{"id":38407,"date":"2018-09-01T01:00:00","date_gmt":"2018-09-17T06:20:31","guid":{"rendered":"http:\/\/www.05921.net\/index.php\/2018\/09\/17\/%e6%9d%8e%e6%96%8c%e6%95%99%e6%8e%88%e5%9b%a2%e9%98%9f%e7%9c%bc%e7%a7%91%e5%9f%ba%e5%9b%a0%e6%b2%bb%e7%96%97-lhon-%e5%ae%89%e5%85%a8%e6%80%a7%e8%8e%b7%e8%ae%a4%e5%8f%af%ef%bc%8clhon-%e6%82%a3%e8%80%85\/"},"modified":"2018-09-17T14:20:32","modified_gmt":"2018-09-17T06:20:32","slug":"%e6%9d%8e%e6%96%8c%e6%95%99%e6%8e%88%e5%9b%a2%e9%98%9f%e7%9c%bc%e7%a7%91%e5%9f%ba%e5%9b%a0%e6%b2%bb%e7%96%97-lhon-%e5%ae%89%e5%85%a8%e6%80%a7%e8%8e%b7%e8%ae%a4%e5%8f%af%ef%bc%8clhon-%e6%82%a3%e8%80%85","status":"publish","type":"post","link":"https:\/\/www.05921.net\/index.php\/38407\/","title":{"rendered":"\u674e\u658c\u6559\u6388\u56e2\u961f\u773c\u79d1\u57fa\u56e0\u6cbb\u7597 LHON \u5b89\u5168\u6027\u83b7\u8ba4\u53ef\uff0cLHON \u60a3\u8005\u770b\u5230\u66d9\u5149"},"content":{"rendered":"
\n

\u8d85\u8d8a\u6b27\u7f8e\uff0c\u674e\u658c\u6559\u6388\u56e2\u961f\u57fa\u56e0\u6cbb\u7597 LHON \u5b89\u5168\u6027\u83b7\u8ba4\u53ef\uff0cLHON \u60a3\u8005\u770b\u5230\u66d9\u5149<\/span>
<\/p>\n


<\/p>\n

Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u662f\u4e00\u79cd\u89c6\u795e\u7ecf\u9000\u884c\u6027\u53d8\u7684\u6bcd\u7cfb\u9057\u4f20\u6027\u75be
<\/p>\n

\u75c5\uff0c\u7531\u5fb7\u56fd\u5b66\u8005 Leber \u4e8e 1871 \u5e74\u9996\u5148\u53d1\u73b0\u5e76\u516c\u5e03\u3002\u8be5\u75c5\u662f\u4e00\u79cd\u6700\u5e38\u89c1\u7684\u7ebf\u7c92\u4f53\u76f8\u5173\u75be\u75c5\uff0c\u7edd\u5927\u591a\u6570\u7684 LHON \u60a3\u8005\u90fd\u662f G11778A \u57fa\u56e0\u4f4d\u70b9\u7a81\u53d8\u5f15\u8d77\u7684\u3002\u8be5\u75c5\u8f83\u4e3a\u7f55\u89c1\uff0c\u5e38\u5728 15-35 \u5c81\u671f\u95f4\u53d1\u75c5\uff0c\u60a3\u8005\u591a\u89c6\u529b\u6025\u5267\u4e0b\u964d\u76f4\u81f3\u53cc\u773c\u5931\u660e\uff0c\u81f3\u4eca\u5728\u5168\u7403\u8303\u56f4\u5185\u5c1a\u65e0\u6709\u6548\u7684\u6cbb\u7597\u65b9\u6cd5\u3002<\/p>\n


<\/p>\n

\u65e9\u5728\u56db\u5341\u51e0\u5e74\u524d\uff0c\u57fa\u56e0\u7597\u6cd5\u754c\u7684\u9886\u519b\u4eba\u7269 Theodore Friedmann \u535a\u58eb\u66fe\u8bbe\u60f3\u8fc7\u901a\u8fc7\u57fa\u56e0\u6cbb\u7597\u6765\u6539\u5584\u7531\u5355\u4e2a\u57fa\u56e0\u5f15\u8d77\u7684\u9057\u4f20\u6027\u75be\u75c5\uff0c\u5373\u7528\u5177\u6709\u6b63\u5e38\u529f\u80fd\u7684\u57fa\u56e0\u7f6e\u6362\u6216\u589e\u8865\u60a3\u8005\u4f53\u5185\u6709\u7f3a\u9677\u7684\u57fa\u56e0\uff0c\u56e0\u800c\u8fbe\u5230\u6cbb\u7597\u75be\u75c5\u7684\u76ee\u7684\u30022007 \u5e74\uff0c\u7f8e\u56fd spark \u516c\u53f8\u6210\u529f\u5730\u5f00\u5c55\u4e86 Leber \u5148\u5929\u9ed1\u77c7\u75c7\uff08LCA\uff09\u7684\u57fa\u56e0\u6cbb\u7597\u4e34\u5e8a\u8bd5\u9a8c\uff0c\u5e76\u53d6\u5f97\u4e86\u521d\u6b65\u6210\u6548\uff0c\u5f00\u542f\u4e86\u773c\u90e8\u75be\u75c5\u57fa\u56e0\u6cbb\u7597\u7684\u5148\u6cb3\u3002<\/p>\n


<\/p>\n


<\/p>\n

\"\u674e\u658c\u6559\u6388\u56e2\u961f\u773c\u79d1\u57fa\u56e0\u6cbb\u7597<\/p>\n


<\/p>\n

\u81ea 2008 \u5e74\u8d77\uff0c\u674e\u658c\u6559\u6388\u56e2\u961f\u5f00\u59cb\u7740\u624b Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09 \u7684\u57fa\u56e0\u6cbb\u7597\u7814\u7a76\uff0c\u5728\u505a\u4e86\u5927\u91cf\u7684\u57fa\u7840\u7814\u7a76\u548c\u4e34\u5e8a\u8bd5\u9a8c\u540e\uff0c\u53d1\u660e\u4e86\u4e00\u79cd\u57fa\u56e0\u7597\u6cd5\u6cbb\u7597 Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u30022011 \u5e74\u674e\u658c\u6559\u6388\u56e2\u961f\u5b8c\u6210\u5168\u7403\u9996\u4f8bLeber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u60a3\u8005\u7684\u57fa\u56e0\u6cbb\u7597\u4e34\u5e8a\u8bd5\u9a8c\uff0c\u5e76\u5728\u672f\u540e 7 \u5e74\u5185\u5bf9\u53c2\u4e0e\u4e34\u5e8a\u7814\u7a76\u7684 9 \u4f8b LHON \u57fa\u56e0\u6cbb\u7597\u60a3\u8005\u8fdb\u884c\u4e34\u5e8a\u89c2\u5bdf\uff0c\u53d1\u73b0 6 \u4f8b Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u60a3\u8005\u89c6\u529b\u663e\u8457\u63d0\u9ad8\uff0c3 \u4f8bLeber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u60a3\u8005\u89c6\u91ce\u5f97\u5230\u6539\u5584\u3002\u5e76\u4e14\u6ce8\u5c04 AAV-2 \u836f\u7269\u540e\u60a3\u8005\u7684\u89c6\u7f51\u819c\u5916\u89c2\u548c\u5f62\u6001\u6ca1\u6709\u5f02\u5e38\uff0c\u5145\u5206\u8bc1\u660e\u4e86\u8be5\u7597\u6cd5\u5b89\u5168\u6027\u597d\u5e76\u4e14\u6709\u6548\u6027\u663e\u8457\u3002<\/p>\n


<\/p>\n

2017 \u5e74 3 \u6708\u5f00\u59cb\uff0c\u674e\u6559\u6388\u56e2\u961f\u987a\u5229\u542f\u52a8\u201c\u5168\u7403\u6837\u672c\u91cf\u6700\u5927\u7684\u57fa\u56e0\u6cbb\u7597 Leber\u2019<\/p>\n

s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\u4e34\u5e8a\u8bd5\u9a8c\u201d\uff0c\u622a\u6b62 2018 \u5e74 6 \u6708 5 \u65e5\u5171\u6cbb\u7597 143 \u4f8b\u60a3\u8005\uff0c\u5176\u4e2d\u5305\u542b 3 \u540d\u4e2d\u56fd\u53f0\u6e7e\u540c\u80de\u548c 7 \u540d\u963f\u6839\u5ef7\u56fd\u9645\u60a3\u8005\u3002<\/p>\n

             <\/p>\n

\"\u674e\u658c\u6559\u6388\u56e2\u961f\u773c\u79d1\u57fa\u56e0\u6cbb\u7597<\/p>\n

\u674e\u658c\u6559\u6388\u7814\u7a76\u56e2\u961f\u628a\u8fd9\u6279\u5165\u7ec4\u7684 Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\u60a3\u8005\u6309\u53d1\u75c5\u65f6\u95f4\u5206\u4e3a 2 \u5e74\u4ee5\u5185\u548c 2 \u5e74\u4ee5\u4e0a\u7b49\uff0c\u5e76\u5206\u522b\u5728\u672f\u540e\u524d\u4e09\u5929\uff0c\u4e00\u4e2a\u6708\u548c\u4e09\u4e2a\u6708\u8fdb\u884c\u5b89\u5168\u6027\u548c\u6709\u6548\u6027\u68c0\u6d4b\u3002\u6cd5\u56fd Gensight \u516c\u53f8\u548c\u7f8e\u56fd Guy \u6559\u6388\u56e2\u961f\u4e5f\u8fdb\u884c\u4e86 Leber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u57fa\u56e0\u6cbb\u7597\u4e34\u5e8a\u8bd5\u9a8c\u3002\u6cd5\u56fd Gensight \u516c\u53f8\u4e34\u5e8a\u6cbb\u7597 15 \u4f8bLeber\u2019s \u9057\u4f20\u6027\u89c6\u795e\u7ecf\u75c5\u53d8\uff08LHON\uff09\u60a3\u8005\uff0c\u60a3\u8005\u6839\u636e\u53d1\u75c5\u65f6\u95f4\u88ab\u5206\u4e3a 6 \u4e2a\u6708\u4ee5\u5185\u7ec4\u548c 6 \u4e2a\u6708\u5230 12 \u4e2a\u6708\u7ec4\u8fdb\u884c\u5b89\u5168\u6027\u68c0\u6d4b\u3002\u7f8e\u56fd Guy \u6559\u6388\u56e2\u961f\u4e34\u5e8a\u6cbb\u7597 15 \u4f8b LHON \u60a3\u8005\uff0c\u60a3\u8005\u6839\u636e\u53d1\u75c5\u65f6\u95f4\u88ab\u5206\u4e3a\u5c0f\u4e8e 12 \u4e2a\u6708\u548c\u5927\u4e8e 12 \u4e2a\u6708\u7ec4\u8fdb\u884c\u5b89\u5168\u6027\u68c0\u6d4b\u3002<\/p>\n


<\/p>\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n\n

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\u672f\u540e\u524d\u4e09\u5929<\/p>\n<\/td>\n

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\u672f\u540e\u7b2c\u4e00\u6708<\/p>\n<\/td>\n

\n

\u672f\u540e\u7b2c\u4e09\u6708<\/p>\n<\/td>\n

\n

\u6cd5\u56fd<\/p>\n<\/td>\n

\n

\u7f8e\u56fd<\/p>\n<\/td>\n<\/tr>\n

\n

Gensight<\/p>\n<\/td>\n

\n

Guy<\/p>\n<\/td>\n<\/tr>\n

\n

\u75c5\u4f8b\u6570<\/p>\n<\/td>\n

\n

\uff08n=141\uff09<\/p>\n<\/td>\n

\n

\uff08n=102\uff09<\/p>\n<\/td>\n

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\uff08n=41\uff09<\/p>\n<\/td>\n

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\uff08n=15\uff09<\/p>\n<\/td>\n

\n

\uff08n=14\uff09<\/p>\n<\/td>\n<\/tr>\n

\n

\u767d\u5185\u969c\u6458\u9664<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

\n

13.33%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

\n

\u8461\u8404\u819c\u708e<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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14.29%<\/p>\n<\/td>\n<\/tr>\n

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\u73bb\u7483\u4f53\u708e<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

\n

86.67%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

\n

\u89d2\u819c\u708e<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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46.67%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

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\u524d\u623f\u708e\u75c7<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

\n

93.33%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

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\u73bb\u7483\u4f53\u79ef\u8840<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

\n

13.33%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

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\u773c\u75db*<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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13.33%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

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\u8fc7\u654f\u6027\u7ed3\u819c\u708e*<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n

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20%<\/p>\n<\/td>\n

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0<\/p>\n<\/td>\n<\/tr>\n

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\u9ad8\u773c\u538b*<\/p>\n<\/td>\n

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14.89%<\/p>\n<\/td>\n

\n

16.67%<\/p>\n<\/td>\n

\n

12.19%<\/p>\n<\/td>\n

\n

66.67%<\/p>\n<\/td>\n

\n

7.14%<\/p>\n<\/td>\n<\/tr>\n

\n

\u7ed3\u819c\u4e0b\u51fa\u8840*<\/p>\n<\/td>\n

\n

20.56%<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n

\n

20%<\/p>\n<\/td>\n

\n

0<\/p>\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n

\u5b89\u5168\u7ed3\u679c\u5206\u6790\u5982\u4e0b\uff1a<\/p>\n

 <\/p>\n

*\u5c5e\u4e8e\u8f7b\u5ea6\u5e76\u53d1\u75c7\uff08\u53ef\u81ea\u884c\u6062\u590d\uff09\uff0c\u5176\u4f59\u90fd\u5c5e\u4e8e\u91cd\u5ea6\u5e76\u53d1\u75c7\uff0c\u9700\u8981\u8fdb\u884c\u5e72\u9884\u6cbb<\/strong><\/span><\/p>\n

 <\/p>\n

\u5b89\u5168\u6027\u662f\u57fa\u56e0\u6cbb\u7597\u5e94\u7528\u4e8e\u4e34\u5e8a\u7684\u9996\u8981\u51b3\u5b9a\u6027\u56e0\u7d20\uff0c\u867d\u7136\u4e09\u4e2a\u56e2\u961f\u90fd\u5bf9 LHON \u60a3\u8005\u8fdb\u884c\u4e86\u57fa\u56e0\u6cbb\u7597\uff0c\u4f46\u4ece\u4ee5\u4e0a\u6837\u672c\u6570\u91cf\u53ca\u5b89\u5168\u6027\u7ed3\u679c\u6bd4\u8f83\u7684\u6570\u636e\u53ef\u4ee5\u770b\u51fa\uff0c\u674e\u658c\u6559\u6388\u56e2\u961f\u7684\u57fa\u56e0\u6cbb\u7597\u5b89\u5168\u6027\u663e\u8457\u9ad8\u4e8e\u6cd5\u56fd Gensight \u516c\u53f8\u4e0e\u7f8e\u56fd Guy \u6559\u6388\u56e2\u961f\u3002<\/p>\n


<\/p>\n

2017 \u5e74\u5e95\uff0c\u9488\u5bf9 Leber \u5148\u5929\u6027\u9ed1\u6726 2 \u578b\u7684\u9996\u4e2a\u57fa\u56e0\u66ff\u4ee3\u7597\u6cd5\u836f\u7269 Luxturna \u83b7<\/p>\n

FDA \u6279\u51c6\u4e0a\u5e02!\u8fd9\u4e00\u836f\u7269\u7684\u6210\u529f\u83b7\u6279\uff0c\u4f7f\u539f\u672c\u65e0\u836f\u53ef\u6cbb\u7684\u773c\u9057\u4f20\u75c5\u60a3\u8005\u770b\u5230\u4e86\u66d9\u5149\uff0c \u4e5f\u8ba9\u7814\u7a76\u8005\u4eec\u51e0\u5341\u5e74\u7684\u52aa\u529b\u9996\u6b21\u5f97\u5230\u201c\u5151\u73b0\u201d\u3002\u4ee5\u4e0a\u674e\u658c\u6559\u6388\u7814\u53d1\u56e2\u961f\u8fd1\u5341\u5e74\u6765\u503e\u5c3d\u5fc3\u8840\u7684\u79d1\u7814\u6210\u679c\u8868\u660e\uff0cLHON \u60a3\u8005\u5b8c\u5168\u80fd\u591f\u88ab\u6cbb\u6108\uff0c\u5f85\u8be5\u836f\u7269\u5e7f\u6cdb\u5e94\u7528\u4e8e\u4e34\u5e8a\u4e4b\u65f6\uff0c\u4fbf\u662f\u5168\u7403\u5e7f\u5927 LHON \u60a3\u8005\u91cd\u89c1\u5149\u660e\u4e4b\u65e5\u3002<\/p>\n


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 \u6b66\u6c49\u7ebd\u798f\u65af\u751f\u7269\u79d1\u6280\u6709\u9650\u516c\u53f8\u5168\u7a0b\u53c2\u4e0e\u674e\u658c\u6559\u6388\u56e2\u961f\u57fa\u56e0\u6cbb\u7597LHON\u4e34\u5e8a\u8bd5\u9a8c\u7814\u7a76\u5de5\u4f5c\uff0c\u5168\u7a0b\u7ed9\u6765\u81ea\u56fd\u5185\uff0c\u4e2d\u56fd\u53f0\u6e7e\u3001\u56fd\u9645LHON\u60a3\u8005\u63d0\u4f9b\u652f\u6301\u548c\u5e2e\u52a9\u4ee5\u53ca\u6280\u672f\u670d\u52a1\uff0c\u613f\u66f4\u5feb\u66f4\u597d\u7684\u63a8\u52a8\u773c\u9057\u4f20\u75c5\u57fa\u56e0\u6cbb\u7597\u6280\u672f\u8fdb\u6b65\uff0c\u4e3a\u5e7f\u5927\u773c\u9057\u4f20\u75c5\u60a3\u8005\u5e26\u6765\u5149\u660e\u548c\u5e0c\u671b\u3002<\/p>\n

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