2014
Risk 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 IIIPatients
2012
The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe
Nouraie M, Lee J, Zhang Y, Kanias T, Zhao X, Xiong Z, Oriss T, Zeng Q, Kato G, Gibbs J, Hildesheim M, Sachdev V, Barst R, Machado R, Hassell K, Little J, Schraufnagel D, Krishnamurti L, Novelli E, Girgis R, Morris C, Rosenzweig E, Badesch D, Lanzkron S, Castro O, Goldsmith J, Gordeuk V, Gladwin M. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica 2012, 98: 464-472. PMID: 22983573, PMCID: PMC3659937, DOI: 10.3324/haematol.2012.068965.Peer-Reviewed Original ResearchConceptsRisk of deathSickle cell diseaseSickle cell anemiaClinical outcomesHemolytic componentPulse pressureCell diseaseOxygen saturationCell anemiaDirect markerRed blood cell microparticlesCutaneous leg ulcerationTricuspid regurgitation velocityIndependent risk factorCell-free hemoglobin concentrationsPulmonary systolic pressureVentricular diastolic dimensionSerum lactate dehydrogenaseSystemic pulse pressureLowest oxygen saturationTwo-year followHemoglobin SS patientsTotal bilirubin concentrationCertain clinical complicationsHigh hemoglobin F
2011
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
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 markersHospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity
Machado R, Barst R, Yovetich N, Hassell K, Kato G, Gordeuk V, Gibbs J, Little J, Schraufnagel D, Krishnamurti L, Girgis R, Morris C, Rosenzweig E, Badesch D, Lanzkron S, Onyekwere O, Castro O, Sachdev V, Waclawiw M, Woolson R, Goldsmith J, Gladwin M. Hospitalization for pain in patients with sickle cell disease treated with sildenafil for elevated TRV and low exercise capacity. Blood 2011, 118: 855-864. PMID: 21527519, PMCID: PMC3148167, DOI: 10.1182/blood-2010-09-306167.Peer-Reviewed Original ResearchConceptsTricuspid regurgitation velocitySickle cell diseaseLower exercise capacityExercise capacityCell diseaseN-terminal pro-brain natriuretic peptideElevated tricuspid regurgitation velocityPro-brain natriuretic peptideSerious adverse eventsPulmonary arterial hypertensionSildenafil trialArterial hypertensionAdverse eventsWalk distanceDoppler echocardiographySCD patientsHospitalization ratesNatriuretic peptidePatientsSildenafilPredominant causePainHospitalizationTreatment effectsDisease
2010
Predictors of Six-Minute Walk Distance In Adults with Sickle Cell Anemia In the Walk-PHaSST Study
Barst R, Kato G, Sachdev V, Nouraie M, Machado R, Hassell K, Gibbs S, Little J, Schraufnagel D, Krishnamurti L, Girgis R, Morris C, Badesch D, Lanzkron S, Castro O, Rosenzweig E, Goldsmith J, Gladwin M, Gordeuk V. Predictors of Six-Minute Walk Distance In Adults with Sickle Cell Anemia In the Walk-PHaSST Study. Blood 2010, 116: 947. DOI: 10.1182/blood.v116.21.947.947.Peer-Reviewed Original ResearchHigher tricuspid regurgitation velocityTricuspid regurgitation velocitySickle cell diseaseSickle cell anemiaRight ventricular systolic pressureVentricular filling pressureVentricular systolic pressureCell anemiaCell diseaseEa ratioExercise capacitySystolic pressureFilling pressureFunctional capacityHemolytic componentElevated tricuspid regurgitant jet velocityTricuspid regurgitant jet velocitySix-minute walk distanceSix-minute walk testSickle cell disease patientsDecreased exercise capacityHemoglobin oxygen desaturationBaseline clinical characteristicsImpaired exercise capacitySickle cell anemia patients