Risk factors for mortality in patients with sickle cell disease: an integrative review
Factores de riesgo para la mortalidad en pacientes con enfermedad falciforme: una revisión integradora
Fatores de risco para mortalidade em pacientes com doença falciforme: uma revisão integrativa
Carolina Mariano Pompeo; Andreia Insabralde de Queiroz Cardoso; Mercy da Costa Souza; Mayara Bontempo Ferraz; Marcos Antonio Ferreira Júnior; Maria Lúcia Ivo
Abstract
Objective: To summarize the risk factors and mortality indicators in sickle cell disease patients.
Method: Integrative review searched publications in journals in CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO, and Web of Science databases. The guiding question was elaborated through the Population, Variable, Outcome (PVO) strategy and the search was from October-to-November 2018, at the Coordination of Higher Level Personnel Improvement Periodicals’ Gate. The keywords anemia, sickle cell “and” mortality “and” survival and their synonyms were used.
Results: From 18/19 articles were cohort and one randomized controlled trial. The sample consisted mostly of females and HbSS genotype. The cumulative mortality rate and the overall mortality curve were the most repeated. Seven studies identified risk factors with a statistically significant association with death. The most frequent were low hemoglobin level, liver variables (alkaline phosphatase and oxalacetic glutamic transaminase enzymes) and cardiovascular variables (tricuspid valve regurgitation speed ≥ 2.5m/s).
Conclusion and implications for practice: Mortality indicators are tools for better management of sickle cell disease’s patient, prevention of risks and complications. There is a need for further studies on the factors related to mortality of these patients. Preventing the causes that lead to death will certainly improve the quality of life and survival of this population.
Keywords
Resumen
Palabras clave
Resumo
Objetivo: Sumarizar fatores de risco e indicadores de mortalidade em pacientes com doença falciforme.
Método: Revisão integrativa em periódicos indexados nas bases de dados CINAHL, PubMed/MEDLINE, Science Direct/SCOPUS, SciELO e Web of Science. A questão norteadora foi elaborada por meio da estratégia Population, variable, outcome (PVO). A busca ocorreu no portal de periódicos da Coordenação de Aperfeiçoamento de Pessoal de Nível Superior entre outubro e novembro de 2018.
Resultados: Dos 19 artigos, 18 eram coorte e um ensaio clínico randomizado. A amostra foi constituída, em sua maioria, pelo sexo feminino e genótipo HbSS. Se repetiram mais a taxa de mortalidade cumulativa e a curva de mortalidade global. Sete estudos identificaram fatores de risco com associação estatisticamente significativa para morte. Os mais frequentes foram o baixo nível de hemoglobina, variáveis hepáticas (enzimas fosfatase alcalina e transaminase glutâmico oxalacética) e cardiovasculares (velocidade de regurgitação da válvula tricúspide ≥ 2,5m/s).
Conclusão e implicações para a prática: Indicadores de mortalidade constituem ferramentas de manejo de pacientes com doença falciforme e prevenção de riscos e complicações. Há necessidade de estudos sobre os fatores relacionados à mortalidade desses pacientes. A prevenção do óbito, certamente, promoverá uma melhoria na qualidade de vida e na sobrevida dessa população.
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Referencias
1 Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-73.
2 Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-23.
3 Ministério da Saúde (BR), Departamento de Informática do SUS [Internet]. 2016 [citado 2018 jul 19]. Disponível em:
4 Arduini GA, Rodrigues LP, Trovo de Marqui AB. Mortality by sickle cell disease in Brazil. Rev Bras Hematol Hemoter. 2017;39(1):52-6.
5 Hopia H, Latvala E, Liimatainen L. Reviewing the methodology of an integrative review. Scand J Caring Sci. 2016;30(4):662-9.
6 Whittemore R, Chao A, Jang M, Minges KE, Park C. Methods for knowledge synthesis: an overview. Heart Lung. 2014;43(5):453-61.
7 Galvão TF, Pereira MG. Revisões sistemáticas da literatura: passos para sua elaboração. Epidemiol Serv Saude. 2014;23(1):183-4.
8 Andrade da Silva G, Otta E. Revisão sistemática e meta-análise de estudos observacionais em Psicologia. Rev Costarric Psicol [Internet]. 2014; [citado 2019 jan 2];33(2):137-53. Disponível em:
9 Almeida Fo N, Barreto ML. Desenhos de pesquisa em epidemiologia. In: Almeida Fo N, Barreto ML, organizadores. Epidemiologia e saúde: fundamentos, métodos e aplicações. Rio de Janeiro: Guanabara Koogan; 2011. p. 165-74.
10 Telfer P, Coen P, Chakravorty S, Wilkey O, Evans J, Newell H et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica. 2007;92(7):905-12.
11 Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003;289(13):1645-51.
12 Mehari A, Alam S, Tian X, Cuttica MJ, Barnett CF, Miles G et al. Hemodynamic predictors of mortality in adults with sickle cell disease. Am J Respir Crit Care Med. 2013;187(8):840-7.
13 McClellan AC, Luthi JC, Lynch JR, Soucie JM, Kulkarni R, Guasch A et al. High one year mortality in adults with sickle cell disease and end‐stage renal disease. Br J Haematol. 2012;159(3):360-7.
14 King LG, Bortolusso-Ali S, Cunningham-Myrie CA, Reid ME. Impact of a comprehensive sickle cell center on early childhood mortality in a developing country: the Jamaican experience. J Pediatr. 2015;167(3):702-5.e1. PMid:26163082.
15 Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 2010;115(17):3447-52.
16 Van Beers EJ, Yang Y, Raghavachari N, Tian X, Allen DT, Nichols JS et al. Iron, inflammation, and early death in adults with sickle cell disease. Circ Res. 2015;116(2):298-306.
17 Shankar SM, Arbogast PG, Mitchel E, Cooper WO, Wang WC, Griffin MR. Medical care utilization and mortality in sickle cell disease: a population‐based study. Am J Hematol. 2005;80(4):262-70.
18 Van der Plas EM, Van den Tweel XW, Geskus RB, Heijboer H, Biemond BJ, Peters M et al. Mortality and causes of death in children with sickle cell disease in the Netherlands, before the introduction of neonatal screening. Br J Haematol. 2011;155(1):106-10.
19 Makani J, Cox SE, Soka D, Komba AN, Oruo J, Mwamtemi H et al. Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PLoS One. 2011;6(2):e14699.
20 Knight-Madden JM, Barton-Gooden A, Weaver SR, Reid M, Greenough A. Mortality, asthma, smoking and acute chest syndrome in young adults with sickle cell disease. Lung. 2013;191(1):95-100.
21 Cox SE, Makani J, Fulford AJ, Komba AN, Soka D, Williams TN et al. Nutritional status, hospitalization and mortality among patients with sickle cell anemia in Tanzania. Haematol. 2011;96(7):948-53.
22 Akinyanju O, Otaigbe A, Ibidapo M. Outcome of holistic care in Nigerian patients with sickle cell anaemia. Clin Lab Haematol. 2005;27(3):195-9.
23 Mekontso Dessap A, Leon R, Habibi A, Nzouakou R, Roudot-Thoraval F, Adnot S et al. Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2008;177(6):646-53.
24 Meier ER, Wright EC, Miller JL. Reticulocytosis and anemia are associated with an increased risk of death and stroke in the newborn cohort of the Cooperative Study of Sickle Cell Disease. Am J Hematol. 2014;89(9):904-6.
25 Wierenga KJ, Hambleton IR, Lewis NA, Unit SC. Survival estimates for patients with homozygous sickle-cell disease in Jamaica: a clinic-based population study. Lancet. 2001;357(9257):680-3.
26 Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 2004;103(11):4023-7.
27 Zimbarra Cabrita I, Mohammed A, Layton M, Ghorashian S, Gilmore A, Cho G et al. The association between tricuspid regurgitation velocity and 5‐year survival in a North West London population of patients with sickle cell disease in the United Kingdom. Br J Haematol. 2013;162(3):400-8.
28 Steinberg MH, McCarthy WF, Castro O, Ballas SK, Armstrong FD, Smith W et al. The risks and benefits of long‐term use of hydroxyurea in sickle cell anemia: a 17.5 year follow‐up. Am J Hematol. 2010;85(6):403-8.
29 Sant’Ana PG, Araujo AM, Pimenta CT, Bezerra ML, Borges Jr SP, Martins No VM et al. Clinical and laboratory profile of patients with sickle cell anemia. Rev Bras Hematol Hemoter. 2017;39(1):40-5.
30 Araujo OMR, Ivo ML, Ferreira Jr MA, Pontes ERJC, Bispo IMGP, Oliveira ECL. Survival and mortality among users and non-users of hydroxyurea with sickle cell disease. Rev Lat Am Enfermagem. 2015;23(1):67-73.
31 Streetly A, Sisodia R, Dick M, Latinovic R, Hounsell K, Dormandy E. Evaluation of newborn sickle cell screening programme in England: 2010-2016. Arch Dis Child. 2018;103(7):648-53. PMid:29104181.
32 Ramos JT, de Amorim FS, Pedroso FKF, Nunes ACC, Rios MA. Mortalidade por doença falciforme em estado do nordeste brasileiro. Rev Enferm Cent-Oeste Min. 2015;5(2):1604-12.
33 Ministério da Saúde (BR). Triagem neonatal biológica: manual técnico [Internet]. Brasilia: Ministério da Saúde; 2016 [citado 2019 jan 2]. Disponível em:
34 Maitra P, Caughey M, Robinson L, Desai PC, Jones S, Nouraie M et al. Risk factors for mortality in adult patients with sickle cell disease: a meta-analysis of studies in North America and Europe. Haematol. 2017;102(4):626-36.
35 Gladwin MT, Barst RJ, Gibbs JSR, Hildesheim M, Sachdev V, Nouraie M et al. Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom. PLoS One. 2014;9(7):e99489.
36 Kassim AA, Payne AB, Rodeghier M, Macklin EA, Strunk RC, DeBaun MR. Forced expiratory volume in 1 second is associated with earlier death in sickle cell anemia. Blood. 2015;126(13):1544-50.
37 Gladwin MT. Cardiovascular complications in patients with sickle cell disease. Hematology. 2017;2017(1):423-30.
38 Lobo CLC, Nascimento EM, Jesus LJC, Freitas TG, Lugon JR, Ballas SK. Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. Hematol Transfus Cell Ther. 2018;40(1):37-42.
39 Hamideh D, Alvarez O. Sickle cell disease related mortality in the United States (1999-2009). Pediatr Blood Cancer. 2013;60(9):1482-6.
40 Gardner K, Douiri A, Drasar E, Allman M, Mwirigi A, Awogbade M et al. Survival in adults with sickle cell disease in a high-income setting. Blood. 2016;128(10):1436-8.
41 Chaturvedi S, Labib Ghafuri D, Kassim A, Rodeghier M, DeBaun MR. Elevated tricuspid regurgitant jet velocity, reduced forced expiratory volume in 1 second, and mortality in adults with sickle cell disease. Am J Hematol. 2017;92(2):125-30.
42 Costa FF, Conran N, Fertrin KY. Anemia falciforme. In: Zago MA, Falcão RP, Pasquini R, organizadores. Tratado de hematologia. São Paulo: Atheneu; 2013. p. 205-23.
43 Nevitt SJ, Jones AP, Howard J. Hydroxyurea (hydroxycarbamide) for sickle cell disease. Cochrane Database Syst Rev. 2017;(4).
44 Serjeant GR, Chin N, Asnani MR, Serjeant BE, Mason KP, Hambleton IR et al. Causes of death and early life determinants of survival in homozygous sickle cell disease: the Jamaican cohort study from birth. PLoS One. 2018;13(3):e0192710.
45 Elmariah H, Garrett ME, Castro LM, Jonassaint JC, Ataga KI, Eckman JR et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. 2014;89(5):530-5.
46 Li X, Du E, Lei H, Tang Y-H, Dao M, Suresh S et al. Patient-specific blood rheology in sickle-cell anaemia. Interface Focus. 2016;6(1):20150065.
47 Karacaoglu PK, Asma S, Korur A, Solmaz S, Buyukkurt NT, Gereklioglu C et al. East Mediterranean region sickle cell disease mortality trial: retrospective multicenter cohort analysis of 735 patients. Ann Hematol. 2016;95(6):993-1000.
48 Curtis SA, Danda N, Etzion Z, Cohen HW, Billett HH. Longitudinal analysis of patient specific predictors for mortality in sickle cell disease. PLoS One. 2016;11(10):e0164743.
49 Howard J. Sickle cell disease: when and how to transfuse. ASH Education Program Book [Internet]. 2016;2016(1):625-31.
50 Carden MA, Fasano RM, Meier ER. Not all red cells sickle the same: contributions of the reticulocyte to disease pathology in sickle cell anemia. Blood Rev. 2019;100637.
51 Feld JJ, Kato GJ, Koh C, Shields T, Hildesheim M, Kleiner DE et al. Liver injury is associated with mortality in sickle cell disease. Aliment Pharmacol Ther. 2015;42(7):912-21.
52 Darbari DS, Wang Z, Kwak M, Hildesheim M, Nichols J, Allen D et al. Severe painful vaso-occlusive crises and mortality in a contemporary adult sickle cell anemia cohort study. PLoS One. 2013;8(11):e79923.
53 Nouraie M, Lee JS, Zhang Y, Kanias T, Zhao X, Xiong Z et al. The relationship between the severity of hemolysis, clinical manifestations and risk of death in 415 patients with sickle cell anemia in the US and Europe. Haematologica. 2013;98(3):464-72.
54 Shah R, Taborda C, Chawla S. Acute and chronic hepatobiliary manifestations of sickle cell disease: a review. World J Gastrointest Pathophysiol. 2017;8(3):108-16.
55 Kato GJ, Steinberg MH, Gladwin MT. Intravascular hemolysis and the pathophysiology of sickle cell disease. J Clin Invest. 2017;127(3):750-60.
56 Gladwin MT. Cardiovascular complications and risk of death in sickle-cell disease. Lancet. 2016;387(10037):2565-74.
57 Cohen RT, Madadi A, Blinder MA, DeBaun MR, Strunk RC, Field JJ. Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. Am J Hematol. 2011;86(9):756-61.
Submitted date:
03/07/2019
Accepted date:
20/12/2019