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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]
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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]
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“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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“High steady-state WBC counts in SCD patients are associated with recurrent pain [20], acute chest syndrome [21], stroke [22], early death [23,24], and other adverse events [25].”
How we prevent and manage infection in sickle cell disease
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Review on screening and analysis techniques for hemoglobin variants and thalassemia
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Band 3 Peptides Block the Adherence of Sickle Cells to Endothelial Cells In Vitro
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“to a rapidly progressive and invalidating disease with median mortality in the fourth decade of life.(5) The phenotypic heterogeneity of SCD is the result of genetic and epigenetic factors that modulate the pathogenic processes of SCD, from the degree of HbS polymerization to the intensity and the relative contributio…”
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“The median survival for men and women with SCD remains dismally low, at ;42 and 48 years, respectively, in high-income settings but remains unknown in low-resource settings.(87) Understanding the patterns of morbidity, mortality, HRQoL, and health care utilization in individuals with SCD living in low-resource setting…”
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“Epidemiological data indicate that 5% of the patients with SCD experience 3–10 crises a year (24, 25).”
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“Median survival of SS patients in the US now exceeds 40 years (42), and in Jamaica 50 years (43).”
BackgroundCentral nervous system abnormalities in asymptomatic young patients with Sβ‐thalassemia
Zafeiriou, D., Prengler, M., Gombakis, N., Kouskouras, K., Economou, M. P., Kardoulas, A., Tsantali, C., Dimitriadis, A., Athanasiou, M. A., & Kirkham, F. J. (2004) · Annals of Neurology · doi:10.1002/ana.20104 · Cited by 39
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ResultInfluentialSickle Cell Disease and the Kidney
Scheinman, J. I. (2003) · Seminars in Nephrology · doi:10.1053/snep.2003.50006 · Cited by 39
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“Higher HbF levels are known to prolong survival (Platt et al, 1994), protect against vaso-occlusive crisis and acute chest syndrome (Platt et al, 1991; Bailey et al, 1992) and decrease the risk of stroke (Powars et al, 1980).”
Background14q32 and let-7 microRNAs regulate transcriptional networks in fetal and adult human erythroblasts
Lessard, S., Beaudoin, M., Orkin, S. H., Bauer, D. E., & Lettre, G. (2018) · Human Molecular Genetics · doi:10.1093/hmg/ddy051 · Cited by 38
Gender differences in severity of sickle cell diseases in non-smokers
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“7 (13-59), and it was significantly lower than the cases without any crisis in their lives (p<0.”
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Helvacı, M. R., Gökçe, C., Davran, R., Akküçük, S., Uğur, M., & Oruç, C. (2015) · PubMed Central · Cited by 38
Sickle cell disease classification using deep learning
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“It was reported by Platt et al. [11] from an investigation on 3685 patients that the typical life expectancy for males and females with SCD was 42 and 48 years, respectively.”
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“In SCA, low HbF levels are associated with a more severe disease phenotype demonstrated by frequent vaso-occlusive events and higher mortality (8, 9).”
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“These problems often lead to several other complications of the disease, such as anemia and stroke; consequently, most patients with SCD live to between the ages of 40 and 50 years.(1) Surprisingly, the most common cause of death in those with SCD is infection, not stroke or organ failure.”
BackgroundRight Ventricular Systolic Strain Is Altered in Children with Sickle Cell Disease
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“Half of ACS episodes developed during hospitalization for another complication of SCD, most frequently VOC (5).”
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Stem Cell Transplant for Children with Sickle Cell Anemia: Parent and Patient Interest
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Complications of sickle cell anaemia in children in Northwestern Tanzania
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“Despite these limitations we feel that the prevalence and rate of progression of organ damage in our study cohort of adult sickle cell patients reinforces the need for systematic screening and/or timely detection (like for the occurrence of PHT) of organ damage in order to prevent further deterioration of organ damage…”
InfluentialSharma, D., Day, M. E., Stimpson, S.-J., Rodeghier, M., Ghafuri, D. L., Callaghan, M. U., Zaidi, A. U., Hannan, B., Kassim, A. A., Zempsky, W. T., et al. (2019) · Journal of Women s Health · doi:10.1089/jwh.2018.7147 · Cited by 38
Considering the spleen in sickle cell disease
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“The Cnaan and Ryan approach is appropriate for analyses of data in studies, similar to this one, in which patients enter and leave the study cohort and observation begins only at enrolment and not at the onset of the disease.(7,8) Univariate analysis.”
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“Women in this population are older than men [8,45], African-Americans comprise the vast majority of patients, and Hispanic patients account for 8% of the cohort.”
BackgroundBloodstream Infection in Adults With Sickle Cell Disease
Zarrouk, V., Habibi, A., Zahar, J., Roudot‐Thoraval, F., Bachir, D., Brun‐Buisson, C., Legrand, P., Godeau, B., Galactéros, F., & Lesprit, P. (2006) · Medicine · doi:10.1097/01.md.0000197023.46846.1c · Cited by 38
“Previous osteonecrosis 11 (61) 7 (24) 0.”
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“Moreover, increases in HbF have been shown to decrease the severity of the disease (1–6) and enhance survival in patients with HbSS (5).”
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Patterns of opioid use in sickle cell disease
Han, J., Saraf, S. L., Zhang, X., Gowhari, M., Molokie, R. E., Hassan, J., Alhandalous, C., Jain, S., Younge, J. S., Abbasi, T. A., et al. (2016) · American Journal of Hematology · doi:10.1002/ajh.24498 · Cited by 38
MANAGEMENT OF SICKLE CELL DISEASE: RECENT ADVANCES AND CONTROVERSIES
Castro, O. L. (1999) · British Journal of Haematology · doi:10.1046/j.1365-2141.1999.01565.x · Cited by 38
“In the U.S. Cooperative Study of Sickle Cell Disease high steady-state leucocyte counts were associated with higher frequency of painful events (Platt et al, 1991) and chest syndrome (Castro et al, 1994), and with mortality (Platt et al, 1994).”
Self-citationGoers, T. A., Panepinto, J. A., DeBaun, M. R., Blinder, M. A., Foglia, R. P., Oldham, K. T., & Field, J. J. (2007) · Pediatric Blood & Cancer · doi:10.1002/pbc.21245 · Cited by 38
Asthma in the pediatric sickle cell patient with acute chest syndrome
Bryant, R. (2005) · Journal of Pediatric Health Care · doi:10.1016/j.pedhc.2004.12.003 · Cited by 38
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Prediction of Adverse Outcomes in Children with Sickle Cell Disease
Horan, J., & Lerner, N. B. (2000) · New England Journal of Medicine · doi:10.1056/nejm200005253422114 · Cited by 40
Vascular complications of sickle cell disease
Usmani, A., & Machado, R. F. (2018) · Clinical Hemorheology and Microcirculation · doi:10.3233/ch-189008 · Cited by 38
“In fact, the median survival of adults with SCD was 42 in men and 48 in women in the 1990s [6].”
CRISPR/Cas9-based gene-editing technology for sickle cell disease
Ma, L., Yang, S., Peng, Q., Zhang, J., & Zhang, J. (2023) · Gene · doi:10.1016/j.gene.2023.147480 · Cited by 45
Ahmad, H., Gayat, É., Yodwut, C., Abduch, M. C. D., Patel, A. R., Weinert, L., Desai, A. A., Tsang, W., Garcia, J. G. N., Lang, R. M., et al. (2012) · Echocardiography · doi:10.1111/j.1540-8175.2012.01710.x · Cited by 37
“3DSTE Indices of Systolic Function: The following parameters of LV deformation were obtained using the TomTec analysis software: (1) global longitudinal strain (GLS), (2) twist angle, defined as the maximum difference between basal and apical rotation, and (3) torsion, defined as the twist angle divided by the end-dia…”
MethodDedeken, L., Lê, P.-Q., Rozen, L., Kenz, H. E., Huybrechts, S., Devalck, C., Diallo, S., Heijmans, C., & Ferster, A. (2018) · Transfusion · doi:10.1111/trf.14575 · Cited by 37
Genetic therapies for sickle cell disease
Esrick, E. B., & Bauer, D. E. (2018) · Seminars in Hematology · doi:10.1053/j.seminhematol.2018.04.014 · Cited by 39
Shah, N., Bhor, M., Latrémouille-Viau, D., Sharma, V. K., Puckrein, G. A., Gagnon‐Sanschagrin, P., Khare, A., Singh, M. K., Serra, E., Davidson, M., et al. (2020) · Journal of Medical Economics · doi:10.1080/13696998.2020.1813144 · Cited by 41
Gene Therapy of the Hemoglobinopathies
Kunz, J. B., & Kulozik, A. E. (2020) · HemaSphere · doi:10.1097/hs9.0000000000000479 · Cited by 40
de la Fuente, J., Gluckman, É., Makani, J., Telfer, P. T., Faulkner, L., Corbacioglu, S., Amrolia, P. J., Ansari, M., Balduzzi, A., Dalassier, A., et al. (2020) · The Lancet Haematology · doi:10.1016/s2352-3026(20)30283-0 · Cited by 38
Glaros, A. K., Razvi, R., Shah, N., & Zaidi, A. U. (2021) · Therapeutic Advances in Hematology · doi:10.1177/20406207211001136 · Cited by 46
Cell and Gene Therapy for Anemia: Hematopoietic Stem Cells and Gene Editing
Anurogo, D., Budi, N. Y. P., Ngo, M.-H. T., Huang, Y., & Pawitan, J. A. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms22126275 · Cited by 43
“In the Unites States, the median survival is 48 years for women and 42 years for men [18], though advances in treatment options may increase survival, and a report from a London Hospital showed a median survival of 67 years [12].”
BackgroundHannemann, A., Rees, D. C., Tewari, S., & Gibson, J. S. (2015) · EBioMedicine · doi:10.1016/j.ebiom.2015.09.026 · Cited by 41
“For example, life expectancy of HbSC individuals is markedly reduced (Platt et al., 1994), and some complications of SCD, like proliferative retinopathy (Condon and Serjeant, 1970), are over-represented in HbSC patients.”
BackgroundHahn, C. K., & Lowrey, C. H. (2013) · Blood · doi:10.1182/blood-2013-03-491043 · Cited by 37
Haemoglobin F modulation in childhood sickle cell disease
Trompeter, S., & Roberts, I. A. G. (2008) · British Journal of Haematology · doi:10.1111/j.1365-2141.2008.07482.x · Cited by 37
“Even in cohorts of SCD patients without these variants, HbF levels predict the frequency of vaso-occlusive crises, acute chest syndrome, osteonecrosis and death (Castro et al, 1994; Platt et al, 1994) and, in young children, dactylitis and splenic sequestration (Bailey et al, 1992).”
BackgroundRaphael, J. L., Kamdar, A., Wang, T., Liu, H., Mahoney, D. H., & Mueller, B. U. (2008) · Pediatric Blood & Cancer · doi:10.1002/pbc.21537 · Cited by 38
“This has the potential to improve patient satisfaction and reduce healthcare costs associated with SCD. Future studies should prospectively compare DH care to inpatient hospitalization in order to make stronger conclusions regarding the utility of the DH as an alternate delivery care system for children with uncomplic…”
BackgroundInfluentialInhaled bronchodilators for acute chest syndrome in people with sickle cell disease
Knight‐Madden, J., & Hambleton, I. R. (2016) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd003733.pub4 · Cited by 37
Pain Management in Adults With Sickle Cell Disease in a Medical Center Emergency Department
Solomon, L. R. (2010) · Journal of the National Medical Association · doi:10.1016/s0027-9684(15)30729-x · Cited by 37
Baskin, M. L., Collins, M. H., Brown, F., Griffith, J. R., Samuels, D., Moody, A. L., Thompson, M. P., Eckman, J. R., & Kaslow, N. J. (1998) · Journal of Clinical Psychology in Medical Settings · doi:10.1023/a:1026206204009 · Cited by 37
“A recent study (Platt et al., 1994) found the median age of death for patients who suffer from the most frequently occurring and severe disease type (hemoglobin SS) was 42 years for males and 48 years for females.”
BackgroundOutcomes of universal antenatal screening for haemoglobinopathies
Greengross, P., Hickman, M., Gill, M. J., Dugan, B., & Davies, S. C. (1999) · Journal of Medical Screening · doi:10.1136/jms.6.1.3 · Cited by 37
Serum cystatin C levels in children with sickle cell disease
Alvarez, O. A., B, G. Z., Wright, D., Montané, B., & Lopez‐Mitnik, G. (2006) · Pediatric Nephrology · doi:10.1007/s00467-006-0033-6 · Cited by 37
Deferiprone as an oral iron chelator in sickle cell disease
Voskaridou, E., Douskou, M., Terpos, E., Stamoulakatou, A., Meletis, J., Ourailidis, A., Papassotiriou, I., & Loukopoulos, D. (2005) · Annals of Hematology · doi:10.1007/s00277-005-1015-7 · Cited by 37
“However, the increase in longevity during the last few decades (42 years in men and 48 years in women) has been associated with clinical evidence of iron overload in some patients [28].”
BackgroundSocial disparities producing health inequities and shaping sickle cell disorder in Brazil
Mota, C. S., Atkin, K. M., Trad, L. A. B., & Dias, A. L. A. (2017) · Health Sociology Review · doi:10.1080/14461242.2017.1361855 · Cited by 36
“Historically, SCD was considered a ‘childhood disease’ that produced premature mortality, mostly due to infection, which can be directly related to social and environmental disadvantages (Platt et al., 1994).”
BackgroundModifier genes in Mendelian disorders: the example of hemoglobin disorders
Sankaran, V. G., Lettre, G., Orkin, S. H., & Hirschhorn, J. N. (2010) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.2010.05821.x · Cited by 37
Induction of fetal hemoglobin through enhanced translation efficiency of γ-globin mRNA
Hahn, C. K., & Lowrey, C. H. (2014) · Blood · doi:10.1182/blood-2014-03-564302 · Cited by 37
Intzes, S., Kalpatthi, R. V., Short, R., & Imran, H. (2013) · Pediatric Hematology and Oncology · doi:10.3109/08880018.2012.756961 · Cited by 36
“In 1994, the Cooperative Study of Sickle Cell Disease noted that patients with SCA had a median age at death of 42 years for males and 48 years for females, with SCDrelated mortality affecting survival [2].”
BackgroundImproving Clinician Attitudes of Respect and Trust for Persons With Sickle Cell Disease
Haywood, C., Williams‐Reade, J., Rushton, C. H., Beach, M. C., & Geller, G. (2015) · Hospital Pediatrics · doi:10.1542/hpeds.2014-0171 · Cited by 39
Lim, S. H., Methé, B. A., Knoll, B. M., Morris, A., & Obaro, S. (2018) · Journal of Translational Medicine · doi:10.1186/s12967-018-1622-4 · Cited by 37
Hoppe, C. C., & Neumayr, L. (2019) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2019.01.014 · Cited by 39
Bakshi, N., Katoch, D., Sinha, C. B., Ross, D. C., Quarmyne, M., Loewenstein, G. F., & Krishnamurti, L. (2020) · JAMA Network Open · doi:10.1001/jamanetworkopen.2020.6742 · Cited by 36
Sinha, C. B., Bakshi, N., Ross, D. C., Loewenstein, G. F., & Krishnamurti, L. (2020) · Pediatric Blood & Cancer · doi:10.1002/pbc.28749 · Cited by 36
“(2) Caregivers are motivated to seek consultation because of their child’s diminished quality of life, recent complications, the need to make a major medical decision, or concern about future severe complications.”
BackgroundRen, H., Okpala, I. E., Ghebremeskel, K., Ugochukwu, C. C., Ibegbulam, O. G., & Crawford, M. A. (2005) · Annals of Hematology · doi:10.1007/s00277-005-1023-7 · Cited by 36
“The clinical severity of sickle cell disease (SCD) increases with blood neutrophil count [2], and leukocytosis is a risk factor for early disease-related death [23].”
BackgroundDNA hypo‐methylating agents and sickle cell disease
Saunthararajah, Y., Lavelle, D., & DeSimone, J. (2004) · British Journal of Haematology · doi:10.1111/j.1365-2141.2004.05064.x · Cited by 36
“The unknown risks of treating sickle cell disease with decitabine (or hydroxyurea) need to be balanced against the significant known risks of early mortality and chronic morbidity (Platt et al, 1994) which may be ameliorated with effective HbF elevation.”
BackgroundFrei‐Jones, M. J., Baxter, A. L., Rogers, Z. R., & Buchanan, G. R. (2007) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2007.06.040 · Cited by 38
“5oC (5), trauma (5), constipation (4), transfer from another hospital (3), cholecystitis (2), spontaneous abortion (1), and tuberculous abscess of the clavicle (1).”
BackgroundInduced Sputum versus Bronchoalveolar Lavage during Acute Chest Syndrome in Sickle Cell Disease
Lechapt, E., Habibi, A., Bachir, D., Galactéros, F., Schaeffer, A., Desvaux, D., Brochard, L. J., HOUSSET, B., Godeau, B., & Maître, B. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200302-174oc · Cited by 36
“It is a complication of sickle cell disease and is the leading cause of death from this disease in adults (1, 2 ). Several pathologic processes are recognized causes of ACS, including infectious diseases, in situ thrombosis, hypoventilation secondary to chest pain, and pulmonary fat embolism (3–12).”
BackgroundMethodOkito, J. P. K., Damme, O. V., & Calliauw, L. (2004) · Acta Neurochirurgica · doi:10.1007/s00701-003-0214-z · Cited by 36
“The life expectancy of these patients is per se already lower than in the normal population [8].”
BackgroundMazumdar, M., Heeney, M. M., Sox, C. M., & Lieu, T. A. (2007) · PEDIATRICS · doi:10.1542/peds.2006-2002 · Cited by 36
Markham, M. J., Lottenberg, R., & Zumberg, M. S. (2003) · American Journal of Hematology · doi:10.1002/ajh.10328 · Cited by 35
“In homozygous sickle cell disease (hemoglobin SS) many factors including infection, leukocytosis, low fetal hemoglobin, pregnancy, and cold weather are known to correlate with an increased frequency of vaso-occlusive pain crises and some with mortality as well [1,2,3,16,17].”
BackgroundAlpha-2-Macroglobulin and Interleukin-6 Levels in Steady-State Sickle Cell Disease Patients
Makis, A. C., Hatzimichael, E. C., Mavridis, A. K., & Bourantas, K. L. (2000) · Acta Haematologica · doi:10.1159/000046509 · Cited by 37
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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