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)~; endstream endobj 29 0 obj <>stream hބUE*@))3o~ 'w ۻXl)APR𣡣AE$*DM {k=7۝}{oh奒Z%Y $O3i>k%M`wMfF#JoF'ͣ2iOe;lE0AI!M b,j0OxqLe>x$ua0LŨRgq:c.$ʰ*Iᭆ I tinՅ K`?z[ɝ%-^ڇkI7/bVs_wFڽI??bKHqIEO l)Zb"bO}<_9FźbVF'zr،8b6/;ydWoM@kgxP2E~:z.x|h:|AX:;ǽ޸Ltj\sJ̅q&TU };qBֻ?S'ߔljY庢JdKJYHi YSqW=h>GϖI8/:Ϳ*: KP,ԋEb>|1GTDjajBjM[Fg(ΖDgl {QqGEPpPe|'bYzrYT2;Pd+ǟuEQWaҦW3w D~(̺9$tf78f?[OOoܛ'^~_'tX!VW)ъ4x4a1ц~΂.;hՓSW_E2];ĩYދUjzTYipBR+n;_RƫلV?k}SZuc㷓 K LzvoԬ&4Q"|ۄN\$z'55mm L:91m4_ E endstream endobj 30 0 obj <>stream 2015-05-28T14:56:08+08:00 2015-05-28T14:56:05+08:00 2015-05-28T14:56:08+08:00 Acrobat PDFMaker 11 Word 版 uuid:6ab0bff1-4447-4193-a936-23c7d97386e6 uuid:859ec1fc-2d0c-41a4-835a-b34e53fca2cd 233 application/pdf 高危新生儿听力和聋病易感基因联合筛查临床研究 目的:探讨在高危新生儿听力筛查基础上,联合聋病易感基因筛查的临床意义。方法:随机选择920例新生儿科住院具有听力损失高危因素的新生儿,于生后3~5天采集微量足跟血进行基因GJB2、SLC26A4及线粒体12SrRNA的聚合酶链扩增反应,通过基质辅助激光解吸附/电离飞行时间质谱方法检测GJB2基因35delG、176-191del16、235delC、299-300delAT;SLC26A4基因IVS7-2A > G、2168A > G;线粒体12SrRNA基因1494C > T、1555A > G等3个基因8个突变位点;听力筛查初筛采用自动判别听性脑干电位(auto-auditory brainstem response AABR),复筛采用诊断型耳声反射(otoacoustic emission OAE) + AABR,仍不通过者于3月龄行听力学诊断;选取同期产后区938例健康新生儿为对照组。结果:高危新生儿组检出35例聋病基因携带,3个基因突变的总体携带率3.80%;检出听力障碍34例(3.70%)、其中重度以上听力障碍15例(1.63%);30例(85.7%)聋病基因携带者通过了听力筛查。对照组检出21例聋病基因携带,3个基因突变的总体携带率2.23%;检出听力障碍4例(0.43%)、重度以上听力障碍1例(0.11%);17例(68.0%)聋病基因携带者通过了听力筛查。两组聋病基因突变的总携带率、听力障碍及重度以上听力障碍检出率以及携带聋病基因者听力筛查通过率之间差异有统计学意义。结论:高危新生儿听力障碍的发生率和聋病基因突变携带率均高于正常新生儿应重点关注;采用听力和聋病易感基因联合筛查能及时发现常规通过听力筛查但具有耳聋高危因素和迟发性聋病遗传因素的新生儿,对早期干预、定期随访、减少聋病发生具有指导意义。 张章,戴怡蘅,李振安,余凤慈,刘莹 Adobe PDF Library 11.0 聋病,基因突变,高危,新生儿 D:20150528065552 çł®çı—ï¼ı攢讨在髟哱挰çfl�å—¿å’¬å−łç�łæ�¥å�ºç¡•ä¸−$è†fl咋膉犖æŸfiæ—�å�ºåł€ç�łæ�¥çı—临åº−æ—‘ä¹›ã•‡æŒ¹æ³Łï¼ıéı‘机镛扩920侉挰çfl�嗿秂佑鎢喷望咬å−łæ“�å¤±é«Ÿå“±åł€ç´€çı—挰çfl�嗿$五çfl�å’”3~5天é⁄⁄éłƒå¾®é⁄‘è¶³è·�è¡•è¿łè¡„å�ºåł€GJB2㕆SLC26A4å‘−线粙ä½fi12SrRNAçı—è†ıå’‹é–¶éfi¾æ›©å¢žå‘“åºfl$é•ıè¿⁄å�ºè´¨è¾–å−©æ¿•喛解咸鎗/çflµç¦»é£žè¡„æŠ¶éŠ´è´¨è°±æŒ¹æ³Łæ£•æµ‰GJB2å�ºåł€35delG㕆176-191del16㕆235delC㕆299-300delATï¼łSLC26A4å�ºåł€IVS7-2A > G㕆2168A > Gï¼łçº¿ç²™ä½fi12SrRNAå�ºåł€1494C > T㕆1555A > Gç�›3个å�ºåł€8ä¸ªçª†å‘Ÿä½“ç‡¹ï¼łå’¬å−łç�łæ�¥å‹šç�łé⁄⁄çfl¨è⁄ªå−¨å‹¤å‹«å’¬æ•§è—‚å¹²çflµä½“(auto-auditory brainstem response AABR)$夓ç�łé⁄⁄çfl¨è¯−æŒ�垉蕳声呓尗(otoacoustic emission OAE) + AABR$仓专é•ıè¿⁄蕖五3朋龗衄咬å−łå�¦è¯−æŒ�ï¼łé•›å‘Œå’„æœ�产咔儺938侉冥康挰çfl�嗿为对疧绗㕇ç»fiæžœï¼ı髟哱挰çfl�嗿绗棕å⁄º35侉膉犖å�ºåł€æ’ºå¸¦ï¼„3个å�ºåł€çª†å‘Ÿçı—æ•»ä½fi撺带ç”⁄3.80%ï¼łæ£•å⁄ºå’¬å−łéıœç¢“34侉(3.70%)㕆営ä¸�é⁄“度以ä¸−å’¬å−łéıœç¢“15侉(1.63%)ï¼ł30侉(85.7%)膉犖å�ºåł€æ’ºå¸¦è•–é•ıè¿⁄亃咬å−łç�łæ�¥ã•‡å¯¹ç–§ç»—棕å⁄º21侉膉犖å�ºåł€æ’ºå¸¦ï¼„3个å�ºåł€çª†å‘Ÿçı—æ•»ä½fi撺带ç”⁄2.23%ï¼łæ£•å⁄ºå’¬å−łéıœç¢“4侉(0.43%)㕆é⁄“度以ä¸−å’¬å−łéıœç¢“1侉(0.11%)ï¼ł17侉(68.0%)膉犖å�ºåł€æ’ºå¸¦è•–é•ıè¿⁄亃咬å−łç�łæ�¥ã•‡ä¸¤ç»—膉犖å�ºåł€çª†å‘Ÿçı—敻撺带ç”⁄㕆咬å−łéıœç¢“å‘−é⁄“度以ä¸−å’¬å−łéıœç¢“棕å⁄ºç”⁄以å‘−撺带膉犖å�ºåł€è•–å’¬å−łç�łæ�¥é•ıè¿⁄ç”⁄义銴差弇望ç»�计å�¦æ—‘乛㕇ç»fi论ï¼ı髟哱挰çfl�å—¿å’¬å−łéıœç¢“çı—å‘‚çfl�ç”⁄噄膉犖å�ºåł€çª†å‘Ÿæ’ºå¸¦ç”⁄åš⁄髟五æ�£å¸¸æŒ°çfl�å—¿åºflé⁄“ç‡¹å–³æ³¨ï¼łé⁄⁄çfl¨å’¬å−łå™„膉犖æŸfiæ—�å�ºåł€è†flå’‹ç�łæ�¥è…½å‘−抶呂田常觗é•ıè¿⁄å’¬å−łç�łæ�¥ä½ƒå–·æœ›è•³è†‰é«Ÿå“±åł€ç´€å™„è¿�å‘‚æ•§è†‰çŠ–é†Šä¼€åł€ç´€çı—挰çfl�嗿$对抩æœ�干颗㕆å®ıæœ�éı‘访㕆å⁄‘専膉犖呂çfl�喷望æ„⁄导旑乛㕇 endstream endobj 31 0 obj <>stream h241W0Pw/+Q0L)645)@HKBB( RYZlg`P endstream endobj 32 0 obj <>stream hތU]lU>mlC1;&hw3SJJmg;3]VNnBq)mMiH%}P|А`PmҤ! ѐBBG`nvw{c9v?9'F/{<}/1w-z`Ͽ;pC#vj4҆Zx/uxn|Xf8A BGX౱B `Cg:rBi(td7ủH ft`kY[UKKlķ[ٸ -ݭTL#,NIquR)5Fkz.$dco Z=!N>,{e` l?qO3B;WhO'[&jca7KȒ(N'$)ﰹ0b&o7ۣ8&jcwF3F;Ft]mjo36Vꊣl" O2 ˛\B7RƪT%x2?Uo%G&?wF`],IEMD^6}s݉KF%WN!K2v9ir1p D܌SuHd_kPK g+U[ə`D\*߉pSvUm*.rx,j~gy:\Q1iY*Ѥu:upOZ04K|[)}ҷk (W9ZKPmxg͉LM. f5r LV K #i)}-;?G*&Ńt܅*_#-5DHd߹M藋\Ux&i/]:([h 'Ῥ\N9Pñ>ߍsr{`I= endstream endobj 33 0 obj <>/Filter/FlateDecode/ID[<54318CBC2478154E993D951F0AF57E8B>]/Info 148 0 R/Length 175/Root 150 0 R/Size 149/Type/XRef/W[1 3 1]>>stream hbb&F ? Rt d@$c?d`QGd"`//=`)&fkI )_23|Xl)0yDf3E|@+f"YAAy 5 .g`$dN_B>Y?dϝ{H҂d;@M endstream endobj startxref 116 %%EOF 30 0 obj <>stream 2015-05-28T14:57:01+08:00 2015-05-28T14:56:05+08:00 2015-05-28T14:57:01+08:00 Acrobat PDFMaker 11 Word 版 uuid:6ab0bff1-4447-4193-a936-23c7d97386e6 uuid:93ab3351-abd7-4785-8d2c-eb602df20278 233 application/pdf 高危新生儿听力和聋病易感基因联合筛查临床研究 目的:探讨在高危新生儿听力筛查基础上,联合聋病易感基因筛查的临床意义。方法:随机选择920例新生儿科住院具有听力损失高危因素的新生儿,于生后3~5天采集微量足跟血进行基因GJB2、SLC26A4及线粒体12SrRNA的聚合酶链扩增反应,通过基质辅助激光解吸附/电离飞行时间质谱方法检测GJB2基因35delG、176-191del16、235delC、299-300delAT;SLC26A4基因IVS7-2A > G、2168A > G;线粒体12SrRNA基因1494C > T、1555A > G等3个基因8个突变位点;听力筛查初筛采用自动判别听性脑干电位(auto-auditory brainstem response AABR),复筛采用诊断型耳声反射(otoacoustic emission OAE) + AABR,仍不通过者于3月龄行听力学诊断;选取同期产后区938例健康新生儿为对照组。结果:高危新生儿组检出35例聋病基因携带,3个基因突变的总体携带率3.80%;检出听力障碍34例(3.70%)、其中重度以上听力障碍15例(1.63%);30例(85.7%)聋病基因携带者通过了听力筛查。对照组检出21例聋病基因携带,3个基因突变的总体携带率2.23%;检出听力障碍4例(0.43%)、重度以上听力障碍1例(0.11%);17例(68.0%)聋病基因携带者通过了听力筛查。两组聋病基因突变的总携带率、听力障碍及重度以上听力障碍检出率以及携带聋病基因者听力筛查通过率之间差异有统计学意义。结论:高危新生儿听力障碍的发生率和聋病基因突变携带率均高于正常新生儿应重点关注;采用听力和聋病易感基因联合筛查能及时发现常规通过听力筛查但具有耳聋高危因素和迟发性聋病遗传因素的新生儿,对早期干预、定期随访、减少聋病发生具有指导意义。 张章,戴怡蘅,李振安,余凤慈,刘莹 Adobe PDF Library 11.0 聋病,基因突变,高危,新生儿 D:20150528065552 çł®çı—ï¼ı攢讨在髟哱挰çfl�å—¿å’¬å−łç�łæ�¥å�ºç¡•ä¸−$è†fl咋膉犖æŸfiæ—�å�ºåł€ç�łæ�¥çı—临åº−æ—‘ä¹›ã•‡æŒ¹æ³Łï¼ıéı‘机镛扩920侉挰çfl�嗿秂佑鎢喷望咬å−łæ“�å¤±é«Ÿå“±åł€ç´€çı—挰çfl�嗿$五çfl�å’”3~5天é⁄⁄éłƒå¾®é⁄‘è¶³è·�è¡•è¿łè¡„å�ºåł€GJB2㕆SLC26A4å‘−线粙ä½fi12SrRNAçı—è†ıå’‹é–¶éfi¾æ›©å¢žå‘“åºfl$é•ıè¿⁄å�ºè´¨è¾–å−©æ¿•喛解咸鎗/çflµç¦»é£žè¡„æŠ¶éŠ´è´¨è°±æŒ¹æ³Łæ£•æµ‰GJB2å�ºåł€35delG㕆176-191del16㕆235delC㕆299-300delATï¼łSLC26A4å�ºåł€IVS7-2A > G㕆2168A > Gï¼łçº¿ç²™ä½fi12SrRNAå�ºåł€1494C > T㕆1555A > Gç�›3个å�ºåł€8ä¸ªçª†å‘Ÿä½“ç‡¹ï¼łå’¬å−łç�łæ�¥å‹šç�łé⁄⁄çfl¨è⁄ªå−¨å‹¤å‹«å’¬æ•§è—‚å¹²çflµä½“(auto-auditory brainstem response AABR)$夓ç�łé⁄⁄çfl¨è¯−æŒ�垉蕳声呓尗(otoacoustic emission OAE) + AABR$仓专é•ıè¿⁄蕖五3朋龗衄咬å−łå�¦è¯−æŒ�ï¼łé•›å‘Œå’„æœ�产咔儺938侉冥康挰çfl�嗿为对疧绗㕇ç»fiæžœï¼ı髟哱挰çfl�嗿绗棕å⁄º35侉膉犖å�ºåł€æ’ºå¸¦ï¼„3个å�ºåł€çª†å‘Ÿçı—æ•»ä½fi撺带ç”⁄3.80%ï¼łæ£•å⁄ºå’¬å−łéıœç¢“34侉(3.70%)㕆営ä¸�é⁄“度以ä¸−å’¬å−łéıœç¢“15侉(1.63%)ï¼ł30侉(85.7%)膉犖å�ºåł€æ’ºå¸¦è•–é•ıè¿⁄亃咬å−łç�łæ�¥ã•‡å¯¹ç–§ç»—棕å⁄º21侉膉犖å�ºåł€æ’ºå¸¦ï¼„3个å�ºåł€çª†å‘Ÿçı—æ•»ä½fi撺带ç”⁄2.23%ï¼łæ£•å⁄ºå’¬å−łéıœç¢“4侉(0.43%)㕆é⁄“度以ä¸−å’¬å−łéıœç¢“1侉(0.11%)ï¼ł17侉(68.0%)膉犖å�ºåł€æ’ºå¸¦è•–é•ıè¿⁄亃咬å−łç�łæ�¥ã•‡ä¸¤ç»—膉犖å�ºåł€çª†å‘Ÿçı—敻撺带ç”⁄㕆咬å−łéıœç¢“å‘−é⁄“度以ä¸−å’¬å−łéıœç¢“棕å⁄ºç”⁄以å‘−撺带膉犖å�ºåł€è•–å’¬å−łç�łæ�¥é•ıè¿⁄ç”⁄义銴差弇望ç»�计å�¦æ—‘乛㕇ç»fi论ï¼ı髟哱挰çfl�å—¿å’¬å−łéıœç¢“çı—å‘‚çfl�ç”⁄噄膉犖å�ºåł€çª†å‘Ÿæ’ºå¸¦ç”⁄åš⁄髟五æ�£å¸¸æŒ°çfl�å—¿åºflé⁄“ç‡¹å–³æ³¨ï¼łé⁄⁄çfl¨å’¬å−łå™„膉犖æŸfiæ—�å�ºåł€è†flå’‹ç�łæ�¥è…½å‘−抶呂田常觗é•ıè¿⁄å’¬å−łç�łæ�¥ä½ƒå–·æœ›è•³è†‰é«Ÿå“±åł€ç´€å™„è¿�å‘‚æ•§è†‰çŠ–é†Šä¼€åł€ç´€çı—挰çfl�嗿$对抩æœ�干颗㕆å®ıæœ�éı‘访㕆å⁄‘専膉犖呂çfl�喷望æ„⁄导旑乛㕇 endstream endobj 150 0 obj <> endobj 223 0 obj <>stream hތU]LU>%j}0cRe-ݙ-6E`ݙt;.ϰ]K:nKז@CIS>h}4JBBh6>4>4MJ93,fw|ν=>cu6jÞ^13b9GLg΃{Jj͑!x/ux|W[d1A(D`XfhN|;*cc!vuS(kO`0+G;st`iuS?8ҵx:Uٮ##09SzLEd_R^ ;jldgFuµb' yW࣑Pnp~A0,o!}Gld3y0ƕ?3=.Ă%6$3QD%zy tx=6#\H,x̏=_Of|yl/cZM8. $考7#-do{Qp]8Chso[2ӏ#Ki+_jJM۹dL38/:֡> yJ7xqCUVtC:Y88Z򗴨(P(͔0I&ϚI|O?ɲ (ݝkEURdC}Ѩ~MB*5'V G'9<{;qը^;2ٹ7bN -j17}MOEnN^1jh.zjqX@L+g]?,-֒dPd7޹G꽄M \YJ63QuvKQOgC[:rr_|7JS/8Wט7 endstream endobj 224 0 obj <>/Filter/FlateDecode/ID[<7721205B81A91448BB1EBE5BB02B72D2>]/Index[30 1 148 1 150 1 223 2]/Info 148 0 R/Length 34/Prev 116/Root 150 0 R/Size 225/Type/XRef/W[1 3 0]>>stream hbbd}[ 6ηL 5@5 R endstream endobj startxref 397218 %%EOF