Document Detail


Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease.
MedLine Citation:
PMID:  20029950     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of hematology     Volume:  85     ISSN:  1096-8652     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2010 Jan 
Date Detail:
Created Date:  2009-12-28     Completed Date:  2010-01-13     Revised Date:  2014-01-10    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  36-40     Citation Subset:  IM    
Copyright Information:
(c) 2009 Wiley-Liss, Inc.
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MeSH Terms
Descriptor/Qualifier:
Academic Medical Centers / 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:
R01 HL068959/HL/NHLBI NIH HHS; R01 HL079915/HL/NHLBI NIH HHS; R01HL68959/HL/NHLBI NIH HHS; R01HL79915/HL/NHLBI NIH HHS; U54 HL070769/HL/NHLBI NIH HHS; U54HL070769/HL/NHLBI NIH HHS
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