Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005

Lanzkron, Sophie; Carroll, C. Patrick; Haywood, Carlton

doi:10.1177/003335491312800206

Cited by 395 publications

(386 citation statements)

References 19 publications

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“…In the most comprehensive study describing the survival of men and women with sickle cell anemia (SCA) 1978 to 1988, the median survival was 42 and 48 years, respectively [14]. In a more recent adult cohort from the National Center for Health Statistics including over 16,000 adults with SCD from 1979 to 2005, the median age at death in 2005 was 38 years for males and 42 years for females [15], not dramatically different from the previous era. In adults with Hb SS and Sb(0) followed at a tertiary care medical care center, the median age of death was 58 years of age [22], still significantly lower than that contemporary life-expectancy for African-American adults without SCD.…”

Section: Mortality In Scdmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

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Section: Mortality In Scdmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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“…QTc prolongation, cardiac ischemic events, and fatal arrhythmias are evident in antemortem EKG tracings of sickle mice. Compared with the normal sinus rhythm in a 2-mo-old WT control (1), antemortem EKG tracings of an age-matched Berk-SS mouse shows ST depression (2). Another 2-mo-old Berk-SS mouse's antemortem EKG tracings show slower heart rate and P-wave irregularity (3), which progressed into outright atrial flutter with 4-5:1 AV conduction (4), and eventual ventricular fibrillation (5).…”

Section: Berk-ss Mice Develop Corrected Qt Prolongation and Widening mentioning

confidence: 99%

“…Recurrent cycles of HbS polymerization result in a host of acute and chronic complications, including vaso-occlusive pain crisis, chronic hemolytic anemia, and organ damage. SCA-associated mortality rates have improved because of early diagnosis with universal newborn screening, immunization, and penicillin prophylaxis, which has changed the natural history of SCA (1,2) and the impact of SCA on organ pathologies is now becoming evident.…”

mentioning

confidence: 99%

Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology

Bakeer

James

Roy

et al. 2016

Proc. Natl. Acad. Sci. U.S.A.

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Cardiopulmonary complications are the leading cause of mortality in sickle cell anemia (SCA). Elevated tricuspid regurgitant jet velocity, pulmonary hypertension, diastolic, and autonomic dysfunction have all been described, but a unifying pathophysiology and mechanism explaining the poor prognosis and propensity to sudden death has been elusive. Herein, SCA mice underwent a longitudinal comprehensive cardiac analysis, combining state-of-the-art cardiac imaging with electrocardiography, histopathology, and molecular analysis to determine the basis of cardiac dysfunction. We show that in SCA mice, anemia-induced hyperdynamic physiology was gradually superimposed with restrictive physiology, characterized by progressive left atrial enlargement and diastolic dysfunction with preserved systolic function. This phenomenon was absent in WT mice with experimentally induced chronic anemia of similar degree and duration. Restrictive physiology was associated with microscopic cardiomyocyte loss and secondary fibrosis detectable as increased extracellular volume by cardiac-MRI. Ultrastructural mitochondrial changes were consistent with severe chronic hypoxia/ischemia and sarcomere diastolic-length was shortened. Transcriptome analysis revealed up-regulation of genes involving angiogenesis, extracellular-matrix, circadian-rhythm, oxidative stress, and hypoxia, whereas ion-channel transport and cardiac conduction were down-regulated. Indeed, progressive corrected QT prolongation, arrhythmias, and ischemic changes were noted in SCA mice before sudden death. Sudden cardiac death is common in humans with restrictive cardiomyopathies and long QT syndromes. Our findings may thus provide a unifying cardiac pathophysiology that explains the reported cardiac abnormalities and sudden death seen in humans with SCA.sickle cell anemia | cardiomyopathy | restrictive physiology | arrhythmias | sudden death S ickle cell anemia (SCA) results from a point mutation in the β-globin gene and affects millions world-wide. It is characterized by production of the mutant hemoglobin S (HbS), which polymerizes upon deoxygenation and distorts the shape of RBCs, increasing their propensity to hemolysis and microvascular occlusion. Recurrent cycles of HbS polymerization result in a host of acute and chronic complications, including vaso-occlusive pain crisis, chronic hemolytic anemia, and organ damage. SCA-associated mortality rates have improved because of early diagnosis with universal newborn screening, immunization, and penicillin prophylaxis, which has changed the natural history of SCA (1, 2) and the impact of SCA on organ pathologies is now becoming evident.Despite improved early survival, nearly one-third of young adults with SCA die suddenly (3-9). The sickle myocardium has been presumed to be relatively resistant to the effects of sickling (10), and studies/autopsies show little evidence of atherosclerosis or coronary disease (11)(12)(13)(14). Mildly increased systolic pulmonary arterial pressure (PAP) estimated by tricuspid regurgitant jet vel...

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“…A post-mortem, performed in 1898, of a patient who died in hospital after being admitted for pains, jaundice and previous leg ulcers shows that these symptoms are suggestive of SCD [22]. It has also been proposed that the clinical illness had been previously recognised in Africa as "cold-season rheumatism" and that the possibility of genetic inheritance of the illness had even been noted [10].…”

Section: Early Descriptionmentioning

confidence: 99%

Introductory Chapter: Introduction to the History, Pathology and Clinical Management of Sickle Cell Disease

Inusa¹,

Casale²,

Ward³

2016

Sickle Cell Disease - Pain and Common Chronic Complications

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Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005

Cited by 395 publications

References 19 publications

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology

Introductory Chapter: Introduction to the History, Pathology and Clinical Management of Sickle Cell Disease

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