PittsburghV1, Main, Exploration, bibRecord, 001548

Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom

Identifieur interne : 001548 ( Main/Exploration ); précédent : 001547; suivant : 001549

Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom

Auteurs : Mark T. Gladwin [États-Unis] ; Robyn J. Barst [États-Unis] ; J. Simon R. Gibbs [Royaume-Uni] ; Mariana Hildesheim [États-Unis] ; Vandana Sachdev [États-Unis] ; Mehdi Nouraie [États-Unis] ; Kathryn L. Hassell [États-Unis] ; Jane A. Little [États-Unis] ; Dean E. Schraufnagel [États-Unis] ; Lakshmanan Krishnamurti [États-Unis] ; Enrico Novelli [États-Unis] ; Reda E. Girgis [États-Unis] ; Claudia R. Morris [États-Unis] ; Erika Berman Rosenzweig [États-Unis] ; David B. Badesch [États-Unis] ; Sophie Lanzkron [États-Unis] ; Oswaldo L. Castro [États-Unis] ; James G. Taylor [États-Unis] ; Jonathan C. Goldsmith [États-Unis] ; Gregory J. Kato [États-Unis] ; Victor R. Gordeuk [États-Unis] ; Roberto F. Machado [États-Unis]

Source :

PLoS ONE [ 1932-6203 ] ; 2014.

RBID : PMC:4079316

Descripteurs français

KwdFr :
- Adulte, Adulte d'âge moyen, Créatinine (sang), Drépanocytose (anatomopathologie), Drépanocytose (mortalité), Estimation de Kaplan-Meier, Facteurs de risque, Femelle, Ferritines (analyse), Fragments peptidiques (sang), Humains, Hypertension pulmonaire (anatomopathologie), Hémolyse, Indice de gravité médicale, Marche à pied, Modèles de hasards proportionnels, Mâle, Peptide natriurétique cérébral (sang), Pression sanguine, Royaume-Uni, Vitesse du flux sanguin, États-Unis d'Amérique, Études de cohortes, Études de suivi.
MESH :
- analyse : Ferritines.
- anatomopathologie : Drépanocytose, Hypertension pulmonaire.
- mortalité : Drépanocytose.
- sang : Créatinine, Fragments peptidiques, Peptide natriurétique cérébral.
- Adulte, Adulte d'âge moyen, Estimation de Kaplan-Meier, Facteurs de risque, Femelle, Humains, Hémolyse, Indice de gravité médicale, Marche à pied, Modèles de hasards proportionnels, Mâle, Pression sanguine, Royaume-Uni, Vitesse du flux sanguin, États-Unis d'Amérique, Études de cohortes, Études de suivi.

English descriptors

KwdEn :
- Adult, Anemia, Sickle Cell (mortality), Anemia, Sickle Cell (pathology), Blood Flow Velocity, Blood Pressure, Cohort Studies, Creatinine (blood), Female, Ferritins (analysis), Follow-Up Studies, Hemolysis, Humans, Hypertension, Pulmonary (pathology), Kaplan-Meier Estimate, Male, Middle Aged, Natriuretic Peptide, Brain (blood), Peptide Fragments (blood), Proportional Hazards Models, Risk Factors, Severity of Illness Index, United Kingdom, United States, Walking.
MESH :
- chemical , analysis : Ferritins.
- chemical , blood : Creatinine, Natriuretic Peptide, Brain, Peptide Fragments.
- mortality : Anemia, Sickle Cell.
- pathology : Anemia, Sickle Cell, Hypertension, Pulmonary.
- Adult, Blood Flow Velocity, Blood Pressure, Cohort Studies, Female, Follow-Up Studies, Hemolysis, Humans, Kaplan-Meier Estimate, Male, Middle Aged, Proportional Hazards Models, Risk Factors, Severity of Illness Index, United Kingdom, United States, Walking.

Abstract

Background

The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial.

Methods and Results

We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67–75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95% CI 4.1–30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8–11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5–39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III–IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death.

Conclusions

A TRV≥3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable.

The study is registered in ClinicalTrials.gov with identifier

NCT00492531

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4079316

DOI: 10.1371/journal.pone.0099489
PubMed: 24988120
PubMed Central: 4079316

Affiliations:

Le document en format XML

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<author><name sortKey="Little, Jane A" sort="Little, Jane A" uniqKey="Little J" first="Jane A." last="Little">Jane A. Little</name>
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<author><name sortKey="Taylor, James G" sort="Taylor, James G" uniqKey="Taylor J" first="James G." last="Taylor">James G. Taylor</name>
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<wicri:regionArea>University of Illinois, Chicago, Illinois</wicri:regionArea>
<placeName><region type="state">Illinois</region>
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<author><name sortKey="Machado, Roberto F" sort="Machado, Roberto F" uniqKey="Machado R" first="Roberto F." last="Machado">Roberto F. Machado</name>
<affiliation wicri:level="2"><nlm:aff id="aff9"><addr-line>University of Illinois, Chicago, Illinois, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Illinois, Chicago, Illinois</wicri:regionArea>
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom</title>
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<author><name sortKey="Hildesheim, Mariana" sort="Hildesheim, Mariana" uniqKey="Hildesheim M" first="Mariana" last="Hildesheim">Mariana Hildesheim</name>
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</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania</wicri:regionArea>
<placeName><region type="state">Pennsylvanie</region>
<settlement type="city">Pittsburgh</settlement>
</placeName>
<orgName type="university">Université de Pittsburgh</orgName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="aff2"><addr-line>Division of Pulmonary Allergy and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Division of Pulmonary Allergy and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania</wicri:regionArea>
<placeName><region type="state">Pennsylvanie</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Sachdev, Vandana" sort="Sachdev, Vandana" uniqKey="Sachdev V" first="Vandana" last="Sachdev">Vandana Sachdev</name>
<affiliation wicri:level="2"><nlm:aff id="aff5"><addr-line>Cardiovascular Branch, NHLBI, Bethesda, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Cardiovascular Branch, NHLBI, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Nouraie, Mehdi" sort="Nouraie, Mehdi" uniqKey="Nouraie M" first="Mehdi" last="Nouraie">Mehdi Nouraie</name>
<affiliation wicri:level="2"><nlm:aff id="aff6"><addr-line>Howard University, Washington, DC, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Howard University, Washington, DC</wicri:regionArea>
<placeName><region type="state">District de Columbia</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Hassell, Kathryn L" sort="Hassell, Kathryn L" uniqKey="Hassell K" first="Kathryn L." last="Hassell">Kathryn L. Hassell</name>
<affiliation wicri:level="2"><nlm:aff id="aff7"><addr-line>University of Colorado HSC, Denver, Colorado, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Colorado HSC, Denver, Colorado</wicri:regionArea>
<placeName><region type="state">Colorado</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Little, Jane A" sort="Little, Jane A" uniqKey="Little J" first="Jane A." last="Little">Jane A. Little</name>
<affiliation wicri:level="2"><nlm:aff id="aff8"><addr-line>Case Western Reserve University, Cleveland, Ohio, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Case Western Reserve University, Cleveland, Ohio</wicri:regionArea>
<placeName><region type="state">Ohio</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Schraufnagel, Dean E" sort="Schraufnagel, Dean E" uniqKey="Schraufnagel D" first="Dean E." last="Schraufnagel">Dean E. Schraufnagel</name>
<affiliation wicri:level="2"><nlm:aff id="aff9"><addr-line>University of Illinois, Chicago, Illinois, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Illinois, Chicago, Illinois</wicri:regionArea>
<placeName><region type="state">Illinois</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Krishnamurti, Lakshmanan" sort="Krishnamurti, Lakshmanan" uniqKey="Krishnamurti L" first="Lakshmanan" last="Krishnamurti">Lakshmanan Krishnamurti</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania</wicri:regionArea>
<placeName><region type="state">Pennsylvanie</region>
<settlement type="city">Pittsburgh</settlement>
</placeName>
<orgName type="university">Université de Pittsburgh</orgName>
</affiliation>
<affiliation wicri:level="2"><nlm:aff id="aff10"><addr-line>Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania</wicri:regionArea>
<placeName><region type="state">Pennsylvanie</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Novelli, Enrico" sort="Novelli, Enrico" uniqKey="Novelli E" first="Enrico" last="Novelli">Enrico Novelli</name>
<affiliation wicri:level="4"><nlm:aff id="aff1"><addr-line>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania</wicri:regionArea>
<placeName><region type="state">Pennsylvanie</region>
<settlement type="city">Pittsburgh</settlement>
</placeName>
<orgName type="university">Université de Pittsburgh</orgName>
</affiliation>
</author>
<author><name sortKey="Girgis, Reda E" sort="Girgis, Reda E" uniqKey="Girgis R" first="Reda E." last="Girgis">Reda E. Girgis</name>
<affiliation wicri:level="2"><nlm:aff id="aff11"><addr-line>Johns Hopkins University, Baltimore, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Johns Hopkins University, Baltimore, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Morris, Claudia R" sort="Morris, Claudia R" uniqKey="Morris C" first="Claudia R." last="Morris">Claudia R. Morris</name>
<affiliation wicri:level="2"><nlm:aff id="aff12"><addr-line>Emory University School of Medicine, Atlanta, Georgia, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Emory University School of Medicine, Atlanta, Georgia</wicri:regionArea>
<placeName><region type="state">Géorgie (États-Unis)</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Berman Rosenzweig, Erika" sort="Berman Rosenzweig, Erika" uniqKey="Berman Rosenzweig E" first="Erika" last="Berman Rosenzweig">Erika Berman Rosenzweig</name>
<affiliation wicri:level="4"><nlm:aff id="aff3"><addr-line>Columbia University, New York, New York, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Columbia University, New York, New York</wicri:regionArea>
<placeName><region type="state">État de New York</region>
<settlement type="city">New York</settlement>
</placeName>
<orgName type="university">Université Columbia</orgName>
</affiliation>
</author>
<author><name sortKey="Badesch, David B" sort="Badesch, David B" uniqKey="Badesch D" first="David B." last="Badesch">David B. Badesch</name>
<affiliation wicri:level="2"><nlm:aff id="aff7"><addr-line>University of Colorado HSC, Denver, Colorado, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Colorado HSC, Denver, Colorado</wicri:regionArea>
<placeName><region type="state">Colorado</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Lanzkron, Sophie" sort="Lanzkron, Sophie" uniqKey="Lanzkron S" first="Sophie" last="Lanzkron">Sophie Lanzkron</name>
<affiliation wicri:level="2"><nlm:aff id="aff11"><addr-line>Johns Hopkins University, Baltimore, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Johns Hopkins University, Baltimore, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Castro, Oswaldo L" sort="Castro, Oswaldo L" uniqKey="Castro O" first="Oswaldo L." last="Castro">Oswaldo L. Castro</name>
<affiliation wicri:level="2"><nlm:aff id="aff6"><addr-line>Howard University, Washington, DC, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Howard University, Washington, DC</wicri:regionArea>
<placeName><region type="state">District de Columbia</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Taylor, James G" sort="Taylor, James G" uniqKey="Taylor J" first="James G." last="Taylor">James G. Taylor</name>
<affiliation wicri:level="2"><nlm:aff id="aff5"><addr-line>Cardiovascular Branch, NHLBI, Bethesda, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Cardiovascular Branch, NHLBI, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Goldsmith, Jonathan C" sort="Goldsmith, Jonathan C" uniqKey="Goldsmith J" first="Jonathan C." last="Goldsmith">Jonathan C. Goldsmith</name>
<affiliation wicri:level="2"><nlm:aff id="aff13"><addr-line>National Heart Lung and Blood Institute/NIH, Bethesda, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>National Heart Lung and Blood Institute/NIH, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Kato, Gregory J" sort="Kato, Gregory J" uniqKey="Kato G" first="Gregory J." last="Kato">Gregory J. Kato</name>
<affiliation wicri:level="2"><nlm:aff id="aff5"><addr-line>Cardiovascular Branch, NHLBI, Bethesda, Maryland, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Cardiovascular Branch, NHLBI, Bethesda, Maryland</wicri:regionArea>
<placeName><region type="state">Maryland</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Gordeuk, Victor R" sort="Gordeuk, Victor R" uniqKey="Gordeuk V" first="Victor R." last="Gordeuk">Victor R. Gordeuk</name>
<affiliation wicri:level="2"><nlm:aff id="aff9"><addr-line>University of Illinois, Chicago, Illinois, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Illinois, Chicago, Illinois</wicri:regionArea>
<placeName><region type="state">Illinois</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Machado, Roberto F" sort="Machado, Roberto F" uniqKey="Machado R" first="Roberto F." last="Machado">Roberto F. Machado</name>
<affiliation wicri:level="2"><nlm:aff id="aff9"><addr-line>University of Illinois, Chicago, Illinois, United States of America</addr-line>
</nlm:aff>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>University of Illinois, Chicago, Illinois</wicri:regionArea>
<placeName><region type="state">Illinois</region>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j">PLoS ONE</title>
<idno type="eISSN">1932-6203</idno>
<imprint><date when="2014">2014</date>
</imprint>
</series>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term>
<term>Anemia, Sickle Cell (mortality)</term>
<term>Anemia, Sickle Cell (pathology)</term>
<term>Blood Flow Velocity</term>
<term>Blood Pressure</term>
<term>Cohort Studies</term>
<term>Creatinine (blood)</term>
<term>Female</term>
<term>Ferritins (analysis)</term>
<term>Follow-Up Studies</term>
<term>Hemolysis</term>
<term>Humans</term>
<term>Hypertension, Pulmonary (pathology)</term>
<term>Kaplan-Meier Estimate</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Natriuretic Peptide, Brain (blood)</term>
<term>Peptide Fragments (blood)</term>
<term>Proportional Hazards Models</term>
<term>Risk Factors</term>
<term>Severity of Illness Index</term>
<term>United Kingdom</term>
<term>United States</term>
<term>Walking</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Créatinine (sang)</term>
<term>Drépanocytose (anatomopathologie)</term>
<term>Drépanocytose (mortalité)</term>
<term>Estimation de Kaplan-Meier</term>
<term>Facteurs de risque</term>
<term>Femelle</term>
<term>Ferritines (analyse)</term>
<term>Fragments peptidiques (sang)</term>
<term>Humains</term>
<term>Hypertension pulmonaire (anatomopathologie)</term>
<term>Hémolyse</term>
<term>Indice de gravité médicale</term>
<term>Marche à pied</term>
<term>Modèles de hasards proportionnels</term>
<term>Mâle</term>
<term>Peptide natriurétique cérébral (sang)</term>
<term>Pression sanguine</term>
<term>Royaume-Uni</term>
<term>Vitesse du flux sanguin</term>
<term>États-Unis d'Amérique</term>
<term>Études de cohortes</term>
<term>Études de suivi</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Ferritins</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="blood" xml:lang="en"><term>Creatinine</term>
<term>Natriuretic Peptide, Brain</term>
<term>Peptide Fragments</term>
</keywords>
<keywords scheme="MESH" qualifier="analyse" xml:lang="fr"><term>Ferritines</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Drépanocytose</term>
<term>Hypertension pulmonaire</term>
</keywords>
<keywords scheme="MESH" qualifier="mortality" xml:lang="en"><term>Anemia, Sickle Cell</term>
</keywords>
<keywords scheme="MESH" qualifier="mortalité" xml:lang="fr"><term>Drépanocytose</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Anemia, Sickle Cell</term>
<term>Hypertension, Pulmonary</term>
</keywords>
<keywords scheme="MESH" qualifier="sang" xml:lang="fr"><term>Créatinine</term>
<term>Fragments peptidiques</term>
<term>Peptide natriurétique cérébral</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adult</term>
<term>Blood Flow Velocity</term>
<term>Blood Pressure</term>
<term>Cohort Studies</term>
<term>Female</term>
<term>Follow-Up Studies</term>
<term>Hemolysis</term>
<term>Humans</term>
<term>Kaplan-Meier Estimate</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Proportional Hazards Models</term>
<term>Risk Factors</term>
<term>Severity of Illness Index</term>
<term>United Kingdom</term>
<term>United States</term>
<term>Walking</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Estimation de Kaplan-Meier</term>
<term>Facteurs de risque</term>
<term>Femelle</term>
<term>Humains</term>
<term>Hémolyse</term>
<term>Indice de gravité médicale</term>
<term>Marche à pied</term>
<term>Modèles de hasards proportionnels</term>
<term>Mâle</term>
<term>Pression sanguine</term>
<term>Royaume-Uni</term>
<term>Vitesse du flux sanguin</term>
<term>États-Unis d'Amérique</term>
<term>Études de cohortes</term>
<term>Études de suivi</term>
</keywords>
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<front><div type="abstract" xml:lang="en"><sec><title>Background</title>
<p>The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial.</p>
</sec>
<sec><title>Methods and Results</title>
<p>We evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥3.0 m/s cuttof, which has a 67–75% positive predictive value for mean pulmonary artery pressure ≥25 mm Hg was used. Among 572 subjects, 11.2% had TRV≥3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV≥3.0 m/sec. At 24 months the cumulative survival was 83% with TRV≥3.0 m/sec and 98% with TRV<3.0 m/sec (p<0.0001). The hazard ratios for death were 11.1 (95% CI 4.1–30.1; p<0.0001) for TRV≥3.0 m/sec, 4.6 (1.8–11.3; p = 0.001) for NT-proBNP≥160 pg/mL, and 14.9 (5.5–39.9; p<0.0001) for both TRV≥3.0 m/sec and NT-proBNP≥160 pg/mL. Age >47 years, male gender, chronic transfusions, WHO class III–IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death.</p>
</sec>
<sec><title>Conclusions</title>
<p>A TRV≥3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable.</p>
</sec>
<sec><title>The study is registered in ClinicalTrials.gov with identifier</title>
<p><ext-link ext-link-type="uri" xlink:href="http://clinicaltrials.gov/ct2/show/NCT00492531">NCT00492531</ext-link>
</p>
</sec>
</div>
</front>
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<biblStruct><analytic><author><name sortKey="Rees, Dc" uniqKey="Rees D">DC Rees</name>
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</analytic>
</biblStruct>
<biblStruct><analytic><author><name sortKey="Gladwin, Mt" uniqKey="Gladwin M">MT Gladwin</name>
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</author>
<author><name sortKey="Jison, Ml" uniqKey="Jison M">ML Jison</name>
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<author><name sortKey="Strayhorn, D" uniqKey="Strayhorn D">D Strayhorn</name>
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<author><name sortKey="Jonassaint, Jc" uniqKey="Jonassaint J">JC Jonassaint</name>
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<author><name sortKey="Graham, Fl" uniqKey="Graham F">FL Graham</name>
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<author><name sortKey="Ashley Koch, A" uniqKey="Ashley Koch A">A Ashley-Koch</name>
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<author><name sortKey="Telen, Mj" uniqKey="Telen M">MJ Telen</name>
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<biblStruct><analytic><author><name sortKey="Parent, F" uniqKey="Parent F">F Parent</name>
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<biblStruct><analytic><author><name sortKey="Nathan, Dg" uniqKey="Nathan D">DG Nathan</name>
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</analytic>
</biblStruct>
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   |texte=   Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
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Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom

Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom

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