Pulmonary hypertension in patients with sickle cell disease: a longitudinal study
Summary Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow‐up period of 2·6 years (range 0·2–5·1 years). Da...
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| Veröffentlicht in: | British journal of haematology 2006-07, Vol.134 (1), p.109-115 |
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| creator | Ataga, Kenneth I. Moore, Charity G. Jones, Susan Olajide, Oludamilola Strayhorn, Dell Hinderliter, Alan Orringer, Eugene P. |
| description | Summary
Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow‐up period of 2·6 years (range 0·2–5·1 years). Data were censored at the time of death or loss to follow‐up. Pulmonary hypertension was associated with an increased risk of death (relative risk, 9·24; 95% confidence interval: 1·2–73·3; P = 0·01). There was no difference in the risk of death when patients with different degrees of PHT were compared. Lactate dehydrogenase and blood urea nitrogen were significantly associated with PHT in a logistic regression model. Higher levels of fetal haemoglobin and treatment with hydroxycarbamide were observed more frequently in patients without PHT. Thirteen per cent of patients with no previous evidence of PHT developed PHT following 3 years of observation. In conclusion: (1) PHT, regardless of severity, is associated with an increased risk of death in SCD patients; (2) haemolysis is strongly associated with PHT in SCD; (3) high levels of fetal haemoglobin and hydroxycarbamide therapy may decrease the occurrence of PHT; (4) screening for PHT is indicated for SCD patients in their non‐crisis, steady states. |
| doi_str_mv | 10.1111/j.1365-2141.2006.06110.x |
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Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow‐up period of 2·6 years (range 0·2–5·1 years). Data were censored at the time of death or loss to follow‐up. Pulmonary hypertension was associated with an increased risk of death (relative risk, 9·24; 95% confidence interval: 1·2–73·3; P = 0·01). There was no difference in the risk of death when patients with different degrees of PHT were compared. Lactate dehydrogenase and blood urea nitrogen were significantly associated with PHT in a logistic regression model. Higher levels of fetal haemoglobin and treatment with hydroxycarbamide were observed more frequently in patients without PHT. Thirteen per cent of patients with no previous evidence of PHT developed PHT following 3 years of observation. In conclusion: (1) PHT, regardless of severity, is associated with an increased risk of death in SCD patients; (2) haemolysis is strongly associated with PHT in SCD; (3) high levels of fetal haemoglobin and hydroxycarbamide therapy may decrease the occurrence of PHT; (4) screening for PHT is indicated for SCD patients in their non‐crisis, steady states.</description><identifier>ISSN: 0007-1048</identifier><identifier>EISSN: 1365-2141</identifier><identifier>DOI: 10.1111/j.1365-2141.2006.06110.x</identifier><identifier>PMID: 16803576</identifier><identifier>CODEN: BJHEAL</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>Adult ; Anemia, Sickle Cell - complications ; Anemia, Sickle Cell - mortality ; Anemias. Hemoglobinopathies ; Antisickling Agents - therapeutic use ; Biological and medical sciences ; Blood Urea Nitrogen ; Chi-Square Distribution ; Diseases of red blood cells ; Echocardiography, Doppler ; Female ; Fetal Hemoglobin - analysis ; Follow-Up Studies ; haemolysis ; Hematologic and hematopoietic diseases ; Humans ; Hydroxyurea - therapeutic use ; Hypertension, Pulmonary - complications ; Hypertension, Pulmonary - mortality ; L-Lactate Dehydrogenase - blood ; Logistic Models ; Male ; Medical sciences ; Middle Aged ; mortality ; Pneumology ; progression ; pulmonary hypertension ; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases ; Risk ; sickle cell disease</subject><ispartof>British journal of haematology, 2006-07, Vol.134 (1), p.109-115</ispartof><rights>2006 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3270-e8bc9bf81260fa717cf61a8bb468836be3388061963b2d2cb83970b5dc4e04c23</citedby><cites>FETCH-LOGICAL-c3270-e8bc9bf81260fa717cf61a8bb468836be3388061963b2d2cb83970b5dc4e04c23</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1365-2141.2006.06110.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1365-2141.2006.06110.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,1433,27924,27925,45574,45575,46409,46833</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=17828592$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16803576$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ataga, Kenneth I.</creatorcontrib><creatorcontrib>Moore, Charity G.</creatorcontrib><creatorcontrib>Jones, Susan</creatorcontrib><creatorcontrib>Olajide, Oludamilola</creatorcontrib><creatorcontrib>Strayhorn, Dell</creatorcontrib><creatorcontrib>Hinderliter, Alan</creatorcontrib><creatorcontrib>Orringer, Eugene P.</creatorcontrib><title>Pulmonary hypertension in patients with sickle cell disease: a longitudinal study</title><title>British journal of haematology</title><addtitle>Br J Haematol</addtitle><description>Summary
Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow‐up period of 2·6 years (range 0·2–5·1 years). Data were censored at the time of death or loss to follow‐up. Pulmonary hypertension was associated with an increased risk of death (relative risk, 9·24; 95% confidence interval: 1·2–73·3; P = 0·01). There was no difference in the risk of death when patients with different degrees of PHT were compared. Lactate dehydrogenase and blood urea nitrogen were significantly associated with PHT in a logistic regression model. Higher levels of fetal haemoglobin and treatment with hydroxycarbamide were observed more frequently in patients without PHT. Thirteen per cent of patients with no previous evidence of PHT developed PHT following 3 years of observation. In conclusion: (1) PHT, regardless of severity, is associated with an increased risk of death in SCD patients; (2) haemolysis is strongly associated with PHT in SCD; (3) high levels of fetal haemoglobin and hydroxycarbamide therapy may decrease the occurrence of PHT; (4) screening for PHT is indicated for SCD patients in their non‐crisis, steady states.</description><subject>Adult</subject><subject>Anemia, Sickle Cell - complications</subject><subject>Anemia, Sickle Cell - mortality</subject><subject>Anemias. Hemoglobinopathies</subject><subject>Antisickling Agents - therapeutic use</subject><subject>Biological and medical sciences</subject><subject>Blood Urea Nitrogen</subject><subject>Chi-Square Distribution</subject><subject>Diseases of red blood cells</subject><subject>Echocardiography, Doppler</subject><subject>Female</subject><subject>Fetal Hemoglobin - analysis</subject><subject>Follow-Up Studies</subject><subject>haemolysis</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Humans</subject><subject>Hydroxyurea - therapeutic use</subject><subject>Hypertension, Pulmonary - complications</subject><subject>Hypertension, Pulmonary - mortality</subject><subject>L-Lactate Dehydrogenase - blood</subject><subject>Logistic Models</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>mortality</subject><subject>Pneumology</subject><subject>progression</subject><subject>pulmonary hypertension</subject><subject>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</subject><subject>Risk</subject><subject>sickle cell disease</subject><issn>0007-1048</issn><issn>1365-2141</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkF1PwyAUhonRuPnxFww3etd5KC0wEy908TMmaqLXBChVJqOztNn2723d4m7lhpOc58B7HoQwgRHpzvl0RCjLk5RkZJQCsBEw0vWWO2j419hFQwDgCYFMDNBBjFMAQiEn-2hAmACaczZEry-tn1VB1Sv8uZrburEhuipgF_BcNc6GJuKFaz5xdObLW2ys97hw0apoL7DCvgofrmkLF5THsStWR2ivVD7a4819iN5vb94m98nT893D5OopMTTlkFihzViXgqQMSsUJNyUjSmidMSEo05ZSIbq1xozqtEiNFnTMQeeFySxkJqWH6Gz97ryuvlsbGzlzsY-ngq3aKJnIBecAHSjWoKmrGGtbynntZt3GkoDsdcqp7K3J3prsdcpfnXLZjZ5s_mj1zBbbwY2_DjjdACoa5ctaBePiluMiFfm4D3u55hbO29W_A8jrx_u-oj98aJB1</recordid><startdate>200607</startdate><enddate>200607</enddate><creator>Ataga, Kenneth I.</creator><creator>Moore, Charity G.</creator><creator>Jones, Susan</creator><creator>Olajide, Oludamilola</creator><creator>Strayhorn, Dell</creator><creator>Hinderliter, Alan</creator><creator>Orringer, Eugene P.</creator><general>Blackwell Publishing Ltd</general><general>Blackwell</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200607</creationdate><title>Pulmonary hypertension in patients with sickle cell disease: a longitudinal study</title><author>Ataga, Kenneth I. ; Moore, Charity G. ; Jones, Susan ; Olajide, Oludamilola ; Strayhorn, Dell ; Hinderliter, Alan ; Orringer, Eugene P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3270-e8bc9bf81260fa717cf61a8bb468836be3388061963b2d2cb83970b5dc4e04c23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2006</creationdate><topic>Adult</topic><topic>Anemia, Sickle Cell - complications</topic><topic>Anemia, Sickle Cell - mortality</topic><topic>Anemias. Hemoglobinopathies</topic><topic>Antisickling Agents - therapeutic use</topic><topic>Biological and medical sciences</topic><topic>Blood Urea Nitrogen</topic><topic>Chi-Square Distribution</topic><topic>Diseases of red blood cells</topic><topic>Echocardiography, Doppler</topic><topic>Female</topic><topic>Fetal Hemoglobin - analysis</topic><topic>Follow-Up Studies</topic><topic>haemolysis</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Humans</topic><topic>Hydroxyurea - therapeutic use</topic><topic>Hypertension, Pulmonary - complications</topic><topic>Hypertension, Pulmonary - mortality</topic><topic>L-Lactate Dehydrogenase - blood</topic><topic>Logistic Models</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>mortality</topic><topic>Pneumology</topic><topic>progression</topic><topic>pulmonary hypertension</topic><topic>Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases</topic><topic>Risk</topic><topic>sickle cell disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ataga, Kenneth I.</creatorcontrib><creatorcontrib>Moore, Charity G.</creatorcontrib><creatorcontrib>Jones, Susan</creatorcontrib><creatorcontrib>Olajide, Oludamilola</creatorcontrib><creatorcontrib>Strayhorn, Dell</creatorcontrib><creatorcontrib>Hinderliter, Alan</creatorcontrib><creatorcontrib>Orringer, Eugene P.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>British journal of haematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ataga, Kenneth I.</au><au>Moore, Charity G.</au><au>Jones, Susan</au><au>Olajide, Oludamilola</au><au>Strayhorn, Dell</au><au>Hinderliter, Alan</au><au>Orringer, Eugene P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pulmonary hypertension in patients with sickle cell disease: a longitudinal study</atitle><jtitle>British journal of haematology</jtitle><addtitle>Br J Haematol</addtitle><date>2006-07</date><risdate>2006</risdate><volume>134</volume><issue>1</issue><spage>109</spage><epage>115</epage><pages>109-115</pages><issn>0007-1048</issn><eissn>1365-2141</eissn><coden>BJHEAL</coden><abstract>Summary
Although pulmonary hypertension (PHT) is a common complication in patients with sickle cell disease (SCD), the rate of development of PHT and the factors that affect disease progression are unknown. We observed 93 patients over a median follow‐up period of 2·6 years (range 0·2–5·1 years). Data were censored at the time of death or loss to follow‐up. Pulmonary hypertension was associated with an increased risk of death (relative risk, 9·24; 95% confidence interval: 1·2–73·3; P = 0·01). There was no difference in the risk of death when patients with different degrees of PHT were compared. Lactate dehydrogenase and blood urea nitrogen were significantly associated with PHT in a logistic regression model. Higher levels of fetal haemoglobin and treatment with hydroxycarbamide were observed more frequently in patients without PHT. Thirteen per cent of patients with no previous evidence of PHT developed PHT following 3 years of observation. In conclusion: (1) PHT, regardless of severity, is associated with an increased risk of death in SCD patients; (2) haemolysis is strongly associated with PHT in SCD; (3) high levels of fetal haemoglobin and hydroxycarbamide therapy may decrease the occurrence of PHT; (4) screening for PHT is indicated for SCD patients in their non‐crisis, steady states.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>16803576</pmid><doi>10.1111/j.1365-2141.2006.06110.x</doi><tpages>7</tpages></addata></record> |
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| subjects | Adult Anemia, Sickle Cell - complications Anemia, Sickle Cell - mortality Anemias. Hemoglobinopathies Antisickling Agents - therapeutic use Biological and medical sciences Blood Urea Nitrogen Chi-Square Distribution Diseases of red blood cells Echocardiography, Doppler Female Fetal Hemoglobin - analysis Follow-Up Studies haemolysis Hematologic and hematopoietic diseases Humans Hydroxyurea - therapeutic use Hypertension, Pulmonary - complications Hypertension, Pulmonary - mortality L-Lactate Dehydrogenase - blood Logistic Models Male Medical sciences Middle Aged mortality Pneumology progression pulmonary hypertension Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases Risk sickle cell disease |
| title | Pulmonary hypertension in patients with sickle cell disease: a longitudinal study |
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