\u8102\u80aa\u6d78\u6da6\u4e25\u91cd\u7a0b\u5ea6\u7684\u8fdb\u4e00\u6b65\u8bc4\u4f30\u6709\u534a\u5b9a\u91cf\u8bc4\u5206\u6cd5\u548c\u5b9a\u91cf\u6cd5\u3002\u534a\u5b9a\u91cf\u8bc4\u5206\u6cd5\u57fa\u4e8e\u5e38\u89c4 MRI \u626b\u63cf\u5e8f\u5217\uff0c \u5c06\u808c\u8089\u8102\u80aa\u6d78\u6da6\u7a0b\u5ea6\u4eba\u4e3a\u5206\u4e3a 5\u7ea7\uff080\u5206\uff1a\u65e0\u8102\u80aa\u6d78\u6da6\uff1b1\u5206\uff1a\uff1c30%\u8102\u80aa\u6d78\u6da6\uff1b2\u5206\uff1a30%\uff5e60%\u8102\u80aa\u6d78\u6da6\uff1b3\u5206\uff1a\uff1e60%\u8102\u80aa\u6d78\u6da6\uff1b4\u5206\uff1a\u5168\u90e8\u808c\u8089\u5747\u4e3a\u8102\u80aa\u6240\u4ee3\u66ff\uff09<sup>\uff3b29\uff3d<\/sup>\u3002\u5b9a\u91cf\u6cd5\u53ef\u4f7f\u7528 MRI\u8bbe\u5907\u4f9b\u5e94\u5546\u63d0\u4f9b\u7684\u8102\u80aa\u5b9a\u91cf\u5e8f\u5217\uff0c\u4ee5\u6d4b\u91cf\u808c\u8089\u8102\u80aa\u5206\u6570\uff08fat fraction\uff0cFF\uff09\u3002\u65e0\u8bba\u662f\u534a\u5b9a\u91cf\u8bc4\u5206\u6cd5\u8fd8\u662f\u5b9a\u91cf\u6cd5\uff0c\u5747\u4ec5\u9650\u4e8e\u79d1\u7814\u9636\u6bb5\uff0c\u5c1a\u672a\u5e38\u89c4\u5e94\u7528\u4e8e\u4e34\u5e8a\u8bc4\u4ef7\u3002<\/p>\n<p>\u5173\u4e8eSMA\u60a3\u8005\u8111\u7ec4\u7ec7\u5f71\u50cf\u6539\u53d8\u7684\u7814\u7a76\u5f88\u5c11\uff0c\u53ea\u6709\u5c11\u6570\u62a5\u9053\u3002\u5bf9 SMA 0\u578b\u548c1\u578b\u7684\u4e2a\u6848\u62a5\u9053\u663e\u793a\uff0c\u90e8\u5206\u60a3\u8005\u53ef\u89c1\u5e7f\u6cdb\u7684\u5927\u8111\u534a\u7403\u76ae\u5c42\u840e\u7f29\uff0c\u6709\u7684\u60a3\u8005\u53ef\u89c1\u5e7f\u6cdb\u7684\u767d\u8d28\u840e\u7f29\uff0c\u4e5f\u6709\u60a3\u8005\u53ef\u89c1\u4e18\u8111\u548c\u58f3\u6838\u7684\u5f02\u5e38\u4fe1\u53f7<sup>\uff3b30\uff3d<\/sup>\u3002\u800c\u5173\u4e8e\u6210\u4eba\u578bSMA\uff0c\u4e00\u9879\u7814\u7a76\u6bd4\u8f83\u4e8625\u4f8bSMA 3\u578b\u548c4\u578b\u60a3\u8005\u4e0e\u6b63\u5e38\u5bf9\u7167\uff0c\u60a3\u8005\u4e0e\u5bf9\u7167\u7ec4\u4e4b\u95f4\u76ae\u5c42\u539a\u5ea6\u3001\u7070\u8d28\u4f53\u79ef\u53ca\u767d\u8d28\u4f53\u79ef\u5747\u65e0\u660e\u663e\u7edf\u8ba1\u5b66\u5dee\u5f02\uff0cSMA \u60a3\u8005\u8fd0\u52a8\u76ae\u5c42\u7070\u8d28\u5bc6\u5ea6\u9ad8\u4e8e\u6b63\u5e38\u5bf9\u7167\u8005\uff0c\u53ef\u80fd\u4e3a\u4ee3\u507f\u6027\u6539\u53d8<sup>\uff3b31\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u6210\u4ebaSMA 3\u578b\u62164\u578b\u60a3\u8005\u5c0f\u8111\u7684\u67d0\u4e9b\u533a\u57df\u4e5f\u6709\u660e\u663e\u7684\u840e\u7f29<sup>\uff3b32\uff3d<\/sup>\u3002<\/p>\n<p>\u5173\u4e8eSMA\u60a3\u8005\u810a\u9ad3\u5f71\u50cf\u5b66\u6539\u53d8\u7684\u62a5\u9053\u8f83\u5c11\u3002\u6709\u7814\u7a76\u663e\u793a\u6210\u4ebaSMA\u60a3\u8005\u7684\u9888\u9ad3\u6a2a\u622a\u9762\u79ef\uff0c\u4e0e\u6b63\u5e38\u5bf9\u7167\u8005\u4e4b\u95f4\u6709\u7edf\u8ba1\u5b66\u5dee\u5f02\uff0c\u660e\u663e\u5c0f\u4e8e\u6b63\u5e38\u5bf9\u7167\u8005<sup>\uff3b31\uff3d<\/sup>\uff1b\u4e5f\u6709\u7814\u7a76\uff08\u5305\u62ec\u513f\u7ae5\u53ca\u6210\u4eba\uff0cSMA 2\u578b\u548c3\u578b\uff09\u7ed3\u679c\u663e\u793a SMA \u60a3\u8005\u548c\u6b63\u5e38\u5bf9\u7167\u8005\u4e4b\u95f4\u9888\u9ad3\u6a2a\u622a\u9762\u79ef\u65e0\u7edf\u8ba1\u5b66\u5dee\u5f02<sup>\uff3b33\uff3d<\/sup>\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aMRI\u53ef\u7528\u4e8eSMA\u60a3\u8005\u808c\u8089\u840e\u7f29\u7a0b\u5ea6\u3001\u75c5\u60c5\u8fdb\u5c55\u7684\u5ba2\u89c2\u8bc4\u4f30\u624b\u6bb5\uff0c\u5728\u79ef\u7d2f\u66f4\u591a\u6570\u636e\u540e\uff0c\u6709\u671b\u7528\u4e8e\u4e34\u5e8a\u8bc4\u4f30\uff08\u2162\u7ea7\u63a8\u8350\uff0cD\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>\u5728\u5f71\u50cf\u5b66\u8bc4\u4f30\u65b9\u9762\uff0c\u5173\u4e8eSMA\u60a3\u8005\u795e\u7ecf\u7cfb\u7edf\u5f71\u50cf\u6539\u53d8\u7684\u7814\u7a76\u6709\u9650\uff0c\u75c5\u4f8b\u6570\u8f83\u5c11\uff0c\u6709\u5f85\u4e8e\u8fdb\u4e00\u6b65\u7814\u7a76\u3002<\/p>\n<p>\uff08\u672c\u90e8\u5206\u6267\u7b14\u4eba\uff1a\u738b\u51e4\u4e39&nbsp;&nbsp;\u5b8b\u5170&nbsp;&nbsp;\u6709\u6167\uff09<\/p>\n<p>6.3&nbsp;&nbsp;\u5fc3\u7406\u533b\u5b66\u79d1\u8bca\u7597\u7ba1\u7406<\/p>\n<p>\u7531\u4e8e\u5173\u4e8eSMA\u76f8\u5173\u7cbe\u795e\u5fc3\u7406\u76d1\u6d4b\u548c\u7ba1\u7406\u7684\u53c2\u8003\u6587\u732e\u6709\u9650\uff0c\u56e0\u6b64\u672c\u90e8\u5206\u5185\u5bb9\u7684\u5f62\u6210\u4e3b\u8981\u662f\u53c2\u8003\u5176\u4ed6\u6162\u6027\u75be\u75c5\u7684\u7cbe\u795e\u5fc3\u7406\u95ee\u9898\u8fdb\u884c\u5236\u5b9a\u3002\u672c\u5185\u5bb9\u5f3a\u8c03\u652f\u6301\u6027\u3001\u8d44\u6e90\u53d6\u5411\u5fc3\u7406\u6cbb\u7597\u7684\u539f\u5219\uff0c\u91cd\u89c6\u60a3\u8005\u3001\u533b\u751f\u7684\u611f\u53d7\uff0c\u4ee5\u53ca\u533b\u60a3\u5173\u7cfb\u4f53\u9a8c\u3002<\/p>\n<p>\u5bf9SMA\u60a3\u8005\u53ca\u5176\u5bb6\u4eba\u7684\u5168\u9762\u7167\u987e\u548c\u62a4\u7406\u987b\u5305\u62ec\u5bf9\u5176\u7cbe\u795e\u5fc3\u7406\u5065\u5eb7\u7684\u76d1\u6d4b\u548c\u7ba1\u7406\u3002\u7efc\u5408\u5fc3\u7406\u7ba1\u7406\u5e94\u89e3\u51b3\u60a3\u8005\u793e\u4f1a\u548c\u8ba4\u77e5\u53d1\u5c55\u3001\u751f\u6d3b\u8d28\u91cf\uff0c\u4ee5\u53ca\u5f71\u54cd\u60a3\u8005\u5bb6\u5ead\u529f\u80fd\u7684\u56e0\u7d20\uff0c\u5177\u4f53\u9700\u6c42\u6839\u636e\u60a3\u8005\u5e74\u9f84\u548c SMA\u4e9a\u578b\u800c\u5f02\u3002\u5728SMA\u7ba1\u7406\u56e2\u961f\u4e2d\u5e94\u5f53\u914d\u5907\u5fc3\u7406\u5065\u5eb7\u65b9\u9762\u7684\u4e34\u5e8a\u533b\u751f\uff0c\u5305\u62ec\u795e\u7ecf\u79d1\u3001\u7cbe\u795e\u79d1\u3001\u8425\u517b\u79d1\u3001\u8fd0\u52a8\u5eb7\u590d\u79d1\u7b49\uff0c\u4ee5\u53ca\u5728\u5e2e\u52a9\u6162\u6027\u75be\u75c5\u60a3\u8005\u65b9\u9762\u6709\u4e13\u4e1a\u7ecf\u9a8c\u7684\u5fc3\u7406\u6cbb\u7597\u5e08\u548c\u793e\u4f1a\u5de5\u4f5c\u8005\u7b49\u3002<\/p>\n<p>6.3.1&nbsp;&nbsp;\u57fa\u672c\u5fc3\u7406\u76d1\u6d4b\u548c\u7ba1\u7406<\/p>\n<p>\u57fa\u672c\u5fc3\u7406\u76d1\u6d4b\u548c\u7ba1\u7406\u5305\u62ec<sup>\uff3b34\uff3d<\/sup>\uff1a\u5728\u60a3\u8005\u75be\u75c5\u8bca\u65ad\u524d\u3001\u521a\u521a\u83b7\u5f97\u75be\u75c5\u8bca\u65ad\u540e\u3001\u6cbb\u7597\u8fc7\u7a0b\u4e2d\u3001\u4ee5\u53ca\u75be\u75c5\u53d1\u751f\u8fdb\u5c55\u7b49\u5173\u952e\u65f6\u95f4\u8282\u70b9\u4e0a\u8fdb\u884c\u5fc3\u7406\u8bc4\u4f30\uff1b\u5728\u5404\u79d1\u5c31\u8bca\u8fc7\u7a0b\u4e2d\uff0c\u6ce8\u610f\u8bc4\u4f30\u60a3\u8005\u7cbe\u795e\u5fc3\u7406\u5065\u5eb7\u6c34\u5e73\u548c\u751f\u6d3b\u8d28\u91cf\uff1b\u975e\u7cbe\u795e\u79d1\u533b\u751f\u53ef\u4ee5\u4f7f\u7528\u7ed3\u6784\u5316\u8bc4\u4f30\u5de5\u5177\uff0c\u5bf9\u60a3\u8005\u5b8c\u6210\u5fc3\u7406\u8bc4\u4f30\uff0c\u4f8b\u5982\uff0c9\u9879\u60a3\u8005\u5065\u5eb7\u95ee\u5377\uff08patient health questionnire-9,PHQ-9)\u30017\u9879\u5e7f\u6cdb\u6027\u7126\u8651\u969c\u788d\u91cf\u8868\uff08generalized anxiety disorde-7,GAD-7)\u300190\u9879\u75c7\u72b6\u6e05\u5355\uff08symptom check list 90,SCL-90)\u3001\u7126\u8651\u81ea\u8bc4\u91cf\u8868\uff08self-rating anxiety scale,SAS)\u3001\u6291\u90c1\u81ea\u8bc4\u91cf\u8868\uff08self-rating depression scale,SDS)\u7b49\u3002\u5728\u6761\u4ef6\u8bb8\u53ef\u7684\u60c5\u51b5\u4e0b\uff0c\u53ef\u4ee5\u8003\u8651\u4f7f\u7528MINI\u5bf9\u60a3\u8005\u8fdb\u884c\u7cbe\u795e\u5fc3\u7406\u6d4b\u8bc4\u3002\u5bf9\u4e8e\u8bc4\u5206\u5f02\u5e38\u8005\uff0c\u9f13\u52b1\u8f6c\u8bca\u7cbe\u795e\u79d1\uff1b\u540c\u65f6\u5173\u6ce8\u60a3\u8005\u5bb6\u5c5e\uff08\u7236\u6bcd\u3001\u5144\u5f1f\u59d0\u59b9\u7b49\uff09\u53ca\u7167\u62a4\u8005\u7684\u5fc3\u7406\u5065\u5eb7\u72b6\u51b5\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1aSMA\u60a3\u8005\u53ca\u5bb6\u5ead\u9700\u5728\u8bca\u65ad\u5173\u952e\u65f6\u95f4\u70b9\u53ca\u957f\u671f\u968f\u8bca\u6cbb\u7597\u8fc7\u7a0b\u4e2d\u8fdb\u884c\u5fc3\u7406\u76d1\u6d4b\u548c\u7ba1\u7406\uff0c\u5e38\u89c4\u7ed3\u6784\u5316\u8bc4\u4f30\u5de5\u5177\u53d1\u73b0\u5f02\u5e38\u8005\uff0c\u5efa\u8bae\u8f6c\u8bca\u7cbe\u795e\u79d1\uff08\u2161\u7ea7\u63a8\u8350\uff0cD\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>6.3.2&nbsp;&nbsp;\u4e0d\u540c\u5e74\u9f84\u548c\u4e9a\u578bSMA\u60a3\u8005\u7684\u7cbe\u795e\u5fc3\u7406\u7279\u70b9<\/p>\n<p>6.3.2.1&nbsp;&nbsp;SMA\u513f\u7ae5\u3001\u9752\u5c11\u5e74\u60a3\u8005&nbsp; \u513f\u7ae5\u548c\u9752\u5c11\u5e74\u5904\u4e8e\u8ba4\u77e5\u3001\u5fc3\u7406\u9010\u6b65\u53d1\u80b2\u6210\u719f\u7684\u7279\u6b8a\u65f6\u671f\uff0c\u7ed9\u4e88\u5fc3\u7406\u6cbb\u7597\u524d\uff0c\u9700\u5145\u5206\u4e86\u89e3\u8fd9\u4e00\u5e74\u9f84\u6bb5\u60a3\u8005\u7684\u7cbe\u795e\u5fc3\u7406\u7279\u70b9\u3002<\/p>\n<p>\uff081\uff09\u5173\u6ce8SMA\u513f\u7ae5\u7684\u8ba4\u77e5\u53d1\u5c55\u548c\u793e\u4f1a\u529f\u80fd\u3002SMA\u60a3\u513f\u51fa\u73b0\u8fd0\u52a8\u969c\u788d\u5f80\u5f80\u65e9\u4e8e\u5176\u667a\u529b\u3001\u884c\u4e3a\u548c\u8bed\u8a00\u53d1\u80b2\uff0c\u8fd0\u52a8\u969c\u788d\u6240\u5bfc\u81f4\u7684\u6d3b\u52a8\u53d7\u9650\uff0c\u53ef\u80fd\u8fdb\u800c\u5f71\u54cd\u513f\u7ae5\u7684\u7cbe\u795e\u5fc3\u7406\u53ca\u793e\u4f1a\u8ba4\u77e5\u7684\u53d1\u5c55<sup>\uff3b12\uff3d<\/sup>\u3002\u4f46\u6709\u7814\u7a76\u663e\u793a\uff0c\u90e8\u5206SMA 1~3\u578b\u60a3\u513f\u7684\u8ba4\u77e5\u529f\u80fd\u4e0e\u6b63\u5e38\u513f\u7ae5\u5e76\u65e0\u663e\u8457\u5dee\u5f02\uff0c\u5176\u539f\u56e0\u53ef\u80fd\u5728\u4e8eSMA\u60a3\u8005\u901a\u8fc7\u57f9\u517b\u4ee5\u73af\u5883\u4e3a\u4e2d\u4ecb\u7684\u8ba4\u77e5\u6280\u80fd\uff0c\u5f25\u8865\u4e86\u8eab\u4f53\u7f3a\u9677\u9020\u6210\u7684\u9650\u5236\u3002\u4e5f\u6709\u7814\u7a76\u663e\u793a\uff0cSMA 1\u578b\u60a3\u513f\u6ce8\u610f\u529b\u548c\u6267\u884c\u529f\u80fd\u53d7\u635f\u98ce\u9669\u53ef\u80fd\u66f4\u5927\u3002\u56e0\u6b64\uff0c\u5bf9\u60a3\u513f\u8fdb\u884c\u7efc\u5408\u6027\u795e\u7ecf\u5fc3\u7406\u76d1\u6d4b\u662f\u5341\u5206\u5fc5\u8981\u7684\uff0c\u76d1\u6d4b\u5185\u5bb9\u53ef\u5305\u62ec\uff1a\u6807\u51c6\u5316\u795e\u7ecf\u5fc3\u7406\u5b66\u6d4b\u8bd5\uff0c\u4ee5\u53ca\u9488\u5bf9\u60a3\u513f\u7684\u8ba4\u77e5\u53d1\u5c55\u3001\u5b66\u4e60\u6280\u80fd\u3001\u793e\u4f1a\u529f\u80fd\u3001\u60c5\u7eea\u8c03\u6574\u548c\u884c\u4e3a\u8c03\u8282\u80fd\u529b\u8fdb\u884c\u8bbf\u8c08\u8bc4\u4f30\u3002\u57fa\u4e8e\u4ee5\u4e0a\u8bc4\u4f30\u7ed3\u679c\uff0c\u5e76\u7ed3\u5408\u60a3\u513f\u7684\u7efc\u5408\u533b\u5b66\u60c5\u51b5\uff0c\u53ca\u65f6\u4e3a\u60a3\u513f\u5236\u8ba2\u9002\u5408\u60a3\u513f\u9700\u6c42\u548c\u76ee\u6807\u7684\u5bb6\u5ead\u6216\u5b66\u6821\u6559\u80b2\u8ba1\u5212\uff0c\u63d0\u4f9b\u53ef\u9002\u5e94\u7684\u6559\u80b2\u73af\u5883\uff0c\u5305\u62ec\u63d0\u4f9b\u5fc5\u8981\u7684\u8bbe\u5907\uff0c\u914d\u5907\u76f8\u5e94\u7684\u62a4\u7406\u4eba\u5458\u548c\u6280\u672f\uff0c\u4fdd\u8bc1\u4ea4\u901a\u4fbf\u5229\u6027\u7b49\u3002\u5b9a\u671f\u7531\u4e34\u5e8a\u533b\u751f\u3001\u6821\u533b\u52a1\u4eba\u5458\u548c\u60a3\u8005\u5bb6\u5c5e\u5171\u540c\u7ef4\u62a4\u66f4\u65b0\uff0c\u4ee5\u4fdd\u8bc1\u60a3\u513f\u66f4\u597d\u5730\u5411\u6210\u5e74\u671f\u8fc7\u5ea6\u3002<\/p>\n<p>\uff082\uff09\u8212\u7f13\u6cbb\u7597\u670d\u52a1\u3002\u5c06\u8212\u7f13\u6cbb\u7597\u670d\u52a1\u7eb3\u5165SMA\u60a3\u513f\u65e5\u5e38\u62a4\u7406<sup>\uff3b12-13\uff3d<\/sup>\u3002\u5728\u533b\u7597\u62a4\u7406\u8fc7\u7a0b\u4e2d\uff0c\u5e94\u8003\u8651\u5e76\u5c0a\u91cd\u60a3\u513f\u7684\u60c5\u7eea\u611f\u53d7\u3002\u4f8b\u5982\uff0c\u8170\u690e\u7a7f\u523a\u524d\u3001\u4e2d\u3001\u540e\u5f80\u5f80\u4f1a\u51fa\u73b0\u5e94\u6fc0\u53cd\u5e94\u3001\u7126\u8651\u53cd\u5e94\u6216\u6050\u60e7\uff0c\u53ef\u5b9e\u65bd\u7279\u5b9a\u7684\u652f\u6301\u6027\u5fc3\u7406\u5e72\u9884\uff0c\u5305\u62ec\u901a\u8fc7\u8046\u542c\u5b69\u5b50\u559c\u7231\u7684\u7ae5\u8bdd\u6545\u4e8b\u6216\u5361\u901a\u4e3b\u9898\u97f3\u4e50\uff0c\u8fdb\u884c\u60c5\u5883\u6e38\u620f\u548c\u6709\u8da3\u7684\u8c1c\u8bed\u6d4b\u9a8c\u7b49\uff0c\u5206\u6563\u6ce8\u610f\u529b\uff0c\u53ef\u6709\u6548\u51cf\u8f7b\u513f\u7ae5\u53ca\u7236\u6bcd\u7684\u7126\u8651\uff0c\u4e5f\u53ef\u91c7\u7528\u6b63\u5ff5\u6cbb\u7597\uff0c\u5e2e\u52a9\u513f\u7ae5\u7f13\u89e3\u7126\u8651\uff0c\u51cf\u8f7b\u75bc\u75db\u3002<\/p>\n<p>\uff083\uff09\u4e34\u7ec8\u5173\u6000\u3002\u4e3a\u5904\u4e8e\u4e34\u5e8a\u7ec8\u672b\u671f\u7684SMA\u60a3\u513f\u63d0\u4f9b\u4e34\u7ec8\u5173\u6000\u7684\u57fa\u7840\u5728\u4e8e\u60a3\u513f\u7684\u75c7\u72b6\u7ba1\u7406\u548c\u5bf9\u60a3\u8005\u53ca\u5176\u5bb6\u5ead\u7684\u5fc3\u7406\u652f\u6301<sup>\uff3b35\uff3d<\/sup>\u3002\u901a\u5e38\uff0c\u547c\u5438\u56f0\u96be\u53ca\u75bc\u75db\u7ba1\u7406\u3001\u8425\u517b\u548c\u4f53\u4f4d\u62a4\u7406\u662f\u60a3\u513f\u4e34\u7ec8\u65f6\u4e3b\u8981\u4e34\u5e8a\u9700\u6c42\uff0c\u5e94\u5f53\u79ef\u6781\u52a0\u5f3a\u5e72\u9884\uff0c\u7f13\u89e3\u60a3\u513f\u75db\u82e6\uff0c\u5fc5\u8981\u65f6\u53ef\u63d0\u9ad8\u836f\u7269\u5242\u91cf\u53ca\u7ed9\u836f\u9891\u7387\u3002\u5982\u5bf9\u75c7\u6cbb\u7597\u5728\u51cf\u8f7b\u75db\u82e6\u65b9\u9762\u65e0\u6548\uff0c\u5219\u53ef\u8003\u8651\u6df1\u5ea6\u8212\u7f13\u9547\u9759\u3002\u6b64\u65f6\u4e3a\u60a3\u513f\u5bb6\u5c5e\u63d0\u4f9b\u5fc3\u7406\u652f\u6301\u81f3\u5173\u91cd\u8981\u3002\u5728\u5fc3\u7406\u65b9\u9762\uff0c\u53ef\u4f7f\u7528\u9057\u613f\u6e05\u5355\u7b49\u6280\u672f\uff0c\u548c\u60a3\u8005\u4ea4\u6d41\u4e34\u7ec8\u524d\u7684\u613f\u671b\uff0c\u4f8b\u5982\uff0c\u7a7f\u4ec0\u4e48\u8863\u670d\u3001\u662f\u5426\u6709\u5916\u51fa\u65c5\u884c\u7684\u613f\u671b\u53ca\u7275\u6302\u7684\u4e8b\u60c5\u548c\u5fc3\u613f\u7b49\u3002\u6b64\u5916\uff0c\u5e94\u4e0e\u60a3\u8005\u63d0\u524d\u4ea4\u6d41\u5bf9\u6709\u521b\u6cbb\u7597\u548c\u62a2\u6551\u7684\u6001\u5ea6\u3002\u8003\u8651\u5230\u60a3\u513f\u5982\u679c\u5728\u5bb6\u4e2d\u6b7b\u4ea1\uff0c\u5bf9\u5bb6\u5ead\u6210\u5458\u7684\u5fc3\u7406\u5f71\u54cd\u53ef\u80fd\u66f4\u52a0\u6301\u4e45\uff0c\u4e14\u6ca1\u6709\u533b\u62a4\u4eba\u5458\u652f\u6301\uff0c\u60a3\u513f\u7236\u6bcd\u5728\u60c5\u611f\u4e0a\u66f4\u96be\u627f\u53d7\uff0c\u53ef\u4e0e\u60a3\u513f\u53ca\u5176\u5bb6\u5c5e\u4ea4\u6d41\uff0c\u63d0\u524d\u9009\u62e9\u60a3\u513f\u6b7b\u4ea1\u7684\u5730\u70b9\u3002<\/p>\n<p>\u6b64\u5916\uff0c\u5e94\u4e3a\u5bb6\u5c5e\u63d0\u4f9b\u5c45\u5bb6\u62a4\u7406\u65b9\u9762\u7684\u76f8\u5173\u8d44\u6e90\u548c\u8bad\u7ec3\uff0c\u5982\u5438\u75f0\u3001\u4f53\u4f4d\u62a4\u7406\u7b49\u57f9\u8bad\u3002<\/p>\n<p>6.3.2.2&nbsp;&nbsp;SMA\u6210\u4eba\u60a3\u8005&nbsp; SMA\u6210\u4eba\u60a3\u8005\u7684\u5fc3\u7406\u5065\u5eb7\u6c34\u5e73\u53ef\u901a\u8fc7\u589e\u52a0\u60a3\u8005\u7684\u793e\u4f1a\u53c2\u4e0e\u53ca\u5bf9\u57fa\u672c\u5fc3\u7406\u9700\u6c42\u7684\u6ee1\u8db3\u6765\u63d0\u5347\u3002\u5176\u4e2d\uff0c\u57fa\u672c\u5fc3\u7406\u9700\u6c42\u5305\u62ec\u81ea\u4e3b\u6027\uff08\u5982\u6709\u9650\u7684\u72ec\u7acb\u6027\uff0c\u53ef\u8fc7\u81ea\u4e3b\u7684\u751f\u6d3b\uff09\u3001\u793e\u4f1a\u76f8\u5173\u6027\uff08\u5982\u793e\u4f1a\u53d1\u5c55\u3001\u803b\u8fb1\u611f\uff09\u3001\u80fd\u529b\uff08\u5982\u81ea\u6211\u63a7\u5236\u3001\u81ea\u4fe1\u3001\u6709\u793e\u4f1a\u4ef7\u503c\uff09\u7b49<sup>\uff3b36\uff3d<\/sup>\u3002\u5728\u5c1d\u8bd5\u5e2e\u52a9\u60a3\u6709\u6162\u6027\u3001\u5a01\u80c1\u751f\u547d\u7684\u8eaf\u4f53\u75be\u75c5\u60a3\u8005\u65f6\uff0c\u5e94\u91c7\u53d6\u4ee5\u652f\u6301\u6027\u7684\u3001\u8d44\u6e90\u53d6\u5411\u5fc3\u7406\u5e72\u9884\u4e3a\u4e3b\u7684\u6cbb\u7597\u65b9\u6cd5\uff0c\u5e2e\u52a9\u60a3\u8005\u53d1\u73b0\u8d44\u6e90\uff0c\u89e3\u51b3\u95ee\u9898\uff0c\u83b7\u53d6\u652f\u6301\uff0c\u6709\u6548\u6ee1\u8db3\u81ea\u8eab\u9700\u6c42\uff0c\u5982\u8d44\u6e90\u6e05\u5355\u6280\u672f\u3002\u6761\u4ef6\u5141\u8bb8\u7684\u60c5\u51b5\u4e0b\uff0c\u53ef\u5728\u7cbe\u795e\u5fc3\u7406\u4e13\u4e1a\u4eba\u5458\u7684\u5e26\u9886\u4e0b\uff0c\u5f00\u5c55\u60a3\u8005\u6216\u60a3\u8005\u5bb6\u5c5e\u7fa4\u4f53\u95f4\u7684\u76f8\u4e92\u652f\u6301\uff0c\u5982\u4e92\u52a9\u4f1a\u3001\u56e2\u4f53\u6cbb\u7597\u7b49\u3002<\/p>\n<p>6.3.2.3&nbsp;&nbsp;SMA\u60a3\u8005\u5bb6\u5c5e\u53ca\u7167\u62a4\u8005&nbsp; \u5728\u513f\u7ae5\u60a3\u8005\u4e2d\uff0c\u5e94\u5173\u6ce8\u60a3\u75c5\u513f\u7ae5\u7684\u5144\u5f1f\u59d0\u59b9\u7fa4\u4f53<sup>\uff3b37\uff3d<\/sup>\u3002\u7814\u7a76\u663e\u793a\uff0c\u8be5\u7fa4\u4f53\u51fa\u73b0\u884c\u4e3a\u95ee\u9898\u3001\u4f4e\u81ea\u5c0a\u3001\u793e\u4f1a\u5b64\u7acb\u548c\u51fa\u73b0\u6291\u90c1\u3001\u7126\u8651\u7b49\u5fc3\u7406\u969c\u788d\u7684\u98ce\u9669\u589e\u52a0\uff0c\u539f\u56e0\u53ef\u80fd\u5305\u62ec\u5bf9\u7236\u6bcd\u7167\u987e\u60a3\u513f\u7684\u4e0d\u6ee1\u3001\u8fc7\u591a\u7684\u5bb6\u5ead\u8d23\u4efb\u7b49\u3002\u533b\u751f\u5e94\u5728\u5bb6\u5ead\u80cc\u666f\u4e0b\u7406\u89e3\u75be\u75c5\u5bf9 SMA \u60a3\u513f\u53ca\u5176\u5144\u5f1f\u59d0\u59b9\u7684\u4e0d\u540c\u5f71\u54cd\u3002SMA\u662f\u4e00\u79cd\u9010\u6e10\u81f4\u6b8b\u7684\u75be\u75c5\uff0c\u9700\u8981\u6240\u6709\u5bb6\u5ead\u6210\u5458\u8fdb\u884c\u5371\u673a\u7ba1\u7406\u548c\u5fc3\u7406\u5e72\u9884\uff0c\u5e2e\u52a9\u5bb6\u5ead\u6210\u5458\u5e94\u5bf9\u5bb6\u4eba\u4e27\u5931\u529f\u80fd\u7684\u60b2\u75db\u53ca\u53ef\u80fd\u65e9\u901d\u7684\u9884\u671f\u3002\u975e\u7cbe\u795e\u5fc3\u7406\u79d1\u533b\u751f\u53ef\u4ee5\u4f7f\u7528\u5404\u7c7b\u5fc3\u7406\u8bc4\u4f30\u5de5\u5177\uff0c\u5982PHQ-9\u3001GAD-7\u7b49\u60c5\u7eea\u7b5b\u67e5\u5de5\u5177\u3001\u4e8b\u4ef6\u5f71\u54cd\u91cf\u8868\u7b49\u8bc4\u4ef7\u4eb2\u4eba\u60a3\u75c5\u5bf9\u5bb6\u5c5e\u5fc3\u7406\u7684\u5f71\u54cd\uff0c\u4ee5\u53ca\u81ea\u5c0a\u548c\u793e\u4f1a\u5b64\u7acb\u95ee\u9898\u7684\u76f8\u5173\u7b5b\u67e5\u5de5\u5177\uff0c\u8bc4\u4ef7\u76f8\u5173\u5fc3\u7406\u6307\u6807<sup>\uff3b35\uff3d<\/sup>\u3002<\/p>\n<p>SMA\u60a3\u8005\u7167\u987e\u8005\u7684\u5fc3\u7406\u56f0\u6270\u548c\u8d1f\u62c5\u611f\u4e0e\u60a3\u8005\u7684\u75be\u75c5\u7279\u5f81\uff08\u5373\u8eab\u4f53\u548c\u884c\u4e3a\u95ee\u9898\uff09\u6709\u5173\uff0c\u4e5f\u4e0e\u7167\u62a4\u8005\u81ea\u8eab\u7279\u5f81\u6709\u5173\uff0c\u5982\u5176\u5e94\u5bf9\u65b9\u5f0f\u3001\u662f\u5426\u53d1\u73b0\u7167\u62a4\u4e2d\u7684\u79ef\u6781\u610f\u4e49\u7b49\u3002\u968f\u7740\u75be\u75c5\u8fdb\u5c55\uff0cSMA\u60a3\u8005\u5bf9\u7167\u62a4\u8005\u7684\u5fc3\u7406\u548c\u751f\u7406\u9700\u6c42\u589e\u52a0\uff0c\u7167\u62a4\u8005\u5fc5\u987b\u627f\u62c5\u8d23\u4efb\uff0c\u63a5\u53d7\u4eb2\u4eba\u7684\u75be\u75c5\u548c\u5373\u5c06\u79bb\u4e16\u7684\u73b0\u5b9e\u3002\u6b64\u5916\uff0c\u8fd8\u53ef\u80fd\u9762\u4e34\u7f3a\u4e4f\u76f8\u5173\u77e5\u8bc6\u548c\u6280\u80fd\u7684\u95ee\u9898\uff0c\u5982\u6c9f\u901a\u75be\u75c5\u548c\u6b7b\u4ea1\u3001\u5904\u7406\u60a3\u8005\u884c\u4e3a\u53d8\u5316\u3001\u5904\u7406\u81ea\u5df1\u60c5\u7eea\u6216\u8868\u8fbe\u81ea\u5df1\u7684\u754c\u9650\u7b49\u3002<\/p>\n<p>\u5f53\u5bf9\u7167\u62a4\u8005\u8fdb\u884c\u5fc3\u7406\u5e72\u9884\u65f6\uff0c\u53ef\u4ee5\u91c7\u7528\u4e00\u822c\u6027\u5fc3\u7406\u652f\u6301\u3001\u8d44\u6e90\u53d6\u5411\u5fc3\u7406\u5e72\u9884\uff0c\u4ee5\u53ca\u63a5\u7eb3\u548c\u627f\u8bfa\u8ba4\u77e5\u884c\u4e3a\u7597\u6cd5\u7b49\uff0c\u9f13\u52b1\u7167\u62a4\u8005\u660e\u786e\u751f\u6d3b\u4e2d\u7684\u91cd\u8981\u4ef7\u503c\u548c\u610f\u4e49\uff0c\u4ee5\u5e2e\u52a9\u5176\u66f4\u597d\u5730\u9002\u5e94\u73b0\u5b9e\u60c5\u51b5\u3001\u7ee7\u7eed\u751f\u6d3b\uff0c\u5e76\u63d0\u5347\u5fc3\u7406\u5065\u5eb7\u6c34\u5e73\u3002<\/p>\n<p>\u63a8\u8350\u610f\u89c1\uff1a\u9488\u5bf9\u4e0d\u540c\u5e74\u9f84\u3001\u4e0d\u540c\u75c5\u60c5\u9636\u6bb5\u7684\u60a3\u8005\u53ca\u5176\u5bb6\u5ead\u6210\u5458\u4e0d\u540c\u7684\u5fc3\u7406\u9700\u6c42\uff0c\u7ed9\u4e88\u652f\u6301\uff0c\u6709\u5229\u4e8e\u63d0\u9ad8SMA\u5bb6\u5ead\u7684\u6574\u4f53\u751f\u6d3b\u8d28\u91cf\uff08\u2161\u7ea7\u63a8\u8350\uff0cD\u7ea7\u8bc1\u636e\uff09\u3002<\/p>\n<p>\uff08\u672c\u90e8\u5206\u6267\u7b14\u4eba\uff1a\u59dc\u5fc6\u5357&nbsp; 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study\uff3bJ\uff3d.Neuroimage Clin\uff0c2019\uff0c21\uff1a101618.<\/p>\n<p>\uff3b32\uff3dde Borba FC\uff0cQuerin G\uff0cFran\ue578a MC Jr\uff0cet al.Cerebellar degeneration in adult spinal muscular atrophy patients\uff3bJ\uff3d.J Neurol\uff0c2020\uff0c267\uff089\uff09\uff1a2625-2631.<\/p>\n<p>\uff3b33\uff3dStam M\uff0cHaakma W\uff0cKuster L\uff0cet al.Magnetic resonance imaging of the cervical spinal cord in spinal muscular atrophy\uff3bJ\uff3d.Neuroimage Clin\uff0c2019\uff0c24\uff1a102002.<\/p>\n<p>\uff3b34\uff3dLaufersweiler-Plass C\uff0cRudnik-Schneborn S\uff0cZerres K\uff0cet al.Behavioural problems in children and adolescents with spinal muscular atrophy and their siblings\uff3bJ\uff3d.Dev Med Child Neurol\uff0c2003\uff0c45\uff081\uff09\uff1a44-49.<\/p>\n<p>\uff3b35\uff3dStefania LF\uff0cCristina F\uff0cMaria S\uff0cet al.Intrathecal administration of Nusinersen in type 1 SMA\uff1asuccessful psychological program in a single Italian center\uff3bJ\uff3d.Neurol Sci\uff0c2018\uff0c39\uff0811\uff09\uff1a1961-1964.<\/p>\n<p>\uff3b36\uff3dRopars J\uff0cPeudenier S\uff0cGenot A\uff0cet al.Multidisciplinary approach and psychosocial management of spinal muscular atrophy\uff08SMA\uff09\uff3bJ\uff3d.Arch de Pediatr\uff0c2020\uff0c27\uff087S\uff09\uff1a7S45-7S49.<\/p>\n<p>\uff3b37\uff3dFischer MJ\uff0cAsselman FL\uff0cKruitwagen-Van Reenen ET\uff0cet al.Psychological well-being in adults with spinal muscular atrophy\uff1athe contribution of participation and psychological needs\uff3bJ\uff3d.Disabil Rehabil\uff0c2020\uff0c42\uff0816\uff09\uff1a2262-2270.<\/p>\n<p>\u7f16\u8f91 |&nbsp;\u90dd\u607a\u96ef&nbsp; \u8463\u96ea<\/p>\n<p>\u76d1\u5236 | \u674e\u6797\u5eb7<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u9752\u5c11\u5e74\u6210\u4eba\u810a\u9ad3\u6027\u808c\u840e\u7f29\u75c7\u4e34\u5e8a\u8bca\u7597\u6307\u5357 \u4e2d\u56fd\u7814\u7a76\u578b\u533b\u9662\u5b66\u4f1a\u7f55\u89c1\u75c5\u5206\u4f1a\uff0c\u4e2d\u56fd\u7f55\u89c1\u75c5\u8054 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