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3.
Blood adv. (Online) ; 4(2): [327-355], Jan. 27, 2020.
Artigo em Inglês | BIGG - guias GRADE | ID: biblio-1117242

RESUMO

Red cell transfusions remain a mainstay of therapy for patients with sickle cell disease (SCD), but pose significant clinical challenges. Guidance for specific indications and administration of transfusion, as well as screening, prevention, and management of alloimmunization, delayed hemolytic transfusion reactions (DHTRs), and iron overload may improve outcomes. Our objective was to develop evidence-based guidelines to support patients, clinicians, and other healthcare professionals in their decisions about transfusion support for SCD and the management of transfusion-related complications. The American Society of Hematology formed a multidisciplinary panel that was balanced to minimize bias from conflicts of interest and that included a patient representative. The panel prioritized clinical questions and outcomes. The Mayo Clinic Evidence-Based Practice Research Program supported the guideline development process. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to form recommendations, which were subject to public comment. The panel developed 10 recommendations focused on red cell antigen typing and matching, indications, and mode of administration (simple vs red cell exchange), as well as screening, prevention, and management of alloimmunization, DHTRs, and iron overload. The majority of panel recommendations were conditional due to the paucity of direct, high-certainty evidence for outcomes of interest. Research priorities were identified, including prospective studies to understand the role of serologic vs genotypic red cell matching, the mechanism of HTRs resulting from specific alloantigens to inform therapy, the role and timing of regular transfusions during pregnancy for women, and the optimal treatment of transfusional iron overload in SCD.


Assuntos
Humanos , Transfusão de Sangue/métodos , Técnica de Placa Hemolítica/métodos , Anemia Falciforme/complicações , Anemia Falciforme/prevenção & controle , Anemia Falciforme/sangue
4.
Brasília, DF; Ministério da Saúde; 1; 2020. 51 p. ilus.
Monografia em Português | LILACS, Inca, PIE | ID: biblio-1099590

RESUMO

Essa síntese teve como objetivo levantar opções para prevenção de complicações da doença falciforme, e informar sobre avanços na implementação da política no país. Seguem as opções descritas na síntese que já fazem parte das recomendações do Ministério da Saúde: Promover a antibioticoterapia profilática e a vacinação anti-pneumocócica; Promover o uso de hidroxiureia para prevenção e tratamento de complicações da doença falciforme; Promover o uso de ultrassonografia Doppler Transcraniano (DTC) e transfusão sanguínea para a prevenção primária de acidente vascular cerebral (AVC); Promover a educação em saúde para as pessoas com doença falciforme e seus cuidadores sobre medidas para reduzir as complicações. .


Assuntos
Humanos , Transfusão de Sangue , Ultrassonografia Doppler Transcraniana , Antibioticoprofilaxia , Vacinas Pneumocócicas , Hidroxiureia/uso terapêutico , Anemia Falciforme/complicações , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Educação em Saúde
5.
Rev. Pesqui. (Univ. Fed. Estado Rio J., Online) ; 11(5): 1213-1218, out.-dez. 2019.
Artigo em Inglês, Português | LILACS, BDENF - Enfermagem | ID: biblio-1022326

RESUMO

Objetivo: Descrever a tipologia do cuidado realizado pelo familiar à criança com doença falciforme segundo Colliére. Método: Estudo qualitativo, descritivo, desenvolvido com familiares de crianças com doença falciforme, em um hospital geral de Vitória por meio da entrevista semiestruturada. Os dados foram interpretados à luz do Referencial Teórico de Collière e submetidos à Análise Temática. Resultados: a família se deparada com profissionais despreparados no momento da descoberta da doença. Quanto aos cuidados, houve predomínio dos cuidados de manutenção relacionados ao momento do diagnostico, brincadeiras, medicação diária, hidratação corporal, alimentação, eliminação e roupas. Já os reparadores ficaram restritos a situações emergenciais. Conclusão: é necessário capacitar os profissionais que atendem as crianças com doença falciforme para melhor satisfação das suas necessidades e de sua família


Objective: This work aims to describe the type of care provided by relatives for children with sickle cell anemia according to Collière. Methods: It is descriptive study with a qualitative approach, which was carried out with relatives of children bearing sickle cell anemia in a general hospital in Vitória city, Espírito Santo State, Brazil, by using semi-structured interviews. Data were interpreted in light of the Collière's Theoretical Reference and submitted to Thematic Analysis. Results: Families had to deal with the health care professionals' lack of skill needed to convey the diagnostic. Also, there was a predominance of the maintenance care related to the time of diagnosis, playing, daily medication, body hydration, diet, elimination, and clothing. Nonetheless, repair care was restricted to emergency situations. Conclusion: It is necessary to train the professionals who care for children with sickle cell anemia to better meet their needs and those of their families


Objetivo: Describir la tipología del cuidado realizado por el familiar al niño con enfermedad falciforme según Colliére. Método: Estudio cualitativo, descriptivo, desarrollado con familiares de niños con enfermedad falciforme, en un hospital general de Vitória por medio de la entrevista semiestructurada. Los datos fueron interpretados a la luz del Referencial Teórico de Collière y sometidos al Análisis Temático. Resultados: la familia se deparó con profesionales despreparados en el momento del descubrimiento de la enfermedad. En cuanto a los cuidados, hubo predominio de los cuidados de mantenimiento relacionados al momento del diagnóstico, bromas, medicación diaria, hidratación corporal, alimentación, eliminación y ropa. Los reparadores quedaron restringidos a situaciones de emergencia. Conclusión: es necesario capacitar a los profesionales que atienden a los niños con enfermedad falciforme para mejor satisfacción de sus necesidades y de su familia


Assuntos
Humanos , Masculino , Feminino , Criança Hospitalizada , Cuidadores/psicologia , Pessoal Técnico de Saúde/educação , Anemia Falciforme/prevenção & controle , Brasil , Família , Capacitação Profissional
6.
Orphanet J Rare Dis ; 14(1): 120, 2019 05 30.
Artigo em Inglês | MEDLINE | ID: mdl-31146777

RESUMO

Sickle cell disease (SCD) is an inherited red blood cell disorder caused by a structural abnormality of hemoglobin called sickle hemoglobin (HbS). Clinical manifestations of SCD are mainly characterized by chronic hemolysis and acute vaso-occlusive crisis, which are responsible for severe acute and chronic organ damage. SCD is widespread in sub-Saharan Africa, in the Middle East, Indian subcontinent, and some Mediterranean regions. With voluntary population migrations, people harboring the HbS gene have spread globally. In 2006, the World Health Organization recognized hemoglobinopathies, including SCD, as a global public health problem and urged national health systems worldwide to design and establish programs for the prevention and management of SCD. Herein we describe the historical experience of the network of hemoglobinopathy centers and their approach to SCD in Italy, a country where hemoglobinopathies have a high prevalence and where SCD, associated with different genotypes including ß-thalassemia, is present in the native population.


Assuntos
Anemia Falciforme/prevenção & controle , Gerenciamento Clínico , Anemia Falciforme/diagnóstico , Anemia Falciforme/metabolismo , Doenças Hematológicas/diagnóstico , Doenças Hematológicas/metabolismo , Doenças Hematológicas/prevenção & controle , Hemoglobinopatias/diagnóstico , Hemoglobinopatias/metabolismo , Hemoglobinopatias/prevenção & controle , Humanos , Hidroxiureia/metabolismo , Itália , Saúde Pública
7.
BMC Med Ethics ; 20(1): 37, 2019 05 29.
Artigo em Inglês | MEDLINE | ID: mdl-31142291

RESUMO

BACKGROUND: Sickle cell anemia (SCA) is a major genetic disease with the greatest burden in sub-Saharan Africa. To try to help reduce this burden, some churches in Nigeria conduct premarital sickle cell hemoglobin screening and refuse to conduct weddings when both individuals are identified as carriers of sickle cell trait. MAIN BODY: This paper explores the ethical challenges involved in such denials. We assess whether churches have the right to decline to marry adults who understand the risks and still prefer to get married, and whether couples should be denied church weddings based on the risk that their child may suffer from sickle cell anemia. We examine the moral and ethical dimensions of such denials and explore the underlying socio-cultural context involving the purpose of marriage and the meaning of the wedding ceremony in societies where premarital screening is one of the few tools available to reduce the risk of having children with SCA. The potential role of the church is also examined against the background of church beliefs, the duty of the church to its members and its role in reducing the suffering of its members and /or their children. CONCLUSION: We argue that the church should impose these burdens on couples only if doing so promotes a sufficiently compelling goal and there is no less burdensome way to achieve it. We then argue that the goal of reducing the number of individuals in Nigeria who have SCA is compelling. However, testing earlier in life offers a less burdensome and potentially even more effective means of achieving this goal. This suggests that, advocating for earlier screening and helping to support these programs, would likely better promote the church's own goals of helping its parishioners, increasing the number of church weddings, and reducing the burden of SCA in Nigeria.


Assuntos
Testes Genéticos/ética , Hemoglobinas/genética , Casamento , Exames Pré-Nupciais/ética , Religião e Medicina , Traço Falciforme/genética , Adolescente , Adulto , Anemia Falciforme/genética , Anemia Falciforme/prevenção & controle , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nigéria , Adulto Jovem
8.
Bioethics ; 33(6): 661-668, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31107563

RESUMO

CRISPR/Cas9 is quickly becoming one of the most influential biotechnologies of the last five years. Clinical trials will soon be underway to test whether CRISPR/Cas9 can edit away the genetic mutations that cause sickle cell disease (SCD). This article will present the background of CRISPR/Cas9 gene editing and SCD, highlighting research that supports the application of CRISPR/Cas9 to SCD. While much has been written on why SCD is a good biological candidate for CRISPR/Cas9, less has been written on the ethical implications of including SCD in CRISPR/Cas9 research. This article will argue that there is a strong case in favor of including SCD. Three benefits are achieving distributive justice in research, continuing to repair the negative relationship between patients with SCD and the health-care system, and benefit-sharing for those who do not directly participate in CRISPR/Cas9 research. Opponents will argue that SCD is a risky candidate, that researchers will not find willing participants, and that the burden of SCD is low. Of this set of arguments, the first gives pause. However, on balance, the case in favor of including SCD in CRISPR/Cas9 research is stronger than the case against. Ultimately, this article will show that the historic and sociopolitical injustices that impede progress in treating and curing SCD can be alleviated through biotechnology.


Assuntos
Anemia Falciforme/prevenção & controle , Proteína 9 Associada à CRISPR/genética , Sistemas CRISPR-Cas , Edição de Genes , Justiça Social , Anemia Falciforme/terapia , Pesquisa Biomédica , Grupos Étnicos , Terapia Genética , Humanos
9.
MEDICC Rev ; 21(4): 34-38, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-32335567

RESUMO

Sickle cell anemia is the most common hereditary disease in Cuba. On average, 1 in 33 Cubans is a carrier of this severe hemolytic anemia that can cause early death. In early 1980, its incidence in Cuba was calculated at 1 in 1600 births. In 1982, the Cuban public health system established the Sickle Cell Anemia Prevention Program, which aims to prevent the disease through identification of carrier couples and antenatal diagnosis of fetuses with disease-associated genotypes. In 1982-2018, hemoglobin genotypes were tested in 4,847,239 pregnant women. Of these, 168,865 (3.5%) were found to be carriers or to have sickle cell disease. During the same period, 8180 at-risk couples were identified, of whom 79.2% agreed to an antenatal study for detection of the sickle cell gene in the fetus. Among fetuses diagnosed, 20.1% had the SS genotype, the most clinically severe; 76.2% of the associated couples decided to interrupt the pregnancy. This program has resulted in a 3-fold reduction in prevalence of sickle cell disease in Cuba, a 10-fold reduction in the number of infants born with it each year, and a 16-year average increase in life expectancy of sickle cell disease patients of both sexes. Key contributors to these results have been universal screening of pregnant women in primary care, installation of diagnostic laboratories in every province, genetic counseling for couples, testing of fetal DNA (allowing couples to decide whether to continue the pregnancy if the fetus tests positive for the disease) and guaranteed multidisciplinary clinical care for patients. The Cuban experience shows that a middle-income country can mitigate the impact of a genetic disease through a universal preventive program based in primary care, which also pays particular attention to afflicted patients. KEYWORDS Sickle cell anemia, sickle cell disease, sickle cell disorders, hemolytic anemia, sickle cell trait, sickle cell hemoglobin C disease, HbS disease, prevention, antenatal screening, preventive health services, Cuba.


Assuntos
Anemia Falciforme/epidemiologia , Anemia Falciforme/prevenção & controle , Anemia Falciforme/genética , Pesquisa Biomédica , Região do Caribe , Cuba/epidemiologia , Feminino , Aconselhamento Genético , História do Século XX , História do Século XXI , Humanos , Programas de Rastreamento/tendências , Gravidez , Diagnóstico Pré-Natal/tendências
10.
Hemoglobin ; 42(4): 257-263, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30501529

RESUMO

Hemoglobinopathies constitute the most frequent monogenic disorders worldwide and in Greece. In Greece, carrier frequency is estimated at about 8.0%, resulting in a heavy disease burden in the past. Therefore, the implementation of a national prevention program of the disease was an urgent necessity. Moreover, due to migration flow from different geographic areas in the last two decades, the observed spectrum of underlying mutations was expanded, leading to the adaptation of diagnostic approaches. We report the results of the National Thalassaemia Prevention Programme in Northern Greece, over a 15-year period (2001-2015). In total 33,837 healthy at-risk individuals (individuals or couples, 91.0% Greeks) were screened. We have screened 1598 pregnancies in 371 (23.0%) (10.0% non Greeks), of whom both parents carried gene defects and were offered genetic counseling. Seventy-six fetuses (23.0%) were predicted to be affected by severe forms of the disease. Following informed parental choices, 73 of the above pregnancies were terminated. Meanwhile, within the study period, 58 new thalassemic babies (five non Greeks) were referred to the Thalassaemia and Sickle Cell Disease Care Unit of Northern Greece, reflecting mostly parental unawareness, choice or the program failure. Based on the region's population, the birth rate and the prevalence of the disease, the anticipated number of new cases is about 45 annually. According to our data, four thalassemic newborns were registered annually at a stable rate in the last 15 years, reaching a reduction of 90.0% of new affected births. Overall, the National Thalassaemia Prevention Programme effectively decreased the incidence of affected newborns in our region.


Assuntos
Anemia Falciforme/prevenção & controle , Programas de Triagem Diagnóstica , Aconselhamento Genético/normas , Avaliação de Programas e Projetos de Saúde , Talassemia/prevenção & controle , Anemia Falciforme/diagnóstico , Anemia Falciforme/genética , Feminino , Triagem de Portadores Genéticos , Grécia , Humanos , Recém-Nascido , Masculino , Gravidez , Diagnóstico Pré-Natal , Talassemia/diagnóstico , Talassemia/genética , Migrantes
13.
Curr Opin Hematol ; 25(6): 459-467, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30124474

RESUMO

PURPOSE OF REVIEW: The aim of this study was to summarize the basic epidemiology, pathophysiology and management of delayed serologic and delayed haemolytic transfusion reactions (DHTRs), as well as recent developments in our understanding of these adverse events. RECENT FINDINGS: Several studies have identified risk factors for DHTRs, including high alloantibody evanescence rates among both general patient groups and those with sickle cell disease (SCD). Antibody detection is also hampered by the phenomenon of transfusion record fragmentation. There have also been enhancements in understanding of what may contribute to the more severe, hyperhaemolytic nature of DHTRs in SCD, including data regarding 'suicidal red blood cell death' and immune dysregulation amongst transfusion recipients with SCD. With growing recognition and study of hyperhaemolytic DHTRs, there have been improvements in management strategies for this entity, including a multitude of reports on using novel immunosuppressive agents for preventing or treating such reactions. SUMMARY: Delayed serologic and haemolytic reactions remain important and highly relevant transfusion-associated adverse events. Future directions include further unravelling the basic mechanisms, which underlie DHTRs and developing evidence-based approaches for treating these reactions. Implementing practical preventive strategies is also a priority.


Assuntos
Anemia Falciforme , Transfusão de Sangue , Hemólise , Imunossupressores/uso terapêutico , Anemia Falciforme/fisiopatologia , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Humanos
14.
Soc Work Public Health ; 33(5): 299-316, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29768104

RESUMO

Sickle cell disease (SCD) is a widespread inherited blood disorder, which leaves lasting effects on the health, social functioning, and finances of individuals, families, communities, and health care systems. A nonexperimental, cross-sectional research design was used to assess 415 college students' knowledge about SCD. Data was obtained through an online survey derived from a modified version of the SCD Knowledge Assessment Tool. The majority of participants (79%) reported previous SCD knowledge; however, 21% of the participants reported no previous SCD knowledge. Results support the need for improved education and awareness for at risk groups. The lack of SCD knowledge among African Americans shows a need for improved, nongendered specific education, awareness, and screening efforts geared toward at-risk populations.


Assuntos
Anemia Falciforme/prevenção & controle , Conhecimentos, Atitudes e Prática em Saúde , Estudantes/psicologia , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Inquéritos e Questionários
15.
Hosp Pract (1995) ; 46(3): 144-151, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29648482

RESUMO

Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay. The main goal is to prevent and treat acute respiratory failure and, thus, minimize irreversible lung damage. This review focuses on the risk factors, pathogenesis, clinical presentation, and management of ACS.


Assuntos
Síndrome Torácica Aguda/etiologia , Anemia Falciforme/complicações , Síndrome Torácica Aguda/prevenção & controle , Síndrome Torácica Aguda/terapia , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Feminino , Humanos , Masculino
16.
PLoS One ; 13(4): e0196455, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29694434

RESUMO

During hemolysis, hemoglobin and heme released from red blood cells promote oxidative stress, inflammation and thrombosis. Plasma haptoglobin and hemopexin scavenge free hemoglobin and heme, respectively, but can be depleted in hemolytic states. Haptoglobin and hemopexin supplementation protect tissues, including the vasculature, liver and kidneys. It is widely assumed that these protective effects are due primarily to hemoglobin and heme clearance from the vasculature. However, this simple assumption does not account for the consequent cytoprotective adaptation seen in cells and organs. To further address the mechanism, we used a hyperhemolytic murine model (Townes-SS) of sickle cell disease to examine cellular responses to haptoglobin and hemopexin supplementation. A single infusion of haptoglobin or hemopexin (± equimolar hemoglobin) in SS-mice increased heme oxygenase-1 (HO-1) in the liver, kidney and skin several fold within 1 hour and decreased nuclear NF-ĸB phospho-p65, and vaso-occlusion for 48 hours after infusion. Plasma hemoglobin and heme levels were not significantly changed 1 hour after infusion of haptoglobin or hemopexin. Haptoglobin and hemopexin also inhibited hypoxia/reoxygenation and lipopolysaccharide-induced vaso-occlusion in SS-mice. Inhibition of HO-1 activity with tin protoporphyrin blocked the protections afforded by haptoglobin and hemopexin in SS-mice. The HO-1 reaction product carbon monoxide, fully restored the protection, in part by inhibiting Weibel-Palade body mobilization of P-selectin and von Willebrand factor to endothelial cell surfaces. Thus, the mechanism by which haptoglobin and hemopexin supplementation in hyperhemolytic SS-mice induces cytoprotective cellular responses is linked to increased HO-1 activity.


Assuntos
Anemia Falciforme/prevenção & controle , Haptoglobinas/uso terapêutico , Heme Oxigenase-1/metabolismo , Hemopexina/uso terapêutico , Inflamação/prevenção & controle , Aldeídos/análise , Anemia Falciforme/patologia , Animais , Monóxido de Carbono/farmacologia , Citocinas/análise , Modelos Animais de Doenças , Feminino , Expressão Gênica/efeitos dos fármacos , Haptoglobinas/farmacologia , Hemopexina/farmacologia , Molécula 1 de Adesão Intercelular , Masculino , Metaloporfirinas/farmacologia , Camundongos , Microssomos Hepáticos/metabolismo , Protoporfirinas/farmacologia , Pele/metabolismo , Pele/patologia , Fator de Transcrição RelA/metabolismo , Molécula 1 de Adesão de Célula Vascular/metabolismo
17.
Sultan Qaboos Univ Med J ; 18(1): e24-e29, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29666677

RESUMO

Due to the high rate of consanguineous marriages in Oman, there is a correspondingly high prevalence of hereditary blood disorders, particularly sickle cell disease and ß-thalassaemia. This article proposes the possibility of implementing mandatory premarital carrier screening for blood disorders in Oman, while giving due consideration to potential social and cultural obstacles. Although the implementation of such legislation would require collaboration between different sectors and may negatively affect the autonomy of certain individuals, mandatory premarital screening would help to alleviate the burden of hereditary blood disorders on the national healthcare system, as well as reduce avoidable suffering among carriers and their families.


Assuntos
Programas de Rastreamento/métodos , Exames Pré-Nupciais/métodos , Anemia Falciforme/genética , Anemia Falciforme/prevenção & controle , Consanguinidade , Testes Genéticos/métodos , Humanos , Programas de Rastreamento/tendências , Omã , Exames Pré-Nupciais/tendências , Prevalência , Talassemia beta/genética , Talassemia beta/prevenção & controle
18.
Int J Immunogenet ; 44(5): 219-224, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28815969

RESUMO

Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) molecule is expressed on T-lymphocyte membrane and negatively influences the antigen-presenting process. Reduced expression of CTLA-4 due to gene polymorphisms is associated with increased risk of autoimmune disorders, whose physiopathology is similar to that of post-transfusion red blood cell (RBC) alloimmunization. Our goal was to evaluate if polymorphisms of CTLA-4 gene that affect protein expression are associated with RBC alloimmunization. This was a case-control study in which 134 sickle cell disease (SCD) patients and 253 non-SCD patients were included. All patients were genotyped for the polymorphisms 49A/G and -318C/T of CTLA-4 gene. The genotype frequency of -318C/T differed significantly between alloimmunized and nonalloimmunized SCD patients, irrespective of clinical confounders (p = .016). SCD patients heterozygous for -318T allele presented higher risk of alloantibody development (OR: 5.4, CI: 1.15-25.6). In conclusion, the polymorphism -318C/T of CTLA-4 gene is associated with RBC alloimmunization among SCD patients. This highlights the role played by CTLA-4 on post-transfusion alloantibody development.


Assuntos
Anemia Falciforme/genética , Doenças Autoimunes/genética , Antígeno CTLA-4/genética , Eritrócitos/imunologia , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/imunologia , Anemia Falciforme/prevenção & controle , Células Apresentadoras de Antígenos/imunologia , Células Apresentadoras de Antígenos/patologia , Doenças Autoimunes/sangue , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Antígeno CTLA-4/imunologia , Eritrócitos/patologia , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Imunização/efeitos adversos , Isoanticorpos/imunologia , Masculino , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo Único/genética , Fatores de Risco , Linfócitos T/imunologia , Linfócitos T/patologia
19.
Pediatr Blood Cancer ; 64(11)2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28556441

RESUMO

Sickle cell disease (SCD) results in end organ damage and a shortened lifespan. Both the pathophysiology of the disease and the social determinants of health affect patient outcomes. Randomized controlled trials have been completed among this population and resulted in medical advances; however, the gestation of these advances and the lack of penetrance into clinical practice have limited advancements in clinical improvements for many people with SCD. We discuss the role of implementation science in SCD and highlight the need for this science to shorten the length of time to implement evidence-based care for more people with SCD.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/prevenção & controle , Antidrepanocíticos/uso terapêutico , Humanos , Prognóstico
20.
Recurso na Internet em Português | LIS - Localizador de Informação em Saúde, LIS-bvsms | ID: lis-45233

RESUMO

Página da Federação Nacional das Associações de Pessoas com Doença Falciforme (FENAFAL) com informações e orientações para pessoas portadoras da doença.


Assuntos
Anemia Falciforme/prevenção & controle , Doenças Hematológicas
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