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  1.灏忎笅棰�

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  2.鑵�瑁�

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2.杩涢�熼殰纰嶃�傜敱浜庡彛銆侀蓟銆佽垖鍙婂捊閮ㄧ暩褰�銆傝繘椋熸椂鎮ｅ効鐨勫惛鍚�鍔熻兘鍜屽悶鍜藉姛鑳藉潎涓嶅叏銆傞�熺墿寰堝�规槗鍙戞祦鍒伴蓟鑵旓紝骞跺惛鍒版皵绠″紩璧峰憶鍜崇敋鑷冲潬绉�鎬ц偤鐐庛��

3.鍙戦煶闅滅�嶃�傚�傛灉灏忎笅棰屽強鍞囪叚瑁傚緱涓嶅埌娌荤枟锛屾偅鍎跨殑鍙戦煶鍙婅��瑷�琛ㄨ揪鍔熻兘浼氬彈鍒板奖鍝嶃��

4.鍏跺畠鍣ㄥ畼鍔熻兘闅滅�嶃�傛牴鎹�鍏跺畠鍣ㄥ畼鐣稿舰鐨勫瓨鍦ㄤ笌鍚﹀彲鑳戒細鍑虹幇寰�鐜�绯荤粺鐨勫姛鑳藉奖鍝嶃��

涓婅堪鐢变簬鍏堝ぉ鐣稿舰閫犳垚鐨勫悇绉嶅姛鑳介殰纰嶏紝灏ゅ叾鏄�鍛煎惛鍙婅繘椋熼殰纰嶄細褰卞搷鎮ｅ効鐨勭敓闀垮彂鑲层��

璇婃柇鍜岄壌鍒�璇婃柇

灏忎笅棰岀暩褰㈢患鍚堢棁鐨勮瘖鏂�涓昏�佷互鍏稿瀷浣撳緛鍜屼复搴婅〃鐜拌繘琛岃瘖鏂�銆傝儙鍎垮彂鑲插悗鏈熶篃鍙�浠ラ�氳繃B瓒呮祴閲忎笅棰岄�ㄧ殑澶у皬杩涜�屼骇鍓嶇瓫鏌ワ紝瀵圭枒浼肩梾渚嬪仛濂界�＄悊鍛煎惛鍜岃繘椋熺殑鍑嗗�囥��

閴村埆璇婃柇娉ㄦ剰涓庡叾瀹冨厛澶╃暩褰�锛屾瘮濡傗�滃攼姘忕患鍚堢棁锛堢��21瀵规煋鑹蹭綋涓変綋鐣稿舰锛夆�濄�佲�滈厭绮惧┐鍎库�濄�佲�滃攪鑵�瑁傗�濄�佲�淪tickler syndrome鈥濄�佲�淭reacher Collins Syndrome鈥濈瓑杩涜�岄壌鍒�銆�

涓婅堪鍏堝ぉ鐤剧梾鐨勬偅鑰呬篃鏈夊彲鑳戒即鏈夊皬涓嬮�岀殑涓村簥琛ㄧ幇锛屽�规槗涓庡皬涓嬮�岀暩褰㈢患鍚堢棁鍦ㄨ瘖鏂�涓婃贩娣嗭紝搴旀敞鎰忛壌鍒�銆�

鍞愭皬缁煎悎鐥囦笌灏忎笅棰岀暩褰㈢患鍚堢棁鏈�澶х殑鍖哄埆鏄�鏈夋煋鑹蹭綋涓変綋鐣稿舰锛岄�氳繃鏌撹壊浣撶殑妫�鏌ュ氨鍙�浠ヨ繘琛屼袱鑰呯殑閴村埆銆�

閰掔簿濠村効涓庡皬涓嬮�岀暩褰㈢患鍚堢棁鐨勯壌鍒�涓昏�佹牴鎹�瀛曟湡鐥呭彶锛屼粩缁嗗垎鏋愬瓡濡囧湪鎬�瀛曟湡闂寸殑楗�椋熸儏鍐碉紝澶氬彲浣滃嚭鍒ゆ柇銆�

鍗曠函鍞囪叚瑁傜暩褰㈣�呭�氭病鏈夊皬涓嬮�岀殑琛ㄧ幇锛屽彲鑳芥湁鍛煎惛闅滅�嶅拰杩涢�熷洶闅撅紝浣嗗�氫笉浼氬嚭涓�鑸屽悗鍧犲強鑸屼笅鍨傘��

Stickler syndrome鏄�閬椾紶鎬х粨缂旂粍缁囧悎鎴愰殰纰嶆�х柧鐥咃紝闈㈤儴鐨勪富瑕佽〃鐜版槸鎵佸钩缂轰箯绔嬩綋鎰燂紝鍚屾椂鍏ㄨ韩鍏宠妭澶氭湁杩涜�屾�х殑杞�楠ㄧ牬鍧忔�х梾鍙樸��

Treacher Collins Syndrome鍏稿瀷鐨勮〃鐜版槸澶栦晶鐪艰�掍笅鍨傦紝骞舵湁澶栫溂瑙掑�勭殑楠ㄩ�肩己鎹熴�傚�氭湁棰ч�ㄩⅶ寮撳彂鑲蹭笉鑹�鍜屼笅鍨傦紝浠庤�屽嚭鐜板吀鍨嬬殑涓嬪瀭闈㈠�广��

浣嗗皬涓嬮�岀患鍚堢棁鎮ｈ�呭�氫笉浼存湁绁炵粡绯荤粺鍙婂叾瀹冨唴鑴忓櫒瀹樺紓甯革紝鑰屼笖寰堝�氭偅鑰呭湪鍚庡ぉ鐢熼暱鍙戣偛鐨勮繃绋嬩腑涓嬮�岄�ㄨ兘澶熷姞閫熷彂鑲插埌姝ｅ父灏哄�搞�傚洜姝わ紝濡傛灉鎮ｅ効鑳藉�熷彂鑲叉垚浜猴紝闄ゅ彲鑳藉瓨鍦ㄧ殑鍙�浠ヤ慨澶嶇殑闈㈤儴澶栧舰寮傚父澶栵紝鑳藉�熸�ｅ父鍙備笌绀句細娲诲姩鍙婂伐浣溿�傚強鏃╃殑浠庡熀鍥犳按骞虫槑纭�璇婃柇瀵归拡瀵规�х殑娌荤枟鍜屼紭鐢熶紭鑲叉彁楂樹汉鍙ｈ川閲忔湁寰堥噸瑕佺殑鎰忎箟銆�

灏忎笅棰岀暩褰㈢患鍚堢棁鐨勬不鐤�

灏忎笅棰岀患鍚堢棁涓�鏃︾‘璇婃不鐤椾富瑕佷互瀵圭棁娌荤枟涓轰富锛屾不鐤楁帾鏂藉彲浠ュ垎涓轰繚瀹堝拰鎵嬫湳娌荤枟銆傜洰鐨勬槸鍑忚交鍛煎惛閬撴�楅樆锛屾敼鍠勯�氭皵鍜岃繘椋熷姛鑳姐��

鍦ㄥ┐骞煎効鏈熷彲浠ラ噰鐢ㄩ蓟鍜介�氭皵閬撹緟鍔╅�氭皵[6,7]锛岃儍绠″杺椋焄8]锛屼笅棰岀壍寮曟垚楠╗9,10]璇卞�间笅棰屽彂鑲插噺杞诲懠鍚搁亾姊楅樆鐥囩姸銆傚繀瑕佹椂瀵规湁涓ラ噸閫氭皵闅滅�嶇殑鍎跨�ワ紝鍙�浠ヨ繘琛屾皵绠″垏寮�銆傚�傛灉鎴愬勾鍚庝笅棰岄�ㄤ粛鐒跺彂鑲蹭笉鑹�鍒欏彲杩涜�屼笅棰岀壍寮曟垚楠ㄥ強棰忓墠绉荤瓑鎵嬫湳娌荤枟浠ユ不鐤楃潯鐪犲懠鍚搁殰纰嶅強鏀瑰杽瀹硅矊[11]銆�

灏忎笅棰岀暩褰㈡垚鍚庡彲浠ヨ繘琛岄�忓墠绉绘暣褰㈡不鐤�

灏忎笅棰岀暩褰㈢患鍚堢棁鐨勯�勫悗

灏忎笅棰岀患鍚堢棁鎮ｈ�呭�備笉浼存湁绁炵粡绯荤粺鍙婂叾瀹冨唴鑴忓櫒瀹樺紓甯革紝鎮ｈ�呭湪鍚庡ぉ鐢熼暱鍙戣偛鐨勮繃绋嬩腑涓嬮�岄�ㄨ兘澶熷姞閫熷彂鑲插埌姝ｅ父灏哄�搞�傚洜姝わ紝鍙�瑕佹偅鍎胯兘澶熸浮杩囧┐骞煎効鏈燂紝闀垮ぇ鎴愪汉鍚庯紝闄ゅ彲鑳藉瓨鍦ㄧ殑鍙�浠ヤ慨澶嶇殑闈㈤儴澶栧舰寮傚父澶栵紝鑳藉�熸�ｅ父鍙備笌绀句細娲诲姩鍙婂伐浣溿�傚強鏃╄瘖鏂�杩涜�岄拡瀵规�х殑娌荤枟鏈夊緢閲嶈�佺殑鎰忎箟銆�

濡傛灉鎮ｇ梾濠村効鍑虹敓鍚庢病鏈夊緱鍒板強鏃剁殑澶勭悊锛屾湁鏄庢樉鍛煎惛閬撴�楅樆鐥囩姸鑰呭瓨娲荤巼寰堜綆銆�

缁间笂鎵�杩帮紝灏忛�岀暩褰㈢患鍚堢棁鏄�涓�绉嶆瘮杈冨父瑙佸厛澶╃暩褰�锛屽叾鑷存畫绋嬪害涓嶉珮锛屽叾鎵�鑷寸暩褰�涔熼兘鑳介�氳繃鐜颁唬澶栫�戞墜娈佃繘琛屾暣澶嶏紝濡傝兘鍙婃椂璇婃柇鍙婃椂娌荤枟鎮ｅ効涓嶄粎鑳芥�ｅ父瀛樻椿锛岃�屼笖鍙�浠ユ�ｅ父鐢熸椿骞跺疄鐜扮ぞ浼氬姛鑳姐�備絾濡傛灉瀵硅繖涓�鐤剧梾鐨勮�よ瘑涓嶈冻锛屼笉鑳芥棭鏈熻瘖鏂�鍜岄噰鍙栨湁鏁堢殑鎵嬫�垫不鐤楋紝寰堝�氬彈绱�鑰呭洜寰椾笉鍒板強鏃舵湁鏁堢殑娌荤枟鍏虫敞鑰屼抚澶辩敓瀛樼殑鏈轰細銆�

鍙傝�冩枃鐚�

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[2].B眉tow KW, Hoogendijk CF, Zwahlen RA. Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol. J Pediatr Surg. Nov 2009;44(11):2112-8.

[3]. Dr. L. J. Sheffield, J. A. Reiss, K. Strohm, M. Gilding, John M. Opitz and James F. Reynolds. A genetic follow-up study of 64 patients with the Pierre Robin complex. American Journal of Medical Genetics. Volume 28, Issue 1,  pages 25鈥�36, September 1987.

[4]. Jakobsen LP, Knudsen MA, Lespinasse J. The genetic basis of the Pierre Robin Sequence. Cleft Palate Craniofac J. 2006 Mar;43(2):155-9.

[5]鏉ㄧ惓锛屽��閿︽枃锛岃┕鍥芥爧绛夈��6渚嬫湁Pierre Robin搴忓垪寰佽〃鍨嬬殑鏂扮敓鍎垮叏鍩哄洜缁勬嫹璐濇暟鍙樺紓鍒嗘瀽,涓�鍗庡洿浜у尰瀛︽潅蹇椼��2011 骞�14鍗�11鏈�  670-675椤点��

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[8].Lidsky ME, Lander TA, Sidman JD. Resolving feeding difficulties with early airway intervention in Pierre Robin Sequence. Laryngoscope. Jan 2008;118(1):120-3.

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[10].Hong P, Brake MK, Cavanagh JP, Bezuhly M, Magit AE. Feeding and mandibular distraction osteogenesis in children with Pierre Robin sequence: A case series of functional outcomes. Int J Pediatr Otorhinolaryngol. Mar 2012;76(3):414-8.

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