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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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Cites
Pain in Sickle Cell Disease: Rates and Risk Factors
Platt, O. S., Thorington, B. D., Brambilla, D. J., Milner, P. F., Rosse, W. F., Vichinsky, E., & Kinney, T. R. (1991) · New England Journal of Medicine · doi:10.1056/nejm199107043250103 · Cited by 1,546
Self-citationLEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P., & RIDA, W. N. (1989) · PubMed · Cited by 361
Self-citationMortality in Children and Adolescents With Sickle Cell Disease
LEIKIN, S. L., Gallagher, D. M., Kinney, T. R., Sloane, D., Klug, P. P., & RIDA, W. N. (1989) · PEDIATRICS · doi:10.1542/peds.84.3.500 · Cited by 319
Self-citationCauses of death in sickle-cell disease in Jamaica.
Thomas, A. N., Pattison, C. W., & Serjeant, G. R. (1982) · BMJ · doi:10.1136/bmj.285.6342.633 · Cited by 273
Embury, S. H., Miller, J., Dozy, A. M., Kan, Y. W., Chan, V., & Todd, D. (1980) · Journal of Clinical Investigation · doi:10.1172/jci109984 · Cited by 260
α-Globin gene organisation in blacks precludes the severe form of α-thalassaemia
Dozy, A. M., Kan, Y. W., Embury, S. H., Mentzer, W. C., Wang, W. C., Lubin, B. H., DAVIS, J. R., & Koenig, H. M. (1979) · Nature · doi:10.1038/280605a0 · Cited by 221
The Haemolytic Anaemias: Congenital and Acquired
Signy, A. G. (1955) · Journal of Clinical Pathology · doi:10.1136/jcp.8.4.353-a · Cited by 174
Steinberg, M. H., Rosenstock, W., Coleman, M. B., Adams, J. D., Platica, O., Cedeno, M., Rieder, R. F., Wilson, J., Milner, P., & West, S. (1984) · Blood · doi:10.1182/blood.v63.6.1353.1353 · Cited by 161
Self-citationThe variable expression of sickle cell disease is genetically determined.
Powars, D., Chan, L. S., & Schroeder, W. (1990) · PubMed · Cited by 155
The cooperative study of sickle cell disease: review of study design and objectives.
Gaston, M. H., & Rosse, W. F. (1982) · PubMed · Cited by 129
Self-citation
Research works printed but not linked: 4
- Diggs LM. Anatomic lesions in sickle cell disease. In: Abramson H, Bertles JF, Wethers DL, eds. Sickle cell disease: diagnosis, management, education, and research. St. Louis: C.V. Mosby, 1973:189-229.
- Powars D, 1990, Semin Hematol, 27, 360
- Gaston M, 1982, Am J Pediatr Hematol Oncol, 4, 197
- Cole TB, 1986, Am J Dis Child, 140, 1255
Cited by
Sorted by citations
Items 401–500 of 1,015
Emerging disease-modifying therapies for sickle cell disease
Carden, M. A., & Little, J. A. (2019) · Haematologica · doi:10.3324/haematol.2018.207357 · Cited by 100
“Clinical manifestations of the presence of HbS polymerization are wide-ranging and include chronic hemolytic anemia, episodic microcirculatory vaso-occlusion with tissue ischemia and pain, and ultimately chronic end-organ damage that can reduce the lifespan of an individual with SCD.(14) Due to its impact on morbidity…”
BackgroundEvidence for a metabolic shift of arginine metabolism in sickle cell disease
Schnog, J. B., Jager, E. H., van der Dijs, F. P. L., Duits, A. J., Moshage, H. J., Muskiet, F. D., & Muskiet, F. A. J. (2004) · Annals of Hematology · doi:10.1007/s00277-004-0856-9 · Cited by 94
“of NO synthesis [28].”
Mortality of children with sickle cell disease: a population study
Fernandes, A. P. P. C., Januário, J. N., Cangussu, C. B., de Macedo, D. L., & Viana, M. B. (2010) · Jornal de Pediatria · doi:10.2223/jped.2005 · Cited by 93
Reliability and validity of a self-efficacy instrument specific to sickle cell disease
Edwards, R. R., Telfair, J., Cecil, H., & Lenoci, J. M. (2000) · Behaviour Research and Therapy · doi:10.1016/s0005-7967(99)00140-0 · Cited by 93
“Indeed, life expectancies for these individuals now approach those for the general population, with more than half surviving into their 60's (Platt et al., 1994).”
Vaso‐occlusive painful events in sickle cell disease: Impact on child well‐being
Brandow, A. M., Brousseau, D. C., Pajewski, N. M., & Panepinto, J. A. (2009) · Pediatric Blood & Cancer · doi:10.1002/pbc.22222 · Cited by 92
Susceptibility to invasive bacterial infections in children with sickle cell disease
Battersby, A., Knox-Macaulay, H., & Carrol, E. D. (2010) · Pediatric Blood & Cancer · doi:10.1002/pbc.22461 · Cited by 92
“individuals in North America, Europe, and the Caribbean of between 45 and 55 years [5,8], but data from sub-Saharan Africa indicate that half of the SCD patients in that region die from”
BackgroundChronic Opioid Therapy and Central Sensitization in Sickle Cell Disease
Carroll, C. P., Lanzkron, S., Haywood, C., Kiley, K. B., Pejsa, M., Moscou-Jackson, G., Haythornthwaite, J. A., & Campbell, C. M. (2016) · American Journal of Preventive Medicine · doi:10.1016/j.amepre.2016.02.012 · Cited by 92
“Patients experiencing VOC are at increased risk for subsequent acute chest syndrome or stroke, both potentially life-threatening complications.(18) The medical literature for some time used acute care visits for VOC as a proxy measure for the pain burden of SCD.”
BackgroundMortality from sickle cell disease in Africa
Serjeant, G. R. (2005) · BMJ · doi:10.1136/bmj.330.7489.432 · Cited by 92
“Median survival for patients with SS disease in North America, Europe, and the Caribbean is now estimated to be 45-55 years.”
BackgroundSteele, C., Sinski, A., Asibey, J. G., Hardy‐Dessources, M., Elana, G., Brennan, C., Odame, I., Hoppe, C. C., Geisberg, M., Serrao, E., et al. (2018) · American Journal of Hematology · doi:10.1002/ajh.25305 · Cited by 91
“Manifestations of SCD include painful episodes, cardiopulmonary disease, stroke, nephropathy, susceptibility to invasive bacterial infections, and early mortality.(1,2) In many highresource regions, universal newborn screening programs coupled with prophylactic interventions have dramatically reduced the mortality and…”
BackgroundEffects of a long-term transfusion regimen on sickle cell-related illnesses
Styles, L. A., & Vichinsky, E. P. (1994) · The Journal of Pediatrics · doi:10.1016/s0022-3476(05)82006-2 · Cited by 91
Bhatnagar, P., Purvis, S. H., Barron‐Casella, E. A., DeBaun, M. R., Casella, J. F., Arking, D. E., & Keefer, J. R. (2011) · Journal of Human Genetics · doi:10.1038/jhg.2011.12 · Cited by 89
Sinha, C. B., Bakshi, N., Ross, D. C., & Krishnamurti, L. (2019) · JAMA Network Open · doi:10.1001/jamanetworkopen.2019.4410 · Cited by 94
Acute chest syndrome: sickle cell disease
Paul, R. N., Castro, O. L., Aggarwal, A., & Oneal, P. A. (2011) · European Journal Of Haematology · doi:10.1111/j.1600-0609.2011.01647.x · Cited by 91
“Thus, moderate pulmonary pressure elevation may be poorly tolerated by patients with SCD and may contribute to their high risk of sudden death particularly during crises or ACS (10, 104).”
BackgroundInfluentialSelf-citationQuinn, C. T., Lee, N. J., Shull, E. P., Ahmad, N., Rogers, Z. R., & Buchanan, G. R. (2007) · Blood · doi:10.1182/blood-2007-07-100719 · Cited by 90
Guasch, A., Zayas, C. F., Eckman, J. R., Muralidharan, K., Zhang, W., & Elsas, L. J. (1999) · Journal of the American Society of Nephrology · doi:10.1681/asn.v1051014 · Cited by 90
Genome-wide detection of natural selection in African Americans pre- and post-admixturePreprint
Jin, W., Xu, S., Wang, H., Yu, Y., Shen, Y., Wu, B.-L., & Li, J. (2011) · Genome Research · doi:10.1101/gr.124784.111 · Cited by 91
“However, these mutations that defend against malaria could become a disadvantage in AfA because malaria is no longer a strong selection pressure in North America, and these mutations could even lead to morbidity or mortality (Platt et al. 1994).”
BackgroundWonkam, A., Bitoungui, V. J. N., Vorster, A. A., Ramesar, R., Cooper, R., Tayo, B. O., Lettre, G., & Ngogang, J. Y. (2014) · PLoS ONE · doi:10.1371/journal.pone.0092506 · Cited by 92
“Previous analyses of the CSSCD dataset showed that increased HbF levels correlate with less severe complications, fewer pain crises [33] and improved survival [34].”
ResultPaulukonis, S. T., Eckman, J. R., Snyder, A., Hagar, W., Feuchtbaum, L., Zhou, M., Grant, A. M., & Hulihan, M. (2016) · Public Health Reports · doi:10.1177/003335491613100221 · Cited by 89
“65–74 164 32 (5) 83 19 (6) 81 13 (4) 39.”
Tebbi, C. K. (2022) · Hemato · doi:10.3390/hemato3020024 · Cited by 110
“This is collaborated with other studies [48].”
BackgroundGENITOURINARY COMPLICATIONS OF SICKLE CELL DISEASE
BRUNO, D., Wigfall, D. R., Zimmerman, S. A., Rosoff, P. M., & Wiener, J. S. (2001) · The Journal of Urology · doi:10.1016/s0022-5347(05)65841-7 · Cited by 89
Berry, P., Cross, T., Thein, S. L., Portmann, B. C., Wendon, J. A., Karani, J. B., Heneghan, M. A., & Bomford, A. B. (2007) · Clinical Gastroenterology and Hepatology · doi:10.1016/j.cgh.2007.08.009 · Cited by 89
Hebbel, R. P., Vercellotti, G. M., & Nath, K. A. (2009) · Cardiovascular & Haematological Disorders - Drug Targets · doi:10.2174/1871529x10909040271 · Cited by 91
“, leukocytosis as a predictor of mortality and morbidity [31] and of a worse predicted course for children [32].”
Estepp, J. H., Smeltzer, M. P., Kang, G., Li, C., Wang, W. C., Abrams, C., Aygun, B., Ware, R. E., Nottage, K., & Hankins, J. S. (2017) · American Journal of Hematology · doi:10.1002/ajh.24906 · Cited by 88
“days, with a median (IQR, range) duration of 30 (28-30, 1-60) days.”
BackgroundPecker, L. H., & Lanzkron, S. (2021) · Annals of Internal Medicine · doi:10.7326/aitc202101190 · Cited by 100
Wahlberg, K. E., Jiang, J., Rooks, H., Jawaid, K., Matsuda, F., Yamaguchi, M., Lathrop, M. G., Thein, S. L., & Best, S. (2009) · Blood · doi:10.1182/blood-2009-03-210146 · Cited by 94
Hankins, J. S., Estepp, J. H., Hodges, J., Villavicencio, M., Robison, L. L., Weiss, M. J., Kang, G., Schreiber, J. E., Porter, J. S., Kaste, S. C., et al. (2018) · Pediatric Blood & Cancer · doi:10.1002/pbc.27228 · Cited by 87
“anti-inflammatory agents,68,69 Hb affinity modulators,70–72 anti-oxidants,73 and gene therapy74) in cumulative organ damage; (2) cognitive impairment, both its underlyingmechanisms and the impact on social function; (3) the impact of the environment on the disease course, including poverty, which is prevalent in the S…”
BackgroundAzar, S., & Wong, T. E. (2016) · Medical Clinics of North America · doi:10.1016/j.mcna.2016.09.009 · Cited by 86
“recurrent episodes of acute chest syndrome, low Hb F concentration, severe anemia, higher rates of hemolysis, and dactylitis before 1 year of age.(25,40,41)”
A Model Linking Sickle Cell Hemoglobinopathies and SMARCB1 Loss in Renal Medullary Carcinoma
Msaouel, P., Tannir, N. M., & Walker, C. L. (2018) · Clinical Cancer Research · doi:10.1158/1078-0432.ccr-17-3296 · Cited by 90
“In addition, sex differences in the frequency of microvascular occlusions due to sickling (45, 46) may explain why men are twice as likely to be affected by RMC than women (3, 4).”
BackgroundNeuropsychological Aspects of Pediatric Sickle Cell Disease
Kral, M. C., Brown, R. T., & Hynd, G. W. (2001) · Neuropsychology Review · doi:10.1023/a:1012901124088 · Cited by 86
“One recent investigation of sickle cell anemia (HbSS) documented a median age of death at 42 years for males and 48 years for females, ages that are 25–30 years less than expected for the general African American population (Platt et al., 1994).”
BackgroundInduction of human fetal hemoglobin via the NRF2 antioxidant response signaling pathway
Macari, E. R., & Lowrey, C. H. (2011) · Blood · doi:10.1182/blood-2010-10-314096 · Cited by 87
The Association of Provider Communication with Trust among Adults with Sickle Cell Disease
Haywood, C., Lanzkron, S., Ratanawongsa, N., Bediako, S. M., Lattimer, L., Powe, N. R., & Beach, M. C. (2010) · Journal of General Internal Medicine · doi:10.1007/s11606-009-1247-7 · Cited by 87
Hematopoietic stem-cell transplantation for sickle cell disease: current evidence and opinions
Shenoy, S. S. (2013) · Therapeutic Advances in Hematology · doi:10.1177/2040620713483063 · Cited by 87
“The average life expectancy despite modernization of supportive care has remained approximately 40 years for men and women with SCD over two decades [Fitzhugh et al. 2010; Platt et al. 1994].”
BackgroundThe Role of Inflammation and Leukocytes in the Pathogenesis of Sickle Cell Disease
Wun, T. (2000) · Hematology · doi:10.1080/10245332.2000.11746536 · Cited by 87
Hematopoietic stem cell transplantation for sickle cell disease
Kassim, A. A., & Sharma, D. (2017) · Hematology/Oncology and Stem Cell Therapy · doi:10.1016/j.hemonc.2017.05.008 · Cited by 86
The Not-So-Simple Process of Sickle Cell Vasoocclusion
Embury, S. H. (2004) · Microcirculation · doi:10.1080/10739680490278277 · Cited by 88
The intersection between asthma and acute chest syndrome in children with sickle-cell anaemia
DeBaun, M. R., & Strunk, R. C. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00145-8 · Cited by 82
Gene therapy of hemoglobinopathies: progress and future challenges
Ikawa, Y., Miccio, A., Magrin, E., Kwiatkowski, J. L., Rivella, S., & Cavazzana, M. (2019) · Human Molecular Genetics · doi:10.1093/hmg/ddz172 · Cited by 86
Strouse, J. J., Cox, C., Melhem, E. R., Lu, H., Kraut, M. A., Razumovsky, A. Y., Yohay, K. H., van Zijl, P. C. M., & Casella, J. F. (2006) · Blood · doi:10.1182/blood-2005-10-4029 · Cited by 85
“%) and elevated platelet count (> 500,000/ul) are also associated with decreased IQ in children with SCA.3,5 CBF may also affect neurocognitive function.”
BackgroundInfluentialInflammatory potential of neutrophils detected in sickle cell disease
Lum, A. F. H., Wun, T., Staunton, D. E., & Simon, S. I. (2004) · American Journal of Hematology · doi:10.1002/ajh.20059 · Cited by 85
Total THA in Adult Osteonecrosis Related to Sickle Cell Disease
Hernigou, P., Zilber, S., Filippini, P., Mathieu, G., Poignard, A., & Galactéros, F. (2008) · Clinical Orthopaedics and Related Research · doi:10.1007/s11999-007-0069-3 · Cited by 85
“With pharmacologic treatments including analgesia, oxygen therapy, hydration, and hydroxyuera [6, 40], the lifespan of the patient with SCD can be extended into the 60 s [38].”
Detecting white matter injury in sickle cell disease using voxel‐based morphometry
Baldeweg, T., Hogan, A. M., Saunders, D. E., Telfer, P. T., Gadian, D. G., Vargha‐Khadem, F., & Kirkham, F. J. (2006) · Annals of Neurology · doi:10.1002/ana.20790 · Cited by 85
Guidelines for the use of hydroxycarbamide in children and adults with sickle cell disease
Qureshi, A., Kaya, B., Pancham, S., Keenan, R., Anderson, J., Akanni, M., Howard, J., & the British Society for Haematology (2018) · British Journal of Haematology · doi:10.1111/bjh.15235 · Cited by 90
Pathophysiology of Stroke in Sickle Cell Disease
HILLERY, C. A., & PANEPINTO, J. A. (2004) · Microcirculation · doi:10.1080/10739680490278600 · Cited by 84
“The leukocyte count is elevated in SCD and correlates with a more severe clinical course, including increased risk of stroke and early death (9,52,60,73).”
BackgroundOrthopaedic Manifestations of Sickle Cell Disease
Vanderhave, K. L., Perkins, C. A., Scannell, B. P., & Brighton, B. K. (2018) · Journal of the American Academy of Orthopaedic Surgeons · doi:10.5435/jaaos-d-16-00255 · Cited by 84
Nordness, M. E., Lynn, J., Zacharisen, M. C., Scott, P. J., & Kelly, K. J. (2005) · Clinical and Molecular Allergy · doi:10.1186/1476-7961-3-2 · Cited by 84
“mortality [1,2].”
Bonds, D. R. (2004) · Blood Reviews · doi:10.1016/j.blre.2004.04.002 · Cited by 84
Epidemiologia de internações por doença falciforme no Brasil
Loureiro, M. M., & Rozenfeld, S. (2005) · Revista de Saúde Pública · doi:10.1590/s0034-89102005000600012 · Cited by 84
Determinants of severity in sickle cell disease
Rees, D. C., Brousse, V. A. M., & Brewin, J. N. (2022) · Blood Reviews · doi:10.1016/j.blre.2022.100983 · Cited by 93
Sarray, S., Saleh, L. R., Saldanha, F. L., Al‐Habboubi, H. H., Mahdi, N., & Almawi, W. Y. (2015) · Cytokine · doi:10.1016/j.cyto.2014.11.030 · Cited by 83
“SCD is characterized by hemolytic anemia, occlusions of the microcirculation, frequent infections and fever, joint pain, lethargy, and weakness, resulting in painful vaso-occlusive crisis (VOC) and chronic organ injury [4–7].”
BackgroundGlomerular Lesions in Patients With Sickle Cell Disease
Maigné, G., Ferlicot, S., Galactéros, F., Belenfant, X., Ulinski, T., Niaudet, P., Ronco, P. M., Godeau, B., Dürrbach, A., Sahali, S., et al. (2010) · Medicine · doi:10.1097/md.0b013e3181ca59b6 · Cited by 83
A simple, rapid, low-cost diagnostic test for sickle cell disease
Yang, X., Kanter, J., Piety, N. Z., Benton, M. S., Vignes, S. M., & Shevkoplyas, S. S. (2013) · Lab on a Chip · doi:10.1039/c3lc41302k · Cited by 83
Maïer-Redelsperger, M., de Montalembert, M., Flahault, A., Neonato, M. G., Ducrocq, R., Masson, M.-P., Girot, R., Élion, J., & the French Study Group on Sickle Cell Disease (1998) · Blood · doi:10.1182/blood.v91.12.4472 · Cited by 83
Malaria chemoprophylaxis in sickle cell disease
Oniyangi, O., & Omari, A. A. (2006) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd003489.pub2 · Cited by 82
Sharan, K. P., Surrey, S., Ballas, S. K., Borowski, M. K., Devoto, M., Wang, K. ‐., Sandler, E. S., & Keller, M. (2003) · British Journal of Haematology · doi:10.1046/j.1365-2141.2003.04762.x · Cited by 82
“(Platt et al, 1994).”
Setty, Y. B. N., & Betal, S. G. (2007) · Blood · doi:10.1182/blood-2007-07-099465 · Cited by 87
Fisiopatologia das doenças falciformes: da mutação genética à insuficiência de múltiplos órgãos
Zago, M. A., & Pinto, A. C. S. (2007) · Revista Brasileira de Hematologia e Hemoterapia · doi:10.1590/s1516-84842007000300003 · Cited by 82
Characteristics of inner-city children with asthma: The National Cooperative Inner-City Asthma Study
Kattan, M., Mitchell, H., Eggleston, P. A., Gergen, P. J., Crain, E. F., Redline, S. S., Weiss, K. B., Evans, R., Kaslow, R. A., Kercsmar, C. M., et al. (1997) · Pediatric Pulmonology · doi:10.1002/(sici)1099-0496(199710)24:4 · Cited by 349
Genetic variants and cell-free hemoglobin processing in sickle cell nephropathy
Saraf, S. L., Zhang, X., Shah, B., Kanias, T., Gudehithlu, K. P., Kittles, R. A., Machado, R. F., Arruda, J. A. L., Gladwin, M. T., Singh, A. K., et al. (2015) · Haematologica · doi:10.3324/haematol.2015.124875 · Cited by 84
Hospital utilization patterns and costs for adult sickle cell patients in Illinois.
Woods, K., Karrison, T. G., Koshy, M., Patel, A. A., Friedmann, P., & Cassel, C. K. (1997) · Europe PMC (PubMed Central) · Cited by 82
Sickle cell disease: a natural model of acute and chronic pain
Brandow, A. M., Zappia, K. J., & Stucky, C. L. (2017) · Pain · doi:10.1097/j.pain.0000000000000824 · Cited by 82
“Patients with more frequent pain crises have a higher mortality rate.(50) The backbone of acute pain crisis treatment includes intravenous opioids.”
BackgroundAfenyi‐Annan, A., Kail, M. E., Combs, M. R., Orringer, E. P., Ashley‐Koch, A. E., & Telen, M. J. (2008) · Transfusion · doi:10.1111/j.1537-2995.2007.01622.x · Cited by 81
“High WBC counts are a strong risk factor for hemorrhagic stroke in both children and adults,(43,44) acute chest syndrome,(45) and early death.(8) Previous studies have also shown that steady state leukocytosis greater than 13,000 ¥ 10(9) per L is associated with more frequent vasoocclusive events.”
BackgroundCardiovascular autonomic dysfunction in sickle cell anemia: A possible risk factor for sudden death?
Mestre, J. C. R., Hernández, A. H., Agramonte, O., & Hernández, P. (1997) · Clinical Autonomic Research · doi:10.1007/bf02308838 · Cited by 81
Thrombin generation and cell‐dependent hypercoagulability in sickle cell disease
Whelihan, M. F., Lim, M. Y., Mooberry, M. J., Piegore, M., Ilich, A., Wogu, A. F., Cai, J., Monroe, D. M., Ataga, K. I., Mann, K. G., et al. (2016) · Journal of Thrombosis and Haemostasis · doi:10.1111/jth.13416 · Cited by 81
The cost of health care for patients with sickle cell disease
Ballas, S. K. (2009) · American Journal of Hematology · doi:10.1002/ajh.21443 · Cited by 81
“Given that the average life span of patients with sickle cell anemia is 42 years for men and 46 years for women [2], the total charge per adult male patient would be about $2,696,400 for 24 years (between the ages of 18 and 42 years) and about $3,145,800 for an adult female patient (between the ages of 18 and 46 years…”
BackgroundLebensburger, J. D., Miller, S. T., Howard, T. H., Casella, J. F., Brown, C., Lü, M., Iyer, R. V., Sarnaik, S. A., Rogers, Z. R., Wang, W. C., et al. (2011) · Pediatric Blood & Cancer · doi:10.1002/pbc.24037 · Cited by 80
Nucleosomes and neutrophil activation in sickle cell disease painful crisis
Schimmel, M., Nur, E., Biemond, B. J., van Mierlo, G. J., Solati, S., Brandjes, D. P. M., Otten, H.-M. M. B., Schnog, J. B., Zeerleder, S. S., & on behalf of the Curama Study Group (2013) · Haematologica · doi:10.3324/haematol.2013.088021 · Cited by 85
Sickle red cells induce adhesion of lymphocytes and monocytes to endothelium
Zennadi, R., Chien, A. C., Xu, K., Batchvarova, M., & Telen, M. J. (2008) · Blood · doi:10.1182/blood-2008-01-134346 · Cited by 80
SICKLE CELL DISEASE: A STIGMATIZING CONDITION THAT MAY LEAD TO DEPRESSION
Jenerette, C. M., Funk, M., & Murdaugh, C. L. (2005) · Issues in Mental Health Nursing · doi:10.1080/01612840500280745 · Cited by 80
“The average lifespan of individuals with SCD is 42 to 48 years (Platt et al., 1994), compared to healthy African Americans who have an average lifespan of 67 to 75 years (Smith & Horton, 1996).”
BackgroundHydroxyurea for Children with Sickle Cell Disease
Heeney, M. M., & Ware, R. E. (2010) · Hematology/Oncology Clinics of North America · doi:10.1016/j.hoc.2009.11.002 · Cited by 80
“The percentage of fetal hemoglobin (HbF), however, is perhaps the most important laboratory parameter influencing clinical severity in SCD [6, 7].”
BackgroundRoseff, S. D. (2009) · Immunohematology · doi:10.21307/immunohematology-2019-234 · Cited by 79
Sabarense, A. P., Lima, G. O., Silva, L. M. L., & Viana, M. B. (2014) · Jornal de Pediatria · doi:10.1016/j.jped.2014.08.006 · Cited by 79
Pulmonary arterial hypertension
Raiesdana, A., & Loscalzo, J. (2006) · Annals of Medicine · doi:10.1080/07853890600622143 · Cited by 81
Sickle Cell Disease in Pregnancy
Rogers, D. T., & Molokie, R. E. (2010) · Obstetrics and Gynecology Clinics of North America · doi:10.1016/j.ogc.2010.02.015 · Cited by 80
McClish, D. K., Levenson, J. L., Penberthy, L. T., Roseff, S. D., Bovbjerg, V. E., Roberts, J. D., Aisiku, I. P., & Smith, W. R. (2006) · Journal of Women s Health · doi:10.1089/jwh.2006.15.146 · Cited by 79
Cardiopulmonary Responses to Exercise in Women with Sickle Cell Anemia
Callahan, L. A., Woods, K. F., Mensah, G. A., Ramsey, L. T., Barbeau, P., & Gutin, B. (2002) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.2002036 · Cited by 79
Early markers of renal dysfunction in patients with sickle cell/β-thalassemia
Voskaridou, E., Terpos, E., Michail, S., Hantzi, E., Anagnostopoulos, A., Margeli, A. P., Simirloglou, D., Loukopoulos, D., & Papassotiriou, I. (2006) · Kidney International · doi:10.1038/sj.ki.5000248 · Cited by 79
Jiao, B., JOHNSON, K., Ramsey, S. D., Bender, M. A., Devine, B., & Basu, A. (2023) · Blood Advances · doi:10.1182/bloodadvances.2022009202 · Cited by 78
How I treat renal complications in sickle cell disease
Sharpe, C. C., & Thein, S. L. (2014) · Blood · doi:10.1182/blood-2014-02-557439 · Cited by 78
“5% of deaths in adult patients with SCDwere associated with renal impairment.(3) In keeping with this trend, in 2008, advanced CKD was reported as present in 24% of SCD patients who had survived to 60 years of age or more and was the cause of death in 45% in those who died above this age.”
BackgroundSobota, A. E., Graham, D. A., Heeney, M. M., & Neufeld, E. J. (2009) · American Journal of Hematology · doi:10.1002/ajh.21565 · Cited by 78
“Given the amazing variation we demonstrate without clear medical reasons, we agree with other authors that a multicenter, prospective study is urgently needed to clarify both the potential efficacy and toxicity of corticosteroids for treatment of ACS in children with SCD.”
BackgroundInfluentialAlapan, Y., Kim, C., Adhikari, A., Gray, K. E., Gürkan-Çavusoglu, E., Little, J. A., & Gurkan, U. A. (2016) · Translational research · doi:10.1016/j.trsl.2016.03.008 · Cited by 77
“In this work, we identified a unique adherent nondeformable RBC population, significantly correlated with serum LDH levels, which reflects a functional outcome in terms of both deformability and adhesion of RBCs and is a pathophysiologically plausible source for active hemolysis in SCD.”
BackgroundInfluentialElevated IL‐8 levels during sickle cell crisis
Duits, A. J., Schnog, J. B., Lard, L. R., Saleh, A. W., & Rojer, R. A. (1998) · European Journal Of Haematology · doi:10.1111/j.1600-0609.1998.tb01092.x · Cited by 80
Bone marrow transplantation in sickle cell anemia
Hoppe, C. C., & Walters, M. C. (2001) · Current Opinion in Oncology · doi:10.1097/00001622-200103000-00001 · Cited by 79
Danielson, C. (2002) · Therapeutic Apheresis and Dialysis · doi:10.1046/j.1526-0968.2002.00396.x · Cited by 75
Kang, E. M., Areman, E. M., David‐Ocampo, V., Fitzhugh, C. D., Link, M. E., Read, E. J., Leitman, S. F., Rodgers, G. P., & Tisdale, J. F. (2002) · Blood · doi:10.1182/blood.v99.3.850 · Cited by 77
Barakat, L. P., Schwartz, L. A., Salamon, K. S., & Radcliffe, J. (2010) · Journal of Pediatric Hematology/Oncology · doi:10.1097/mph.0b013e3181e793f9 · Cited by 77
“Chronic, unpredictable pain is the most common complication of SCD, contributing to hospitalizations and interference with daily activities(2) and often increasing during adolescence.(3) In addition to pain, adolescents with SCD may experience chronic anemia, susceptibility to infection, pulmonary complications and ac…”
BackgroundBrousseau, D. C., Scott, J. P., Badaki‐Makun, O., Darbari, D. S., Chumpitazi, C. E., Airewele, G. E., Ellison, A. M., Smith‐Whitley, K., Mahajan, P., Sarnaik, S. A., et al. (2015) · Blood · doi:10.1182/blood-2015-05-647107 · Cited by 76
COVID‐19‐induced endotheliitis: emerging evidence and possible therapeutic strategies
Calabretta, E., Moraleda, J. M., Iacobelli, M., Jara, R., Vlodavsky, I., O’Gorman, P., Pagliuca, A., Mo, C. C., Baron, R. M., Aghemo, A., et al. (2021) · British Journal of Haematology · doi:10.1111/bjh.17240 · Cited by 75
“Vaso-occlusive phenomena commonly affect the lung vasculature, provoking acute chest syndrome (ACS), a spectrum of diseases ranging from mild pneumonia to ARDS and MOF, which is the leading cause of morbidity and mortality in SCD.(53) Lung specimens from ACS cases showed micro-thrombotic occlusion, endothelial VWF dep…”
ResultPATHOPHYSIOLOGY OF VASO-OCCLUSION
Ballas, S. K., & Mohandas, N. (1996) · Hematology/Oncology Clinics of North America · doi:10.1016/s0889-8588(05)70396-8 · Cited by 76
Instability of dinucleotide repeats in Hodgkin's disease
Mark, Z., Toren, A., Amariglio, N., Schiby, G., Brok‐Simoni, F., & Rechavi, G. (1998) · American Journal of Hematology · doi:10.1002/(sici)1096-8652(199802)57:2 · Cited by 16
Najaf-Zadeh, A., & Leclerc, F. (2011) · Annals of Intensive Care · doi:10.1186/2110-5820-1-15 · Cited by 76
“Acute chest syndrome (ACS) is one of the leading causes of death and hospitalization among patients with sickle cell disease [42] .”
BackgroundPreventive Services Received by Adolescents With Cystic Fibrosis and Sickle Cell Disease
Britto, M. T., Garrett, J. M., Dugliss, M. A. J., Johnson, C. A., Majure, J. M., & Leigh, M. W. (1999) · Archives of Pediatrics and Adolescent Medicine · doi:10.1001/archpedi.153.1.27 · Cited by 76
Dynamic deformability of sickle red blood cells in microphysiological flow
Alapan, Y., Matsuyama, Y., Little, J. A., & Gurkan, U. A. (2016) · TECHNOLOGY · doi:10.1142/s2339547816400045 · Cited by 76
Hydroxyurea Treatment for Sickle Cell Disease
Steinberg, M. H. (2002) · The Scientific World JOURNAL · doi:10.1100/tsw.2002.295 · Cited by 74
“1)[133].”
Self-citationQuarmyne, M., Wei, D., Theodore, R. S., Anand, S. S., Barry, V., Adisa, O. A., Buchanan, I. D., Bost, J. E., Brown, R. C., Joiner, C. H., et al. (2016) · American Journal of Hematology · doi:10.1002/ajh.24587 · Cited by 75
Testing the theory of self‐care management for sickle cell disease
Jenerette, C. M., & Murdaugh, C. L. (2008) · Research in Nursing & Health · doi:10.1002/nur.20261 · Cited by 75
“The majority of admissions are related to acute painful crises (Houston-Yu, Rana, Beyer, & Castro, 2003; Platt et al., 1994; Prasad, Hasan, Castro, Perlin, & Kim, 2003).”
BackgroundDevelopment of the PedsQL™ sickle cell disease module items: qualitative methods
Panepinto, J. A., Torres, S., & Varni, J. W. (2011) · Quality of Life Research · doi:10.1007/s11136-011-9941-4 · Cited by 81
“The evaluation of the disease burden and treatment effect on those with sickle cell disease has traditionally focused on measuring the number of hospitalizations, occurrence of painful crises, determining laboratory values or calculating mortality data [1–3].”
BackgroundBuchanan, I. D., Woodward, M., & Reed, G. W. (2005) · Pediatric Blood & Cancer · doi:10.1002/pbc.20403 · Cited by 75
Couque, N., Girard, D., Ducrocq, R., Boizeau, P., Haouari, Z., Missud, F., Holvoet, L., Ithier, G., Belloy, M., Odièvre, M., et al. (2016) · British Journal of Haematology · doi:10.1111/bjh.14015 · Cited by 76
“The rates of recurrent VOC, recurrent severe ACS and severe chronic anaemia in our cohort were comparable to published reports (Platt et al, 1991, 1994; Vichinsky et al, 1997).”
BackgroundResultCauses and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease
(2000) · New England Journal of Medicine · doi:10.1056/nejm200009143431122 · Cited by 76
Occurrence of hemophilia in the United States
Soucie, J. M., Evatt, B. L., Jackson, D. J., & the Hemophilia Surveillance System Project Investigators (1998) · American Journal of Hematology · doi:10.1002/(sici)1096-8652(199812)59:4 · Cited by 347
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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