Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom
Identifieur interne : 000702 ( Pmc/Checkpoint ); précédent : 000701; suivant : 000703Risk 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.
Abstract
The role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial.
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.
A TRV≥3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable.
Url:
DOI: 10.1371/journal.pone.0099489
PubMed: 24988120
PubMed Central: 4079316
Affiliations:
- Royaume-Uni, États-Unis
- Angleterre, Colorado, District de Columbia, Grand Londres, Géorgie (États-Unis), Illinois, Maryland, Ohio, Pennsylvanie, État de New York
- Londres, New York, Pittsburgh
- Université Columbia, Université de Pittsburgh
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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></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">24988120</idno><idno type="pmc">4079316</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4079316</idno><idno type="RBID">PMC:4079316</idno><idno type="doi">10.1371/journal.pone.0099489</idno><date when="2014">2014</date><idno type="wicri:Area/Pmc/Corpus">001D67</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">001D67</idno><idno type="wicri:Area/Pmc/Curation">001D42</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">001D42</idno><idno type="wicri:Area/Pmc/Checkpoint">000702</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">000702</idno></publicationStmt><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><author><name sortKey="Gladwin, Mark T" sort="Gladwin, Mark T" uniqKey="Gladwin M" first="Mark T." last="Gladwin">Mark T. Gladwin</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="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="Barst, Robyn J" sort="Barst, Robyn J" uniqKey="Barst R" first="Robyn J." last="Barst">Robyn J. Barst</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="Gibbs, J Simon R" sort="Gibbs, J Simon R" uniqKey="Gibbs J" first="J. Simon R." last="Gibbs">J. Simon R. Gibbs</name><affiliation wicri:level="3"><nlm:aff id="aff4"><addr-line>National Heart & Lung Institute, Imperial College London, London, United Kingdom</addr-line></nlm:aff><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>National Heart & Lung Institute, Imperial College London, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author><author><name sortKey="Hildesheim, Mariana" sort="Hildesheim, Mariana" uniqKey="Hildesheim M" first="Mariana" last="Hildesheim">Mariana Hildesheim</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="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></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><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><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Gladwin, Mt" uniqKey="Gladwin M">MT Gladwin</name></author><author><name sortKey="Vichinsky, E" uniqKey="Vichinsky E">E Vichinsky</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Rees, Dc" uniqKey="Rees D">DC Rees</name></author><author><name sortKey="Williams, Tn" uniqKey="Williams T">TN 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<front><journal-meta><journal-id journal-id-type="nlm-ta">PLoS One</journal-id><journal-id journal-id-type="iso-abbrev">PLoS ONE</journal-id><journal-id journal-id-type="publisher-id">plos</journal-id><journal-id journal-id-type="pmc">plosone</journal-id><journal-title-group><journal-title>PLoS ONE</journal-title></journal-title-group><issn pub-type="epub">1932-6203</issn><publisher><publisher-name>Public Library of Science</publisher-name><publisher-loc>San Francisco, USA</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">24988120</article-id><article-id pub-id-type="pmc">4079316</article-id><article-id pub-id-type="publisher-id">PONE-D-14-00813</article-id><article-id pub-id-type="doi">10.1371/journal.pone.0099489</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="Discipline-v2"><subject>Biology and Life Sciences</subject><subj-group><subject>Cell Biology</subject><subj-group><subject>Cellular Types</subject><subj-group><subject>Animal Cells</subject><subj-group><subject>Blood Cells</subject><subj-group><subject>Red Blood Cells</subject></subj-group></subj-group></subj-group></subj-group></subj-group></subj-group><subj-group subj-group-type="Discipline-v2"><subject>Medicine and Health Sciences</subject><subj-group><subject>Cardiology</subject></subj-group><subj-group><subject>Hematology</subject></subj-group><subj-group><subject>Pulmonology</subject><subj-group><subject>Pulmonary Vascular Diseases</subject></subj-group></subj-group></subj-group></article-categories><title-group><article-title>Risk Factors for Death in 632 Patients with Sickle Cell Disease in the United States and United Kingdom</article-title><alt-title alt-title-type="running-head">PH and Mortality in the Walk-PHASST Screening Cohort</alt-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Gladwin</surname><given-names>Mark T.</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref><xref ref-type="corresp" rid="cor1"><sup>*</sup></xref></contrib><contrib contrib-type="author"><name><surname>Barst</surname><given-names>Robyn J.</given-names></name><xref ref-type="aff" rid="aff3"><sup>3</sup></xref></contrib><contrib contrib-type="author"><name><surname>Gibbs</surname><given-names>J. Simon R.</given-names></name><xref ref-type="aff" rid="aff4"><sup>4</sup></xref></contrib><contrib contrib-type="author"><name><surname>Hildesheim</surname><given-names>Mariana</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff2"><sup>2</sup></xref></contrib><contrib contrib-type="author"><name><surname>Sachdev</surname><given-names>Vandana</given-names></name><xref ref-type="aff" rid="aff5"><sup>5</sup></xref></contrib><contrib contrib-type="author"><name><surname>Nouraie</surname><given-names>Mehdi</given-names></name><xref ref-type="aff" rid="aff6"><sup>6</sup></xref></contrib><contrib contrib-type="author"><name><surname>Hassell</surname><given-names>Kathryn L.</given-names></name><xref ref-type="aff" rid="aff7"><sup>7</sup></xref></contrib><contrib contrib-type="author"><name><surname>Little</surname><given-names>Jane A.</given-names></name><xref ref-type="aff" rid="aff8"><sup>8</sup></xref></contrib><contrib contrib-type="author"><name><surname>Schraufnagel</surname><given-names>Dean E.</given-names></name><xref ref-type="aff" rid="aff9"><sup>9</sup></xref></contrib><contrib contrib-type="author"><name><surname>Krishnamurti</surname><given-names>Lakshmanan</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref><xref ref-type="aff" rid="aff10"><sup>10</sup></xref></contrib><contrib contrib-type="author"><name><surname>Novelli</surname><given-names>Enrico</given-names></name><xref ref-type="aff" rid="aff1"><sup>1</sup></xref></contrib><contrib contrib-type="author"><name><surname>Girgis</surname><given-names>Reda E.</given-names></name><xref ref-type="aff" rid="aff11"><sup>11</sup></xref></contrib><contrib contrib-type="author"><name><surname>Morris</surname><given-names>Claudia R.</given-names></name><xref ref-type="aff" rid="aff12"><sup>12</sup></xref></contrib><contrib contrib-type="author"><name><surname>Berman Rosenzweig</surname><given-names>Erika</given-names></name><xref ref-type="aff" rid="aff3"><sup>3</sup></xref></contrib><contrib contrib-type="author"><name><surname>Badesch</surname><given-names>David B.</given-names></name><xref ref-type="aff" rid="aff7"><sup>7</sup></xref></contrib><contrib contrib-type="author"><name><surname>Lanzkron</surname><given-names>Sophie</given-names></name><xref ref-type="aff" rid="aff11"><sup>11</sup></xref></contrib><contrib contrib-type="author"><name><surname>Castro</surname><given-names>Oswaldo L.</given-names></name><xref ref-type="aff" rid="aff6"><sup>6</sup></xref></contrib><contrib contrib-type="author"><name><surname>Taylor</surname><given-names>James G.</given-names><suffix>VI</suffix></name><xref ref-type="aff" rid="aff5"><sup>5</sup></xref></contrib><contrib contrib-type="author"><name><surname>Goldsmith</surname><given-names>Jonathan C.</given-names></name><xref ref-type="aff" rid="aff13"><sup>13</sup></xref></contrib><contrib contrib-type="author"><name><surname>Kato</surname><given-names>Gregory J.</given-names></name><xref ref-type="aff" rid="aff5"><sup>5</sup></xref></contrib><contrib contrib-type="author"><name><surname>Gordeuk</surname><given-names>Victor R.</given-names></name><xref ref-type="aff" rid="aff9"><sup>9</sup></xref></contrib><contrib contrib-type="author"><name><surname>Machado</surname><given-names>Roberto F.</given-names></name><xref ref-type="aff" rid="aff9"><sup>9</sup></xref></contrib><contrib contrib-type="author"><collab>on behalf of the walk-PHaSST Investigators and Patients</collab></contrib></contrib-group><aff id="aff1"><label>1</label><addr-line>Vascular Medicine Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, United States of America</addr-line></aff><aff id="aff2"><label>2</label><addr-line>Division of Pulmonary Allergy and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States of America</addr-line></aff><aff id="aff3"><label>3</label><addr-line>Columbia University, New York, New York, United States of America</addr-line></aff><aff id="aff4"><label>4</label><addr-line>National Heart & Lung Institute, Imperial College London, London, United Kingdom</addr-line></aff><aff id="aff5"><label>5</label><addr-line>Cardiovascular Branch, NHLBI, Bethesda, Maryland, United States of America</addr-line></aff><aff id="aff6"><label>6</label><addr-line>Howard University, Washington, DC, United States of America</addr-line></aff><aff id="aff7"><label>7</label><addr-line>University of Colorado HSC, Denver, Colorado, United States of America</addr-line></aff><aff id="aff8"><label>8</label><addr-line>Case Western Reserve University, Cleveland, Ohio, United States of America</addr-line></aff><aff id="aff9"><label>9</label><addr-line>University of Illinois, Chicago, Illinois, United States of America</addr-line></aff><aff id="aff10"><label>10</label><addr-line>Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania, United States of America</addr-line></aff><aff id="aff11"><label>11</label><addr-line>Johns Hopkins University, Baltimore, Maryland, United States of America</addr-line></aff><aff id="aff12"><label>12</label><addr-line>Emory University School of Medicine, Atlanta, Georgia, United States of America</addr-line></aff><aff id="aff13"><label>13</label><addr-line>National Heart Lung and Blood Institute/NIH, Bethesda, Maryland, United States of America</addr-line></aff><contrib-group><contrib contrib-type="editor"><name><surname>West</surname><given-names>James</given-names></name><role>Editor</role><xref ref-type="aff" rid="edit1"></xref></contrib></contrib-group><aff id="edit1"><addr-line>Vanderbilt University Medical Center, United States of America</addr-line></aff><author-notes><corresp id="cor1">* E-mail: <email>gladwinmt@upmc.edu</email></corresp><fn fn-type="conflict"><p><bold>Competing Interests: </bold>The following authors have no conflicts to report: Castro, Girgis, Goldsmith, Hildesheim, Kato, Krishnamurti, Little, Machado, Nouraie, and Sachdev. Badesch has received honoraria for service on steering committees or advisory boards (or as a consultant) to the following companies working in the area of pulmonary hypertension: Actelion/CoTherix, Gilead, Pfizer, Mondo-Biotech/Mondogen, United Therapeutics/Lung Rx, GlaxoSmithKline, Lilly/ICOS, Bayer, Ikaria, and Arena. He has received grant support for clinical studies from GlaxoSmithKline, Actelion/CoTHerix, Gilead, Pfizer, United Therapeutics/Lung Rx, Lilly/ICOS, Bayer, and Novartis. He provided information pertinent to a legal matter for Actelion. Barst has received honoraria for consulting for: Actelion, Eli Lilly, Gilead, Ikaria, Novartis, Pfizer, VentriPoint, and owns stock/stock options in VentriPoint. Gibbs serves on advisory boards and/or performs consultancy work for Actelion, Bayer, Gilead, GSK, Novartis, Pfizer, and United Therapeutics. Mark Gladwin receives grant support from the NIH, Bayer Corporation, and Gilead Sciences. He is listed as a co-inventor on a US government patent for the use of nitrate salts and is a co-author on patent applications related to treating hemolysis. He also performs consulting for Bayer Corp and serves in an advisory capacity for Aires Pharmaceuticals. The Vascular Medicine Institute is funded by the Institute for Transfusion Medicine and the Hemophilia Center of Western Pennsylvania. Victor Gordeuk serves as a consultant for AesRx Pharmaceutical Company regarding developing a drug for sickle cell disease. He has also received an honorarium from AesRx and provided a testimonial review during a legal case involving sickle cell disease. Hassell has received research support for a multicenter study from Glycomimetics, Inc and Emmaus, Inc. and served on the advisory board for ApoPharma; AGA, Inc. Lanzkron serves on the scientific advisory board of Hemaquest. She also receives K award funding from the NHLBI K23HL083089. Morris is the inventor or co-inventor of patent applications owned by Children's Hospital & Research Center Oakland (CHRCO) involving biomarkers and therapies that target arginine dysregulation, and has received royalties from IP licensed from CHRCO to NourishLife for a nutritional supplement that targets oxidative stress. Novelli receives research funding from the American Society of Hematology Scholar Award and the Gilead Sciences Research Award. Berman Rosenzweig has received honoraria for consultation with Actelion, Gilead, and United Therapeutics in the past 2 years. She has received research funding from Actelion, Gilead, United Therapeutics, GSK, Eli Lily, Bayer, and Novartis in the past 2 years. Schraufnagel, MD serves on the Board of Directors for the International Union Against Tuberculosis and Lung Disease; Union North America. This does not alter the authors' adherence to PLOS ONE policies on sharing data and materials.</p></fn><fn fn-type="con"><p>Performed the experiments: RJB JSRG VS MN KLH JAL DES LK EN REG CRM EBR DBB SL OLC JGT JCG GJK VRG. Analyzed the data: MTG RFM MH. Contributed reagents/materials/analysis tools: MTG RFM MH RJB JSRG VS MN KLH JAL DES LK EN REG CRM EBR DBB SL OLC JGT JCG GJK VRG. Wrote the manuscript: MTG MH RFM. Edited the manuscript: RJB JSRG VS MN KLH JAL DES LK EN REG CRM EBR DBB SL OLC JGT JCG GJK VRG.</p></fn></author-notes><pub-date pub-type="collection"><year>2014</year></pub-date><pub-date pub-type="epub"><day>2</day><month>7</month><year>2014</year></pub-date><volume>9</volume><issue>7</issue><elocation-id>e99489</elocation-id><history><date date-type="received"><day>24</day><month>3</month><year>2014</year></date><date date-type="accepted"><day>15</day><month>5</month><year>2014</year></date></history><permissions><copyright-year>2014</copyright-year><license><license-p>This is an open-access article, free of all copyright, and may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose. The work is made available under the Creative Commons CC0 public domain dedication.</license-p></license></permissions><abstract><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></abstract><funding-group><funding-statement>This project has been founded with federal funds from the National Heart, Lung, and Blood Institute, National Institutes of Health, Department of Health and Human Services, under contract HHSN268200617182C. NHLBI was involved in the design and conduct of the original study and their staff had input in the writing of the manuscript.</funding-statement></funding-group><counts><page-count count="8"></page-count></counts></article-meta></front></pmc><affiliations><list><country><li>Royaume-Uni</li><li>États-Unis</li></country><region><li>Angleterre</li><li>Colorado</li><li>District de Columbia</li><li>Grand Londres</li><li>Géorgie (États-Unis)</li><li>Illinois</li><li>Maryland</li><li>Ohio</li><li>Pennsylvanie</li><li>État de New York</li></region><settlement><li>Londres</li><li>New York</li><li>Pittsburgh</li></settlement><orgName><li>Université Columbia</li><li>Université de Pittsburgh</li></orgName></list><tree><country name="États-Unis"><region name="Pennsylvanie"><name sortKey="Gladwin, Mark T" sort="Gladwin, Mark T" uniqKey="Gladwin M" first="Mark T." last="Gladwin">Mark T. Gladwin</name></region><name sortKey="Badesch, David B" sort="Badesch, David B" uniqKey="Badesch D" first="David B." last="Badesch">David B. Badesch</name><name sortKey="Barst, Robyn J" sort="Barst, Robyn J" uniqKey="Barst R" first="Robyn J." last="Barst">Robyn J. Barst</name><name sortKey="Berman Rosenzweig, Erika" sort="Berman Rosenzweig, Erika" uniqKey="Berman Rosenzweig E" first="Erika" last="Berman Rosenzweig">Erika Berman Rosenzweig</name><name sortKey="Castro, Oswaldo L" sort="Castro, Oswaldo L" uniqKey="Castro O" first="Oswaldo L." last="Castro">Oswaldo L. Castro</name><name sortKey="Girgis, Reda E" sort="Girgis, Reda E" uniqKey="Girgis R" first="Reda E." last="Girgis">Reda E. Girgis</name><name sortKey="Gladwin, Mark T" sort="Gladwin, Mark T" uniqKey="Gladwin M" first="Mark T." last="Gladwin">Mark T. Gladwin</name><name sortKey="Goldsmith, Jonathan C" sort="Goldsmith, Jonathan C" uniqKey="Goldsmith J" first="Jonathan C." last="Goldsmith">Jonathan C. Goldsmith</name><name sortKey="Gordeuk, Victor R" sort="Gordeuk, Victor R" uniqKey="Gordeuk V" first="Victor R." last="Gordeuk">Victor R. Gordeuk</name><name sortKey="Hassell, Kathryn L" sort="Hassell, Kathryn L" uniqKey="Hassell K" first="Kathryn L." last="Hassell">Kathryn L. Hassell</name><name sortKey="Hildesheim, Mariana" sort="Hildesheim, Mariana" uniqKey="Hildesheim M" first="Mariana" last="Hildesheim">Mariana Hildesheim</name><name sortKey="Hildesheim, Mariana" sort="Hildesheim, Mariana" uniqKey="Hildesheim M" first="Mariana" last="Hildesheim">Mariana Hildesheim</name><name sortKey="Kato, Gregory J" sort="Kato, Gregory J" uniqKey="Kato G" first="Gregory J." last="Kato">Gregory J. Kato</name><name sortKey="Krishnamurti, Lakshmanan" sort="Krishnamurti, Lakshmanan" uniqKey="Krishnamurti L" first="Lakshmanan" last="Krishnamurti">Lakshmanan Krishnamurti</name><name sortKey="Krishnamurti, Lakshmanan" sort="Krishnamurti, Lakshmanan" uniqKey="Krishnamurti L" first="Lakshmanan" last="Krishnamurti">Lakshmanan Krishnamurti</name><name sortKey="Lanzkron, Sophie" sort="Lanzkron, Sophie" uniqKey="Lanzkron S" first="Sophie" last="Lanzkron">Sophie Lanzkron</name><name sortKey="Little, Jane A" sort="Little, Jane A" uniqKey="Little J" first="Jane A." last="Little">Jane A. Little</name><name sortKey="Machado, Roberto F" sort="Machado, Roberto F" uniqKey="Machado R" first="Roberto F." last="Machado">Roberto F. Machado</name><name sortKey="Morris, Claudia R" sort="Morris, Claudia R" uniqKey="Morris C" first="Claudia R." last="Morris">Claudia R. Morris</name><name sortKey="Nouraie, Mehdi" sort="Nouraie, Mehdi" uniqKey="Nouraie M" first="Mehdi" last="Nouraie">Mehdi Nouraie</name><name sortKey="Novelli, Enrico" sort="Novelli, Enrico" uniqKey="Novelli E" first="Enrico" last="Novelli">Enrico Novelli</name><name sortKey="Sachdev, Vandana" sort="Sachdev, Vandana" uniqKey="Sachdev V" first="Vandana" last="Sachdev">Vandana Sachdev</name><name sortKey="Schraufnagel, Dean E" sort="Schraufnagel, Dean E" uniqKey="Schraufnagel D" first="Dean E." last="Schraufnagel">Dean E. Schraufnagel</name><name sortKey="Taylor, James G" sort="Taylor, James G" uniqKey="Taylor J" first="James G." last="Taylor">James G. Taylor</name></country><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Gibbs, J Simon R" sort="Gibbs, J Simon R" uniqKey="Gibbs J" first="J. Simon R." last="Gibbs">J. Simon R. Gibbs</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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