Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death

Platt, Orah S.; Brambilla, Donald; Rosse, Wendell F.; Milner, Paul F.; Castro, Oswaldo; Steinberg, Martin H.; Klug, Panpit

doi:10.1056/nejm199406093302303

Cited by 2,902 publications

(2,415 citation statements)

References 14 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.…”

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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“…Although its course is unpredictable, the disease is often associated with substantial morbidity, a decreased life span, and a poor quality of life. The risk of early death is highest among patients who have had severe complications, such as recurrent acute chest syndrome, renal failure, and pulmonary hypertension 2829. In contrast, many affected individuals have a good quality of life, and additional genetic and environmental factors may reduce the severity of the disease in some parts of the world.…”

Section: Clinical Epidemiology Of Sickle Cell Disease In Saudi Arabiamentioning

confidence: 99%

Epidemiology of sickle cell disease in Saudi Arabia

Jastaniah

2011

Annals of Saudi Medicine

210

187

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Sickle cell disease (SCD) is an autosomal recessive disorder characterized by production of abnormal hemoglobin S and is associated with high morbidity and mortality. Information about the prevalence of SCD in Saudi Arabia is patchy and probably underestimated, but studies have reported that SCD is a relatively common genetic disorder in this part of the world. The prevalence of SCD in Saudi Arabia varies significantly in different parts of the country, with the highest prevalence is in the Eastern province, followed by the southwestern provinces. The reported prevalence for sickle-cell trait ranges from 2% to 27%, and up to 2.6% will have SCD in some areas. Clinical and hematological variability exists in SCD in Saudi Arabia with two major phenotypes: a mild phenotype and a severe phenotype. Further studies on the prevalence, molecular and clinical epidemiology of SCD may help predict disease severity and risk stratification of patients to determine whether to receive early intensive care or continued symptomatic care.

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“…1 As many of the complications of sickle cell anemia (homozygosity for HBB, glu6val), like osteonecrosis, acute chest syndrome and painful episode, are associated with the level of HbF, and, HbF is inversely associated with mortality, investigators have assiduously sought pharmacological means of increasing HbF production. [2][3][4][5][6] Hydroxyurea (HU), a ribonucleotide reductase inhibitor, is one drug that increases HbF concentration in patients with sickle cell anemia [7][8][9][10] and it is the sole FDA-approved agent for treating sickle cell anemia. Most, but not all patients respond to HU treatment with an increase in HbF, but as with the baseline HbF concentration, which varies widely among patients, the magnitude of the HbF response to HU is also variable.…”

Section: Introductionmentioning

confidence: 99%

Fetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea

et al. 2007

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The increase in fetal hemoglobin (HbF) in response to hydroxyurea (HU) varies among patients with sickle cell anemia. Twenty-nine candidate genes within loci previously reported to be linked to HbF level (6q22.3-q23.2, 8q11-q12 and Xp22.2-p22.3), involved in metabolism of HU and related to erythroid progenitor proliferation were studied in 137 sickle cell anemia patients treated with HU. Three-hundred and twenty tagging single nucleotide polymorphisms (SNPs) for genotyping were selected based on HapMap data. Multiple linear regression and the nonlinear regression Random Forest method were used to investigate the association between SNPs and the change in HbF level after 2 years of treatment with HU. Both methods revealed that SNPs in genes within the 6q22.3-23.2 and 8q11-q12 linkage peaks, and also the ARG2, FLT1, HAO2 and NOS1 genes were associated with the HbF response to HU. Polymorphisms in genes regulating HbF expression, HU metabolism and erythroid progenitor proliferation might modulate the patient response to HU.

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Mortality In Sickle Cell Disease -- Life Expectancy and Risk Factors for Early Death

Cited by 2,902 publications

References 14 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

Epidemiology of sickle cell disease in Saudi Arabia

Fetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea

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