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Mortality in sickle cell disease. Life expectancy and risk factors for early death
Open accessAbstract
BACKGROUND: Information on life expectancy and risk factors for early death among patients with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and the sickle cell-beta-thalassemias) is needed to counsel patients, target therapy, and design clinical trials. METHODS: We followed 3764 patients who ranged from birth to 66 years of age at enrollment to determine the life expectancy and calculate the median age at death. In addition, we investigated the circumstances of death for all 209 adult patients who died during the study, and used proportional-hazards regression analysis to identify risk factors for early death among 964 adults with sickle cell anemia who were followed for at least two years. RESULTS: Among children and adults with sickle cell anemia (homozygous for sickle hemoglobin), the median age at death was 42 years for males and 48 years for females. Among those with sickle cell-hemoglobin C disease, the median age at death was 60 years for males and 68 years for females. Among adults with sickle cell disease, 18 percent of the deaths occurred in patients with overt organ failure, predominantly renal. Thirty-three percent were clinically free of organ failure but died during an acute sickle crisis (78 percent had pain, the chest syndrome, or both; 22 percent had stroke). Modeling revealed that in patients with sickle cell anemia, the acute chest syndrome, renal failure, seizures, a base-line white-cell count above 15,000 cells per cubic millimeter, and a low level of fetal hemoglobin were associated with an increased risk of early death. CONCLUSIONS: Fifty percent of patients with sickle cell anemia survived beyond the fifth decade. A large proportion of those who died had no overt chronic organ failure but died during an acute episode of pain, chest syndrome, or stroke. Early mortality was highest among patients whose disease was symptomatic. A high level of fetal hemoglobin predicted improved survival and is probably a reliable childhood forecaster of adult life expectancy.
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“Elevated levels of fetal hemoglobin (consisting of two alpha and two gamma chains) are associated with improved morbidity and mortality in patients with TDT and SCD.[20][21][22]”
From CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia (2021) · cites this paper as [21]
Experimental — accuracy not yet measuredThe source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
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“the presence of chronic kidney disease is an independent predictor of pulmonary vascular disease and early mortality in adults with SCD (68, 202).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [68]
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“In 3764 patients in the pre-hydroxyurea era, HbF .8.6% was associated with improved survival.60”
From Fetal hemoglobin in sickle cell anemia (2020) · cites this paper as [60]
Experimental — accuracy not yet measuredIn the citing paper
“Patients affected by sickle cell anemia and β-thalassemia experience a milder course of the disease if they express elevated levels of fetal hemoglobin in adulthood (Platt et al., 1994; Weatherall, 2001).”
From Reactivation of developmentally silenced globin genes by forced chromatin looping (2014) · cites this paper as Platt et al., 1994
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“…(ACS), characterized by fever, respiratory symptoms and a new pulmonary infiltrate, is responsible for much of the morbidity of sickle cell disease (SCD), is the second most common cause of hospitalization, and is the leading cause of death (Platt, et al 1994, Sprinkle, et al 1986, Vichinsky 1991).”
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Powars, D. R. (2000) · British Journal of Haematology · doi:10.1046/j.1365-2141.2000.01912.x · Cited by 53
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“Moreover, ACS was associated with higher mortality in adult patients with SCD than in pediatric patients with SCD (4.3% vs. 1.1”
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“death of 42 years in males and 48 years for females (4).”
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Differences between males and females in adult sickle cell pain crisis in eastern Saudi Arabia
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“This classification was chosen because these criteria have previously been used to demonstrate the association between frequent painful events and mortality and as an endpoint in two SCA placebo controlled, efficacy trials [3,22,23].”
MethodSelf-citationSerum Interleukin-6 Levels in the Steady State of Sickle Cell Disease
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“The diagnosis of early-stage ACS may be difficult [1, 12 ] as the clinical presentation consists of nonspecific manifestations such as a fever, chest pain, tachypnea, wheezing, coughing, and/or a new pulmonary infiltrate [2, 4]. Hypoxemia is a feature in about 70% of episodes [13, 14].”
MethodSickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events
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“It is thought to favorably modify the course of disease (5, 8, 31).”
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“Mortality is high with a median age at death of 42 years for males and 48 years for females homozygous for the hemoglobin S mutation (HbSS) in the pre-hydroxyurea era [2,3].”
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“The increased indel frequency and size yielded an overall increase in total HbF expression in vitro of as high as 41%, levels well above a clinically relevant threshold of 10%–20%.(7)”
BackgroundBlood transfusion in sickle cell disease
Davies, S. C. (1997) · Blood Reviews · doi:10.1016/s0268-960x(97)90012-6 · Cited by 51
Cord blood stem cell transplantation for haemoglobinopathies
Pinto, F. O., & Roberts, I. A. G. (2008) · British Journal of Haematology · doi:10.1111/j.1365-2141.2008.07016.x · Cited by 51
“…iron chelators and hydroxycarbamide, now offers hope for a better life expectancy for many patients and a much improved quality of life (Borgna-Pignatti et al, 2004; Miller et al, 2000; Modell et al, 2000; Olivieri et al, 1994; Platt et al, 1994; Steinberg et al, 2003; Telfer et al, 2007, 2006).”
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“It is the leading cause of death in sickle cell disease, and many patients suffer from multiple, severe episodes (1,2).”
BackgroundClose, J. P., Gamé, L., Clark, B. E., Bergounioux, J., Gerovassili, A., & Thein, S. L. (2004) · BMC Genomics · doi:10.1186/1471-2164-5-33 · Cited by 51
“In particular, a number of studies have shown that an increased fetal hemoglobin (HbF; α2γ2) response in conjunction with either sickle cell disease or β-thalassemia has an ameliorating effect on the clinical disease [3,4].”
BackgroundSamuelson, C., Radtke, S., Zhu, H., Llewellyn, M. J., Fields, E., Cook, S. M., Huang, M.-L. W., Jerome, K. R., Kiem, H., & Humbert, O. (2021) · Molecular Therapy — Methods & Clinical Development · doi:10.1016/j.omtm.2021.10.008 · Cited by 51
“Where HPFH is co-inherited with SCD or b-thalassemia, it ameliorates the disease phenotype and, in some cases, renders individuals entirely asymptomatic.(6,17,18)”
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“In the United States, survival during childhood has improved significantly due to better supportive care for children with SCA [4,5], but average life expectancy remains half that of the general population (40 years for a male with HbSS and 48 years for a female HbSS patient) [2].”
BackgroundDevelopment and validation of a pediatric severity index for sickle cell patients
van den Tweel, X. W., van der Lee, J. H., Heijboer, H., Peters, M., & Fijnvandraat, K. (2010) · American Journal of Hematology · doi:10.1002/ajh.21846 · Cited by 50
“Patients with a severe clinical course suffer from recurrent painful vaso-occlusive crises, develop irreversible organ damage at an early age, and may die prematurely [1].”
BackgroundAnemia is Associated with Poor Outcomes in Patients with Less Severe Ischemic Stroke
Sico, J. J., Concato, J., Wells, C. K., Lo, A., Nadeau, S. E., Williams, L. S., Peixoto, A. J., Gorman, M., Boice, J. L., & Bravata, D. M. (2011) · Journal of Stroke and Cerebrovascular Diseases · doi:10.1016/j.jstrokecerebrovasdis.2011.09.003 · Cited by 50
Foetal haemoglobin and disease severity in sickle cell anaemia patients in Kampala Uganda
Mpalampa, L., Ndugwa, C. M., Ddungu, H., & Idro, R. (2012) · BMC Blood Disorders · doi:10.1186/1471-2326-12-11 · Cited by 50
“Populations with high HbF levels like those in Eastern Saudi Arabia have been described as having less severe disease with fewer complications [9] and better survival [10].”
BackgroundAnderson, B. R., Howell, D. N., Soldano, K. L., Garrett, M. E., Katsanis, N., Telen, M. J., Davis, E. E., & Ashley‐Koch, A. E. (2015) · PLoS Genetics · doi:10.1371/journal.pgen.1005349 · Cited by 50
Outcomes of Adult Patients With Sickle Cell Disease Admitted to the ICU
Cecchini, J., Lionnet, F., Djibré, M., Parrot, A., Stankovic, K., Girot, R., & Fartoukh, M. S. (2014) · Critical Care Medicine · doi:10.1097/ccm.0000000000000316 · Cited by 50
“Cerebral vasculopathy 12 (9) 9 (8) 3 (11) 0.”
BackgroundMIR-144-mediated NRF2 gene silencing inhibits fetal hemoglobin expression in sickle cell disease
Li, Y., Zhu, X., Ward, C. M., Starlard‐Davenport, A., Takezaki, M., Berry, A., Ward, A., Wilder, C., Neunert, C. E., Kutlar, A., et al. (2018) · Experimental Hematology · doi:10.1016/j.exphem.2018.11.002 · Cited by 51
“Hydroxyurea is the only Food and Drug Administration-approved drug that ameliorates the clinical symptoms of SCD through HbF induction and other beneficial properties such as increasing nitric oxide levels and anti-inflammatory effects [2,3].”
BackgroundHaploidentical CD3 or α/β T-cell depleted HSCT in advanced stage sickle cell disease
Foell, J. L., Schulte, J. H., Pfirstinger, B., Troeger, A., Wolff, D., Edinger, M., Hofmann, P., Aslanidis, C., Lang, P., Holler, E., et al. (2019) · Bone Marrow Transplantation · doi:10.1038/s41409-019-0550-0 · Cited by 50
Type 2 cytokine serum levels in healthy sickle cell disease patients.
Taylor, S. C., SHACKS, S. J., Qu, Z., & Wiley, P. (1997) · PubMed Central · Cited by 50
Nolan, V. G., Ma, Q., Cohen, H. T., Adewoye, A. H., Rybicki, A. C., Baldwin, C. T., Mahabir, R. N., Homan, E. P., Wyszynski, D. F., Fabry, M. E., et al. (2006) · American Journal of Hematology · doi:10.1002/ajh.20800 · Cited by 50
“Renal complications are a well known cause of morbidity and mortality in sickle cell disease, and the incidence of renal failure increases as overall patient survival improves [1–10].”
BackgroundSelf-citationAli, S. B., MooSang, M., King, L., Knight‐Madden, J., & Reid, M. E. (2011) · American Journal of Hematology · doi:10.1002/ajh.22142 · Cited by 50
“Introduction Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by chronic hemolytic anemia, a vasculopathy leading to hypoxic-ischemic tissue injury and ultimately decreased life expectancy [1].”
BackgroundVlieg‐Boerstra, B., der Heide, S. V., Elberink, J. N. G. O., Kluin‐Nelemans, H. C., & Dubois, A. E. J. (2005) · PubMed · Cited by 50
Survival and mortality among users and non-users of hydroxyurea with sickle cell disease
de Araújo, O. M. R., Ivo, M. L., Júnior, M. A. F., Pontes, E. R. J. C., Bispo, I. M. G. P., & de Oliveira, É. C. L. (2015) · Revista Latino-Americana de Enfermagem · doi:10.1590/0104-1169.3385.2526 · Cited by 49
“Actualmente los avances en el tratamiento y los estudios de sobrevida con pacientes falciformes demuestran que la expectativa de vida viene mejorando considerablemente((4)).”
BackgroundInfluentialTransplantation for liver failure in patients with sickle cell disease
Hurtova, M., Bachir, D., Lee, K., Caldéraro, J., Decaens, T., Kluger, M. D., Zafrani, É. S., Cherqui, D., Mallat, A., Galactéros, F., et al. (2011) · Liver Transplantation · doi:10.1002/lt.22257 · Cited by 49
Lower airway obstruction is associated with increased morbidity in children with sickle cell disease
Boyd, J. H., DeBaun, M. R., Morgan, W. J., Mao, J., & Strunk, R. C. (2009) · Pediatric Pulmonology · doi:10.1002/ppul.20998 · Cited by 49
Protein C and S and inflammation in sickle cell disease
Schnog, J. B., Gillavry, M. R. M., van Zanten, A. P., Meijers, J. C. M., Rojer, R. A., Duits, A. J., Cate, H. T., & Brandjes, D. P. M. (2004) · American Journal of Hematology · doi:10.1002/ajh.20052 · Cited by 49
“Low HbF% and high leukocyte counts are associated with early mortality, frequent painful crises, stroke, and ACS; high hemoglobin levels lead to more pain, whereas lower levels are a risk factor for both clinical and subclinical cerebrovascular disease and leg ulceration [38–43].”
Sickle Cell Disease: An Opportunity for Palliative Care Across the Life Span
Wilkie, D. J., Johnson, B., Mack, A. K., Labotka, R., & Molokie, R. E. (2010) · Nursing Clinics of North America · doi:10.1016/j.cnur.2010.03.003 · Cited by 49
“Ethical guidelines require five elements in order for informed consent to occur: (1) person must have capacity to make decisions; (2) all relevant facts must be disclosed; (3) person must understand what has been disclosed; (4) the decision must be voluntary; and (5) the person must give consent.”
BackgroundRecommandations pratiques de prise en charge de la drépanocytose de l’adulte
Lionnet, F., Arlet, J. B., Bartolucci, P., Habibi, A., Ribeil, J., & Stankovic, K. (2009) · La Revue de Médecine Interne · doi:10.1016/j.revmed.2009.07.001 · Cited by 49
“L’amélioration continue de l’espérance de vie a modifié l’histoire naturelle [2].”
Regionally Specific Cortical Thinning in Children with Sickle Cell Disease
Kirk, G. R., Haynes, M. R., Palasis, S., Brown, C., Burns, T. G., McCormick, M. L., & Jones, R. A. (2008) · Cerebral Cortex · doi:10.1093/cercor/bhn193 · Cited by 50
“…pneumococcal vaccination, and routine screening with transcranial Doppler ultrasound—has dramatically increased the life expectancy of patients with SCD (Platt et al. 1994) and has led to increasing attention on the chronic adverse effects of SCD that significantly impair quality of life.”
BackgroundInfluentialHasan, M. K., Aziz, M. H., Zarif, M. I. I., Hasan, M., Hashem, M. M. A., Guha, S., Love, R. R., & Ahamed, S. I. (2020) · JMIR mhealth and uhealth · doi:10.2196/16806 · Cited by 52
“Thus, an Hb disorder diagnosis based on an invasive method is not a perfect solution, especially for people in low- and middle-income countries [9,10].”
BackgroundTeofili, L., Papacci, P., Orlando, N., Bianchi, M., Molisso, A., Purcaro, V., Valentini, C. G., Giannantonio, C., Serrao, F., Chiusolo, P., et al. (2020) · British Journal of Haematology · doi:10.1111/bjh.16851 · Cited by 49
“In recent years, fetal haemoglobin has attracted a renewed attention for its ability to modify the clinical course of sickle cell disease and b thalassemia.(25,26) The evolutionary advantage Fig 1.”
BackgroundSickle cell disease: no longer a single gene disorder
Chui, D. H. K., & Dover, G. J. (2001) · Current Opinion in Pediatrics · doi:10.1097/00008480-200102000-00004 · Cited by 49
Endothelial cell nitric oxide production in acute chest syndrome
Hammerman, S., Klings, E. S., Hendra, K. P., Upchurch, G. R., Rishikof, D. C., Loscalzo, J., & Farber, H. W. (1999) · American Journal of Physiology-Heart and Circulatory Physiology · doi:10.1152/ajpheart.1999.277.4.h1579 · Cited by 48
“Further definition and understanding of these and additional mechanisms involved in ACS are important because recurrent episodes not only develop in 20–80% of patients but also are the major risk for development of pulmonary hypertension, cor pulmonale and eventually death (5, 31, 49, 51).”
BackgroundThe management of crisis in sickle cell disease
Okpala, I. E. (1998) · European Journal Of Haematology · doi:10.1111/j.1600-0609.1998.tb00989.x · Cited by 49
Use of Hydroxyurea in Children Ages 2 to 5 Years With Sickle Cell Disease
Hoppe, C. C., Vichinsky, E. P., Quirolo, K. C., van Warmerdam, J., Allen, K., & Styles, L. A. (2000) · The American journal of pediatric hematology/oncology · doi:10.1097/00043426-200007000-00009 · Cited by 49
Schleuning, M., Stoetzer, O. J., Waterhouse, C., Schlemmer, M., Ledderose, G., & Kolb, H. (2002) · Experimental Hematology · doi:10.1016/s0301-472x(01)00775-5 · Cited by 49
“However, one-third of deaths occur in patients without prior evidence of organ failure [1].”
BackgroundMetabolic persistence of fetal hemoglobin
Little, J., Dempsey, N. J., Tuchman, M., & Ginder, G. (1995) · Blood · doi:10.1182/blood.v85.7.1712.bloodjournal8571712 · Cited by 49
Development of a Severity Classification System for Sickle Cell Disease
Shah, N., Beenhouwer, D. O., Broder, M. S., Bronté-Hall, L., Castro, L. M. D., Gibbs, S. N., Gordeuk, V. R., Kanter, J., Klings, E. S., Lipato, T., et al. (2020) · ClinicoEconomics and Outcomes Research · doi:10.2147/ceor.s276121 · Cited by 51
Haematological actions of androgens
Warren, A. M., & Grossmann, M. (2022) · Best Practice & Research Clinical Endocrinology & Metabolism · doi:10.1016/j.beem.2022.101653 · Cited by 59
A Cell-Permeant Nanobody-Based Degrader That Induces Fetal Hemoglobin
Shen, F., Zheng, G., Setegne, M. T., Tenglin, K., Izadi, M., Xie, H. T. H., Zhai, L., Orkin, S. H., & Dassama, L. M. K. (2022) · ACS Central Science · doi:10.1021/acscentsci.2c00998 · Cited by 49
“γ-globin to 30% of the total beta-like hemoglobin, a level above the threshold for inhibition of red blood cells sickling SCD patients (22) (Fig.”
BackgroundCognitive Testing of PAINReportIt in Adult African Americans With Sickle Cell Disease
Jha, A., Suarez, M. L., Ferrans, C. E., Molokie, R. E., Kim, Y. O., & Wilkie, D. J. (2010) · CIN Computers Informatics Nursing · doi:10.1097/ncn.0b013e3181d7820b · Cited by 48
“Pain from SCD recurs throughout life, often starting in infancy and continuing throughout childhood, adulthood, and the elder years.(23) Pain from SCD is also characterized by marked variations in the character and severity of pain symptoms among individuals.”
BackgroundSickle Cell Disease and the “Difficult Patient” Conundrum
Bergman, E. J., & Diamond, N. J. (2013) · The American Journal of Bioethics · doi:10.1080/15265161.2013.767954 · Cited by 48
“A 1994 study found that an acute pain episode preceded 22% of sickle cell deaths (Platt et al. 1994).”
BackgroundMortalidade de crianças com doença falciforme: um estudo de base populacional
Fernandes, A. P. P. C., Januário, J. N., Cangussu, C. B., de Macedo, D. L., & Viana, M. B. (2010) · Jornal de Pediatria · doi:10.1590/s0021-75572010000400006 · Cited by 48
Pain Management in Sickle Cell Disease: Palliative Care Begins at Birth?
Benjamin, L. J. (2008) · Hematology · doi:10.1182/asheducation-2008.1.466 · Cited by 48
Genetic disruption of the KLF1 gene to overexpress the γ‐globin gene using the CRISPR/Cas9 system
Shariati, L., Khanahmad, H., Salehi, M., Hejazi, Z., Rahimmanesh, I., Tabatabaiefar, M. A., & Modarressi, M. H. (2016) · The Journal of Gene Medicine · doi:10.1002/jgm.2928 · Cited by 48
Khemani, K., Ross, D. C., Sinha, C. B., Haight, A. E., Bakshi, N., & Krishnamurti, L. (2017) · Transplantation and Cellular Therapy · doi:10.1016/j.bbmt.2017.11.018 · Cited by 48
Pro-inflammatory cytokines associate with NETosis during sickle cell vaso-occlusive crises
Barbu, E. A., Mendelsohn, L. G., Samsel, L., & Thein, S. L. (2019) · Cytokine · doi:10.1016/j.cyto.2019.154933 · Cited by 48
“High neutrophil count and their overactivation correlate with poor disease outcome and early death [3,4].”
BackgroundHickman, J. M., & Lachiewicz, P. F. (1997) · The Journal of Arthroplasty · doi:10.1016/s0883-5403(97)90198-4 · Cited by 48
Preventing morbidity and mortality from sickle cell disease:
Olney, R. S. (1999) · American Journal of Preventive Medicine · doi:10.1016/s0749-3797(98)00140-8 · Cited by 48
Medical disorders and orthodontics
Patel, A., Burden, D. J., & Sandler, J. (2009) · Journal of Orthodontics · doi:10.1179/14653120723346 · Cited by 49
“The disease is chronic and life expectancy is shortened to about 45 years.(29) Sickle cell trait is the term given to patients who inherit one of the genes of sickle cell disease but without developing the recurrent symptoms.”
BackgroundTrends in hospital admissions for sickle cell disease in England, 2001/02-2009/10
AlJuburi, G., Laverty, A. A., Green, S. A., Phekoo, K. J., Banarsee, R., Okoye, N., Bell, D., & Majeed, A. (2012) · Journal of Public Health · doi:10.1093/pubmed/fds035 · Cited by 48
Perrine, S. P., Dover, G. H., Daftari, P., Walsh, C. T., Jin, Y., Mays, A. D., & Faller, D. V. (1994) · British Journal of Haematology · doi:10.1111/j.1365-2141.1994.tb05073.x · Cited by 48
High Prevalence of Pulmonary Hypertension in Children With Sickle Cell Disease
Nelson, S. C., Adade, B. B., McDonough, E. A., Moquist, K. L., & Hennessy, J. M. (2007) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0b013e31805d8f32 · Cited by 48
“Now, most of the children with sickle cell disease are living into adulthood.(2) Because of recurrent vaso-occlusive events, the risk of end-organ damage increases as these children age.”
BackgroundPsychosocial challenges of persons with sickle cell anemia: A narrative review
Essien, E., Winter-Eteng, B. F., Onukogu, C. U., Nkangha, D. D., & Daniel, F. (2023) · Medicine · doi:10.1097/md.0000000000036147 · Cited by 39
“[22] 23–25] These mental disorders significantly impact overall health-related QOL more than the genotype.”
Background
Metadata
- Authors
- Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klug, P. P.
- Affiliations
- Boston Children's Hospital (US)
- New England Research Institutes (US)
- Duke University (US)
- Augusta University (US)
- Howard University (US)
- University of Mississippi Medical Center (US)
- Texas Tech University (US)
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1994-06-09
- Volume
- 330
- Issue
- 23
- Pages
- 1639-1644
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2334263927
- Semantic Scholar
- 6bcc7a4d436214c41a4d128f9d221e950eb4962b
- Cited by
- 3,320
- References
- 19
- Citations by year
- 2026: 83 · 2025: 104 · 2024: 123 · 2023: 135 · 2022: 128 · 2021: 167 · 2020: 147 · 2019: 168 · 2018: 140 · 2017: 159 · 2016: 173 · 2015: 165 · 2014: 154 · 2013: 139 · 2012: 115
- FWCI
- 24.43
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Digital Howard (Howard University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Malaria Research and Control
- Health Sciences › Medicine › Hematology › Blood groups and transfusion
- Keywords
- sickle cell disease, sickle cell-hemoglobin C disease, sickle beta-thalassemia, life expectancy, mortality risk factors, acute chest syndrome, vaso-occlusive crisis, stroke, renal failure, seizures, fetal hemoglobin, white blood cell count, organ failure
- MeSH terms
- Adult, Aged, Anemia, Sickle Cell, Cause of Death, Child, Female, Hemoglobin SC Disease, Humans, Life Expectancy, Male, Middle Aged, Probability, Regression Analysis, Risk Factors, Survival Analysis, beta-Thalassemia
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