Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study.
<h4>Background</h4>Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient surv...
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Public Library of Science (PLoS)
2013-01-01
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| Online Access: | https://doi.org/10.1371/journal.pone.0079923 |
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| author | Deepika S Darbari Zhengyuan Wang Minjung Kwak Mariana Hildesheim James Nichols Darlene Allen Catherine Seamon Marlene Peters-Lawrence Anna Conrey Mary K Hall Gregory J Kato James G Taylor |
| author_facet | Deepika S Darbari Zhengyuan Wang Minjung Kwak Mariana Hildesheim James Nichols Darlene Allen Catherine Seamon Marlene Peters-Lawrence Anna Conrey Mary K Hall Gregory J Kato James G Taylor |
| author_sort | Deepika S Darbari |
| collection | DOAJ |
| description | <h4>Background</h4>Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.<h4>Methods</h4>Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation.<h4>Results</h4>Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality.<h4>Conclusions</h4>Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies.<h4>Trial registration</h4>ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/ |
| format | Article |
| id | doaj-art-47cfeb83c7514bb59e15ffefd67f7b5e |
| institution | Kabale University |
| issn | 1932-6203 |
| language | English |
| publishDate | 2013-01-01 |
| publisher | Public Library of Science (PLoS) |
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| spelling | doaj-art-47cfeb83c7514bb59e15ffefd67f7b5e2025-08-20T03:46:23ZengPublic Library of Science (PLoS)PLoS ONE1932-62032013-01-01811e7992310.1371/journal.pone.0079923Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study.Deepika S DarbariZhengyuan WangMinjung KwakMariana HildesheimJames NicholsDarlene AllenCatherine SeamonMarlene Peters-LawrenceAnna ConreyMary K HallGregory J KatoJames G Taylor<h4>Background</h4>Frequent painful vaso-occlusive crises (VOCs) were associated with mortality in the Cooperative Study of Sickle Cell Disease (CSSCD) over twenty years ago. Modern therapies for sickle cell anemia (SCA) like hydroxyurea are believed to have improved overall patient survival. The current study sought to determine the relevance of the association between more frequent VOCs and death and its relative impact upon overall mortality compared to other known risk factors in a contemporary adult SCA cohort.<h4>Methods</h4>Two hundred sixty four SCA adults were assigned into two groups based on patient reported outcomes for emergency department (ED) visits or hospitalizations for painful VOC treatment during the 12 months prior to evaluation.<h4>Results</h4>Higher baseline hematocrit (p = 0.0008), ferritin (p = 0.005), and HDL cholesterol (p = 0.01) were independently associated with 1 or more painful VOCs requiring an ED visit or hospitalization for acute pain. During a median follow-up of 5 years, mortality was higher in the ED visit/hospitalization group (relative risk [RR] 2.68, 95% CI 1.1-6.5, p = 0.03). Higher tricuspid regurgitatant jet velocity (TRV) (RR 2.41, 95% CI 1.5-3.9, p < 0.0001), elevated ferritin (RR 4.00, 95% CI 1.8-9.0, p = 0.001) and lower glomerular filtration rate (RR=2.73, 95% CI 1.6-4.6, p < 0.0001) were also independent risk factors for mortality.<h4>Conclusions</h4>Severe painful VOCs remain a marker for SCA disease severity and premature mortality in a modern cohort along with other known risk factors for death including high TRV, high ferritin and lower renal function. The number of patient reported pain crises requiring healthcare utilization is an easily obtained outcome that could help to identify high risk patients for disease modifying therapies.<h4>Trial registration</h4>ClinicalTrials.gov NCT00011648 http://clinicaltrials.gov/https://doi.org/10.1371/journal.pone.0079923 |
| spellingShingle | Deepika S Darbari Zhengyuan Wang Minjung Kwak Mariana Hildesheim James Nichols Darlene Allen Catherine Seamon Marlene Peters-Lawrence Anna Conrey Mary K Hall Gregory J Kato James G Taylor Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. PLoS ONE |
| title | Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. |
| title_full | Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. |
| title_fullStr | Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. |
| title_full_unstemmed | Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. |
| title_short | Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. |
| title_sort | severe painful vaso occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study |
| url | https://doi.org/10.1371/journal.pone.0079923 |
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