最新Pulmonary Complications of Sickle Cell Disease教学讲义PPT课件.ppt

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1、PulmonaryComplicationsofSickleCellDiseaseWhycareaboutahemoglobinopathy?0.15%ofAfricanAmericansareHbSS(250,000birthsperyearworldwide)8%havesicklecelltraitMedianageatdeath42(males)and48(females)Pulmonarycomplications–majorcauseofmortality(20-30%ofdeaths)Sh

2、ortcomingsinthisFieldSCDisrare,hardtodolargepopulation-basedstudiesDifficultpatientpopulationtostudyLackofplacebo-controlledrandomizedtrialsandoutcomesstudiesACS–CXRFindingsVichinskyEP,etal.Blood1997ACS–EtiologyVichinskyEP,etalNEJM2000VichinskyEP2000Pred

3、ictorsofRespiratoryFailureinACSMulti-lobardisease(>4lobeshighestrisk)HistoryofcardiacdiseaseThrombocytopeniaPre-existentpulmonaryhypertensionACS-EtiologyACS–TreatmentStrategiesPaincontrol/incentivespirometryBronchodilatorsAntibioticsTransfusion-Simpleore

4、xchangeAnti-coagulationifVTEconfirmedOtheragents–inhaledNO,anti-adhesiveagents,steroidsPulmonaryHTNinSCDWasformanyyrsthoughttobepartof“Chronicsicklecelllungdisease”OriginallydefinedbyaTRV>2.5m/secObservedin1/3ofHbSSand10-25%ofHbSCadults10-22%ofHbSSchildr

5、enandadolescentsOverlapinsomewithLVdiastolicdysfunctionGladwinMT,etal.NEJM2004;350:886-95.CastroO,etal.Blood2003;4:1257-1261.KatoGJ,etal.BloodRev2007;21:37-47.KlingsES,etal.AmJHematol2008;83(7):547-553.AnElevatedTRVPredictsMortalityinSCDAdultsGladwinMT,e

6、tal.Pulmonaryhypertensionasariskfactorfordeathinpatientswithsicklecelldisease.NEJM2004.ButanElevatedTRVisNotPHEcho-25%positivepredictivevalueforPH73%falsepositiveratePHprevalence–6%ParentF,etal.NEJM2011;365:44-53.PH(n=56)NoPH(n=30)pvalueCVP(mmHg)10±56±3<

7、0.001mPAP(mmHg)36±919±4<0.001PCWP(mmHg)16±512±3<0.001CO(L/min)8±39±20.14CI(L/min/m2)5±15±10.063PVR(dyne.sec/cm5)229±14974±38<0.001PH(n=24)NoPH(n=72)pvalueCVP(mmHg)10±67±2<0.001mPAP(mmHg)30±619±3<0.001PCWP(mmHg)16±711±3<0.001CO(L/min)8.7±1.98.4±2.10.60PVR

8、(dyne.sec/cm5)138±5872±26<0.001PH(n=8)NoPH(n=18)pvaluemPAP(mmHg)33.1±8.918.7±2.8<0.001PCWP(mmHg)16.0±5.713.3±20.07CI(L/min/m2)5±1.364.9±1.70.85PVR(dyne.sec/cm5)179±12064±480.002NIHBrazilFrenchParentF,etal.NEJM2011;365:44-5