| Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease. | |
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MedLine Citation:
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PMID: 20029950 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled. Forty-three patients (21 males and 22 females) died during the study period. The median age of survival was 39 years for females (95% CI: 34-56), 40 years for males (95% CI: 34-48), and 40 years overall (95% CI: 35-48). Cardiac causes of death accounted for 25.6% (11/43 patients); pulmonary, 14.0% (six patients); other SCD related, 32.6% (14 patients); unknown, 14.0% (six patients); and others, 14.0% (six patients). Pulseless electrical activity arrest, pulmonary emboli, multiorgan failure, and stroke were the most frequent causes of death. Among the deceased patients, the most common premorbid conditions were cardiopulmonary: acute chest syndrome/pneumonia (58.1%), Pulmonary hypertension (pHTN; 41.9%), systemic HTN (25.6%), congestive heart failure (25.6%), myocardial infarction (20.9%), and arrhythmias (14.0%). Tricuspid regurgitant jet velocity was significantly higher (3.1 m/sec vs. 2.6 m/sec, P < 0.001) and hemoglobin significantly lower (8.3 g/dL vs. 9.2 g/dL, P < 0.05) in deceased patients when compared with patients who lived, respectively. With improved preventive and therapeutic advances, including hydroxyurea therapy, acute complications such as infection are no longer the leading cause of death; instead, causes of death and premorbid conditions are shifting to chronic cardiopulmonary complications. Further, arrhythmia leading to premature death is under-recognized in SCD and warrants further investigation. |
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Authors:
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Courtney D Fitzhugh; Naudia Lauder; Jude C Jonassaint; Marilyn J Telen; Xiongce Zhao; Elizabeth C Wright; Francis R Gilliam; Laura M De Castro |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: American journal of hematology Volume: 85 ISSN: 1096-8652 ISO Abbreviation: Am. J. Hematol. Publication Date: 2010 Jan |
Date Detail:
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Created Date: 2009-12-28 Completed Date: 2010-01-13 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7610369 Medline TA: Am J Hematol Country: United States |
Other Details:
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Languages: eng Pagination: 36-40 Citation Subset: IM |
Copyright Information:
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(c) 2009 Wiley-Liss, Inc. |
Affiliation:
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Molecular and Clinical Hematology Branch, National Heart, Lung, and Blood Institute and National Institute of Diabetes, Digestive, and Kidney Diseases/NIH, 9000 Rockville Pike, Bethesda, MD 20892, USA. fitzhughc@nhlbi.nih.gov |
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| MeSH Terms | |
Descriptor/Qualifier:
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Academic Medical Centers
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statistics & numerical data Adult Aged Aged, 80 and over Anemia, Sickle Cell / complications*, mortality Cardiovascular Diseases / complications*, mortality* Female Humans Incidence Lung Diseases / complications*, mortality* Male Middle Aged North Carolina / epidemiology Prospective Studies Young Adult |
| Grant Support | |
ID/Acronym/Agency:
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R01HL68959/HL/NHLBI NIH HHS; R01HL79915/HL/NHLBI NIH HHS; U54HL070769/HL/NHLBI NIH HHS |
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