2014
Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption
Klings E, Machado R, Morris C, Gordeuk V, Kato G, Ataga K, Castro O, Hsu L, Telen M, Krishnamurti L, Steinberg M, Gladwin M. Reply: Practice Guideline for Pulmonary Hypertension in Sickle Cell: Direct Evidence Needed before Universal Adoption. American Journal Of Respiratory And Critical Care Medicine 2014, 190: 238-240. PMID: 25025360, PMCID: PMC4226058, DOI: 10.1164/rccm.201404-0733le.Peer-Reviewed Original ResearchRisk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
Gladwin M, Barst R, Gibbs J, Hildesheim M, Sachdev V, Nouraie M, Hassell K, Little J, Schraufnagel D, Krishnamurti L, Novelli E, Girgis R, Morris C, Rosenzweig E, Badesch D, Lanzkron S, Castro O, Taylor J, Goldsmith J, Kato G, Gordeuk V, Machado R, Investigators and Patients O. Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom. PLOS ONE 2014, 9: e99489. PMID: 24988120, PMCID: PMC4079316, DOI: 10.1371/journal.pone.0099489.Peer-Reviewed Original ResearchMeSH KeywordsAdultAnemia, Sickle CellBlood Flow VelocityBlood PressureCohort StudiesCreatinineFemaleFerritinsFollow-Up StudiesHemolysisHumansHypertension, PulmonaryKaplan-Meier EstimateMaleMiddle AgedNatriuretic Peptide, BrainPeptide FragmentsProportional Hazards ModelsRisk FactorsSeverity of Illness IndexUnited KingdomUnited StatesWalkingConceptsTricuspid regurgitation velocitySickle cell diseaseNT-proBNPCell diseaseMean pulmonary artery pressurePg/Pulmonary artery systolic pressurePulmonary artery pressureRisk of deathCause of mortalityPositive predictive valuePg/mLArtery pressurePulmonary hypertensionChronic transfusionScreening cohortHemolytic markersSystolic pressureCumulative survivalMale genderRisk factorsHigh riskPredictive valueClass IIIPatientsAn Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease
Klings E, Machado R, Barst R, Morris C, Mubarak K, Gordeuk V, Kato G, Ataga K, Gibbs J, Castro O, Rosenzweig E, Sood N, Hsu L, Wilson K, Telen M, Decastro L, Krishnamurti L, Steinberg M, Badesch D, Gladwin M. An Official American Thoracic Society Clinical Practice Guideline: Diagnosis, Risk Stratification, and Management of Pulmonary Hypertension of Sickle Cell Disease. American Journal Of Respiratory And Critical Care Medicine 2014, 189: 727-740. PMID: 24628312, PMCID: PMC3983842, DOI: 10.1164/rccm.201401-0065st.Peer-Reviewed Original ResearchMeSH KeywordsAdultAnemia, Sickle CellAnticoagulantsAntihypertensive AgentsAntisickling AgentsCardiac CatheterizationDecision Support TechniquesEchocardiography, DopplerErythrocyte TransfusionHumansHydroxyureaHypertension, PulmonaryPhosphodiesterase 5 InhibitorsRisk AssessmentSeverity of Illness IndexConceptsRight heart catheterizationTricuspid regurgitant velocitySickle cell diseasePulmonary vascular resistancePulmonary hypertensionManagement of patientsEvidence-based recommendationsStrong recommendationsWedge pressureVascular resistanceRisk stratificationWeak recommendationCell diseaseMortality riskOfficial American Thoracic Society Clinical Practice GuidelineSerum N-terminal pro-brain natriuretic peptide (NT-proBNP) levelsN-terminal pro-brain natriuretic peptide levelsElevated pulmonary artery wedge pressureNormal pulmonary capillary wedge pressurePro-brain natriuretic peptide levelsElevated tricuspid regurgitant velocityLow pulmonary vascular resistanceNT-pro-BNP levelsPhosphodiesterase-5 inhibitor therapyPulmonary artery wedge pressure
2012
Risk Factors for Death in 632 Patients with Sickle Cell Anemia in the United States and United Kingdom
Gladwin M, Barst R, Gibbs J, Hildesheim M, Sachdev V, Nouraie M, Hassell K, Little J, Schraufnagel D, Krishnamurti L, Novelli E, Girgis R, Zhang Y, Morris C, Rosenzweig E, Badesch D, Lanzkron S, Castro O, Taylor J, Goldsmith J, Gordeuk V, Kato G, Machado R. Risk Factors for Death in 632 Patients with Sickle Cell Anemia in the United States and United Kingdom. Blood 2012, 120: 3240. DOI: 10.1182/blood.v120.21.3240.3240.Peer-Reviewed Original ResearchTricuspid regurgitant jet velocityHigher tricuspid regurgitant jet velocityProportional hazards regression analysisHigher NT-proBNPUnadjusted hazard ratioNT-proBNPHazards regression analysisSickle cell diseasePulmonary hypertensionRisk factorsGlaxo Smith KlineAspartate aminotransferaseHazard ratioCell diseaseCox proportional hazards regression analysisMean pulmonary artery pressureSix-minute walk distanceSickle cell disease patientsPopulation screening studiesPulmonary artery pressureRight heart catheterizationRegurgitant jet velocityRisk of deathDoppler echocardiographic measurementsScreening study
2011
Non-Cardiopulmonary Factors Affecting the Six-Minute Walk Distance in Patients with Sickle Cell Disease: Results From the Walk-PHaSST Study
Goel R, Hassell K, Machado R, Barst R, Yovetich N, Kato G, Gordeuk V, Little J, Gibbs J, Schraufnagel D, Girgis R, Rosenzweig E, Morris C, Badesch D, Lanzkron S, Onyekwere O, Nouraie M, Castro O, Sachdev V, Waclawiw M, Woolson R, Goldsmith J, Gladwin M, Krishnamurti L. Non-Cardiopulmonary Factors Affecting the Six-Minute Walk Distance in Patients with Sickle Cell Disease: Results From the Walk-PHaSST Study. Blood 2011, 118: 1074. DOI: 10.1182/blood.v118.21.1074.1074.Peer-Reviewed Original ResearchTR jet velocitySickle cell diseaseSix-minute walk distanceAvascular necrosisMultivariable linear regressionPulmonary hypertensionCell diseasePrimary endpointWalk distanceChronic painSCD patientsSevere genotypeAmerican Thoracic Society guidelinesSix-minute walk testSelf-reported medical historyThoracic Society guidelinesMulti-center studySided p valueGreater predictive valueCardiopulmonary factorsOral sildenafilPain 2NT-proBNPExercise capacityWalk testEchocardiographic Markers of Elevated Pulmonary Pressure and Left Ventricular Diastolic Dysfunction Are Associated With Exercise Intolerance in Adults and Adolescents With Homozygous Sickle Cell Anemia in the United States and United Kingdom
Sachdev V, Kato G, Gibbs J, Barst R, Machado R, Nouraie M, Hassell K, Little J, Schraufnagel D, Krishnamurti L, Novelli E, Girgis R, Morris C, Rosenzweig E, Badesch D, Lanzkron S, Castro O, Taylor J, Hannoush H, Goldsmith J, Gladwin M, Gordeuk V. Echocardiographic Markers of Elevated Pulmonary Pressure and Left Ventricular Diastolic Dysfunction Are Associated With Exercise Intolerance in Adults and Adolescents With Homozygous Sickle Cell Anemia in the United States and United Kingdom. Circulation 2011, 124: 1452-1460. PMID: 21900080, PMCID: PMC3183314, DOI: 10.1161/circulationaha.111.032920.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultAgedAnemia, Sickle CellChildEchocardiographyExercise TestExercise ToleranceFamilial Primary Pulmonary HypertensionFemaleHomozygoteHumansHypertension, PulmonaryMaleMiddle AgedMultivariate AnalysisPredictive Value of TestsProspective StudiesPulmonary ArteryTricuspid Valve InsufficiencyUnited KingdomUnited StatesVentricular Dysfunction, LeftYoung AdultConceptsTricuspid regurgitation velocitySickle cell diseaseSickle cell anemiaVentricular diastolic dysfunctionDiastolic dysfunctionCell diseaseCell anemiaPulmonary hypertensionExercise capacityWalk distanceElevated pulmonary artery systolic pressurePulmonary artery systolic pressureSystolic pulmonary artery pressureHomozygous sickle cell anemiaPulmonary pressure elevationLV diastolic dysfunctionLV filling pressurePoor exercise capacityPulmonary artery pressureElevated pulmonary pressuresLong-term outcomesHomozygous hemoglobin SBlood urea nitrogenArtery pressureEchocardiographic markers
2010
NT-Probnp as a Marker of Cardiopulmonary Compromise and Exercise Limitation In Adults with Sickle Cell Anemia In the Walk-PHaSST Study
Nouraie M, Barst R, Rosenzweig E, Sachdev V, Machado R, Hassell K, Gibbs J, Little J, Schraufnagel D, Krishnamurti L, Novelli E, Girgis R, Morris C, Badesch D, Lanzkron S, Castro O, Goldsmith J, Gladwin M, Gordeuk V, Kato G. NT-Probnp as a Marker of Cardiopulmonary Compromise and Exercise Limitation In Adults with Sickle Cell Anemia In the Walk-PHaSST Study. Blood 2010, 116: 1639. DOI: 10.1182/blood.v116.21.1639.1639.Peer-Reviewed Original ResearchElevated NT-proBNP levelsNT-proBNP levelsRight ventricular systolic pressureSickle cell anemiaVentricular systolic pressureNT-proBNP concentrationsNT-proBNPSickle cell diseasePulmonary hypertensionPositive predictive valueCell anemiaPg/Exercise limitationHeart failureSystolic pressureNatriuretic peptideCell diseaseN-terminal pro-brain natriuretic peptide levelsN-terminal pro-B-type natriuretic peptidePlasma brain natriuretic peptide concentrationPredictive valuePro-brain natriuretic peptide levelsPro-B-type natriuretic peptideCardiac natriuretic peptide systemLower NT-proBNP levels
2009
Safety and Efficacy of Sildenafil Therapy for Doppler-Defined Pulmonary Hypertension in Patients with Sickle Cell Disease: Preliminary Results of the Walk-PHaSST Clinical Trial.
Machado R, Barst R, Yovetich N, Hassell K, Goldsmith J, Woolson R, Gordeuk V, Gibbs S, Little J, Kato G, Schraufnagel D, Krishnamurti L, Girgis R, Morris C, Berman-Rosenzweig E, Badesch D, Waclawiw M, Gladwin M. Safety and Efficacy of Sildenafil Therapy for Doppler-Defined Pulmonary Hypertension in Patients with Sickle Cell Disease: Preliminary Results of the Walk-PHaSST Clinical Trial. Blood 2009, 114: 571. DOI: 10.1182/blood.v114.22.571.571.Peer-Reviewed Original ResearchSerious adverse eventsSickle cell diseaseRight heart catheterizationBrief Pain InventoryPulmonary hypertensionCell diseaseAdverse eventsArterial pressureMean pulmonary arterial pressureSix-minute walk distanceApparent safety issuesDose of sildenafilSecondary endpoint analysisWeeks of sildenafilPulmonary vascular resistancePulmonary arterial pressureTransthoracic Doppler echocardiographySystemic arterial pressurePremature study terminationSingle test doseSignificant differencesSignificant increaseSickle cell anemiaOral sildenafilSildenafil dosing
2007
Hematopoietic cell transplantation for sickle cell disease: state of the art
Krishnamurti L. Hematopoietic cell transplantation for sickle cell disease: state of the art. Expert Opinion On Biological Therapy 2007, 7: 161-172. PMID: 17250455, DOI: 10.1517/14712598.7.2.161.Peer-Reviewed Original ResearchConceptsHematopoietic cell transplantationSickle cell diseaseCell transplantationCell diseaseReduced-intensity conditioning regimenIndication of transplantationImpact of transplantationStem cell transplantationPossibility of cureEffect of treatmentConditioning regimenPreparative regimensPulmonary hypertensionBlood transfusionConsiderable morbidityPediatric patientsOrgan damagePremature mortalityTransplantationNatural historyMorbidityMortalityDiseaseStem cellsSafe useHEMATOPOIETIC CELL TRANSPLANTATION: A Curative Option for Sickle Cell Disease
Krishnamurti L. HEMATOPOIETIC CELL TRANSPLANTATION: A Curative Option for Sickle Cell Disease. Pediatric Hematology And Oncology 2007, 24: 569-575. PMID: 18092247, DOI: 10.1080/08880010701640531.Peer-Reviewed Original ResearchConceptsSickle cell diseaseCell transplantationCell diseaseReduced intensity conditioning regimenIndication of transplantationHematopoietic cell transplantationImpact of transplantationIntensity conditioning regimenStem cell transplantationPossibility of cureEffect of treatmentConditioning regimenPreparative regimenPulmonary hypertensionCurative optionBlood transfusionConsiderable morbidityPediatric patientsRelated morbidityOrgan damagePremature mortalityTransplantationNatural historyMorbidityRegimen
2006
Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease
Ambrusko S, Gunawardena S, Sakara A, Windsor B, Lanford L, Michelson P, Krishnamurti L. Elevation of tricuspid regurgitant jet velocity, a marker for pulmonary hypertension in children with sickle cell disease. Pediatric Blood & Cancer 2006, 47: 907-913. PMID: 16496290, DOI: 10.1002/pbc.20791.Peer-Reviewed Original ResearchConceptsElevated tricuspid regurgitant velocityTricuspid regurgitant velocitySickle cell diseaseAcute chest syndromePulmonary hypertensionCerebrovascular diseaseVasoocclusive crisisSCD patientsReticulocyte countCell diseaseType of SCDTricuspid regurgitant jet velocityLife-threatening complicationsObstructive sleep apneaPediatric SCD patientsRegurgitant jet velocityElevated reticulocyte countSignificant differencesNumber of echocardiogramsChest syndromeEchocardiographic evidenceOutpatient echocardiogramMean hemoglobinClinical characteristicsHydroxyurea therapy
2005
Mixed Hematopoietic Chimerism for Sickle Cell Disease Prevents Intravascular Hemolysis and Corrects Biomarkers of Endothelial Function.
Wu C, Krishnamurti L, Gladwin M, Biernacki M, Rogers S, Wang X, Zahrieh D, Antin J, Ritz J. Mixed Hematopoietic Chimerism for Sickle Cell Disease Prevents Intravascular Hemolysis and Corrects Biomarkers of Endothelial Function. Blood 2005, 106: 2333. DOI: 10.1182/blood.v106.11.2333.2333.Peer-Reviewed Original ResearchSickle cell diseaseMixed hematopoietic chimerismStem cell transplantationEndothelial functionIntravascular hemolysisMixed chimerismPulmonary hypertensionDonor engraftmentCell transplantationVascular functionHematopoietic chimerismNonmyeloablative allogeneic stem cell transplantationChronic end-organ damageAllogeneic stem cell transplantationEnd-organ injuryExperienced graft rejectionEnd-organ damageEnd-organ toxicityPre-transplant levelsDonor cell engraftmentChronic intravascular hemolysisSuccess of therapyFree Hb concentrationEndothelial dysfunctionGraft rejectionPulmonary Hypertension in Children with Sickle Cell Disease: Clinical Characteristics and Co-Morbidities.
Ambrusko S, Gunawardena S, Sakara A, Windsor B, Lanford L, Michelson P, Krishnamurti L. Pulmonary Hypertension in Children with Sickle Cell Disease: Clinical Characteristics and Co-Morbidities. Blood 2005, 106: 3791. DOI: 10.1182/blood.v106.11.3791.3791.Peer-Reviewed Original ResearchSickle cell diseasePulmonary hypertensionCell diseaseSilent strokeHepatitis CClinical characteristicsAplastic crisisCerebrovascular diseaseTransfusion therapyNocturnal enuresisTR jetTricuspid regurgitant jet velocityChronic transfusion programMoya-Moya syndromeTR jet velocityAcute chest syndromeElevated total bilirubinRestrictive lung diseaseMajority of patientsObstructive sleep apneaRegurgitant jet velocityCell-directed therapiesMiddle cerebral arteryRetrospective case reviewSickle cell patients