The DOI matches its Crossref record; 19 of the 19 references it cites were found in a registry or an index.
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.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
The source backs part of the claim, not all of it — or backs it for a different population, condition or comparison.
In the citing paper
“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.
In the citing paper
“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]
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.
In the citing paper
“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
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
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
Kernan, W. N., Ovbiagele, B., Black, H. R., Bravata, D. M., Chimowitz, M. I., Ezekowitz, M. D., Fang, M. C., Fisher, M., Furie, K. L., Heck, D. V., et al. (2014) · Stroke · doi:10.1161/str.0000000000000024 · Cited by 5,170
“Sickle Cell Disease Stroke is a common complication of sickle cell disease, and stroke is a major cause of death in both children and adults with sickle cell disease.(705) The highest risk of stroke is in patients with the SS genotype, but stroke can occur in patients with other genotypes.”
BackgroundRees, D. C., Williams, T. N., & Gladwin, M. T. (2010) · The Lancet · doi:10.1016/s0140-6736(10)61029-x · Cited by 2,504
CRISPR-Cas9 Gene Editing for Sickle Cell Disease and β-Thalassemia
Frangoul, H., Altshuler, D., Cappellini, M. D., Chen, Y.-S., Domm, J., Eustace, B. K., Foell, J., de la Fuente, J., Grupp, S., Handgretinger, R., et al. (2021) · New England Journal of Medicine · doi:10.1056/nejmoa2031054 · Cited by 1,962
Self-citationGlobal epidemiology of haemoglobin disorders and derived service indicators
Modell, B. (2008) · Bulletin of the World Health Organization · doi:10.2471/blt.06.036673 · Cited by 1,932
Gulati, M., Levy, P. D., Mukherjee, D., Amsterdam, E. A., Bhatt, D. L., Birtcher, K. K., Blankstein, R., Boyd, J. H., Bullock-Palmer, R. P., Conejo, T., et al. (2021) · Circulation · doi:10.1161/cir.0000000000001029 · Cited by 1,684
Pulmonary Arterial Hypertension
Farber, H. W., & Loscalzo, J. (2004) · New England Journal of Medicine · doi:10.1056/nejmra035488 · Cited by 1,467
Kato, G. J., Piel, F. B., Reid, C. D., Gaston, M. H., Ohene-Frempong, K., Krishnamurti, L., Smith, W. R., Panepinto, J. A., Weatherall, D. J., Costa, F. F., et al. (2018) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2018.10 · Cited by 1,501
Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Disease
Gladwin, M. T., Sachdev, V., Jison, M. L., Shizukuda, Y., Plehn, J. F., Minter, K., Brown, B., Coles, W. A., Nichols, J. S., Ernst, I., et al. (2004) · New England Journal of Medicine · doi:10.1056/nejmoa035477 · Cited by 1,325
Self-citationCauses and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease
Vichinsky, E. P., Neumayr, L. D., Earles, A. N., Williams, R., Lennette, E. T., Dean, D., Nickerson, B., Orringer, E., McKie, V., Bellevue, R., et al. (2000) · New England Journal of Medicine · doi:10.1056/nejm200006223422502 · Cited by 1,234
Population Estimates of Sickle Cell Disease in the U.S.
Hassell, K. L. (2010) · American Journal of Preventive Medicine · doi:10.1016/j.amepre.2009.12.022 · Cited by 1,214
Piel, F. B., Hay, S. I., Gupta, S., Weatherall, D. J., & Williams, T. N. (2013) · PLoS Medicine · doi:10.1371/journal.pmed.1001484 · Cited by 1,144
“[31].”
Borgna‐Pignatti, C., Rugolotto, S., Stefano, P. D., Zhao, H., Cappellini, M. D., Vecchio, G. C. D., Romeo, M. A., Forni, G. L., Gamberini, M. R., Ghilardi, R., et al. (2004) · PubMed · Cited by 1,119
Ware, R. E., de Montalembert, M., Tshilolo, L. M., & Abboud, M. R. (2017) · The Lancet · doi:10.1016/s0140-6736(17)30193-9 · Cited by 1,036
Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia
Steinberg, M. H., Barton, F. B., Castro, O. L., Pegelow, C. H., Ballas, S. K., Kutlar, A., Orringer, E. P., Bellevue, R., Olivieri, N. F., Eckman, J. R., et al. (2003) · JAMA · doi:10.1001/jama.289.13.1645 · Cited by 928
“Objective To determine whether hydroxyurea attenuates mortality in patients with SCA.”
BackgroundResultInfluentialSelf-citationCrizanlizumab for the prevention of pain crises in sickle cell disease.
Ataga, K. I., Kutlar, A., Kanter, J., Liles, D., Cancado, R., Friedrisch, J., Guthrie, T. H., Knight-Madden, J., Alvarez, O. A., Gordeuk, V. R., et al. (2017) · New England Journal of Medicine · doi:10.1056/nejmoa1611770 · Cited by 914
Stuart, M. J., & Nagel, R. L. (2004) · The Lancet · doi:10.1016/s0140-6736(04)17192-4 · Cited by 867
2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR Guideline for the Evaluation and Diagnosis of Chest Pain
Gulati, M., Levy, P. D., Mukherjee, D., Amsterdam, E. A., Bhatt, D. L., Birtcher, K. K., Blankstein, R., Boyd, J. H., Bullock-Palmer, R. P., Conejo, T., et al. (2021) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2021.07.053 · Cited by 849
Pathophysiology of Sickle Cell Disease
Sundd, P., Gladwin, M. T., & Novelli, E. M. (2019) · Annual Review of Pathology Mechanisms of Disease · doi:10.1146/annurev-pathmechdis-012418-012838 · Cited by 813
Wang, W. C., Ware, R. E., Miller, S. T., Iyer, R. V., Casella, J. F., Minniti, C. P., Rana, S., Thornburg, C. D., Rogers, Z. R., Kalpatthi, R. V., et al. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60355-3 · Cited by 803
Serjeant, G. R. (1997) · The Lancet · doi:10.1016/s0140-6736(97)07330-3 · Cited by 768
Intravascular hemolysis and the pathophysiology of sickle cell disease
Kato, G. J., Steinberg, M. H., & Gladwin, M. T. (2017) · Journal of Clinical Investigation · doi:10.1172/jci89741 · Cited by 748
“Mortality Increases risk (19, 39, 42, 47, 49, 64, 188) Protective (189) Protective (190)”
Self-citationUda, M., Galanello, R., Sanna, S., Lettre, G., Sankaran, V. G., Chen, W., Usala, G., Busonero, F., Maschio, A., Albai, G., et al. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0711566105 · Cited by 672
Pulmonary Arterial Hypertension
McLaughlin, V. V., & McGoon, M. D. (2006) · Circulation · doi:10.1161/circulationaha.104.503540 · Cited by 667
Daily Assessment of Pain in Adults with Sickle Cell Disease
Smith, W. R., Penberthy, L. T., Bovbjerg, V. E., McClish, D. K., Roberts, J. D., Dahman, B. A., Aisiku, I. P., Levenson, J. L., & Roseff, S. D. (2008) · Annals of Internal Medicine · doi:10.7326/0003-4819-148-2-200801150-00004 · Cited by 656
“Caregivers have therefore traditionally used the term crisis to describe these contacts and their underlying episodic, acute pain exacerbations and have reserved the term chronic pain syndrome to describe pain from sickle cell disease complications, such as ankle ulcers or avascular necrosis (13).”
BackgroundVichinsky, E. P., Haberkern, C. M., Neumayr, L., Earles, A. N., Black, D. M., Koshy, M., Pegelow, C. H., Abboud, M. R., Ohene‐Frempong, K., & Iyer, R. V. (1995) · New England Journal of Medicine · doi:10.1056/nejm199507273330402 · Cited by 644
Management of Sickle Cell Disease
Steinberg, M. H. (1999) · New England Journal of Medicine · doi:10.1056/nejm199904013401307 · Cited by 603
Self-citationAcute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course
Vichinsky, E. P., Styles, L. A., Colangelo, L. H., Wright, E. C., Castro, O., Nickerson, B., & Disease, T. C. S. O. S. C. (1997) · Blood · doi:10.1182/blood.v89.5.1787 · Cited by 573
Self-citationOutcome of Sickle Cell Anemia: A 4-Decade Observational Study of 1056 Patients
Powars, D. R., Chan, L. S., Hiti, A., Ramicone, E., & Johnson, C. (2005) · Medicine · doi:10.1097/01.md.0000189089.45003.52 · Cited by 558
Lettre, G., Sankaran, V. G., Bezerra, M., Araujo, A. S., Uda, M., Sanna, S., Cao, A., Schlessinger, D., Costa, F. F., Hirschhorn, J. N., et al. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0804799105 · Cited by 577
“Subsequent analyses in the CSSCD also clarified the natural history and identified predictors of ACS, in which patients suffer from hypoxia and chest pain secondary to sickling in the pulmonary vasculature (3), and mortality (4).”
BackgroundMethodResultInfluentialFetal hemoglobin in sickle cell anemia
Akinsheye, I., Alsultan, A., Solovieff, N., Ngo, D., Baldwin, C. T., Sebastiani, P., Chui, D. H. K., & Steinberg, M. H. (2011) · Blood · doi:10.1182/blood-2011-03-325258 · Cited by 553
“Three possible mechanisms have been proposed to explain the increased HbF associated with these large deletions: (1) the deletion of regulatory sequences affecting HBG expression, (2) juxtaposition of enhancers of HBG normally located downstream of HBB, and (3) LCR interactions with HBG resulting from deletion of HBB.”
Self-citationSickle Hemoglobin (Hb S) Allele and Sickle Cell Disease: A HuGE Review
Ashley-Koch, A., Yang, Q., & Olney, R. S. (2000) · American Journal of Epidemiology · doi:10.1093/oxfordjournals.aje.a010288 · Cited by 528
“Among adults (>20 years) with sickle cell disease, the causes of mortality are more varied (14).”
BackgroundInfluentialPrediction of Adverse Outcomes in Children with Sickle Cell Disease
Miller, S. T., Sleeper, L. A., Pegelow, C. H., Enos, L. E., Wang, W. C., Weiner, S. J., Wethers, D. L., Smith, J., & Kinney, T. R. (2000) · New England Journal of Medicine · doi:10.1056/nejm200001133420203 · Cited by 508
Long-term results of related myeloablative stem-cell transplantation to cure sickle cell disease
Bernaudin, F., Socié, G., Kuentz, M., Chevret, S., Duval, M., Bertrand, Y., Vannier, J., Yakouben, K., Thuret, I., Bordigoni, P., et al. (2007) · Blood · doi:10.1182/blood-2007-03-079665 · Cited by 519
“Median ferritin level, ng/mL (range) 374 (13-3820) 792 (340-2059) 604 (31-3171) 553 (13-3820)”
DeWitt, M. A., Magis, W., Bray, N. L., Wang, T., Berman, J. R., Urbinati, F., Heo, S.-J., Mitros, T., Muñoz, D. P., Boffelli, D., et al. (2016) · Science Translational Medicine · doi:10.1126/scitranslmed.aaf9336 · Cited by 477
American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support
Chou, S. T., Alsawas, M., Fasano, R. M., Field, J. J., Hendrickson, J. E., Howard, J., Kameka, M., Kwiatkowski, J. L., Pirenne, F., Shi, P. A., et al. (2020) · Blood Advances · doi:10.1182/bloodadvances.2019001143 · Cited by 506
Gluckman, É., Cappelli, B., Bernaudin, F., Labopin, M., Volt, F., Carreras, J., Simões, B. P., Ferster, A., Dupont, S., de la Fuente, J., et al. (2016) · Blood · doi:10.1182/blood-2016-10-745711 · Cited by 496
Primary role for adherent leukocytes in sickle cell vascular occlusion: A new paradigm
Turhan, A., Weiss, L. A., Mohandas, N., Coller, B. S., & Frenette, P. S. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.052522799 · Cited by 487
The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up
Steinberg, M. H., McCarthy, W. F., Castro, O., Ballas, S. K., Armstrong, F. D., Smith, W., Ataga, K., Swerdlow, P., Kutlar, A., DeCastro, L., et al. (2010) · American Journal of Hematology · doi:10.1002/ajh.21699 · Cited by 486
“Even with so-called “mild disease,” it is the rare patient that escapes these complications.[29,33,47] Besides drug therapy, transfusions and stem cell transplantation can be efficacious but each has complications.”
Self-citationPulmonary Complications of Sickle Cell Disease
Gladwin, M. T., & Vichinsky, E. (2008) · New England Journal of Medicine · doi:10.1056/nejmra0804411 · Cited by 470
The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy
Hebbel, R. P., Osarogiagbon, R. U., & Kaul, D. K. (2004) · Microcirculation · doi:10.1080/10739680490278402 · Cited by 464
“It is interesting, given that female humans with sickle disease have greater bioavailability of NO (58) and longevity (139) compared to males, that female rodents were selectively protected due to action of hepatic eNOS in a nonsickle model of liver ischemia– reperfusion (69).”
BackgroundMortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005
Lanzkron, S., Carroll, C. P., & Haywood, C. (2013) · Public Health Reports® · doi:10.1177/003335491312800206 · Cited by 414
“Ischemic heart disease 497 (3) 246 (50) 54.”
Mortality in Sickle Cell Disease
Rodgers, G. P., Walker, E. C., & Podgor, M. J. (1994) · New England Journal of Medicine · doi:10.1056/nejm199410133311515 · Cited by 459
Post-Transcriptional Genetic Silencing of BCL11A to Treat Sickle Cell Disease
Esrick, E. B., Lehmann, L. E., Biffi, A., Achebe, M., Brendel, C., Ciuculescu, M. F., Daley, H., MacKinnon, B., Morris, E., Federico, A., et al. (2021) · New England Journal of Medicine · doi:10.1056/nejmoa2029392 · Cited by 449
Reactivation of developmentally silenced globin genes by forced chromatin looping
Deng, W., Rupon, J. W., Krivega, I., Breda, L., Motta, I., Jahn, K. S., Reik, A., Gregory, P. D., Rivella, S., Dean, A., et al. (2014) · Cell · doi:10.1016/j.cell.2014.05.050 · Cited by 443
“Patients affected by sickle cell anemia and b-thalassemia experience a milder course of the disease if they express elevated levels of fetal hemoglobin in adulthood (Platt et al., 1994; Weatherall, 2001).”
BackgroundVoskaridou, E., Christoulas, D., Bilalis, A., Plata, E., Varvagiannis, K., Stamatopoulos, G., Sinopoulou, K., Balassopoulou, A., Loukopoulos, D., & Terpos, E. (2010) · Blood · doi:10.1182/blood-2009-05-221333 · Cited by 430
“001); (3) a significant reduction of hospital admissions from 2.”
BackgroundMethodInfluentialGulati, M., Levy, P. D., Mukherjee, D., Amsterdam, E. A., Bhatt, D. L., Birtcher, K. K., Blankstein, R., Boyd, J. H., Bullock-Palmer, R. P., Conejo, T., et al. (2021) · Circulation · doi:10.1161/cir.0000000000001030 · Cited by 436
Zhang, D., Xu, C., Manwani, D., & Frenette, P. S. (2016) · Blood · doi:10.1182/blood-2015-09-618538 · Cited by 413
Fetal hemoglobin in sickle cell anemia: determinants of response to hydroxyurea
Steinberg, M. H., Lu, Z.-H., Barton, F. B., Terrin, M. L., Charache, S., Dover, G. J., & Hydroxyurea, T. M. S. O. (1997) · Blood · doi:10.1182/blood.v89.3.1078 · Cited by 407
Self-citationSickle cell disease: old discoveries, new concepts, and future promise
Frenette, P. S., & Atweh, G. F. (2007) · Journal of Clinical Investigation · doi:10.1172/jci30920 · Cited by 416
Angelucci, E., Matthes‐Martin, S., BARONCIANI, D., Bernaudin, F., Bonanomi, S., Cappellini, M. D., Dalle, J., Bartolomeo, P. D., de Heredia, C. D., Dickerhoff, R., et al. (2014) · Haematologica · doi:10.3324/haematol.2013.099747 · Cited by 404
How I use hydroxyurea to treat young patients with sickle cell anemia
Ware, R. E. (2010) · Blood · doi:10.1182/blood-2009-04-146852 · Cited by 406
“Low Hb and %HbF, as well as elevated WBC and LDH, are associated with adverse clinical outcomes [1,2,5,7]; hydroxyurea will have”
BackgroundInfluentialThe cost of health care for children and adults with sickle cell disease
Kauf, T. L., Coates, T. D., Liu, H., Mody‐Patel, N., & Hartzema, A. G. (2009) · American Journal of Hematology · doi:10.1002/ajh.21408 · Cited by 388
“Lifetime costs then were estimated by summing annualized costs through age 44, the approximate median survival for SCD patients [10].”
BackgroundMethodThe number of people with sickle‐cell disease in the United States: national and state estimates
Brousseau, D. C., Panepinto, J. A., Nimmer, M., & Hoffmann, R. G. (2009) · American Journal of Hematology · doi:10.1002/ajh.21570 · Cited by 383
Survival of children with sickle cell disease
Quinn, C. T., Rogers, Z. R., & Buchanan, G. R. (2004) · Blood · doi:10.1182/blood-2003-11-3758 · Cited by 376
“The current status of each patient is indicated by continually updated status codes: (1) active (evaluated in our center within the past 2 years); (2) inactive (not evaluated in the past 2-5 years); (3) lost to follow-up (not evaluated in the past 5 or more years); (4) moved; (5) deceased; (6) transitioned to adult me…”
BackgroundMethodInfluentialSickle cell disease: renal manifestations and mechanisms
Nath, K. A., & Hebbel, R. P. (2015) · Nature Reviews Nephrology · doi:10.1038/nrneph.2015.8 · Cited by 367
Sangokoya, C., Telen, M. J., & Chi, J. (2010) · Blood · doi:10.1182/blood-2009-04-214817 · Cited by 363
Hydroxyurea for the Treatment of Sickle Cell Anemia
Platt, O. S. (2008) · New England Journal of Medicine · doi:10.1056/nejmct0708272 · Cited by 357
Self-citationTelfer, P. T., Coen, P. G., Chakravorty, S., Wilkey, O., Evans, J. P. M., Newell, H., Smalling, B., Amos, R. I. J., Stephens, A. D., Rogers, D., et al. (2007) · Haematologica · doi:10.3324/haematol.10937 · Cited by 356
“5) 23 (9) Other adult clinic 5 (3) 5 (8) 1 (12.”
Infection in sickle cell disease: A review
Booth, C., Inusa, B. P. D., & Obaro, S. (2009) · International Journal of Infectious Diseases · doi:10.1016/j.ijid.2009.03.010 · Cited by 355
Causes of death in sickle cell disease: an autopsy study
Manci, E. A., Culberson, D. E., Yang, Y., Gardner, T. M., Powell, R. W., Haynes, J., Shah, A. K., Mankad, V. N., & Investigators of the Cooperative Study of Sickle Cell Disease (2003) · British Journal of Haematology · doi:10.1046/j.1365-2141.2003.04594.x · Cited by 353
“Clinical series have been more common (Seeler, 1972; Serjeant, 1975; Anionwu et al, 1981; Ashcroft & Serjeant, 1981; Mann, 1981; Murthy & Haywood, 1981; Brozovic & Anionwu, 1984; Bainbridge et al, 1985; Sarma et al, 1986; Kehinde et al, 1987; Gill et al, 1989; Leikin et al, 1989; Platt et al, 1994).”
BackgroundResultInfluentialLubeck, D., Agodoa, I., Bhakta, N., Danese, M., Pappu, K., Howard, R., Gleeson, M., Halperin, M., & Lanzkron, S. (2019) · JAMA Network Open · doi:10.1001/jamanetworkopen.2019.15374 · Cited by 336
Hydroxyurea and Sickle Cell Anemia Clinical Utility of a Myelosuppressive “Switching” Agent
Charache, S., Barton, F. B., Moore, R. D., Terrin, M. L., Steinberg, M. H., Dover, G. J., Ballas, S. K., McMahon, R. P., Castro, O. L., & Orringer, E. P. (1996) · Medicine · doi:10.1097/00005792-199611000-00002 · Cited by 335
Self-citationThe switch from fetal to adult hemoglobin.
Sankaran, V. G., & Orkin, S. H. (2013) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a011643 · Cited by 333
“In this article, we discuss historic attempts to induce HbF that have resulted in some therapeutic approaches to manage SCD and b-thalassemia.”
ResultInfluentialGenetic dissection and prognostic modeling of overt stroke in sickle cell anemia
Sebastiani, P., Ramoni, M. F., Nolan, V., Baldwin, C. T., & Steinberg, M. H. (2005) · Nature Genetics · doi:10.1038/ng1533 · Cited by 333
“, and increased fetal hemoglobin (HbF) levels are associated with reduced risks for other complication”
BackgroundSelf-citationCorrection of Sickle Cell Disease in Adult Mice by Interference with Fetal Hemoglobin Silencing
Xu, J., Peng, C., Sankaran, V. G., Shao, Z., Esrick, E. B., Chong, B. G., Ippolito, G. C., Fujiwara, Y., Ebert, B. L., Tucker, P. W., et al. (2011) · Science · doi:10.1126/science.1211053 · Cited by 326
Morris, C. R., Morris, S. M., Hagar, W., van Warmerdam, J., Claster, S., Kepka‐Lenhart, D., Machado, L., Kuypers, F. A., & Vichinsky, E. P. (2003) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200208-967oc · Cited by 328
“Nitric oxide metabolism is altered during acute chest syndrome (42), a common pulmonary complication of sickle cell disease (43) associated with low plasma arginine (20) and nitric oxide metabolite levels (20, 44), that is also often accompanied by pulmonary hypertension.”
Mortality in Sickle Cell Anemia in Africa: A Prospective Cohort Study in Tanzania
Makani, J., Cox, S. E., Soka, D., Komba, A., Oruo, J., Mwamtemi, H., Magesa, P., Rwezaula, S. S., Meda, E., Mgaya, J., et al. (2011) · PLoS ONE · doi:10.1371/journal.pone.0014699 · Cited by 327
“13 per 100 PYO reported from the USA[4,6].”
BackgroundMethodResultInfluentialDiastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease
Sachdev, V., Machado, R. F., Shizukuda, Y., Rao, Y. N., Sidenko, S., Ernst, I., St. Peter, M., Coles, W. A., Rosing, D. R., Blackwelder, W. C., et al. (2007) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2006.09.038 · Cited by 323
Self-citationPredicting clinical severity in sickle cell anaemia
Steinberg, M. H. (2005) · British Journal of Haematology · doi:10.1111/j.1365-2141.2005.05411.x · Cited by 330
“Long range linkage disequilibrium (LD), as seen in this QTL, has been reported for other chromosomal regions (Liu & Barker, 1999).”
BackgroundSelf-citationGenetic modifiers of sickle cell disease
Steinberg, M. H., & Sebastiani, P. (2012) · American Journal of Hematology · doi:10.1002/ajh.23232 · Cited by 315
“Median lifespan for patients with sickle cell anemia in the United States was in the fifth decade [19].”
BackgroundSelf-citationThein, S. L., Menzel, S., Xu, P., Best, S., Jiang, J., Close, J. P., Silver, N., Gerovassili, A., Chen, P., Yamaguchi, M., et al. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0611393104 · Cited by 313
Zimmerman, S. A., Schultz, W. H., Davis, J. S., Pickens, C. V., Mortier, N. A., Howard, T. A., & Ware, R. E. (2003) · Blood · doi:10.1182/blood-2003-07-2475 · Cited by 312
Vermylen, C., Cornu, G., Ferster, A., Brichard, B., Ninane, J., Ferrant, A., Zenebergh, A., Maes, P., Dhooge, C., Benoît, Y., et al. (1998) · Bone Marrow Transplantation · doi:10.1038/sj.bmt.1701291 · Cited by 312
Silent cerebral infarcts in sickle cell anemia: a risk factor analysis
Kinney, T. R., Sleeper, L. A., Wang, W. C., Zimmerman, R. A., Pegelow, C. H., Ohene-Frempong, K., Wethers, D. L., Bello, J. A., Vichinsky, E. P., Moser, F. G., et al. (1999) · PEDIATRICS · doi:10.1542/peds.103.3.640 · Cited by 309
Self-citationThe paradox of the neutrophilˈs role in tissue injury
Segel, G. B., Halterman, M. W., & Lichtman, M. A. (2010) · Journal of Leukocyte Biology · doi:10.1189/jlb.0910538 · Cited by 315
“1 10(9)/L had a higher risk of death [67].”
BackgroundVichinsky, E. P. (2010) · JAMA · doi:10.1001/jama.2010.562 · Cited by 296
“The major findings are that (1) adults with SCA showed poorer performance on neurocognitive tests when compared with community controls; (2) anemia is associated with the age-related decline in cognitive performance; and (3) MRI findings do not explain the performance differences in the subset of patients with neuroim…”
Factors associated with survival in a contemporary adult sickle cell disease cohort
Elmariah, H., Garrett, M. E., Castro, L. M. D., Jonassaint, J., Ataga, K. I., Eckman, J. R., Ashley‐Koch, A. E., & Telen, M. J. (2014) · American Journal of Hematology · doi:10.1002/ajh.23683 · Cited by 300
“in subjects with Hb SS and 60–68 years in subjects with Hb SC [6].”
BackgroundResultInfluentialHealth related quality of life in sickle cell patients: The PiSCES project
McClish, D. K., Penberthy, L. T., Bovbjerg, V. E., Roberts, J. D., Aisiku, I. P., Levenson, J. L., Roseff, S. D., & Smith, W. R. (2005) · Health and Quality of Life Outcomes · doi:10.1186/1477-7525-3-50 · Cited by 308
Population genetics of malaria resistance in humans
Hedrick, P. W. (2011) · Heredity · doi:10.1038/hdy.2011.16 · Cited by 308
Pulmonary hypertension in patients with sickle cell disease: a longitudinal study
Ataga, K. I., Moore, C. G., Jones, S., Olajide, O., Strayhorn, D., Hinderliter, A., & Orringer, E. P. (2006) · British Journal of Haematology · doi:10.1111/j.1365-2141.2006.06110.x · Cited by 298
“Furthermore, high HbF levels decrease the risk of vaso-occlusive complications, such as acute chest syndrome (Castro et al, 1994) and pain crisis (Platt et al, 1991), as well as the risk of early death (Platt et al, 1994).”
BackgroundThe Natural History of Sickle Cell DiseasePreprint
Serjeant, G. R. (2013) · Cold Spring Harbor Perspectives in Medicine · doi:10.1101/cshperspect.a011783 · Cited by 296
“Overall, survival in SS disease in developed societies now shows a median survival of 45– 55 yr in the United States and Jamaica (Platt et al. 1994; Wierenga et al. 2001).”
BackgroundSteinberg, M. H. (2008) · The Scientific World JOURNAL · doi:10.1100/tsw.2008.157 · Cited by 306
“Induction of HbF has been a goal of treating sickle cell disease for decades, since the clinical and laboratory observations that HbF inhibits HbS polymerization and that higher levels of HbF are associated with reduced mortality[137,138].”
BackgroundSelf-citationSickle cell anemia as an inflammatory disease
Platt, O. (2000) · Journal of Clinical Investigation · doi:10.1172/jci10726 · Cited by 293
“Patients with high baseline leukocyte counts are more likely to die at a younger age (5).”
BackgroundSelf-citationIncentive Spirometry to Prevent Acute Pulmonary Complications in Sickle Cell Diseases
Bellet, P. S., Kalinyak, K. A., Shukla, R., Gelfand, M. J., & Rucknagel, D. L. (1995) · New England Journal of Medicine · doi:10.1056/nejm199509143331104 · Cited by 278
Sickle cell disease related mortality in the United States (1999-2009)
Hamideh, D., & Alvarez, O. A. (2013) · Pediatric Blood & Cancer · doi:10.1002/pbc.24557 · Cited by 290
“In 1994, the National Institutes of Health-sponsored multi-center Cooperative Study of Sickle Cell Disease (CSSCD) estimated that the median survival for individuals with Hb SS was 42 years for males and 48 years for female patients followed at the participating sickle cell centers [2].”
BackgroundResultInfluentialEpidemiology of Sickle Cell Disease in Saudi Arabia
Jastaniah, W. A. (2011) · Annals of Saudi Medicine · doi:10.4103/0256-4947.81540 · Cited by 289
Systematic Review: Hydroxyurea for the Treatment of Adults with Sickle Cell Disease
Lanzkron, S., Strouse, J. J., Wilson, R. F., Beach, M. C., Haywood, C., Park, H., Witkop, C., Bass, E. B., & Segal, J. B. (2008) · Annals of Internal Medicine · doi:10.7326/0003-4819-148-12-200806170-00221 · Cited by 288
“Sickle cell disease, which affects nearly 100 000 persons in the United States (1), decreases life expectancy by 25 to 30 years (2) and causes great morbidity (3).”
BackgroundThe mouse as a model for human biology: a resource guide for complex trait analysis
Peters, L. L., Robledo, R. F., Bult, C. J., Churchill, G. A., Paigen, B. J., & Svenson, K. L. (2006) · Nature Reviews Genetics · doi:10.1038/nrg2025 · Cited by 293
Leukocytosis and Ischemic Vascular Disease Morbidity and Mortality: Is It Time to Intervene?
Coller, B. S. (2005) · Arteriosclerosis, Thrombosis, and Vascular Biology · doi:10.1161/01.atv.0000156877.94472.a5 · Cited by 286
Extracellular hemin crisis triggers acute chest syndrome in sickle mice
Ghosh, S., Adisa, O. A., Chappa, P., Tan, F., Jackson, K. A., Archer, D. R., & Ofori-Acquah, S. F. (2013) · Journal of Clinical Investigation · doi:10.1172/jci64578 · Cited by 282
“Acute chest syndrome (ACS) is the second most common reason for hospital admission of SCD patients (2) and the leading cause of referral to intensive care units (3), and it is associated with a high mortality rate (4).”
Ballas, S. K., & Lusardi, M. (2005) · American Journal of Hematology · doi:10.1002/ajh.20336 · Cited by 284
Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study
Hankins, J. S., Ware, R. E., Rogers, Z. R., Wynn, L. W., Lane, P. A., Scott, J. P., & Wang, W. C. (2005) · Blood · doi:10.1182/blood-2004-12-4973 · Cited by 283
Guidelines for the management of the acute painful crisis in sickle cell disease
Rees, D. C., Olujohungbe, A. D., Parker, N. E., Stephens, A. D., Telfer, P. T., & Wright, J. (2003) · British Journal of Haematology · doi:10.1046/j.1365-2141.2003.04193.x · Cited by 280
“Although the pain itself is not directly lifethreatening, inappropriate treatment leads to unnecessary suffering and potentially fatal complications, related both to the disease and the treatment, and repeated admissions with pain are associated with a higher mortality rate (Platt et al, 1994).”
BackgroundCircumstances of death in adult sickle cell disease patients
Darbari, D. S., Kple-Faget, P., Kwagyan, J., RANA, S. R., Gordeuk, V. R., & Castro, O. L. (2006) · American Journal of Hematology · doi:10.1002/ajh.20685 · Cited by 277
“Table V summarizes and compares the seven leading circumstances of death in our study to three other adult SCD mortality series reported in the literature [2,4,5].”
ResultSelf-citationHydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial
Ferster, A., Vermylen, C., Cornu, G., Buyse, M. E., Corazza, F., Devalck, C., Fondu, P., Toppet, M., & Sariban, E. (1996) · Blood · doi:10.1182/blood.v88.6.1960.bloodjournal8861960 · Cited by 276
Cardiovascular Abnormalities in Sickle Cell Disease
Gladwin, M. T., & Sachdev, V. (2012) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2011.10.900 · Cited by 279
“inhibits hemoglobin S polymerization) (10).”
InfluentialSickle cell vaso-occlusion: multistep and multicellular paradigm
Frenette, P. S. (2002) · Current Opinion in Hematology · doi:10.1097/00062752-200203000-00003 · Cited by 275
Fetal hemoglobin in sickle cell anemia: a glass half full?
Steinberg, M. H., Chui, D. H. K., Dover, G. J., Sebastiani, P., & Alsultan, A. (2014) · Blood · doi:10.1182/blood-2013-09-528067 · Cited by 267
“Different concentrations of HbF were postulated to protect against different complications of disease, although 1 study suggested that any increment in HbF was associated with longevity.(22,25) Only the calculated mean value of HbF/F-cell was reported and only in some of these studies.”
Self-citationDivergent Nitric Oxide Bioavailability in Men and Women With Sickle Cell Disease
Gladwin, M. T., Schechter, A. N., Ognibene, F. P., Coles, W. A., Reiter, C. D., Schenke, W. H., Csákó, G., Waclawiw, M. A., Panza, J. A., & Cannon, R. O. (2003) · Circulation · doi:10.1161/01.cir.0000044943.12533.a8 · Cited by 275
The paradox of hemoglobin SC disease
Nagel, R. L., Fabry, M. E., & Steinberg, M. H. (2003) · Blood Reviews · doi:10.1016/s0268-960x(03)00003-1 · Cited by 270
Self-citation
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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
