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姗磱鑲岃倝鐦�(rhabdomyosarcoma锛孯MS)鏄厭绔ユ湡鏈€甯歌(ji脿n)鐨勮粺绲勭箶鑵槫锛屽崰鍏掔鑵槫鐨�6.5%宸﹀彸銆傚叾鑷ㄥ簥琛ㄧ従(xi脿n)澶氭ǎ锛岀暟璩�(zh矛)鎬у挤(qi谩ng)锛岄爯(y霉)鍚庤垏鑵槫鍘熺櫦(f膩)閮ㄤ綅銆佸ぇ灏�銆佸杩強渚电姱鍛ㄥ湇绲勭箶銆佸櫒瀹樼▼搴︿互鍙婄梾鐞嗛鍨嬫湁闂�(gu膩n)銆�

鍘熺櫦(f膩)閮ㄤ綅浠ラ牠闋搁儴鍙婃硨灏跨敓娈栫郴绲�(t菕ng)鏈€甯歌(ji脿n)锛屽叾娆＄偤鍥涜偄銆佽粈骞茬瓑鍏朵綑閮ㄤ綅銆俁MS灏�(du矛)鍖栫檪銆佹斁鐧傛晱鎰燂紝浣嗗柈涓€娌荤檪鏁堟灉宸�锛岄渶瑕佽叓鐦ゅ収(n猫i)銆佸绉戙€佹斁鐧傜瓑澶氬(xu茅)绉戣伅(li谩n)鍚堢殑缍滃悎娌荤檪銆�

鍏掔姗磱鑲岃倝鐦ょ爺绌�(缇庡湅(gu贸))鍗�(xi茅)浣滅祫(IntergroupRMSStudyGroup锛孖RSG)鍜屽厭绔ヨ粺绲勭箶鑲夌槫鐮旂┒(姝愭床)鍗�(xi茅)浣滅祫(EuropeanPediatricsofttissue sarcomasstudygroup锛孍pSSG)绛夎純澶у厭绔ヨ叓鐦ょ爺绌剁祫缍�(j墨ng)閬�(gu貌)20浣欏勾鐨勮嚚搴婄爺绌讹紝骞舵牴鎿�(j霉)鐥呯悊浜炲瀷銆佽(sh霉)鍚庣梾鐞嗗垎鏈熷拰TNM鍒嗘湡锛屽皣RMS鍒嗙偤浣庡嵄銆佷腑鍗卞拰楂樺嵄3绲�锛岄€�(j矛n)琛屽垎灞ゅ拰缍滃悎娌荤檪锛屼笉鏂峰劒(y艒u)鍖栨不鐧傛柟妗�锛岄€�(j矛n)涓€姝ユ敼鍠勯爯(y霉)鍚庯紝浣垮緱RMS鐧傛晥閫愬勾鎻愰珮銆�

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(涓€)鑷ㄥ簥琛ㄧ従(xi脿n)

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闋牳閮細鑵﹁啘鏃佸崁(q奴)RMS鍗犻牠闋搁儴RMS鐨�50%锛屾棭鏈熶笉鏄撶櫦(f膩)鐝�(xi脿n)锛岃€屼笖寰堥洠瀹屽叏鍒囬櫎?锛熸潃鎲〧(xi脿n)鐐洪蓟鑵旀垨鑰呭鑰抽亾鍑虹従(xi脿n)鑶胯鎬у垎娉岀墿锛岃€抽亾鎴栭蓟鑵旈樆濉烇紝鎴栬€呭悶鍜藉洶闆�銆傜櫏鐙€鍙兘鏈�(hu矛)琚瑾�(r猫n)鐐烘槸涓婂懠鍚搁亾鎱㈡€х値鐧�銆�

鍑虹従(xi脿n)椤辩缍�(j墨ng)绯荤当(t菕ng)鐧ョ媭鎴栧叾浠栫缍�(j墨ng)绯荤当(t菕ng)鐧ョ媭锛屾彁绀洪”搴曟垨涓绁炵稉(j墨ng)绯荤当(t菕ng)渚电姱锛岄渶瑕佺珛鍗宠褰卞儚瀛�(xu茅)妾㈡煡銆傝叇鑴婃恫涓叓鐦ょ窗(x矛)鑳為櫧(y谩ng)鎬у彲鑳芥棭浜庡奖鍍忓(xu茅)鐧�(f膩)鐝�(xi脿n)鑵槫渚电姱銆傜溂鐪禦MS鍗犻牠闋搁儴RMS鐨�25%锛岄爯(y霉)鍚庣浉灏�(du矛)鑹ソ锛屾閮ㄤ綅鐨勮叓鐦ゆ棭鏈熷鏄撳嚭鐝�(xi脿n)鐧ョ媭锛屽鐪肩悆绐佸嚭銆佷即鐪肩悆鍥哄畾銆佷竴鍋�(c猫)鐪肩灱澧炲帤銆佺湺鍛ㄥ嚭琛€鎴栨枩瑕栫瓑銆傚叾浠栫殑闋牳閮≧MS鍙綅浜庨牳閮ㄨ粺绲勭箶銆侀”闋傝叡鑶溿€佸彛鑵�銆佸斁娑茶吅銆佸枆銆佸捊閮�銆佽叜鑵哄強闈㈤牥绛夐儴浣嶃€�

娉屽翱鐢熸畺閬擄細鏈€甯歌(ji脿n)浜庤唨鑳卞拰鍓嶅垪鑵�锛岀磩鍗�30%50%銆傝唨鑳辫叓鐦ゅ偩鍚戜簬鍚戣厰鍏�(n猫i)鐢熼暦(zh菐ng)锛屽鍦ㄨ唨鑳变笁瑙掑崁(q奴)鍏�(n猫i)鎴栭檮杩�锛屽亸鍚戜簬灞€闄�锛屼互琛€灏�銆佸翱璺闃诲苟灏夸腑鍋舵湁榛忔恫琛€鎬ф垚鍒嗙偤涓昏琛ㄧ従(xi脿n)銆傚墠鍒楄吅鑵槫甯稿嚭鐝�(xi脿n)宸ㄥぇ楠ㄧ泦鍏�(n猫i)鑵墿锛屽父鏃╂湡杞�(zhu菐n)绉昏嚦鑲洪儴銆傝叓鐦や篃鍙櫦(f膩)鐢熶簬鐫父鏃佹垨濂虫€х敓娈栭亾銆�

鍥涜偄鍜岃粈骞诧細鑲㈤珨灞€閮ㄨ叓鑴规槸鑲夌槫鐨勭壒寰�锛屽彲涔熷嚭鐝�(xi脿n)绱呰叓鍙婅Ц鐥涜〃鐝�(xi脿n)銆傝叓鐦ょ浉灏�(du矛)杓冨ぇ锛屾牴鎿�(j霉)鐩告噳(y墨ng)鐨勫師鐧�(f膩)閮ㄤ綅锛屼篃鍙疮鍙婅嚚杩戣兏鑵版鑴婃煴锛屼絾灞€閮ㄦ穻宸寸祼(ji茅)钄撳欢灏戣(ji脿n)銆傝兏鑵斿収(n猫i)鍜岃吂鑶滃悗楠ㄧ泦鍗€(q奴)鍩燂細鑳歌厰鍏�(n猫i)鍜岃吂鑶滃悗楠ㄧ泦鍗€(q奴)鍩熶綅缃繁锛岃ê鏂峰墠鍙兘鑵槫宸茬稉(j墨ng)寰堝ぇ锛屽父鍖呯篂澶ц绠�锛屼笉鑳藉畬鍏ㄥ垏闄ゃ€�

鍏朵粬閮ㄤ綅锛氭渻(hu矛)闄�-鑲涘懆鍗€(q奴)鍩�锛屽彲椤炰技鑶胯叓鎴栨伅鑲�;鑶介亾鑵槫灏戣(ji脿n)锛屽彲鏈夋闃绘€ч粌鐤歌〃鐝�(xi脿n)锛屽父鏈夎倽鍏�(n猫i)杞�(zhu菐n)绉�銆佽吂鑶滃悗杞�(zhu菐n)绉诲強鑲鸿綁(zhu菐n)绉汇€�

(浜�)鐥呯悊鍒嗗瀷

姗磱鑲岃倝鐦ゆ槸鐢变笉鍚屽垎鍖栭殠娈电殑姗磱鑲屾瘝绱�(x矛)鑳炵祫鎴愭儭鎬ц叓鐦�锛屽爆浜庡皬鍦撹棈(l谩n)绱�(x矛)鑳炶叓鐦�锛岃叓鐦ょ敱灏忕窗(x矛)鑳炵祫鎴愶紝鏈夊ぇ鐨�銆佸湏鐨勬繁鏌撶殑绱�(x矛)鑳炴牳锛岄彙涓嬪彲瑕�(ji脿n)楠ㄩ鑲屾帓鍒椼€傚厤鐤祫鍖栭’绀哄瓨鍦╠esmin銆乵yogenin銆乵yoglobin銆乤ctin銆乿imentin绛夐楠艰倢妯�(bi膩o)瑷�銆俉HO灏嘡MS鍒嗙偤浠ヤ笅涓夌ó鍩烘湰鐥呯悊椤炲瀷銆�

1.鑳氳儙鍨�(ERMS)锛氬寘鎷憽钀勭媭(botryoidcell)鍜屾褰㈢窗(x矛)鑳炲瀷(spindlecell)銆傜窗(x矛)鑳為伜鍌冲(xu茅)鍙婂垎瀛愮敓鐗╁(xu茅)鐮旂┒鎻愮ず锛岄儴鍒嗚儦鑳庡瀷瀛樺湪11铏�(h脿o)鏌撹壊楂旈洔鍚堢己澶�銆�

2.鑵烘场鍨�(ARMS)锛氶儴鍒咥RMS涓瓨鍦ㄦ煋鑹查珨鏄撲綅t(2;13)(q35;q14)鎴杢(1;13)(q36;q14)銆傞€欏叐绋槗浣嶅垎鍒ュ舰鎴愪簡鐩告噳(y墨ng)鐨勮瀺鍚堝熀鍥燩AX3-FKHR鍜孭AX7-FKHR銆傚叾涓�锛孭AX3-FKHR铻嶅悎铔嬬櫧鑸囬爯(y霉)鍚庝笉鑹浉闂�(gu膩n)銆�

3.澶氬舰鍨嬫垨闁撹畩鍨�(pleomorphicoranaplasticRMS)锛氬厭绔ョ綍瑕�(ji脿n)锛岀梾鐞嗕簽鍨嬬偤闁撹畩鑰呴爯(y霉)鍚庝笉浣�銆�

(涓�)杓斿姪妾㈡煡

1.琛€甯歌(gu墨)銆佸翱甯歌(gu墨)銆佽鐢熷寲銆佸嚌琛€妾㈡煡

(1)琛€甯歌(gu墨)妾㈡煡锛氭偅鍏掑彲琛ㄧ従(xi脿n)鐐鸿钵琛€;鏈夐楂撴蹈娼�(r霉n)鑰呭彲鍑虹従(xi脿n)鍏ㄨ绱�(x矛)鑳炴笡灏�銆�

(2)灏垮父瑕�(gu墨)妾㈡煡锛氭硨灏跨敓娈栭亾鑵槫鍙湁琛€灏胯〃鐝�(xi脿n)銆�

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(4)鍑濊鍔熻兘锛氬寘鎷琍T銆丄PTT銆乀T銆丗IB銆丏-浜岃仛楂�銆丗DP銆傛湁楠ㄩ珦娴告饯(r霉n)銆侀珮鑵槫璨�(f霉)鑽�銆佸法澶х槫鐏跺悎骞惰叓鐦ょ牬瑁傚嚭琛€鑰呭彲鍑虹従(xi脿n)FIB涓嬮檷锛孌-浜岃仛楂斿崌楂樼瓑銆�

2.褰卞儚瀛�(xu茅)妾㈡煡

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(3)纾佸叡鎸巸鎻忥細鍙⒑瀹氬師鐧�(f膩)鐦ょ伓浠ュ強灏�(du矛)鍛ㄥ湇閯拌繎绲勭箶鍣ㄥ畼鐨勪镜鐘儏娉侊紝灏ゅ叾閬╃敤浜庣湺鍛�銆佽叇鑶滄梺鍙婅剨鏌辨梺鍗€(q奴)鍩熺殑鑵槫銆�

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(5)PET/CT妾㈡煡锛氭湁姊濅欢鐨勫柈浣嶅彲鑰冩叜琛屽叏韬闆诲瓙鐧�(f膩)灏勮▓(j矛)绠楁(j墨)鏂峰堡椤儚锛屾湁鍔╀簬鍏ㄩ潰瑭�(p铆ng)浼扮槫鐏跺強杞�(zhu菐n)绉婚儴浣�銆�

(6)B瓒呮鏌ワ細鍙敤浜庡師鐧�(f膩)鐦ょ伓鍙婅綁(zhu菐n)绉荤槫鐏剁殑瑭�(p铆ng)浼板強娌荤檪鍙嶆噳(y墨ng)鐨勭洠(ji膩n)娓�(c猫)銆�

(7)蹇冭嚐褰╄壊瓒呰伈锛氱敤浜庡寲鐧傚墠蹇冭嚐鍔熻兘鐨勮⿻(p铆ng)浼�銆�

3.鏈夊壍(chu脿ng)鎿嶄綔妾㈡煡

(1)娲绘锛氶€氬父鎵嬭(sh霉)娲绘鍙嵅鍙栬冻澶犵殑妯�(bi膩o)鏈敤浜庣祫绻旂梾鐞嗗強鍒嗗瓙鐢熺墿瀛�(xu茅)妾㈡煡銆傝嫢鍒濊ê鏅�(sh铆)涓嶈兘琛屾牴娌绘€ф墜琛�(sh霉)鍒囬櫎锛屽彲鍏堜簣杓斿姪鍖栫檪锛屽緟鑵槫绺皬鍚庢搰鏈熸墜琛�(sh霉)娌荤檪銆傛浠跺厑瑷辩殑鎯呮硜涓嬪彲琛岀┛鍒烘椿妾�銆�

(2)楠ㄩ珦绌垮埡鎴栨椿妾細鐢ㄤ簬瑭�(p铆ng)浼版湁鐒�(w煤)楠ㄩ珦娴告饯(r霉n)銆�

(3)鑵﹁剨娑叉鏌ワ細鐥呰畩浣嶄簬鑵﹁啘鏃佸崁(q奴)鑰�锛屽缓璀拌鑵﹁剨娑叉鏌ワ紝鍖呮嫭閺′笅鎵剧槫绱�(x矛)鑳�锛屾湁姊濅欢鐨勫柈浣嶅彲琛屾祦寮忕窗(x矛)鑳炶(sh霉)妾㈡煡銆�

4.鍏朵粬妾㈡煡

(1)鑱�(t墨ng)鍔涙鏌ワ細鐢ㄤ簬娌荤檪鍓嶈伣(t墨ng)鍔涚殑瑭�(p铆ng)浼�锛屼互鍙婇墤椤炶棩鐗╄伣(t墨ng)鍔涙瘨鎬х殑鐩�(ji膩n)娓�(c猫)銆�

(2)UGTIAI鍩哄洜澶氭厠(t脿i)鎬х殑妾㈡脯(c猫)锛歎GTIAI鏄紛绔嬫浛搴风殑涓昏浠ｈ瑵閰�锛屾湁姊濅欢鐨勫柈浣嶆噳(y墨ng)琛孶GTIAI鍩哄洜澶氭厠(t脿i)鎬ф娓�(c猫)銆俇GTIAI*6鍩哄洜鐨勭獊璁婂瀷鍙檷浣嶶GTIAI娲绘€�锛屽鍔犺吀閬撻粡鑶滄悕鍌凤紝鍑虹従(xi脿n)鑵圭€夐ⅷ(f膿ng)闅�(xi菐n);UGTIAI*28鍩哄洜澶氭厠(t脿i)鎬ф槸闋�(y霉)娓�(c猫)鍖栫檪鍚庣櫦(f膩)鐢熷毚(y谩n)閲嶇矑绱�(x矛)鑳炴笡灏戝拰鑵圭€夐ⅷ(f膿ng)闅�(xi菐n)鐨勫垎瀛愭(bi膩o)瑷�銆�

(3)鐔掑厜鍘熶綅闆滀氦妾㈡煡(FISH)锛氬皪(du矛)浜庤吅娉″瀷RMS锛岄€欓爡(xi脿ng)妾㈡煡鐢ㄤ締(l谩i)妾㈡脯(c猫)鏄惁鏈塼(1:13)鎴杢(2:13)鏄撲綅锛屽嵆鏄惁瀛樺湪PAX7-FKHR鍜孭AX3-FKHR铻嶅悎鍩哄洜銆�

(鍥�)閼掑垾瑷烘柗

1.灏ゆ枃鑲夌槫锛氶€氬父琛ㄧ従(xi脿n)鐐鸿粺绲勭箶鍖呭锛岀壒鍒ユ槸鐧�(f膩)鐢熶簬闋牳閮�銆佸洓鑲㈢殑RMS闇€娉ㄦ剰鑸囧挨鏂囪倝鐦ら憭鍒�锛岀祫绻旂梾鐞嗘鏌ュ強FISH妾㈡煡鏈夊姪浜庨憭鍒ヨê鏂枫€�

2.绁炵稉(j墨ng)姣嶇窗(x矛)鑳炵槫锛氱櫦(f膩)鐢熶簬鑵圭泦鑵�銆侀牳閮ㄧ殑RMS闇€娉ㄦ剰鑸囩缍�(j墨ng)姣嶇窗(x矛)鑳炵槫閼掑垾锛屽彲绲�(ji茅)鍚堣叓鐦ゆ(bi膩o)瑷樼墿绁炵稉(j墨ng)鍏冪壒鐣版€х儻閱囧寲閰�(NSE)銆佸翱3-鐢叉哀鍩�4-缇ュ熀鑻︽潖浠侀吀/棣欒崏鎵佹閰�(HVA/VMA)杓斿姪瑷烘柗銆�

3.鐧借?銆傛渤娉勬挰鏋ㄥ潃鍌呰厼MS闇€娉ㄦ剰鑸囩櫧琛€鐥呯浉閼掑垾锛岄楂撹叓鐦ょ窗(x矛)鑳炲厤鐤垎鍨�銆侀楂撴椿妾㈢梾鐞嗘鏌ユ湁鍔╀簬瑷烘柗銆�

4.娣嬪反鐦わ細鐗瑰垾鏄吅娉″瀷姗磱鑲岃倝鐦ゆ湁鏅�(sh铆)鍙瑷虹偤娣嬪反鐦�锛岄渶瑕佺梾鐞嗘渻(hu矛)瑷烘槑纰鸿ê鏂�銆�

5.鍏朵粬杌熺祫绻旇倝鐦わ細濡傜簴缍倝鐦�銆佸钩婊戣倢鑲夌槫銆佹儭鎬у鍛ㄧ缍�(j墨ng)闉樼槫銆侀鑲夌槫銆佹粦鑶滆倝鐦ょ瓑锛岄渶瑕侀€氶亷(gu貌)鐥呯悊瑷烘柗閼掑垾銆�

鍥�銆佽嚚搴婂垎鏈熷強鍗遍毆(xi菐n)搴﹀垎绲�

(涓€)鑷ㄥ簥鍒嗘湡

鍏掔鑵槫鐮旂┒鍦�(gu贸)闅涘崝(xi茅)鏈�(hu矛)锛屾牴鎿�(j霉)娌荤檪鍓嶅熀浜庡奖鍍忓(xu茅)鍒跺畾鐨勮嚚搴婂垎鏈熺郴绲�(t菕ng)(TNM-UICC锛岃〃1)锛屼互鍙婃┇绱嬭倢鑲夌槫鐮旂┒缇庡湅(gu贸)鍗�(xi茅)浣滅祫(IRS)鐨勮(sh霉)鍚�-鐥呯悊鑷ㄥ簥鍒嗙祫绯荤当(t菕ng)(琛�2)锛屽叐绋垎鏈熸柟娉曠浉绲�(ji茅)鍚�銆�

(浜�)鍗遍毆(xi菐n)搴﹀垎绲�

渚濇摎(j霉)鐥呯悊浜炲瀷銆佽(sh霉)鍚庣梾鐞嗗垎鏈熷拰TNM鍒嗘湡锛屽皣鍗遍毆(xi菐n)搴﹀垎鐐轰綆鍗辩祫銆佷腑鍗辩祫銆侀珮鍗辩祫(琛�3)锛屼互渚垮垎灞ゆ不鐧�銆�

浜�銆佺檪鏁堣⿻(p铆ng)浼�

(涓€)鐦ょ伓瑭�(p铆ng)浼版(bi膩o)婧�(zh菙n)

1.瀹屽叏绶╄В(CR)锛氭墍鏈夌梾鐏跺畬鍏ㄦ秷澶卞ぇ浜�4鍛�锛岄楂撹綁(zhu菐n)绉昏€呯窗(x矛)鑳炴秱鐗囨鏌ラ櫚鎬с€�

2.閮ㄥ垎绶╄В(PR)锛氬師鐧�(f膩)鑵槫绺皬≥64%锛岃綁(zhu菐n)绉荤槫鐏剁府灏�≥30%锛屾矑(m茅i)鏈夋柊鐨勭梾鐏�銆�

3.鐤剧梾閫�(j矛n)灞�(PD)锛氬師鐧�(f膩)鑵槫杓冨垵瑷烘檪(sh铆)澧炲ぇ≥40%锛岃綁(zhu菐n)绉荤槫鐏�≥20%锛屾垨鍑虹従(xi脿n)鏂扮梾鐏�銆�

4.鐤剧梾绌�(w臎n)瀹�(SD)锛氳叓鐦ら珨绌嶄粙浜嶱D鍜孭R涔嬮枔銆�

(浜�)绺介珨鐧傛晥瑭�(p铆ng)鍍�(ji脿)妯�(bi膩o)婧�(zh菙n)

1.CR锛氬師鐧�(f膩)鐦ょ伓鍜岃綁(zhu菐n)绉荤槫鐏跺潎CR锛岀劇(w煤)鏂扮櫦(f膩)鐥呯伓銆�

2.PR锛氬師鐧�(f膩)鐦ょ伓CR锛岃綁(zhu菐n)绉荤槫鐏禤R/SD锛岀劇(w煤)鏂扮櫦(f膩)鐥呯伓;鎴栧師鐧�(f膩)鐦ょ伓PR锛岃綁(zhu菐n)绉荤槫CR/PR/SD锛岀劇(w煤)鏂扮櫦(f膩)鐥呯伓銆�

3.SD锛氬師鐧�(f膩)鐦ょ伓SD锛岃綁(zhu菐n)绉荤槫CR/PR/SD锛岀劇(w煤)鏂扮櫦(f膩)鐥呯伓銆�

4.PD锛氬師鐧�(f膩)鐦ょ伓銆佽綁(zhu菐n)绉荤槫鐏朵换浣曡檿閫�(j矛n)灞昉D銆傞”楠�1.5鍘樼背浠ュ収(n猫i)鐥呯伓銆�

(涓�)娌荤檪涓⿻(p铆ng)浼�

1.鎵嬭(sh霉)鍓嶆鏌ャ€傚叏韬鏌�锛屽寘鎷偤CT銆侀牠椤盡RI銆佸師鐧�(f膩)鐦ょ伓B瓒呭拰灞€閮ㄥ寮�(qi谩ng)CT鎴朚RI銆�

2.鍖栫檪姣忛枔闅�2鐧傜▼銆傝⿻(p铆ng)浼板師鐧�(f膩)鐦ょ伓鍙婅綁(zhu菐n)绉荤槫鐏跺ぇ灏忥紝鍗冲京(f霉)鏌ュ眬閮˙瓒�锛岄伕鎿囨€у寮�(qi谩ng)MRI銆�

3.寰�(f霉)鐧�(f膩)瑭�(p铆ng)浼�銆傚京(f霉)鐧�(f膩)鏄寚鑵槫瀹屽叏娑堝け1鍊�(g猫)鏈堝悗锛屽啀娆″嚭鐝�(xi脿n)灞€閮ㄦ垨杞�(zhu菐n)绉绘€ц叓鐦ょ梾鐏躲€傛鑵槫鏄撳師浣嶅京(f霉)鐧�(f膩)锛屽鏋滃京(f霉)鏌ヤ腑鐧�(f膩)鐝�(xi脿n)鍘熶綅鎴栫梾鍒濊綁(zhu菐n)绉荤伓寰�(f霉)鐧�(f膩)锛屾垨鑰呭嚭鐝�(xi脿n)鐩告噳(y墨ng)鐨勭櫏鐙€銆侀珨寰�锛屽墖闇€閫�(j矛n)涓€姝ュ啀鑰冩叜鑲篊T銆侀牠椤盡RI绛夊鏄撹綁(zhu菐n)绉婚儴浣嶇殑妾㈡煡銆�

4.娌荤檪涓娆¤⿻(p铆ng)鍍�(ji脿)鐐篊R銆丳R鍜孭D鐨勬偅鍏�锛岄枔闅�6锝�8鍛ㄥ啀瑭�(p铆ng)鍍�(ji脿)浠ョ⒑瑾�(r猫n)銆�

5.娌荤檪绲�(ji茅)鏉熷墠瑭�(p铆ng)浼�銆傚師鐧�(f膩)鐦ょ伓B瓒�銆佸寮�(qi谩ng)纾佸叡鎸垨CT銆佽兏閮–T銆侀牠椤盡RI锛屽繀瑕佹檪(sh铆)琛岄鎺冩弿銆傛不鐧備腑棣栨瑭�(p铆ng)鍍�(ji脿)鐐篊R銆丳R鍜孭D鐨勬偅鍏�锛岄枔闅�6锝�8鍛ㄥ啀瑭�(p铆ng)鍍�(ji脿)浠ョ⒑瑾�(r猫n)銆傛不鐧傜祼(ji茅)鏉熷悗閲濆皪(du矛)绺介珨鐨勬不鐧傛晥鏋滃啀娆¤⿻(p铆ng)鍍�(ji脿)鍜岀⒑瑾�(r猫n)銆�

鍏�銆佹不鐧�

RMS娌荤檪鍘熷墖鍜岀窗(x矛)鍓囬噸榛�(di菐n)鍙冪収銆婄編鍦�(gu贸)鍏掔姗磱鑲岃倝鐦ょ爺绌跺崝(xi茅)浣滅祫(IRSG)娌荤檪鏂规銆嬪拰銆婁腑鍦�(gu贸)鍏掔鍙婇潚灏戝勾姗磱鑲岃倝鐦よê鐧傚缓璀癈CCG-RMS-2016鍜岃В璁€銆嬨€愪腑鑿厭绉戦洔蹇�2017绗�55鍗风10鏈熴€�銆傛彁渚涚殑浠ヤ笅娌荤檪鏂规鍚勯啱(y墨)闄㈡牴鎿�(j霉)鍚勮嚜鎯呮硜閬告搰鎳�(y墨ng)鐢�锛岄儴鍒嗙梾浜哄彲绲�(ji茅)鍚堟墍鍦ㄩ啱(y墨)闄㈠(sh铆)闅涙儏娉侀仼鐣�(d膩ng)鏀硅壇銆�

(涓€)鎵嬭(sh霉)

鏈€濂借兘鍋氬畬鏁寸殑鑵槫鍒囬櫎鎴栧儏鏈夐彙涓嬫畼鐣�銆傚鏋滀笉鑳藉畬鍏ㄥ垏闄ゆ垨鑰呯梾璁婄疮鍙婄溂鐪�銆侀櫚閬撱€佽唨鑳辨垨鑶介亾锛岀偤浜嗕繚瀛樺櫒瀹樺強鍏跺姛鑳斤紝鍙厛鐢ㄥ寲鐧傛垨鍔犳斁鐧�锛屼娇鑵槫绺皬锛屽啀閫�(j矛n)琛屾墜琛�(sh霉)銆�

濡傜涓€娆℃墜琛�(sh霉)鍍呭仛鑵槫閮ㄥ垎鍒囬櫎锛屽彲缍�(j墨ng)鍖栫檪鍜�(鎴�)鏀剧檪3锝�6鍊�(g猫)鏈�(4锝�8鍊�(g猫)鐧傜▼)鍚庡啀鎵嬭(sh霉)銆傜偤浜嗛仈(d谩)鍒板畬鏁村垏闄よ叓鐦ょ殑鍘熺櫦(f膩)鐥呯伓锛屽彲浠ラ€�(j矛n)琛屼簩娆℃墜琛�(sh霉)锛屽垏闄ゅ師閬虹暀涓嬬殑闄�(y谩ng)鎬ч倞绶ｆ垨鍘熷儏鍋氭椿妾㈤儴浣�銆�

(浜�)鏀剧檪

1.鏀剧檪鎸囧緛锛歊MS鑳氳儙鍨婭RS-鈪犳湡涓嶅仛鏀剧檪锛屸叀锝炩叄鏈熷墖闋堟斁鐧�銆傝吅娉″瀷鏄撴湁灞€閮ㄥ京(f霉)鐧�(f膩)锛屾晠鈪犳湡涔熷仛鏀剧檪銆�

2.鏀剧檪鍔戦噺锛氬父瑕�(gu墨)鍒嗗壊鏀剧檪鑸囪秴鍒嗗壊鏀剧檪鐒�(w煤)鍗€(q奴)鍒�銆傜偤娓涘皯鏅氭湡鎼嶅偡锛屾斁鐧傞渶閲囩敤澶氬垎娆＄収灏�锛屽柈娆″姂閲忎笉瓒呴亷(gu貌)180cGy銆傚缓璀伴噰鐢ㄤ笁缍仼褰㈡垨瑾�(di脿o)寮�(qi谩ng)鏀剧檪鎶€琛�(sh霉)銆俁MS鍘熺櫦(f膩)鍜岃綁(zhu菐n)绉荤槫鐏剁殑鎺ㄨ枽鏀剧檪鍔戦噺(琛�4)銆�

3.鏀剧檪鏅�(sh铆)姗�(j墨)锛氭墜琛�(sh霉)宸茬稉(j墨ng)瀹屽叏鍒囬櫎鐦ょ伓鑰�锛屽彲浜庤(sh霉)鍚�1鍛ㄥ収(n猫i)鏀剧檪;浼撮”搴曚镜鐘殑鎮ｅ厭锛屾湁鏄庨’澹撹揩鐧ョ媭锛岄渶瑕佺穵鎬ユ斁鐧傝€咃紝鍙簬鍖栫檪鍓嶅厛鏀剧檪銆傝叓鐦よ純澶х劇(w煤)娉曟墜琛�(sh霉)鑰�锛屽缓璀版斁鐧傛檪(sh铆)闁撳湪鍘熺櫦(f膩)鐦ょ伓鍖栫檪绗�13鍛�锛岃綁(zhu菐n)绉荤槫鐏跺彲寤堕伈鍒板寲鐧傜25鍛�銆傚湪鏈夋浠剁殑鎯呮硜涓�锛屽彲浠ヨ€冩叜閲囩敤璩�(zh矛)瀛愭斁鐧傦紝鑳芥洿楂樼殑淇濊(h霉)闈跺崁(q奴)鍛ㄥ湇鐨勬甯哥祫绻斿拰鍣ㄥ畼銆�

(涓�)鍖栫檪

1.绺界窗(x矛)鍓�

(1)鏍规摎(j霉)褰卞儚瀛�(xu茅)鍙婂叾浠栨鏌�锛屼及瑷�(j矛)鑵槫鑳藉熀鏈畬鍏ㄥ垏闄よ€呭厛鎵嬭(sh霉);瀹屽叏鍒囬櫎鍥伴洠鑰呭儏娲绘锛屾槑纰鸿ê鏂峰悗鍏堝寲鐧傚啀鎵嬭(sh霉)銆傚閬告搰鎵嬭(sh霉)锛屽墖鍖栫檪鍦ㄨ(sh霉)鍚�7d鍏�(n猫i)闁�(k膩i)濮嬨€傜1娆″寲鐧傛檪(sh铆)娉ㄦ剰鐥呯悊鏈�(hu矛)瑷虹祼(ji茅)鏋滐紝濡傛灉鐐鸿吅娉″瀷寤鸿鍋氳瀺鍚堝熀鍥燩AX3-FKHR鍜孭AX7-FKHR锛岄渶淇鍗遍毆(xi菐n)搴﹀垎绲�銆�

(2)鏀剧檪鏈熼枔閬垮厤鎳�(y墨ng)鐢ㄦ斁绶氳弻绱燚(ACTD)鍜岄樋闇夌礌(ADR)锛屽寲鐧傚姂閲忔笡鐐哄崐閲忋€�

(3)鍚勬湡鍧囨湁蹇呰鍖栫檪銆傛牴鎿�(j霉)鍗遍毆(xi菐n)搴﹀垎绲勶紝閲囩敤涓嶅悓寮�(qi谩ng)搴︾殑鍖栫檪銆�

(4)鍔戦噺鍙婂寲鐧傚墠瑕佹眰锛氶暦(zh菐ng)鏄ユ柊鍫挎渶澶ч噺2.0mg锛屾斁绶氳弻绱燚鏈€澶ч噺2.5mg銆傚湪瀹屽叏绶╄В鍚�4锝�6鍊�(g猫)鐧傜▼鍙€冩叜鍋滆棩锛岀附鐧傜▼鏁�(sh霉)>12鍊�(g猫)鏅�(sh铆)鑰冩叜鍊�(g猫)楂斿寲瑾�(di脿o)鏁存柟妗�銆傚寲鐧�12鍛ㄧ槫鐏惰⿻(p铆ng)浼拌檿浜嶱D(鑵槫澧炲ぇ鎴栧嚭鐝�(xi脿n)鏂扮梾鐏�)鍓囧嚭绲�銆�

骞撮健<12鍊�(g猫)鏈堝寲鐧傚姂閲忔笡鍗婃垨楂旈噸≤12kg鎸夐珨閲嶈▓(j矛)绠�锛屽姂閲�=楂旇〃闈㈢⿳鍔戦噺/30×楂旈噸(kg)銆傛瘡鐧傜▼闁撻殧21d銆傛瘡鐧傜▼鍖栫檪鍓嶄腑鎬х矑绱�(x矛)鑳�(ANC)>0.75×10⁹/L锛岃灏忔澘(PLT)>100×10⁹/L?锛涱嚖鐔僘(ji茅)鏉�24锝�48h锛岄枊(k膩i)濮嬫敞灏勭矑绱�(x矛)鑳炲埡婵€鍥犲瓙(G-CSF)鎴栫矑-宸ㄥ櫖绱�(x矛)鑳炲埡婵€鍥犲瓙(GM-CSF)銆傞楂撴姂鍒舵湡>28澶╄€呬笅涓€鐧傜▼娓涢噺25%銆�

(5)鍖栫檪钘ョ墿姣掑壇鍙嶆噳(y墨ng)鐨勫垽瀹氭(bi膩o)婧�(zh菙n)锛氫互NCI涓嶈壇鍙嶆噳(y墨ng)鐨勫垎绱�(j铆)妯�(bi膩o)婧�(zh菙n)(CTCAEversion5.0锛岃〃5)鐐轰緷鎿�(j霉)?锛涱嚖鐔愭槀缂额嫗姘夆拋閳緕娓�(c猫)蹇�銆佽倽銆佽厧鍔熻兘鍜岃伣(t墨ng)鍔�銆備笝姘ㄩ吀杞�(zhu菐n)姘ㄩ叾(ALT)闇€灏忎簬姝ｅ父鍊�2鍊�锛岀附鑶界磪绱犲皬浜庢甯稿€�1.5鍊嶃€傝厧鍔熻兘涓嶅叏鏅�(sh铆)鎸夎倢閰愭竻闄ょ巼(Ccr)闄嶄綆姣斾緥鐩告噳(y墨ng)娓涢噺銆�

(6)甯歌(gu墨)鍙ｆ湇寰�(f霉)鏂规柊璜炬槑25mg/(kg·d),2娆�/d锛屾渶澶ч噺0.48g/娆�锛�2娆�/澶╋紝鏈�3d/鍛�锛屽寲鐧傞枊(k膩i)濮嬬洿鍒板寲鐧傜祼(ji茅)鏉熷悗3鍊�(g猫)鏈堛€�

2.鍚勫嵄闅�(xi菐n)搴﹀垎绲勬柟妗�

(1)浣庡嵄绲勬柟妗堬細

VAC鏂规(V锛氶暦(zh菐ng)鏄ユ柊鍫�+A锛氭斁绶氳弻绱燚+C锛氱挵(hu谩n)纾烽叞鑳�);VA鏂规(V锛氶暦(zh菐ng)鏄ユ柊鍫�+A锛氭斁绶氳弻绱燚);闀�(zh菐ng)鏄ユ柊鍫匡細1.5mg/m²,闈滆剤婊存敞,d1,d8,d15;鏀剧窔鑿岀礌D锛�0.045mg/(kg·娆�)+鐢熺悊楣芥按5min,d1;鐠�(hu谩n)纾烽叞鑳猴細1.2g/m²,1h,闈滆剤婊存敞锛宒1銆�2-宸板熀涔欏熀纾洪吀閳�(Mesna)360mg/(m²·娆�)+鐢熺悊楣芥按20锝�30min锛屼簬鐠�(hu谩n)纾烽叞鑳�0銆�3銆�6銆�9h,闈滆剤鎺ㄦ敞锛宒1銆�

娉細骞撮健灏忎簬12鍊�(g猫)鏈�锛屾斁绶氳弻绱燚鍔戦噺娓涘崐鎴�≤12kg鎸夐珨閲嶈▓(j矛)绠�锛屽姂閲�=楂旇〃闈㈢⿳鍔戦噺/30×楂旈噸(kg)銆傚寲鐧�4鐧傜▼鍚庡叏闈㈣⿻(p铆ng)浼�锛屽鏋滃畬鍏ㄧ珐瑙�锛�4鐧傜▼鍚庡彲鑰冩叜鍋滆棩锛岀附鐧傜▼涓嶈秴閬�(gu貌)10娆�銆�

(2)涓嵄绲勬柟妗堬細

VAC鏂规锛氬悓浣庡嵄绲勶紝≤12kg鎸夐珨閲嶈▓(j矛)绠�锛屽姂閲�=楂旇〃闈㈢⿳鍔戦噺/30×楂旈噸(kg)銆俈I鏂规(V锛氶暦(zh菐ng)鏄ユ柊鍫�+I锛氫紛绔嬫浛搴�)闀�(zh菐ng)鏄ユ柊鍫垮悓浣庡嵄绲�锛屼紛绔嬫浛搴凤細50mg/m²锛宒1锝瀌5锛孷CR鍚庨潨鑴堟淮娉�90min;鍠鏈€澶ч噺≤100mg/d锛屼紛绔嬫浛搴锋湁鍤�(y谩n)閲嶇矑绱�(x矛)鑳炴笡灏戝拰鑵圭€夌瓑涓嶈壇鍙嶆噳(y墨ng)锛屾湁姊濅欢鑰呭湪鍖栫檪鍓嶆浠惰€呭彲鍋歎GT1A1鍩哄洜妾㈡脯(c猫)銆�

娉細VAC鍜孷I鏂规鍙氦鏇块€�(j矛n)琛�銆傚叏閮ㄥ寲鐧傚湪瀹屽叏绶╄В鍚�4锝�6鍊�(g猫)鐧傜▼鍙€冩叜鍋滆棩锛岀附鐧傜▼鏁�(sh霉)鏈€澶氱偤13鍊�(g猫)鐧傜▼(42鍛�)锛岃秴閬�(gu貌)12鍊�(g猫)鐧傜▼鏅�(sh铆)鑰冩叜鍊�(g猫)楂斿寲瑾�(di脿o)鏁存柟妗�?锛涱嚖?2鍛ㄧ槫鐏惰⿻(p铆ng)浼拌檿浜嶱D(鑵槫澧炲ぇ鎴栧嚭鐝�(xi脿n)鏂扮梾鐏�)鍓囧嚭绲�锛屽彲鑰冩叜閫犺骞茬窗(x矛)鑳炵Щ妞�銆�

(3)楂樺嵄绲勬柟妗堬細

VAC銆乂I鏂规锛氬悓涓嵄绲�锛�≤12kg鎸夐珨閲嶈▓(j矛)绠�锛屽姂閲�=楂旇〃闈㈢⿳鍔戦噺/30×楂旈噸(kg)銆俈DC(V锛氶暦(zh菐ng)鏄ユ柊鍫�+D锛氶樋闇夌礌+C锛氱挵(hu谩n)纾烽叞鑳�)/IE(I锛氱暟鐠�(hu谩n)纾烽叞鑳�+E:渚濇墭娉婅嫹)鏂规銆傞暦(zh菐ng)鏄ユ柊鍫匡細鍚屼綆鍗辩祫;闃块湁绱狅細30mg/m²锛宒1锝瀌2;鐠�(hu谩n)纾烽叞鑳猴細1.2g/銕�锛�1h锛宒1;鐣扮挵(hu谩n)纾烽叞鑳�1.8g/m²锛宒1锝瀌5;渚濇墭娉婅嫹锛�100mg/m²锛宒1锝瀌5;2-宸板熀涔欏熀纾洪吀閳�(Mesna)360mg/(銕�·娆�)+鐢熺悊楣芥按20锝�30min锛岀挵(hu谩n)纾烽叞鑳�/鐣扮挵(hu谩n)纾烽叞鑳�0銆�3銆�6銆�9h,d1銆�

娉細浠ヤ笂鍖栫檪鏂规寤鸿琛�(sh霉)鍓嶄互VAC鍜孷I浜ゆ浛鐐轰富銆傝(sh霉)鍚庝互VDC鍜孖E浜ゆ浛鐐轰富銆傛斁鐧傛湡闁撳缓璀版噳(y墨ng)鐢╒I鏂规銆傚叏閮ㄥ寲鐧傚湪54鍛ㄥ畬鎴愶紝绺界檪绋嬫暩(sh霉)瓒呴亷(gu貌)12鍊�(g猫)鐧傜▼鏅�(sh铆)鍙€冩叜鍊�(g猫)楂斿寲瑾�(di脿o)鏁存柟妗�銆傚寲鐧�12鍛ㄥ悗瑭�(p铆ng)浼拌檿浜嶱D(鑵槫澧炲ぇ鎴栧嚭鐝�(xi脿n)鏂扮梾鐏�)鍓囧嚭绲�锛屽彲鑰冩叜骞茬窗(x矛)鑳炵Щ妞嶃€�

涓�銆佹不鐧傜浉闂�(gu膩n)姣掑壇鍙嶆噳(y墨ng)鐩�(ji膩n)娓�(c猫)鍙婅紨鍔╂不鐧�

(涓€)钘ョ墿杩戞湡姣掓€х洠(ji膩n)娓�(c猫)鍙婅紨鍔╂不鐧�

鍖栫檪钘ョ墿杩戞湡姣掓€т富瑕佽〃鐝�(xi脿n)鍦ㄨ娑层€佸績銆佽偤銆佽倽銆佽厧鍜岃叇绛�锛岃伅(li谩n)鍚堢敤钘ユ檪(sh铆)锛屾噳(y墨ng)娉ㄦ剰鍔犲挤(qi谩ng)灏�(du矛)鑵槫绱�(x矛)鑳炵殑娈哄偡鍔涜€屼笉澧炲姞钘ョ墿鐨勬瘨鎬т綔鐢�銆�

鍦ㄧ敤钘ラ亷(gu貌)绋嬩腑锛岄櫎娉ㄦ剰澧炲挤(qi谩ng)鐕�(y铆ng)椁�(y菐ng)锛岃(b菙)鍏呯董鐢熺礌锛岄槻娌绘按鍜岄浕瑙ｈ唱(zh矛)绱婁簜锛岀煰姝ｈ钵琛€锛屾帶鍒跺拰閬垮厤浜ゅ弶鎰熸煋澶栧苟鏍规摎(j霉)鍖栫檪钘ョ墿鎬ユ€у強浜炴€ユ€ф瘨鎬у弽鎳�(y墨ng)锛屽楠ㄩ珦鎶戝埗鍙婅儍鑵搁亾鍙嶆噳(y墨ng)绛�锛屽姞寮�(qi谩ng)鏀寔鍜屽皪(du矛)鐧ユ不鐧�銆傛湁鐨勮棩鐗╅倓鏈夌壒鐣版€у壇浣滅敤锛屽闀�(zh菐ng)鏄ユ柊鍫垮彲寮曡捣鑵搁夯鐥瑰拰渚跨锛岄樋闇夌礌鍙紩璧峰績鑷熸悕瀹�锛屽潎闋堟敞鎰忔娓�(c猫)鍜岄爯(y霉)闃�銆�

1.蹇冭嚐姣掓€�:涓昏鎸囪捊鐠�(hu谩n)椤炶棩鐗╃殑蹇冭嚐姣掓€э紝鍖呮嫭鎬ユ€у績鑲屾悕鍌峰拰鎱㈡€у績鍔熻兘鎼嶅銆傚墠鑰呯偤鐭毇鑰屽彲閫嗙殑蹇冭倢灞€閮ㄧ己琛€锛屽彲琛ㄧ従(xi脿n)鐐哄績鎱�銆佹埃鐭�銆佽兏鎮�銆佸績鍓嶅崁(q奴)涓嶉仼绛�;鍚庤€呯偤涓嶅彲閫嗙殑鍏呰鎬у績鍔涜“绔紝鑸囪棩鐗╃疮绌嶅姂閲忕浉闂�(gu膩n)銆�

涓€鏃﹀績鍔熻兘妾㈡脯(c猫)鎻愮ず蹇冭嚐灏勮鍒�?j菒n)?sh霉)<55%鎴栬桓绺煭鍒�?j菒n)?sh霉)<28%锛岃嫢鑳借瓑鏄庡乏蹇冨姛鑳界暟甯稿拰绱�(x矛)鑿屾劅鏌撴湁闂�(gu膩n)锛屽彲浠ョ辜绾�(x霉)浣跨敤钂界挵(hu谩n)椤炴姉鐢熺礌锛屽惁鍓囨噳(y墨ng)瑭叉毇鍋�锛岀洿鍒板皠琛€鍒�?j菒n)?sh霉)≥55%鎴栬桓绺煭鍒�?j菒n)?sh霉)≥28%銆傛牴鎿�(j霉)钂界挵(hu谩n)椤炶棩鐗╀娇鐢ㄥ姂閲忔垨蹇冭倢鎼嶅偡绋嬪害閬告搰鍙充笝浜炶兒(Zinecard)銆佸乏鏃嬭倝鍫�銆佽兘閲忓悎鍔戠瓑钘ョ墿銆�

2.杞�(zhu菐n)姘ㄩ叾鍗囬珮锛欰LT/AST閬�(d谩)姝ｅ父楂橀檺10鍊嶆垨浠ヤ笂鏅�(sh铆)寤剁珐鍖栫檪锛�1鍛ㄥ悗浠嶆湁鐣板父鑰呭彲浠ュ湪鍤�(y谩n)瀵嗚瀵熶笅鍖栫檪銆傞暦(zh菐ng)鏄ユ柊鍫匡細闀�(zh菐ng)鏄ユ柊鍫垮姂閲忎笉寰楄秴閬�(gu貌)2mg銆傚父瑕�(ji脿n)鐨勮紩搴︽瘨鎬ф湁涓嬮牅鐤肩棝銆佷究绉樸€佹繁鍙嶅皠娓涘急銆傛湁鏅�(sh铆)鍙互鏈夌櫦(f膩)鑱查殰绀欍€�

鍑虹従(xi脿n)鐩茶吀鐐庤€呮噳(y墨ng)鍋滆棩銆傞憭浜庣洰鍓嶇劇(w煤)闀�(zh菐ng)鏄ュ湴杈涜垏闀�(zh菐ng)鏄ユ柊鍫垮湪娌荤檪鐧傛晥鐨勯毃姗�(j墨)灏�(du矛)鐓�?q菒ng)?b脿o)鍛�锛岄櫎浠ヤ笂鎯呮硜澶栧缓璀伴閬稿湅(gu贸)闅涘叕瑾�(r猫n)钘ョ墿闀�(zh菐ng)鏄ユ柊鍫�銆�

3.闋�(y霉)闃插崱姘忚偤鍥婅煵(ch贸ng)鎰熸煋銆傚缓璀伴暦(zh菐ng)鏈熸湇鐢ㄥ京(f霉)鏂圭：鑳虹敳鍣佸攽鐗�(SMZco)闋�(y霉)闃插崱姘忚偤鍥婅煵(ch贸ng)鎰熸煋锛�25mg/(kg·d)锛屽垎鍏╂锛屾渶澶у姂閲忔瘡娆�0.5锛孊id锛屾瘡鍛�3澶┿€傜洿鍖栫檪绲�(ji茅)鏉熷悗2锝�3鍊�(g猫)鏈�銆�

4.琛€鍒跺搧杓告敞銆傝钵琛€锛氫竴鑸彲浠ラ€氶亷(gu貌)杓告敞绱呯窗(x矛)鑳炵珐瑙ｈ锛岃绱呰泲鐧�<60g/L蹇呴爤杓告敞;琛€灏忔澘娓涘皯锛氳灏忔澘瑷�(j矛)鏁�(sh霉)<20×10⁹/L鏅�(sh铆)鎳�(y墨ng)杓告敞琛€灏忔澘锛屼即鏈夋槑椤嚭琛€鐧ョ媭鎴栨劅鏌撹〃鐝�(xi脿n)鏅�(sh铆)杓告敞鎸囧緛鍙仼鐣�(d膩ng)鏀惧銆�

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