導語：Alagille綜合征圍手(shou)術期護(hu)理(li)研究一(yi)文(wen)來源于網(wang)友上傳，不(bu)代(dai)表本站觀點，若需要原創(chuang)文(wen)章可咨詢(xun)客服老師(shi)，歡迎(ying)參考。

摘要:總結10例alagille綜合征患兒行肝移植術的圍手術期護理經(jing)(jing)驗。護(hu)(hu)理(li)要點包括:多(duo)(duo)器(qi)官功能(neng)評(ping)估,做好(hao)術(shu)前護(hu)(hu)理(li);早期營養(yang)評(ping)估,啟(qi)動個(ge)體化(hua)營養(yang)干預(yu);精準容量管理(li),保證(zheng)臟器(qi)最佳灌注(zhu);采(cai)取多(duo)(duo)形式隔(ge)離(li)措施,加強(qiang)感染預(yu)防與控制(zhi);實施預(yu)見性心(xin)理(li)護(hu)(hu)理(li),緩解患兒不良(liang)情(qing)緒(xu);采(cai)用多(duo)(duo)模式隨訪(fang)(fang)管理(li),提供(gong)延續護(hu)(hu)理(li)服務(wu)。經(jing)(jing)過精心(xin)的治療護(hu)(hu)理(li),10例(li)患兒均康復出(chu)院,隨訪(fang)(fang)2個(ge)月至(zhi)1年,恢(hui)復良(liang)好(hao)。

關鍵詞:Alagille綜合(he)征;肝(gan)移植;先天性(xing)心臟病;皮(pi)膚瘙癢;生長發育(yu);營養;圍手術期(qi)護(hu)理;兒科護(hu)理

Alagille綜合征是一種復雜的累及多系統的常染色體顯性遺傳性疾病,隨著家系研究和基因檢測的(de)開展,近年來報(bao)道病例逐(zhu)漸增多,人群(qun)患(huan)(huan)病率可(ke)達1/50000~1/30000[1]。Alagille綜(zong)合(he)征(zheng)的(de)臨床表現多樣,常(chang)累及多個器(qi)官,膽汁淤積發(fa)生率高(gao)(92%~100%),其余包(bao)括先(xian)天(tian)性心臟病、特殊面容、蝴蝶椎及眼(yan)部異(yi)常(chang),還可(ke)有腎臟異(yi)常(chang)、生長發(fa)育(yu)遲緩(huan)、胰(yi)腺(xian)異(yi)常(chang)等[2]。目前(qian)主(zhu)要(yao)是內科對癥支持治療[1]。但對出現慢性肝衰竭、頑固性皮膚(fu)瘙癢(yang)、生長發(fa)育(yu)顯著落(luo)后及反復骨折者(zhe),肝移(yi)(yi)(yi)(yi)植是當前(qian)治療的(de)唯一有效方法(fa)[3-4]。研究(jiu)表明,多達70%的(de)Alagille綜(zong)合(he)征(zheng)患(huan)(huan)者(zhe)在(zai)兒(er)(er)童(tong)時期(qi)(qi)需要(yao)肝移(yi)(yi)(yi)(yi)植[5]。由于兒(er)(er)童(tong)肝移(yi)(yi)(yi)(yi)植手(shou)術(shu)風險高(gao),并發(fa)癥多,圍術(shu)期(qi)(qi)護(hu)理對保(bao)障(zhang)手(shou)術(shu)效果(guo)與促進患(huan)(huan)兒(er)(er)康復至關重要(yao)。我院2021年3月至2022年1月收治10例Alagille綜(zong)合(he)征(zheng)行肝移(yi)(yi)(yi)(yi)植術(shu)患(huan)(huan)兒(er)(er),現將(jiang)圍手(shou)術(shu)期(qi)(qi)護(hu)理介紹如下。

1臨床資料

本組10例(li)(li)(li),男(nan)4例(li)(li)(li),女6例(li)(li)(li);年齡6個(ge)月至(zhi)12歲,中(zhong)(zhong)(zhong)位年齡28個(ge)月。10例(li)(li)(li)均(jun)因膽汁淤積皮(pi)(pi)(pi)膚鞏膜(mo)黃染加重入院(yuan)。其中(zhong)(zhong)(zhong)1例(li)(li)(li)胸椎呈蝴蝶椎畸形,1例(li)(li)(li)黃色素瘤,2例(li)(li)(li)角膜(mo)后(hou)胚胎環,2例(li)(li)(li)曾行腹(fu)腔(qiang)鏡下膽道(dao)探(tan)查術(shu)。3例(li)(li)(li)已進展為肝(gan)硬(ying)化,4例(li)(li)(li)特(te)殊面容,6例(li)(li)(li)皮(pi)(pi)(pi)膚瘙癢(yang)伴皮(pi)(pi)(pi)疹(zhen),6例(li)(li)(li)生長(chang)(chang)發(fa)(fa)育遲緩(huan)。9例(li)(li)(li)先天性(xing)心(xin)臟(zang)病,其中(zhong)(zhong)(zhong)6例(li)(li)(li)肺動脈狹窄(zhai)、4例(li)(li)(li)動脈導(dao)管未(wei)閉、1例(li)(li)(li)房間(jian)隔缺損(sun)、1例(li)(li)(li)室間(jian)隔缺損(sun)、1例(li)(li)(li)法洛四聯征、1例(li)(li)(li)三(san)尖瓣關閉不全行心(xin)臟(zang)矯正(zheng)術(shu)。入院(yuan)后(hou)進行營養評(ping)估,給予營養支持(chi),完善心(xin)肺功(gong)能(neng)(neng)、肝(gan)功(gong)能(neng)(neng)及全身臟(zang)器(qi)功(gong)能(neng)(neng)評(ping)估,經多學(xue)科聯合會診,確定手術(shu)方案。其中(zhong)(zhong)(zhong)劈裂式(shi)肝(gan)移植5例(li)(li)(li),經典原(yuan)位肝(gan)移植3例(li)(li)(li),減(jian)體(ti)積肝(gan)移植1例(li)(li)(li),活(huo)體(ti)肝(gan)移植1例(li)(li)(li)。經過移植團隊(dui)的精心(xin)護理,10例(li)(li)(li)患兒均(jun)康復出(chu)院(yuan),住院(yuan)時間(jian)21~60d。隨(sui)訪2個(ge)月至(zhi)1年,肝(gan)功(gong)能(neng)(neng)均(jun)正(zheng)常,皮(pi)(pi)(pi)膚瘙癢(yang)、皮(pi)(pi)(pi)疹(zhen)均(jun)消失,未(wei)出(chu)現心(xin)功(gong)能(neng)(neng)衰竭、感染等并發(fa)(fa)癥,其中(zhong)(zhong)(zhong)1例(li)(li)(li)黃色素瘤體(ti)積逐漸減(jian)小(xiao),5例(li)(li)(li)生長(chang)(chang)發(fa)(fa)育出(chu)現明(ming)顯追(zhui)長(chang)(chang)。

2護理

2.1術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)2.1.1多(duo)器(qi)(qi)官(guan)(guan)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu),做好(hao)(hao)(hao)(hao)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)Alagille綜(zong)合(he)(he)征(zheng)常(chang)(chang)(chang)包括肝(gan)(gan)(gan)(gan)(gan)臟(zang)(zang)(zang)(zang)、心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)、骨(gu)骼、眼(yan)部(bu)(bu)(bu)(bu)異(yi)常(chang)(chang)(chang)及(ji)(ji)特(te)(te)殊面(mian)容(rong)五(wu)大(da)(da)臨(lin)床特(te)(te)征(zheng),臨(lin)床表現(xian)(xian)從(cong)沒(mei)有(you)(you)(you)(you)癥狀(zhuang)(zhuang)(zhuang)到(dao)危及(ji)(ji)生(sheng)(sheng)命的(de)(de)(de)(de)(de)(de)(de)情(qing)(qing)(qing)況(kuang)(kuang)均(jun)(jun)(jun)可發(fa)(fa)(fa)(fa)(fa)生(sheng)(sheng)[6]。因此(ci)(ci),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)多(duo)器(qi)(qi)官(guan)(guan)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)尤其(qi)重(zhong)(zhong)要(yao)(yao)(yao)(yao)。①肝(gan)(gan)(gan)(gan)(gan)膽(dan)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu):患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)入(ru)(ru)院(yuan)(yuan)(yuan)2d內(nei)(nei)完善肝(gan)(gan)(gan)(gan)(gan)膽(dan)B超(chao)(chao)(chao)、肝(gan)(gan)(gan)(gan)(gan)臟(zang)(zang)(zang)(zang)CT增(zeng)(zeng)強(qiang)(qiang)、臟(zang)(zang)(zang)(zang)器(qi)(qi)灰階立體(ti)(ti)(ti)(ti)成(cheng)(cheng)像(xiang)、生(sheng)(sheng)化(hua)(hua)(hua)、凝血(xue)(xue)(xue)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)、血(xue)(xue)(xue)常(chang)(chang)(chang)規(gui)等(deng)(deng)(deng)(deng)(deng)檢(jian)查(cha)。每(mei)(mei)日(ri)(ri)系(xi)統評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)有(you)(you)(you)(you)無(wu)腹(fu)(fu)(fu)(fu)脹、腹(fu)(fu)(fu)(fu)痛、惡(e)(e)心(xin)(xin)(xin)(xin)(xin)(xin)嘔吐、納(na)差(cha)等(deng)(deng)(deng)(deng)(deng)情(qing)(qing)(qing)況(kuang)(kuang)。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)入(ru)(ru)院(yuan)(yuan)(yuan)時(shi)(shi)神(shen)志淡漠,皮(pi)(pi)膚(fu)(fu)(fu)鞏膜黃(huang)染(ran)(ran)明(ming)(ming)顯(xian)(xian),急(ji)診(zhen)(zhen)查(cha)總(zong)(zong)膽(dan)紅(hong)素(su)485μmol/L,血(xue)(xue)(xue)氨(an)238μmol/L,立即(ji)予(yu)護(hu)(hu)(hu)(hu)(hu)肝(gan)(gan)(gan)(gan)(gan)退黃(huang)、降(jiang)(jiang)(jiang)血(xue)(xue)(xue)氨(an)、預(yu)(yu)防(fang)感(gan)(gan)染(ran)(ran)等(deng)(deng)(deng)(deng)(deng)治療(liao)(liao),1d后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)未見明(ming)(ming)顯(xian)(xian)好(hao)(hao)(hao)(hao)轉,行(xing)(xing)(xing)(xing)(xing)(xing)人(ren)工肝(gan)(gan)(gan)(gan)(gan)治療(liao)(liao)2次后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)精(jing)神(shen)好(hao)(hao)(hao)(hao)轉,總(zong)(zong)膽(dan)紅(hong)素(su)降(jiang)(jiang)(jiang)至(zhi)205μmol/L,血(xue)(xue)(xue)氨(an)降(jiang)(jiang)(jiang)至(zhi)86μmol/L。②心(xin)(xin)(xin)(xin)(xin)(xin)肺(fei)(fei)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu):由(you)于(yu)(yu)目(mu)前(qian)(qian)(qian)尚無(wu)根據(ju)(ju)Alagille綜(zong)合(he)(he)征(zheng)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)異(yi)常(chang)(chang)(chang)嚴重(zhong)(zhong)程(cheng)(cheng)度判斷能(neng)(neng)(neng)(neng)否(fou)行(xing)(xing)(xing)(xing)(xing)(xing)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)的(de)(de)(de)(de)(de)(de)(de)具(ju)(ju)(ju)體(ti)(ti)(ti)(ti)標準(zhun),因此(ci)(ci)更需(xu)要(yao)(yao)(yao)(yao)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)精(jing)準(zhun)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)畸(ji)形(xing)對(dui)(dui)(dui)(dui)(dui)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)的(de)(de)(de)(de)(de)(de)(de)影(ying)響(xiang),以(yi)(yi)進(jin)(jin)(jin)(jin)(jin)一步(bu)提(ti)高(gao)(gao)(gao)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)療(liao)(liao)效(xiao)[7]。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)入(ru)(ru)院(yuan)(yuan)(yuan)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)綜(zong)合(he)(he)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)心(xin)(xin)(xin)(xin)(xin)(xin)肺(fei)(fei)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)發(fa)(fa)(fa)(fa)(fa)現(xian)(xian),本(ben)(ben)組(zu)(zu)(zu)(zu)9例(li)(li)(li)(li)(li)(li)(li)(li)伴(ban)(ban)有(you)(you)(you)(you)不(bu)(bu)(bu)(bu)同(tong)程(cheng)(cheng)度的(de)(de)(de)(de)(de)(de)(de)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)病(bing)(bing)。采用(yong)(yong)(yong)(yong)改良(liang)Ross評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)[8],4~6分(fen)(fen)(fen)(fen)(fen)(fen)輕(qing)度心(xin)(xin)(xin)(xin)(xin)(xin)力(li)(li)(li)(li)衰(shuai)竭(jie)7例(li)(li)(li)(li)(li)(li)(li)(li),>7分(fen)(fen)(fen)(fen)(fen)(fen)中(zhong)度心(xin)(xin)(xin)(xin)(xin)(xin)力(li)(li)(li)(li)衰(shuai)竭(jie)2例(li)(li)(li)(li)(li)(li)(li)(li)。其(qi)中(zhong)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)彩(cai)色(se)(se)多(duo)普勒超(chao)(chao)(chao)聲示:左(zuo)肺(fei)(fei)動(dong)脈(mo)(mo)(mo)壓(ya)65mmHg;哭(ku)鬧時(shi)(shi)易(yi)出(chu)現(xian)(xian)口(kou)唇發(fa)(fa)(fa)(fa)(fa)紺(gan),甚至(zhi)暈厥,予(yu)持(chi)(chi)續(xu)低(di)(di)(di)流(liu)量(liang)(liang)(liang)(liang)(liang)(liang)氧(yang)療(liao)(liao),并(bing)在(zai)(zai)(zai)全(quan)麻下(xia)行(xing)(xing)(xing)(xing)(xing)(xing)右心(xin)(xin)(xin)(xin)(xin)(xin)漂(piao)浮導管(guan)(guan)(guan)檢(jian)查(cha)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)。請(qing)心(xin)(xin)(xin)(xin)(xin)(xin)胸(xiong)外科(ke)(ke)(ke)、兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)科(ke)(ke)(ke)、麻醉科(ke)(ke)(ke)聯(lian)合(he)(he)會(hui)診(zhen)(zhen)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)風(feng)(feng)險(xian)(xian),制訂(ding)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)方(fang)案。③皮(pi)(pi)膚(fu)(fu)(fu)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li):本(ben)(ben)組(zu)(zu)(zu)(zu)6例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)皮(pi)(pi)膚(fu)(fu)(fu)瘙(sao)癢明(ming)(ming)顯(xian)(xian),嚴重(zhong)(zhong)降(jiang)(jiang)(jiang)低(di)(di)(di)其(qi)生(sheng)(sheng)活質(zhi)(zhi)量(liang)(liang)(liang)(liang)(liang)(liang)。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)入(ru)(ru)院(yuan)(yuan)(yuan)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)修(xiu)剪(jian)指(zhi)甲,使(shi)(shi)(shi)用(yong)(yong)(yong)(yong)防(fang)抓手(shou)(shou)(shou)套(tao),瘙(sao)癢發(fa)(fa)(fa)(fa)(fa)作時(shi)(shi),用(yong)(yong)(yong)(yong)爐甘(gan)石(shi)洗劑濕敷,陪同(tong)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)玩(wan)(wan)耍,用(yong)(yong)(yong)(yong)其(qi)喜歡的(de)(de)(de)(de)(de)(de)(de)玩(wan)(wan)具(ju)(ju)(ju)分(fen)(fen)(fen)(fen)(fen)(fen)散注(zhu)(zhu)(zhu)意力(li)(li)(li)(li)。指(zhi)導家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)每(mei)(mei)日(ri)(ri)早(zao)晚(wan)用(yong)(yong)(yong)(yong)溫水(shui)(shui)給患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)擦拭(shi)皮(pi)(pi)膚(fu)(fu)(fu),涂抹潤(run)膚(fu)(fu)(fu)油,更換棉質(zhi)(zhi)寬松衣物,保(bao)持(chi)(chi)皮(pi)(pi)膚(fu)(fu)(fu)清(qing)潔滋潤(run)。每(mei)(mei)日(ri)(ri)觀(guan)(guan)察(cha)記錄患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)皮(pi)(pi)膚(fu)(fu)(fu)情(qing)(qing)(qing)況(kuang)(kuang)。同(tong)時(shi)(shi)予(yu)消(xiao)(xiao)(xiao)膽(dan)胺(an)口(kou)服(fu)。6例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)瘙(sao)癢較前(qian)(qian)(qian)好(hao)(hao)(hao)(hao)轉。④眼(yan)部(bu)(bu)(bu)(bu)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li):2例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)角(jiao)膜后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)胚胎環(huan)(huan)伴(ban)(ban)視盤(pan)玻璃疣,觀(guan)(guan)察(cha)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)是(shi)否(fou)存在(zai)(zai)(zai)眼(yan)痛、視力(li)(li)(li)(li)障(zhang)礙、分(fen)(fen)(fen)(fen)(fen)(fen)泌物多(duo)等(deng)(deng)(deng)(deng)(deng)情(qing)(qing)(qing)況(kuang)(kuang)。宣教家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)不(bu)(bu)(bu)(bu)用(yong)(yong)(yong)(yong)電(dian)(dian)子(zi)產品(pin)吸引(yin)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er),哭(ku)鬧難(nan)哄時(shi)(shi),連(lian)續(xu)看(kan)(kan)視頻(pin)時(shi)(shi)間(jian)(jian)不(bu)(bu)(bu)(bu)能(neng)(neng)(neng)(neng)超(chao)(chao)(chao)過半小(xiao)時(shi)(shi)。進(jin)(jin)(jin)(jin)(jin)食(shi)(shi)富含(han)(han)維(wei)生(sheng)(sheng)素(su)A等(deng)(deng)(deng)(deng)(deng)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)眼(yan)神(shen)經(jing)(jing)(jing)的(de)(de)(de)(de)(de)(de)(de)食(shi)(shi)物,如(ru)小(xiao)米、瘦肉、胡蘿卜等(deng)(deng)(deng)(deng)(deng)。目(mu)前(qian)(qian)(qian),2例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)視力(li)(li)(li)(li)檢(jian)查(cha)正(zheng)(zheng)常(chang)(chang)(chang),但仍(reng)需(xu)眼(yan)科(ke)(ke)(ke)定(ding)期(qi)(qi)檢(jian)測(ce)(ce)(ce)(ce)以(yi)(yi)篩(shai)(shai)(shai)查(cha)疾病(bing)(bing)進(jin)(jin)(jin)(jin)(jin)展(zhan)。⑤骨(gu)骼護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li):大(da)(da)多(duo)數Alagille綜(zong)合(he)(he)征(zheng)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)具(ju)(ju)(ju)有(you)(you)(you)(you)特(te)(te)征(zheng)性(xing)(xing)(xing)(xing)(xing)的(de)(de)(de)(de)(de)(de)(de)面(mian)容(rong)和(he)蝴蝶椎(zhui)(zhui)骨(gu),骨(gu)質(zhi)(zhi)減少(shao)和(he)骨(gu)折風(feng)(feng)險(xian)(xian)隨(sui)(sui)著患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)年(nian)齡(ling)增(zeng)(zeng)長(chang)而增(zeng)(zeng)加(jia)(jia)(jia)[9]。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)12歲(sui)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)胸(xiong)椎(zhui)(zhui)呈蝴蝶椎(zhui)(zhui)畸(ji)形(xing)伴(ban)(ban)低(di)(di)(di)血(xue)(xue)(xue)鈣,醫(yi)囑予(yu)葡(pu)萄糖酸鈣及(ji)(ji)維(wei)生(sheng)(sheng)素(su)D口(kou)服(fu)。同(tong)時(shi)(shi)對(dui)(dui)(dui)(dui)(dui)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)跌倒風(feng)(feng)險(xian)(xian)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu),提(ti)高(gao)(gao)(gao)室(shi)內(nei)(nei)環(huan)(huan)境安(an)全(quan)性(xing)(xing)(xing)(xing)(xing),對(dui)(dui)(dui)(dui)(dui)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)加(jia)(jia)(jia)強(qiang)(qiang)宣教。本(ben)(ben)組(zu)(zu)(zu)(zu)10例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)均(jun)(jun)(jun)順利進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)。2.1.2早(zao)期(qi)(qi)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu),啟動(dong)個(ge)體(ti)(ti)(ti)(ti)化(hua)(hua)(hua)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)干(gan)(gan)(gan)(gan)預(yu)(yu)慢性(xing)(xing)(xing)(xing)(xing)肝(gan)(gan)(gan)(gan)(gan)病(bing)(bing)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)處于(yu)(yu)高(gao)(gao)(gao)能(neng)(neng)(neng)(neng)量(liang)(liang)(liang)(liang)(liang)(liang)代謝狀(zhuang)(zhuang)(zhuang)態,營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)攝入(ru)(ru)不(bu)(bu)(bu)(bu)足,且肝(gan)(gan)(gan)(gan)(gan)臟(zang)(zang)(zang)(zang)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)性(xing)(xing)(xing)(xing)(xing)惡(e)(e)化(hua)(hua)(hua),大(da)(da)大(da)(da)增(zeng)(zeng)加(jia)(jia)(jia)了感(gan)(gan)染(ran)(ran)和(he)死亡的(de)(de)(de)(de)(de)(de)(de)風(feng)(feng)險(xian)(xian)[10]。術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)干(gan)(gan)(gan)(gan)預(yu)(yu)旨在(zai)(zai)(zai)改善患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)狀(zhuang)(zhuang)(zhuang)況(kuang)(kuang),提(ti)高(gao)(gao)(gao)其(qi)對(dui)(dui)(dui)(dui)(dui)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)創(chuang)傷(shang)耐受(shou)性(xing)(xing)(xing)(xing)(xing),避免(mian)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)并(bing)發(fa)(fa)(fa)(fa)(fa)癥[11]。本(ben)(ben)組(zu)(zu)(zu)(zu)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)采用(yong)(yong)(yong)(yong)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)狀(zhuang)(zhuang)(zhuang)況(kuang)(kuang)和(he)生(sheng)(sheng)長(chang)風(feng)(feng)險(xian)(xian)篩(shai)(shai)(shai)查(cha)工具(ju)(ju)(ju)(STRONGkids)[12]進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)狀(zhuang)(zhuang)(zhuang)況(kuang)(kuang)篩(shai)(shai)(shai)查(cha),0分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)低(di)(di)(di)風(feng)(feng)險(xian)(xian),1~3分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)中(zhong)風(feng)(feng)險(xian)(xian),4~5分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)高(gao)(gao)(gao)風(feng)(feng)險(xian)(xian)。在(zai)(zai)(zai)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)入(ru)(ru)院(yuan)(yuan)(yuan)8h內(nei)(nei)、手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)前(qian)(qian)(qian)、手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)及(ji)(ji)時(shi)(shi)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)風(feng)(feng)險(xian)(xian)篩(shai)(shai)(shai)查(cha),并(bing)每(mei)(mei)周(zhou)(zhou)(zhou)復評(ping)(ping)(ping)(ping)(ping)。入(ru)(ru)院(yuan)(yuan)(yuan)時(shi)(shi)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)風(feng)(feng)險(xian)(xian)篩(shai)(shai)(shai)查(cha)發(fa)(fa)(fa)(fa)(fa)現(xian)(xian)4例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)4分(fen)(fen)(fen)(fen)(fen)(fen)、4例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)3分(fen)(fen)(fen)(fen)(fen)(fen)、2例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)為(wei)(wei)2分(fen)(fen)(fen)(fen)(fen)(fen);9例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)BMI為(wei)(wei)12.89~14.63,低(di)(di)(di)于(yu)(yu)正(zheng)(zheng)常(chang)(chang)(chang)值(zhi),只有(you)(you)(you)(you)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)BMI為(wei)(wei)18.25,在(zai)(zai)(zai)正(zheng)(zheng)常(chang)(chang)(chang)范圍。然后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)評(ping)(ping)(ping)(ping)(ping)定(ding),包括人(ren)體(ti)(ti)(ti)(ti)測(ce)(ce)(ce)(ce)量(liang)(liang)(liang)(liang)(liang)(liang)、實驗室(shi)指(zhi)標、環(huan)(huan)境家(jia)(jia)(jia)(jia)庭(ting)情(qing)(qing)(qing)況(kuang)(kuang)等(deng)(deng)(deng)(deng)(deng)。再請(qing)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)科(ke)(ke)(ke)會(hui)診(zhen)(zhen),協助(zhu)制訂(ding)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)方(fang)案。由(you)于(yu)(yu)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)發(fa)(fa)(fa)(fa)(fa)育落后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)正(zheng)(zheng)常(chang)(chang)(chang)同(tong)齡(ling)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)童(tong)(tong)(tong),要(yao)(yao)(yao)(yao)求(qiu)(qiu)能(neng)(neng)(neng)(neng)量(liang)(liang)(liang)(liang)(liang)(liang)攝入(ru)(ru)≥1.2倍基礎能(neng)(neng)(neng)(neng)量(liang)(liang)(liang)(liang)(liang)(liang)消(xiao)(xiao)(xiao)耗[126~145kJ/(kg·d)],葡(pu)糖糖2~3g/(kg·d),蛋白質(zhi)(zhi)1.5g/(kg·d),脂肪占能(neng)(neng)(neng)(neng)量(liang)(liang)(liang)(liang)(liang)(liang)攝入(ru)(ru)比40%~50%[10]。喂(wei)養(yang)(yang)(yang)(yang)期(qi)(qi)間(jian)(jian)觀(guan)(guan)察(cha)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)的(de)(de)(de)(de)(de)(de)(de)耐受(shou)情(qing)(qing)(qing)況(kuang)(kuang),遵從(cong)由(you)少(shao)到(dao)多(duo)、由(you)細到(dao)粗、由(you)稀到(dao)稠、由(you)一種(zhong)到(dao)多(duo)種(zhong)的(de)(de)(de)(de)(de)(de)(de)輔食(shi)(shi)添加(jia)(jia)(jia)原(yuan)則(ze),確(que)認(ren)無(wu)異(yi)常(chang)(chang)(chang)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)提(ti)升喂(wei)養(yang)(yang)(yang)(yang)劑量(liang)(liang)(liang)(liang)(liang)(liang),直(zhi)至(zhi)達到(dao)最(zui)高(gao)(gao)(gao)喂(wei)養(yang)(yang)(yang)(yang)量(liang)(liang)(liang)(liang)(liang)(liang)。對(dui)(dui)(dui)(dui)(dui)2例(li)(li)(li)(li)(li)(li)(li)(li)12歲(sui)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)采用(yong)(yong)(yong)(yong)高(gao)(gao)(gao)熱量(liang)(liang)(liang)(liang)(liang)(liang)、高(gao)(gao)(gao)維(wei)生(sheng)(sheng)素(su)、低(di)(di)(di)脂、優質(zhi)(zhi)蛋白飲(yin)食(shi)(shi),少(shao)量(liang)(liang)(liang)(liang)(liang)(liang)多(duo)餐。每(mei)(mei)次進(jin)(jin)(jin)(jin)(jin)食(shi)(shi)前(qian)(qian)(qian)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)消(xiao)(xiao)(xiao)化(hua)(hua)(hua)情(qing)(qing)(qing)況(kuang)(kuang)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)再執(zhi)行(xing)(xing)(xing)(xing)(xing)(xing)喂(wei)養(yang)(yang)(yang)(yang)計劃[13],即(ji)用(yong)(yong)(yong)(yong)手(shou)(shou)(shou)輕(qing)輕(qing)觸摸患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)腹(fu)(fu)(fu)(fu)部(bu)(bu)(bu)(bu),腹(fu)(fu)(fu)(fu)部(bu)(bu)(bu)(bu)膨脹、腹(fu)(fu)(fu)(fu)肌緊張說明(ming)(ming)有(you)(you)(you)(you)腸(chang)(chang)(chang)脹氣(qi)(qi)或(huo)消(xiao)(xiao)(xiao)化(hua)(hua)(hua)不(bu)(bu)(bu)(bu)良(liang)的(de)(de)(de)(de)(de)(de)(de)情(qing)(qing)(qing)況(kuang)(kuang),予(yu)開塞(sai)(sai)露灌(guan)腸(chang)(chang)(chang),延遲喂(wei)養(yang)(yang)(yang)(yang);若(ruo)腹(fu)(fu)(fu)(fu)部(bu)(bu)(bu)(bu)柔軟、腸(chang)(chang)(chang)鳴音正(zheng)(zheng)常(chang)(chang)(chang)則(ze)可按(an)(an)計劃喂(wei)養(yang)(yang)(yang)(yang)。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)6月齡(ling)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)腸(chang)(chang)(chang)鳴音弱,予(yu)腹(fu)(fu)(fu)(fu)部(bu)(bu)(bu)(bu)環(huan)(huan)形(xing)按(an)(an)摩(mo)方(fang)式(shi)(shi)(shi)促(cu)進(jin)(jin)(jin)(jin)(jin)胃腸(chang)(chang)(chang)蠕動(dong),每(mei)(mei)4小(xiao)時(shi)(shi)按(an)(an)摩(mo)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)腹(fu)(fu)(fu)(fu)部(bu)(bu)(bu)(bu)1次,每(mei)(mei)次15min,3d后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)腸(chang)(chang)(chang)鳴音正(zheng)(zheng)常(chang)(chang)(chang),解(jie)黃(huang)色(se)(se)糊便2~5次/d。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)出(chu)現(xian)(xian)腹(fu)(fu)(fu)(fu)瀉12次/d,予(yu)改深度水(shui)(shui)解(jie)配方(fang)奶粉(fen)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)好(hao)(hao)(hao)(hao)轉。伴(ban)(ban)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)病(bing)(bing)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)往往營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)不(bu)(bu)(bu)(bu)良(liang)更嚴重(zhong)(zhong),本(ben)(ben)組(zu)(zu)(zu)(zu)2例(li)(li)(li)(li)(li)(li)(li)(li)肺(fei)(fei)動(dong)脈(mo)(mo)(mo)高(gao)(gao)(gao)壓(ya)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)胃腸(chang)(chang)(chang)道功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)極弱,進(jin)(jin)(jin)(jin)(jin)食(shi)(shi)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)常(chang)(chang)(chang)伴(ban)(ban)惡(e)(e)心(xin)(xin)(xin)(xin)(xin)(xin)嘔吐,醫(yi)囑采用(yong)(yong)(yong)(yong)腸(chang)(chang)(chang)外營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)方(fang)式(shi)(shi)(shi),靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)滴注(zhu)(zhu)(zhu)富含(han)(han)氨(an)基酸、葡(pu)萄糖、維(wei)生(sheng)(sheng)素(su)、電(dian)(dian)解(jie)質(zhi)(zhi)等(deng)(deng)(deng)(deng)(deng)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)物質(zhi)(zhi)。經(jing)(jing)(jing)積極營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)干(gan)(gan)(gan)(gan)預(yu)(yu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou),4例(li)(li)(li)(li)(li)(li)(li)(li)高(gao)(gao)(gao)風(feng)(feng)險(xian)(xian)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)風(feng)(feng)險(xian)(xian)篩(shai)(shai)(shai)查(cha)評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)降(jiang)(jiang)(jiang)為(wei)(wei)3分(fen)(fen)(fen)(fen)(fen)(fen),4例(li)(li)(li)(li)(li)(li)(li)(li)中(zhong)風(feng)(feng)險(xian)(xian)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)風(feng)(feng)險(xian)(xian)篩(shai)(shai)(shai)查(cha)評(ping)(ping)(ping)(ping)(ping)分(fen)(fen)(fen)(fen)(fen)(fen)降(jiang)(jiang)(jiang)為(wei)(wei)1~2分(fen)(fen)(fen)(fen)(fen)(fen);BMI達到(dao)16.82~20.05。2.2術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)2.2.1精(jing)準(zhun)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)管(guan)(guan)(guan)理(li)(li)(li)(li),保(bao)證臟(zang)(zang)(zang)(zang)器(qi)(qi)最(zui)佳(jia)灌(guan)注(zhu)(zhu)(zhu)先天(tian)性(xing)(xing)(xing)(xing)(xing)心(xin)(xin)(xin)(xin)(xin)(xin)臟(zang)(zang)(zang)(zang)病(bing)(bing)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)液(ye)(ye)體(ti)(ti)(ti)(ti)管(guan)(guan)(guan)理(li)(li)(li)(li)要(yao)(yao)(yao)(yao)求(qiu)(qiu)嚴格(ge),容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)不(bu)(bu)(bu)(bu)足會(hui)導致(zhi)多(duo)器(qi)(qi)官(guan)(guan)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)損(sun)傷(shang),液(ye)(ye)體(ti)(ti)(ti)(ti)超(chao)(chao)(chao)負(fu)荷又(you)會(hui)造(zao)成(cheng)(cheng)心(xin)(xin)(xin)(xin)(xin)(xin)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)衰(shuai)竭(jie);而肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)通常(chang)(chang)(chang)全(quan)身(shen)液(ye)(ye)體(ti)(ti)(ti)(ti)超(chao)(chao)(chao)負(fu)荷,血(xue)(xue)(xue)管(guan)(guan)(guan)內(nei)(nei)可能(neng)(neng)(neng)(neng)是(shi)低(di)(di)(di)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)、等(deng)(deng)(deng)(deng)(deng)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)或(huo)高(gao)(gao)(gao)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)狀(zhuang)(zhuang)(zhuang)態[14]。因此(ci)(ci)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)精(jing)準(zhun)的(de)(de)(de)(de)(de)(de)(de)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)管(guan)(guan)(guan)理(li)(li)(li)(li)非常(chang)(chang)(chang)重(zhong)(zhong)要(yao)(yao)(yao)(yao)。本(ben)(ben)中(zhong)心(xin)(xin)(xin)(xin)(xin)(xin)采用(yong)(yong)(yong)(yong)Holliday-Segar法計算每(mei)(mei)日(ri)(ri)液(ye)(ye)體(ti)(ti)(ti)(ti)需(xu)要(yao)(yao)(yao)(yao)量(liang)(liang)(liang)(liang)(liang)(liang),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)液(ye)(ye)體(ti)(ti)(ti)(ti)總(zong)(zong)量(liang)(liang)(liang)(liang)(liang)(liang)限制在(zai)(zai)(zai)需(xu)要(yao)(yao)(yao)(yao)量(liang)(liang)(liang)(liang)(liang)(liang)的(de)(de)(de)(de)(de)(de)(de)60%~80%,要(yao)(yao)(yao)(yao)求(qiu)(qiu)尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)>1mL/(kg·h)[10]。密切(qie)監(jian)測(ce)(ce)(ce)(ce)生(sheng)(sheng)命體(ti)(ti)(ti)(ti)征(zheng)、有(you)(you)(you)(you)創(chuang)動(dong)脈(mo)(mo)(mo)血(xue)(xue)(xue)壓(ya)、中(zhong)心(xin)(xin)(xin)(xin)(xin)(xin)靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)壓(ya)、24h出(chu)入(ru)(ru)量(liang)(liang)(liang)(liang)(liang)(liang)。根據(ju)(ju)心(xin)(xin)(xin)(xin)(xin)(xin)率(lv)(lv)、血(xue)(xue)(xue)壓(ya)、中(zhong)心(xin)(xin)(xin)(xin)(xin)(xin)靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)壓(ya)、尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)、引(yin)流(liu)量(liang)(liang)(liang)(liang)(liang)(liang)、血(xue)(xue)(xue)氣(qi)(qi)分(fen)(fen)(fen)(fen)(fen)(fen)析等(deng)(deng)(deng)(deng)(deng)綜(zong)合(he)(he)評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)血(xue)(xue)(xue)管(guan)(guan)(guan)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang),隨(sui)(sui)時(shi)(shi)調(diao)整(zheng)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)補液(ye)(ye)方(fang)案。按(an)(an)醫(yi)囑控(kong)制輸液(ye)(ye)速(su)度,液(ye)(ye)體(ti)(ti)(ti)(ti)以(yi)(yi)靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)泵(beng)(beng)持(chi)(chi)續(xu)泵(beng)(beng)入(ru)(ru),靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)高(gao)(gao)(gao)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)液(ye)(ye)采用(yong)(yong)(yong)(yong)輸液(ye)(ye)泵(beng)(beng)24h勻速(su)泵(beng)(beng)入(ru)(ru)。進(jin)(jin)(jin)(jin)(jin)食(shi)(shi)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)隨(sui)(sui)著腸(chang)(chang)(chang)內(nei)(nei)營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)耐受(shou)情(qing)(qing)(qing)況(kuang)(kuang)逐步(bu)減少(shao)輸液(ye)(ye)量(liang)(liang)(liang)(liang)(liang)(liang)。本(ben)(ben)組(zu)(zu)(zu)(zu)2例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)血(xue)(xue)(xue)壓(ya)、血(xue)(xue)(xue)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)灌(guan)注(zhu)(zhu)(zhu)正(zheng)(zheng)常(chang)(chang)(chang)前(qian)(qian)(qian)提(ti)下(xia),出(chu)現(xian)(xian)煩躁哭(ku)鬧、口(kou)唇干(gan)(gan)(gan)(gan)燥(zao)伴(ban)(ban)尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)少(shao)于(yu)(yu)0.5mL/(kg·h)。醫(yi)囑予(yu)右美托咪定(ding)小(xiao)劑量(liang)(liang)(liang)(liang)(liang)(liang)靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)持(chi)(chi)續(xu)泵(beng)(beng)入(ru)(ru),避免(mian)躁動(dong)誘發(fa)(fa)(fa)(fa)(fa)肺(fei)(fei)動(dong)脈(mo)(mo)(mo)高(gao)(gao)(gao)壓(ya)危象,呋塞(sai)(sai)米3~5mg/d靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)推注(zhu)(zhu)(zhu),1d后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)逐漸增(zeng)(zeng)多(duo),2d后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)恢(hui)復正(zheng)(zheng)常(chang)(chang)(chang)。使(shi)(shi)(shi)用(yong)(yong)(yong)(yong)鎮(zhen)靜(jing)(jing)(jing)(jing)(jing)劑期(qi)(qi)間(jian)(jian)嚴密觀(guan)(guan)察(cha)血(xue)(xue)(xue)壓(ya)、呼吸、氧(yang)飽(bao)和(he)度、瞳孔(kong)的(de)(de)(de)(de)(de)(de)(de)變化(hua)(hua)(hua),防(fang)止鎮(zhen)靜(jing)(jing)(jing)(jing)(jing)劑蓄(xu)積。使(shi)(shi)(shi)用(yong)(yong)(yong)(yong)利尿(niao)(niao)劑后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)密切(qie)關(guan)注(zhu)(zhu)(zhu)尿(niao)(niao)量(liang)(liang)(liang)(liang)(liang)(liang)變化(hua)(hua)(hua),監(jian)測(ce)(ce)(ce)(ce)電(dian)(dian)解(jie)質(zhi)(zhi),使(shi)(shi)(shi)血(xue)(xue)(xue)鉀濃度維(wei)持(chi)(chi)在(zai)(zai)(zai)4.0~4.5mmoL/L。同(tong)時(shi)(shi)密切(qie)觀(guan)(guan)察(cha)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)有(you)(you)(you)(you)無(wu)呼吸困(kun)難(nan)、咳粉(fen)紅(hong)色(se)(se)泡沫樣痰(tan)(tan)、煩躁不(bu)(bu)(bu)(bu)安(an)等(deng)(deng)(deng)(deng)(deng)容(rong)量(liang)(liang)(liang)(liang)(liang)(liang)負(fu)荷過重(zhong)(zhong)表現(xian)(xian)。本(ben)(ben)組(zu)(zu)(zu)(zu)10例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)未出(chu)現(xian)(xian)肺(fei)(fei)水(shui)(shui)腫等(deng)(deng)(deng)(deng)(deng)表現(xian)(xian)。2.2.2采取多(duo)形(xing)式(shi)(shi)(shi)的(de)(de)(de)(de)(de)(de)(de)隔(ge)(ge)(ge)離措施(shi),加(jia)(jia)(jia)強(qiang)(qiang)感(gan)(gan)染(ran)(ran)預(yu)(yu)防(fang)與控(kong)制兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)童(tong)(tong)(tong)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)在(zai)(zai)(zai)我(wo)國發(fa)(fa)(fa)(fa)(fa)展(zhan)迅速(su),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)生(sheng)(sheng)存率(lv)(lv)在(zai)(zai)(zai)部(bu)(bu)(bu)(bu)分(fen)(fen)(fen)(fen)(fen)(fen)中(zhong)心(xin)(xin)(xin)(xin)(xin)(xin)已(yi)(yi)達國際先進(jin)(jin)(jin)(jin)(jin)水(shui)(shui)平(ping),但移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)感(gan)(gan)染(ran)(ran)仍(reng)是(shi)影(ying)響(xiang)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)物存活率(lv)(lv)及(ji)(ji)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)生(sheng)(sheng)存率(lv)(lv)的(de)(de)(de)(de)(de)(de)(de)主要(yao)(yao)(yao)(yao)原(yuan)因[15]。本(ben)(ben)組(zu)(zu)(zu)(zu)10例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)均(jun)(jun)(jun)入(ru)(ru)住肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)監(jian)護(hu)(hu)(hu)(hu)(hu)室(shi),監(jian)護(hu)(hu)(hu)(hu)(hu)室(shi)配有(you)(you)(you)(you)中(zhong)央控(kong)制消(xiao)(xiao)(xiao)毒(du)系(xi)統,其(qi)含(han)(han)濾器(qi)(qi)及(ji)(ji)負(fu)離子(zi)發(fa)(fa)(fa)(fa)(fa)生(sheng)(sheng)器(qi)(qi),雙(shuang)進(jin)(jin)(jin)(jin)(jin)風(feng)(feng)離心(xin)(xin)(xin)(xin)(xin)(xin)風(feng)(feng)機(ji),對(dui)(dui)(dui)(dui)(dui)病(bing)(bing)房(fang)(fang)內(nei)(nei)的(de)(de)(de)(de)(de)(de)(de)空氣(qi)(qi)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)凈化(hua)(hua)(hua)和(he)消(xiao)(xiao)(xiao)毒(du)。肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)專科(ke)(ke)(ke)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)團(tuan)隊落實保(bao)護(hu)(hu)(hu)(hu)(hu)性(xing)(xing)(xing)(xing)(xing)隔(ge)(ge)(ge)離措施(shi),專人(ren)特(te)(te)護(hu)(hu)(hu)(hu)(hu),嚴格(ge)無(wu)菌(jun)操作。責任(ren)護(hu)(hu)(hu)(hu)(hu)士(shi)在(zai)(zai)(zai)進(jin)(jin)(jin)(jin)(jin)入(ru)(ru)病(bing)(bing)房(fang)(fang)前(qian)(qian)(qian)嚴格(ge)手(shou)(shou)(shou)衛生(sheng)(sheng),戴一次性(xing)(xing)(xing)(xing)(xing)橡(xiang)膠(jiao)手(shou)(shou)(shou)套(tao)、帽子(zi),更換經(jing)(jing)(jing)高(gao)(gao)(gao)壓(ya)蒸汽滅(mie)菌(jun)的(de)(de)(de)(de)(de)(de)(de)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)專用(yong)(yong)(yong)(yong)護(hu)(hu)(hu)(hu)(hu)士(shi)服(fu)、護(hu)(hu)(hu)(hu)(hu)士(shi)鞋。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)使(shi)(shi)(shi)用(yong)(yong)(yong)(yong)診(zhen)(zhen)療(liao)(liao)用(yong)(yong)(yong)(yong)具(ju)(ju)(ju)如(ru)體(ti)(ti)(ti)(ti)溫計、聽診(zhen)(zhen)器(qi)(qi)、血(xue)(xue)(xue)壓(ya)計等(deng)(deng)(deng)(deng)(deng)專人(ren)專用(yong)(yong)(yong)(yong),用(yong)(yong)(yong)(yong)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)予(yu)伽(jia)馬濕巾(jin)消(xiao)(xiao)(xiao)毒(du)。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)所用(yong)(yong)(yong)(yong)生(sheng)(sheng)活物品(pin)經(jing)(jing)(jing)嚴格(ge)消(xiao)(xiao)(xiao)毒(du)帶(dai)入(ru)(ru),進(jin)(jin)(jin)(jin)(jin)食(shi)(shi)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)科(ke)(ke)(ke)室(shi)提(ti)供(gong)(gong)一次性(xing)(xing)(xing)(xing)(xing)奶瓶奶嘴(zui)。每(mei)(mei)日(ri)(ri)監(jian)測(ce)(ce)(ce)(ce)血(xue)(xue)(xue)液(ye)(ye)、痰(tan)(tan)液(ye)(ye)、引(yin)流(liu)液(ye)(ye)、大(da)(da)便等(deng)(deng)(deng)(deng)(deng)培養(yang)(yang)(yang)(yang),動(dong)態監(jian)測(ce)(ce)(ce)(ce)體(ti)(ti)(ti)(ti)溫、血(xue)(xue)(xue)常(chang)(chang)(chang)規(gui)、C反(fan)應蛋白、病(bing)(bing)毒(du)DNA等(deng)(deng)(deng)(deng)(deng)。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)第4天(tian)痰(tan)(tan)培養(yang)(yang)(yang)(yang)金黃(huang)色(se)(se)葡(pu)萄球(qiu)菌(jun),體(ti)(ti)(ti)(ti)溫最(zui)高(gao)(gao)(gao)38.5℃,白細胞1.5×109/L,醫(yi)囑予(yu)單間(jian)(jian)飛(fei)沫隔(ge)(ge)(ge)離,調(diao)整(zheng)抗(kang)感(gan)(gan)染(ran)(ran)方(fang)案為(wei)(wei)利奈(nai)唑胺(an)+美羅培南+更昔洛韋靜(jing)(jing)(jing)(jing)(jing)脈(mo)(mo)(mo)注(zhu)(zhu)(zhu)射,吸入(ru)(ru)用(yong)(yong)(yong)(yong)布地(di)奈(nai)德混懸液(ye)(ye)霧化(hua)(hua)(hua),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)15d后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)復測(ce)(ce)(ce)(ce)痰(tan)(tan)培養(yang)(yang)(yang)(yang)無(wu)細菌(jun)生(sheng)(sheng)長(chang)。本(ben)(ben)組(zu)(zu)(zu)(zu)1例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)5d腹(fu)(fu)(fu)(fu)瀉逐漸加(jia)(jia)(jia)重(zhong)(zhong),糞便艱難(nan)梭菌(jun)檢(jian)測(ce)(ce)(ce)(ce)陽性(xing)(xing)(xing)(xing)(xing)。醫(yi)囑予(yu)接(jie)觸隔(ge)(ge)(ge)離,萬古(gu)霉(mei)素(su)口(kou)服(fu)抗(kang)感(gan)(gan)染(ran)(ran),蒙(meng)脫石(shi)散止瀉及(ji)(ji)雙(shuang)歧(qi)桿菌(jun)三聯(lian)活菌(jun)膠(jiao)囊調(diao)節腸(chang)(chang)(chang)道菌(jun)群。由(you)于(yu)(yu)反(fan)復腹(fu)(fu)(fu)(fu)瀉,采用(yong)(yong)(yong)(yong)3M造(zao)口(kou)粉(fen)涂抹后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)再噴賽膚(fu)(fu)(fu)潤(run)液(ye)(ye)體(ti)(ti)(ti)(ti)敷料,隔(ge)(ge)(ge)絕(jue)糞水(shui)(shui)對(dui)(dui)(dui)(dui)(dui)皮(pi)(pi)膚(fu)(fu)(fu)的(de)(de)(de)(de)(de)(de)(de)刺激。術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)20d糞便艱難(nan)梭菌(jun)培養(yang)(yang)(yang)(yang)陰(yin)性(xing)(xing)(xing)(xing)(xing),解(jie)黃(huang)色(se)(se)糊便3次/d,未發(fa)(fa)(fa)(fa)(fa)生(sheng)(sheng)失禁性(xing)(xing)(xing)(xing)(xing)皮(pi)(pi)炎。余8例(li)(li)(li)(li)(li)(li)(li)(li)無(wu)感(gan)(gan)染(ran)(ran)相關(guan)并(bing)發(fa)(fa)(fa)(fa)(fa)癥發(fa)(fa)(fa)(fa)(fa)生(sheng)(sheng)。2.2.3實施(shi)預(yu)(yu)見性(xing)(xing)(xing)(xing)(xing)心(xin)(xin)(xin)(xin)(xin)(xin)理(li)(li)(li)(li)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li),緩解(jie)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)不(bu)(bu)(bu)(bu)良(liang)情(qing)(qing)(qing)緒治療(liao)(liao)過程(cheng)(cheng)中(zhong)有(you)(you)(you)(you)創(chuang)檢(jian)查(cha)等(deng)(deng)(deng)(deng)(deng)會(hui)影(ying)響(xiang)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)身(shen)心(xin)(xin)(xin)(xin)(xin)(xin)舒適度,再加(jia)(jia)(jia)上表達及(ji)(ji)理(li)(li)(li)(li)解(jie)能(neng)(neng)(neng)(neng)力(li)(li)(li)(li)欠(qian)缺,住院(yuan)(yuan)(yuan)過程(cheng)(cheng)會(hui)產生(sheng)(sheng)強(qiang)(qiang)烈(lie)的(de)(de)(de)(de)(de)(de)(de)恐(kong)懼(ju)感(gan)(gan)[16]。責任(ren)護(hu)(hu)(hu)(hu)(hu)士(shi)積極主動(dong)與家(jia)(jia)(jia)(jia)長(chang)溝通,了解(jie)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)心(xin)(xin)(xin)(xin)(xin)(xin)理(li)(li)(li)(li)狀(zhuang)(zhuang)(zhuang)況(kuang)(kuang)、生(sheng)(sheng)活經(jing)(jing)(jing)歷(li)等(deng)(deng)(deng)(deng)(deng),有(you)(you)(you)(you)針(zhen)對(dui)(dui)(dui)(dui)(dui)性(xing)(xing)(xing)(xing)(xing)、計劃性(xing)(xing)(xing)(xing)(xing)地(di)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)心(xin)(xin)(xin)(xin)(xin)(xin)理(li)(li)(li)(li)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)。采取游戲、鼓勵、暗示等(deng)(deng)(deng)(deng)(deng)多(duo)種(zhong)方(fang)法對(dui)(dui)(dui)(dui)(dui)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)激勵,增(zeng)(zeng)進(jin)(jin)(jin)(jin)(jin)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)信任(ren)感(gan)(gan),消(xiao)(xiao)(xiao)除陌(mo)生(sheng)(sheng)恐(kong)懼(ju)感(gan)(gan)。病(bing)(bing)房(fang)(fang)環(huan)(huan)境布置童(tong)(tong)(tong)趣(qu)化(hua)(hua)(hua),對(dui)(dui)(dui)(dui)(dui)配合(he)(he)治療(liao)(liao)表現(xian)(xian)好(hao)(hao)(hao)(hao)的(de)(de)(de)(de)(de)(de)(de)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)給予(yu)棒(bang)棒(bang)糖、貼畫、小(xiao)玩(wan)(wan)具(ju)(ju)(ju)等(deng)(deng)(deng)(deng)(deng)物品(pin)鼓勵。同(tong)時(shi)(shi)提(ti)升患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)父(fu)母的(de)(de)(de)(de)(de)(de)(de)認(ren)知(zhi)(zhi)水(shui)(shui)平(ping),使(shi)(shi)(shi)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)從(cong)父(fu)母的(de)(de)(de)(de)(de)(de)(de)行(xing)(xing)(xing)(xing)(xing)(xing)為(wei)(wei)中(zhong)得(de)到(dao)安(an)全(quan)感(gan)(gan)。學齡(ling)期(qi)(qi)是(shi)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)童(tong)(tong)(tong)心(xin)(xin)(xin)(xin)(xin)(xin)理(li)(li)(li)(li)發(fa)(fa)(fa)(fa)(fa)展(zhan)的(de)(de)(de)(de)(de)(de)(de)重(zhong)(zhong)要(yao)(yao)(yao)(yao)時(shi)(shi)期(qi)(qi),該時(shi)(shi)期(qi)(qi)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)童(tong)(tong)(tong)自尊心(xin)(xin)(xin)(xin)(xin)(xin)較強(qiang)(qiang)。本(ben)(ben)組(zu)(zu)(zu)(zu)2例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)已(yi)(yi)就讀小(xiao)學四年(nian)級,自身(shen)容(rong)貌特(te)(te)殊,前(qian)(qian)(qian)額突出(chu)伴(ban)(ban)耳尖,入(ru)(ru)院(yuan)(yuan)(yuan)時(shi)(shi)均(jun)(jun)(jun)表現(xian)(xian)為(wei)(wei)情(qing)(qing)(qing)緒消(xiao)(xiao)(xiao)極,沉默(mo)寡言,自卑。責任(ren)護(hu)(hu)(hu)(hu)(hu)士(shi)以(yi)(yi)其(qi)能(neng)(neng)(neng)(neng)夠接(jie)受(shou)的(de)(de)(de)(de)(de)(de)(de)話語方(fang)式(shi)(shi)(shi)交流(liu),通過視頻(pin)、宣教手(shou)(shou)(shou)冊等(deng)(deng)(deng)(deng)(deng)易(yi)于(yu)(yu)表達理(li)(li)(li)(li)解(jie)的(de)(de)(de)(de)(de)(de)(de)方(fang)式(shi)(shi)(shi)為(wei)(wei)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)及(ji)(ji)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)介紹手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)過程(cheng)(cheng)、術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)產生(sheng)(sheng)的(de)(de)(de)(de)(de)(de)(de)良(liang)好(hao)(hao)(hao)(hao)效(xiao)果,消(xiao)(xiao)(xiao)除疑慮,緩解(jie)其(qi)對(dui)(dui)(dui)(dui)(dui)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)的(de)(de)(de)(de)(de)(de)(de)擔憂。同(tong)時(shi)(shi),請(qing)預(yu)(yu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)良(liang)好(hao)(hao)(hao)(hao)的(de)(de)(de)(de)(de)(de)(de)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)作為(wei)(wei)志愿者(zhe)(zhe)來分(fen)(fen)(fen)(fen)(fen)(fen)享經(jing)(jing)(jing)驗,增(zeng)(zeng)強(qiang)(qiang)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)及(ji)(ji)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)治療(liao)(liao)信心(xin)(xin)(xin)(xin)(xin)(xin)。2.2.4多(duo)模式(shi)(shi)(shi)隨(sui)(sui)訪(fang)管(guan)(guan)(guan)理(li)(li)(li)(li),提(ti)供(gong)(gong)延續(xu)護(hu)(hu)(hu)(hu)(hu)理(li)(li)(li)(li)服(fu)務兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)童(tong)(tong)(tong)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)手(shou)(shou)(shou)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)技(ji)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)難(nan)度較大(da)(da),國內(nei)(nei)獲得(de)資質(zhi)(zhi)的(de)(de)(de)(de)(de)(de)(de)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)中(zhong)心(xin)(xin)(xin)(xin)(xin)(xin)較少(shao)。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)往往來自于(yu)(yu)全(quan)國各地(di),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)隨(sui)(sui)訪(fang)需(xu)要(yao)(yao)(yao)(yao)花費(fei)(fei)較多(duo)的(de)(de)(de)(de)(de)(de)(de)時(shi)(shi)間(jian)(jian)和(he)費(fei)(fei)用(yong)(yong)(yong)(yong),給患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)家(jia)(jia)(jia)(jia)庭(ting)帶(dai)來很大(da)(da)的(de)(de)(de)(de)(de)(de)(de)不(bu)(bu)(bu)(bu)便。基于(yu)(yu)移(yi)(yi)(yi)(yi)(yi)動(dong)互聯(lian)網平(ping)臺的(de)(de)(de)(de)(de)(de)(de)遠(yuan)程(cheng)(cheng)隨(sui)(sui)訪(fang)能(neng)(neng)(neng)(neng)有(you)(you)(you)(you)效(xiao)減少(shao)受(shou)者(zhe)(zhe)出(chu)行(xing)(xing)(xing)(xing)(xing)(xing)負(fu)擔,經(jing)(jing)(jing)濟(ji)高(gao)(gao)(gao)效(xiao)[17]。我(wo)院(yuan)(yuan)(yuan)采用(yong)(yong)(yong)(yong)線上、線下(xia),門診(zhen)(zhen)與互聯(lian)網相結合(he)(he)的(de)(de)(de)(de)(de)(de)(de)多(duo)模式(shi)(shi)(shi)隨(sui)(sui)訪(fang)。家(jia)(jia)(jia)(jia)長(chang)是(shi)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)出(chu)院(yuan)(yuan)(yuan)管(guan)(guan)(guan)理(li)(li)(li)(li)的(de)(de)(de)(de)(de)(de)(de)主要(yao)(yao)(yao)(yao)執(zhi)行(xing)(xing)(xing)(xing)(xing)(xing)者(zhe)(zhe),術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)即(ji)將如(ru)何預(yu)(yu)防(fang)感(gan)(gan)染(ran)(ran)、免(mian)疫(yi)接(jie)種(zhong)計劃、服(fu)藥(yao)相關(guan)注(zhu)(zhu)(zhu)意事(shi)項、復查(cha)時(shi)(shi)間(jian)(jian)等(deng)(deng)(deng)(deng)(deng)融入(ru)(ru)日(ri)(ri)常(chang)(chang)(chang)宣教,同(tong)時(shi)(shi)發(fa)(fa)(fa)(fa)(fa)放相關(guan)手(shou)(shou)(shou)冊,教會(hui)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)下(xia)載(zai)觀(guan)(guan)看(kan)(kan)相關(guan)視頻(pin)。在(zai)(zai)(zai)出(chu)院(yuan)(yuan)(yuan)前(qian)(qian)(qian)1周(zhou)(zhou)(zhou)左(zuo)右,肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)科(ke)(ke)(ke)、兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)科(ke)(ke)(ke)、營(ying)(ying)(ying)(ying)養(yang)(yang)(yang)(yang)科(ke)(ke)(ke)等(deng)(deng)(deng)(deng)(deng)醫(yi)護(hu)(hu)(hu)(hu)(hu)人(ren)員(yuan)組(zu)(zu)(zu)(zu)成(cheng)(cheng)的(de)(de)(de)(de)(de)(de)(de)專業(ye)團(tuan)隊,根據(ju)(ju)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)的(de)(de)(de)(de)(de)(de)(de)實際情(qing)(qing)(qing)況(kuang)(kuang)量(liang)(liang)(liang)(liang)(liang)(liang)身(shen)定(ding)制出(chu)院(yuan)(yuan)(yuan)計劃,由(you)隨(sui)(sui)訪(fang)護(hu)(hu)(hu)(hu)(hu)士(shi)指(zhi)導患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu)在(zai)(zai)(zai)智能(neng)(neng)(neng)(neng)手(shou)(shou)(shou)機(ji)下(xia)載(zai)安(an)裝醫(yi)院(yuan)(yuan)(yuan)官(guan)(guan)方(fang)隨(sui)(sui)訪(fang)App完成(cheng)(cheng)注(zhu)(zhu)(zhu)冊使(shi)(shi)(shi)用(yong)(yong)(yong)(yong)。App定(ding)期(qi)(qi)自動(dong)發(fa)(fa)(fa)(fa)(fa)送肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)相關(guan)知(zhi)(zhi)識(shi),提(ti)醒患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)復查(cha)。隨(sui)(sui)訪(fang)護(hu)(hu)(hu)(hu)(hu)士(shi)按(an)(an)照隨(sui)(sui)訪(fang)—評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)—干(gan)(gan)(gan)(gan)預(yu)(yu)—再隨(sui)(sui)訪(fang)—再評(ping)(ping)(ping)(ping)(ping)估(gu)(gu)(gu)—再干(gan)(gan)(gan)(gan)預(yu)(yu)的(de)(de)(de)(de)(de)(de)(de)流(liu)程(cheng)(cheng)進(jin)(jin)(jin)(jin)(jin)行(xing)(xing)(xing)(xing)(xing)(xing)隨(sui)(sui)訪(fang),對(dui)(dui)(dui)(dui)(dui)于(yu)(yu)聯(lian)系(xi)困(kun)難(nan)、依從(cong)性(xing)(xing)(xing)(xing)(xing)差(cha)的(de)(de)(de)(de)(de)(de)(de)家(jia)(jia)(jia)(jia)屬(shu)(shu)(shu)(shu),再結合(he)(he)電(dian)(dian)話回(hui)訪(fang)、微信、電(dian)(dian)子(zi)郵件、門診(zhen)(zhen)預(yu)(yu)約等(deng)(deng)(deng)(deng)(deng)方(fang)式(shi)(shi)(shi)執(zhi)行(xing)(xing)(xing)(xing)(xing)(xing)。隨(sui)(sui)訪(fang)內(nei)(nei)容(rong)包括病(bing)(bing)情(qing)(qing)(qing)監(jian)測(ce)(ce)(ce)(ce)、何時(shi)(shi)復診(zhen)(zhen)、血(xue)(xue)(xue)生(sheng)(sheng)化(hua)(hua)(hua)檢(jian)測(ce)(ce)(ce)(ce)、并(bing)發(fa)(fa)(fa)(fa)(fa)癥預(yu)(yu)防(fang)、飲(yin)食(shi)(shi)指(zhi)導、用(yong)(yong)(yong)(yong)藥(yao)指(zhi)導、功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)鍛煉(lian)、疫(yi)苗接(jie)種(zhong)等(deng)(deng)(deng)(deng)(deng)。患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)肝(gan)(gan)(gan)(gan)(gan)移(yi)(yi)(yi)(yi)(yi)植(zhi)(zhi)(zhi)(zhi)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)6個(ge)月內(nei)(nei)復診(zhen)(zhen)頻(pin)率(lv)(lv)高(gao)(gao)(gao),要(yao)(yao)(yao)(yao)求(qiu)(qiu)術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)1個(ge)月每(mei)(mei)周(zhou)(zhou)(zhou)1次,術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)2~3個(ge)月每(mei)(mei)2周(zhou)(zhou)(zhou)1次,術(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)(shu)后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)4~6個(ge)月每(mei)(mei)4周(zhou)(zhou)(zhou)1次,6個(ge)月后(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)(hou)根據(ju)(ju)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)者(zhe)(zhe)具(ju)(ju)(ju)體(ti)(ti)(ti)(ti)情(qing)(qing)(qing)況(kuang)(kuang)調(diao)整(zheng)。醫(yi)院(yuan)(yuan)(yuan)為(wei)(wei)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)家(jia)(jia)(jia)(jia)庭(ting)提(ti)供(gong)(gong)半年(nian)的(de)(de)(de)(de)(de)(de)(de)“愛(ai)心(xin)(xin)(xin)(xin)(xin)(xin)之(zhi)家(jia)(jia)(jia)(jia)”免(mian)費(fei)(fei)租(zu)房(fang)(fang)。目(mu)前(qian)(qian)(qian),10例(li)(li)(li)(li)(li)(li)(li)(li)患(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)(huan)兒(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)(er)隨(sui)(sui)訪(fang)率(lv)(lv)100%,肝(gan)(gan)(gan)(gan)(gan)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)恢(hui)復正(zheng)(zheng)常(chang)(chang)(chang),未出(chu)現(xian)(xian)心(xin)(xin)(xin)(xin)(xin)(xin)功(gong)(gong)(gong)能(neng)(neng)(neng)(neng)衰(shuai)竭(jie)、感(gan)(gan)染(ran)(ran)等(deng)(deng)(deng)(deng)(deng)并(bing)發(fa)(fa)(fa)(fa)(fa)癥,皮(pi)(pi)膚(fu)(fu)(fu)瘙(sao)癢、皮(pi)(pi)疹均(jun)(jun)(jun)消(xiao)(xiao)(xiao)失,生(sheng)(sheng)長(chang)發(fa)(fa)(fa)(fa)(fa)育出(chu)現(xian)(xian)明(ming)(ming)顯(xian)(xian)追長(chang)。

3小結

Alagille綜(zong)合征(zheng)涉及患(huan)(huan)(huan)兒消化(hua)系統(tong)、循環系統(tong)、呼(hu)吸(xi)系統(tong)、皮膚等(deng),給(gei)治(zhi)療(liao)(liao)及護理(li)帶來較大的難(nan)度(du)。應(ying)在術前加(jia)強(qiang)多(duo)(duo)器官功能(neng)監護,促進手術順利進行;早期營養(yang)(yang)篩查,進行營養(yang)(yang)評估,腸內腸外(wai)營養(yang)(yang)相結合,預防營養(yang)(yang)不良;術后(hou)制訂精細的液體管(guan)(guan)理(li)方(fang)案,保證內環境穩定;嚴格落實感(gan)染(ran)預防措施,警(jing)惕多(duo)(duo)重耐藥菌感(gan)染(ran);加(jia)強(qiang)患(huan)(huan)(huan)兒心理(li)護理(li),提高治(zhi)療(liao)(liao)依從(cong)性(xing),樹立戰勝疾病的信(xin)(xin)心;建立信(xin)(xin)息化(hua)網絡管(guan)(guan)理(li)平臺,加(jia)強(qiang)隨訪管(guan)(guan)理(li),促進患(huan)(huan)(huan)兒康復。但病例數量有限,在肝(gan)移植術前評估、術后(hou)管(guan)(guan)理(li)和出(chu)院指(zhi)導等(deng)方(fang)面(mian)需要更(geng)多(duo)(duo)的經驗(yan)積累。

作者：王芳 盧芳燕(yan) 王燕(yan)