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1、兒童重癥監(jiān)護(hù)病房噬血細(xì)胞綜合征22例診治分析張金霞于方(新疆烏魯木齊市第一人民醫(yī)院<兒童醫(yī)院>新疆烏魯木齊830000)【摘要】目的：探討噬血細(xì)胞綜合征合并多臟器功能不全的臨床特征，治療及預(yù)后。方法：回顧性分析2009-2014年兒童重癥監(jiān)護(hù)室(PICU)收治的22例HPS并多臟器功能不全危重患兒的病因、臨床特征、實(shí)驗(yàn)室檢查、治療及轉(zhuǎn)歸。結(jié)果：22例HPS患兒中有13例與感染相關(guān)；其余與非感染相關(guān)，其中家族性1例；川崎病1例，全身型幼年類風(fēng)濕性關(guān)節(jié)炎GIA)1例；藥物1例；原因不明5例。臨床以反復(fù)
2、發(fā)熱伴肝脾淋巴結(jié)腫大，全血細(xì)胞二系及三系減少；彌漫性血管內(nèi)凝血；低纖維蛋白原血癥；肝功能異常，高坫油三酯血癥為主要表現(xiàn)。臟器損害累及肝臟，血液，呼吸，胃腸，心臟，腎臟，中樞等；以肝臟，凝血，呼吸最為明顯。結(jié)論：嗜血細(xì)胞綜合征并多臟器功能不全病因復(fù)雜，病情兇險(xiǎn)，進(jìn)展快，受累臟器多，預(yù)后差，病死率高。早期診斷，聯(lián)合化療可挽救部分患兒生命。血液凈化技術(shù)對(duì)HLH合并MODS患兒是一種有效輔助治療方法?！娟P(guān)鍵詞】淋巴組織細(xì)胞增多癥；噬血細(xì)胞；兒童；多臟器功能不全【中圖分類號(hào)】R720.5【文獻(xiàn)標(biāo)識(shí)碼】A【文章編號(hào)】20
3、95-1752(2015)28-0026-02Children'sintensivecareeatsbloodsyndromediagnosisandtreatmentof22casesanalysisZhangJinxia,YuFang.TheFirstPeople'sHospitalofUrumqiCity(children、hospital),Xinjiang,Urumqi830000,China【Abstract】ObjectiveExplorethewhitebloodcellssyndromean
4、dclinicalfeaturesofcombinedmultipleorgandysfunction,treatmentandprognosis.MethodsChildrenfrom2009to2014wereretrospectivelyanalyzedintheintensivecareunit(PICU)of22casesofHPSandmultipleorgandysfunctioncriticallyillchildrenwiththeetiology,clinicalfeatures,labo
5、ratoryexamination,treatmentandoutcome.ResultsThereare13casesinchildrenwith22casesofHPS,associatedwithinfection;Therestisassociatedwiththeinfection,including1familialcases;Kawasakidiseasein1case,thewholebodytype,1caseofjuvenilerheumatoidarthritis(JIA);Drugsi
6、n1case;Forunknownreasonsin5cases.Forclinicalrecurringfeverassociatedwithliverspleenandlymphnodeenlargement,completebloodtwoandthreeisreduced;Diffuseintravascularcoagulation;Lowfibrinogenconcentration;Liverfunctionisabnormal,hightriglyceridelevels,asthemainp
7、erformance.Visceradamageinvolvingtheliver,blood,respiratory,gastrointestinal,heart,kidney,central,etc.;Intheliver,bloodcoagulation,breathingismostobvious.ConclusionsBloodcellssyndromeandmultipleorgandysfunctioncausescomplex,dangerous,progressisfast,theaffec
8、tedorgans,prognosisispoor,highcasefatalityrate.Earlydiagnosis,MDTcansavesomechildrenlife.ChildrenwithHLHmergeMODSbloodpurificationtechnologyisaneffectiveauxiliarytreatment.【Keywords】Lymphatichistiocyto
