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Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients [ABSTRACT FROM AUTHOR]

Objective: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). Methods: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. Results: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R²=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R²=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R²=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R²=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio — HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). Conclusions: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death. [ABSTRACT FROM AUTHOR]

Na doença falciforme, o genótipo HbSS da anemia falciforme, considerado a forma mais grave, prevalente no continente africano, tem alta morbidade e mortalidade. Objetivo: identificar se a estrutura instalada na atenção às pessoas com anemia falciforme e seus familiares, contribui para a redução da mortalidade precoce em Angola. Material e Método: pesquisa exploratória descritiva, realizada em Luanda-Angola, no período entre agosto e dezembro de 2022. Foram aplicados questionários estruturados para 20 pessoas com doença falciforme e 15 familiares, todos com mais de 21 anos. A metodologia utilizada foi bola de neve. Para análise e discussão dos dados, foi utilizado o referencial teórico de Donabedian, com ênfase no eixo estrutura. Resultados: diagnóstico feito por sinais e sintomas, idade do diagnóstico entre 1 e 5 anos. A crise de dor predominou entre eventos agudos. Conclusão: inexistência de estrutura instalada de saúde em Luanda que garantam no serviço público gratuito o acesso à medicamentos, transfusão de sangue, vacinas especiais e exames complementares. A transferência de tecnologias sociais da organização de sociedade civil brasileira para a de Luanda mostra ser adequada.

Copyright of Odeere is the property of Edicoes UESB and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: The aim of this study was to identify clinical and complete blood count differences between pediatric hospitalized patients with sickle cell disease infected or not by SARS-CoV-2 and compare the complete blood count of patients with sickle cell disease infected by SARS-CoV-2 before hospitalization and on admission. Methods: This study was a single-center prospective cohort. Data were collected from medical records of pediatric inpatients with sickle cell disease under 18 years old infected or not with SARS-CoV-2 from the first visit to the hospital until discharge and from the last medical appointment. All patients were tested for SARS-CoV-2 by the real-time reverse transcription polymerase chain reaction. Results: Among 57 pediatric patients with sickle cell disease hospitalized from March to November 2020 in a Brazilian academic hospital, 11 (19.3%) had a positive result for SARSCoV-2. Patients infected by SARS-CoV-2 had a higher prevalence of comorbidities than the ones who were not infected (63.6 vs. 30.4%; p=0.046). During hospital stay, no clinical or complete blood count differences between groups were found. There was a decrease in eosinophil count on hospital admission in patients with sickle cell disease infected by SARS-CoV-2 (p=0.008). Conclusions: Pediatric hospitalized patients with sickle cell disease infected by SARS-CoV-2 had more comorbidities and had a decrease in eosinophil count between hospital admission and the last medical appointment. [ABSTRACT FROM AUTHOR]

Copyright of Aequitas: Journal of Human Development, Disability & Social Change / Revue de Développement Humain, Handicap et Changement Social is the property of Reseau international sur le Processus de production du handicap, RIPPH and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Scire Salutis is the property of CBPC - Companhia Brasileira de Producao Cientifica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: To systematically establish whether there is an association between polymorphisms and avascular necrosis in patients with sickle cell disease. Data source: The review, conducted according to PRISMA guidelines and registered with PROSPERO, was based on research of studies in PubMed, SciELO, LILACS, BVS databases and in the gray literature (Google Scholar and Open Gray) published until June 2020. The STROBE initiative was used to analyze the articles' quality. Data synthesis: Ten articles were selected from the databases and two were included through manual search, totaling 12 studies. All samples gathered 2,362 patients. According to STROBE, seven studies fully and/or partially covered more than 70% of the essential items and two studies reached less than 60%, with an overall variation of 86.4-54.5%. The results indicate that polymorphisms in the genes of the bone morphogenetic protein 6 (BMP6), Klotho (KL) and Annexin A2 (ANXA2) may be associated with osteonecrosis in the context of sickle cell disease. Six articles addressed the polymorphism in the MTHFR enzyme gene, but only one found a positive association. Polymorphisms associated with the DARC receptor, the ITGA4 gene, CD36 and thrombophilia protein genes were not associated in any of the studies. Conclusions: The results indicate that the polymorphisms in BMP6, Klotho and ANXA2 genes may be associated with avascular necrosis in patients with sickle cell disease. However, in order to confirm these genetic changes as risk factors, further studies with greater statistical power and methodological rigor are needed. [ABSTRACT FROM AUTHOR]

Copyright of Minas Gerais Medical Journal / Revista Médica de Minas Gerais is the property of Associacao Medica de Minas Gerais and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Texto & Contexto Enfermagem is the property of Universidade Federal de Santa Catarina, Programa de Pos-Graduacao de Enfermagem and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Texto & Contexto Enfermagem is the property of Universidade Federal de Santa Catarina, Programa de Pos-Graduacao de Enfermagem and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article reports a case study on Bioanthropology studies in a hematological health context, indicating the main challenges and perspectives of fieldwork during the Masters/Doctorate courses with a concentration in Bioanthropology (Federal University of Pará/UFPA). We describe the step by step of immersion in the field in a blood center in Belém/PA in contact with people with sickle cell disease, presenting the Bioanthropology metier with the different ethical/bioethical paths of the area in question. We conducted qualitative health research with 60 people diagnosed with the most severe form of Sickle Cell Disease, Sickle Cell Anemia (SCA or Hb SS), with a total of 120 interviews in two phases via the Informed Consent Form (ICF) in order to collect data on genetic ancestry with autosomal DNA testing, ethnic-racial identity and socioeconomic aspects, using the technique of participant observation and extensive bibliographic review. The results showed that the researched group has a higher frequency of European DNA (58.2%), faces obstacles linked to poverty and racism and that the bioanthropological analysis is interdisciplinary in the humanities and related sciences, largely exercising the ethical / bioethical character of the research socio-cultural context in the Amazon. [ABSTRACT FROM AUTHOR]

Este estudo utilizou o método de análise de conteúdo para investigar a abordagem sobre a doença falciforme (DF) em livros didáticos (LDs) de Biologia do ensino médio publicados no Brasil. Os resultados mostraram a necessidade de reavaliar informações disponibilizadas pelos LDs para evitar estigmatização e a perpetuação de conceitos que interferem no patrimônio cultural dessas pessoas. Apesar da frequência elevada do alelo que codifica a hemoglobina S em todo o Brasil, dados sobre a epidemiologia desta mutação não foram encontrados nos livros. Além disso, conceitos sobre o autocuidado, a qualidade e a expectativa de vida das pessoas com DF precisam ser revisitados. Uma vez que os LDs constituem um dos meios de disseminação de informações mais utilizados pelos estudantes nas escolas públicas brasileiras, a atualização das informações fornecidas torna-se necessária à luz de novos conhecimentos que têm impactos no entendimento da DF e na percepção das pessoas a seu respeito, tanto aquelas que vivem quanto as que não vivem com DF.

A reconstituição do itinerário terapêutico é uma tecnologia utilizada na prática da pesquisa qualitativa. Este estudo buscou analisar a vulnerabilidade social de pessoas adultas com doença falciforme (DF), por meio da reconstrução de itinerários terapêuticos. Trata-se de uma enfermidade hereditária, crônica, de alta prevalência no Brasil, que pode causar uma série de complicações ao longo da vida do indivíduo acometido. Na pesquisa foram entrevistados sete adultos (cinco mulheres e dois homens), com doença falciforme. Os participantes, selecionados de forma intencional, apresentaram em comum um histórico clínico e laboratorial de DF do tipo grave (SS). O material foi submetido à análise de conteúdo, na modalidade temática, utilizando as categorias indicadas previamente: (a) Renda, trabalho e escolaridade; (b) Interações familiar, social e amorosa; (c) Associação de pacientes e Internet. Os resultados da pesquisa demonstraram que a reconstituição de itinerários terapêuticos possibilita a compreensão dos variados modos da vulnerabilidade social na vida dessas pessoas, no estado de Alagoas, apresentando as diferenças e as similaridades nas formas de seu enfrentamento. Observou-se a vulnerabilidade social associada à precária comunicação sobre a doença, no ato da consulta médica, na escola e no trabalho. A inserção desses indivíduos em grupos de convivência, a exemplo das mídias sociais, trouxe benefícios como fonte de conhecimento, bem como canal de comunicação com outras pessoas com diagnóstico semelhante. Portanto, a reconstrução de itinerários terapêuticos mostrou-se uma importante ferramenta para: conhecer e avaliar esta dimensão analítica da vulnerabilidade e sua relação com o autocuidado em saúde; identificar a necessidade de ações mais efetivas visando oportunidades educacional, econômica, social e cultural; e favorecer a afirmação da experiência trazida pelas pessoas com doença falciforme.

Este artigo discute a exclusão social vivida pelos indivíduos brasileiros com a doença falciforme, sob a perspectiva da bioética de intervenção. Trata-se de uma enfermidade na qual o afetado herda de seus progenitores o gene responsável pelo formato diferenciado da hemoglobina; ademais, apresenta maior incidência na população negra, uma vez que a alteração do formato da hemoglobina foi resultado da evolução do próprio corpo para evitar a contração da malária no continente africano. Além da exclusão social que a população negra sofre, há, ainda, um maior grau de preconceito advindo dos efeitos da doença. Revela-se que as dificuldades enfrentadas pelos acometidos são agravadas em decorrência da COVID-19. Portanto, a criação de políticas públicas que se alicercem nos parâmetros da bioética da intervenção, cujo foco principal é a vulnerabilidade e os desafios persistentes da sociedade, é proposta como possível ação para minorar as consequências da doença falciforme na população por ela afetada.

O documentário Meia lua Falciforme retrata diversas faces e enfrentamentos no cotidiano dos pacientes e familiares que convivem com a doença falciforme no cenário brasileiro. A dor, o racismo e a resistência são pontos-chave discutidos no relevante curta-metragem, de Débora Evellyn Olimpo e Denise Kelm, que traz uma perspectiva para além do adoecimento físico e, com leveza, apresenta produções artísticas dos conviventes com a temática. No tocante à resistência e à luta, o documentário cita a grande conquista no que diz respeito ao reconhecimento e à normatização da doença no Brasil, os confltos enfrentados rotineiramente em busca de acesso aos serviços de saúde, bem como a luta por um SUS que garanta os princípios da universalidade e equidade. A presente resenha traz algumas notas sobre o documentário e os temas que perpassam a vida dos pacientes desde o momento do diagnóstico até os itinerários e decisões terapêuticos.

Objective: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics. Methods: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability. Results: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%. Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1. [ABSTRACT FROM AUTHOR]

Copyright of Revista Brasileira em Promoção da Saúde is the property of Revista Brasileira em Promocao da Saude and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista de Pesquisa: Cuidado é Fundamental is the property of Revista de Pesquisa: Cuidado e Fundamental Online and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista de Pesquisa: Cuidado é Fundamental is the property of Revista de Pesquisa: Cuidado e Fundamental Online and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

A Revista Eletrônica de Comunicação, Informação & Inovação em Saúde – Reciis comunica a publicação formal de retratação para extração do artigo: RAMOS, Edith Maria Barbosa; RAMOS, Paulo Roberto Barbosa; CARVALHO, Márcia Haydee Porto de; SILVA, Delmo Mattos da; DUTRA JÚNIOR, Paulo Henrique de Freitas. Portadores da doença falciforme: reflexos da história da população negra no acesso à saúde. Revista Eletrônica de Comunicação, Informação & Inovação em Saúde, Rio de Janeiro, v. 14, n. 3, p. 681-691, 2020. DOI: https://doi.org/10.29397/reciis.v14i3.1182. desde que foram identificadas inconformidades com a política editorial da revista. Christovam Barcellos Igor Sacramento Kizi Mendonça de Araújo Editores científicos Retratação do artigo: https://www.reciis.icict.fiocruz.br/index.php/reciis/article/view/4118/version/2737

O objetivo deste artigo é problematizar a relação entre eugenia e racismo associada às doenças genéticas, especifiamente à doença falciforme, tendo como referência o caso do Programa de Triagem Populacional (PTP), cuja implantação em Salvador, Bahia, não se concretizou devido às mobilizações sociais. Realizou-se uma pesquisa qualitativa, ancorada na perspectiva feminista e antirracista, que aliou a análise documental da proposta do programa e entrevistas em profundidade com mulheres negras com doença falciforme acerca da percepção sobre o conteúdo do programa. A análise do documento evidenciou conteúdos racistas e eugenistas, relacionados com um discurso de cerceamento do direito reprodutivo de mulheres com este agravo. Entre as mulheres negras entrevistadas, prevaleceu o sentimento de indignação frente à interdição de seu direito reprodutivo. Há necessidade de destituir práticas institucionais que se forjam sob o crivo do racismo institucional e produzem violências sobre os corpos negros.

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.

Copyright of Revista Ciência & Saúde Coletiva is the property of Associacao Brasileira de Pos-Graduacao em Saude Coletiva and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This paper discusses from the perspective of intervention bioethics the social exclusion experienced by Brazilian individuals suffering from sickle cell anemia. It is an inherited disease where the gene responsible for the hemoglobin differential format is generationally passed on. It has more incidence in the afro-descendant population than any other, given that this hemoglobin format alteration was an evolutionary response to prevent malaria in the African continent. Besides the social exclusion that this population suffers, there is an even greater degree of discrimination resulting from the disease's effects. It has been recently discovered that COVID-19 has worsened the difficulties faced by patients with this disease. Therefore, drafting intervention-bioethics-based public policies whose main focus relies on vulnerability and the persistent challenges of society is proposed as a possible action to reduce the consequences of patients suffering from sickle-cell anemia. [ABSTRACT FROM AUTHOR]

Copyright of Jornal de Pediatria is the property of Sociedade Brasileira de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: To use the spatial distribution of the sickle cell trait (SCT) to analyze the frequency of hemoglobin S (HbS) carriers in Sergipe. Methods: The sample consisted of all individuals born in Sergipe from October 2011 to October 2012 who underwent neonatal screening in the public health system. Tests were carried out in basic health units and forwarded to the University Hospital laboratory, where they were analyzed. We used spatial autocorrelation (Moran's index) to assess the spatial distribution of heterozygous individuals with hemoglobinopathies. Results: Among 32,906 newborns, 1,202 showed other types of hemoglobin besides Hemoglobin A. We found a positive correlation between the percentage of black and multiracial people and the incidence of SCT. Most SCT cases occurred in the cities of Aracaju (n=273; 22.7%), Nossa Senhora do Socorro (n=102; 8.4%), São Cristóvão (n=58; 4.8%), Itabaiana (n=39; 4.2%), Lagarto (n=37; 4.01%), and Estância (n=46; 4.9%). Conclusions: The spatial distribution analysis identified regions in the state with a high frequency of HbS carriers. This information is important health care planning. This method can be applied to detect other places that need health units to guide and care for sickle cell disease patients and their families. [ABSTRACT FROM AUTHOR]

O presente estudo teve como objetivo apreender as diversas campanhas virtuais relacionadas à doença falciforme desenvolvidas pela Associação de Pessoas com Doença Falciforme de Ilhéus – APEDFI (Bahia/Brasil) – no Facebook, entre os anos de 2012 a 2016. O potencial deste perfil é que permite externar experiências de adoecimentos, de sofrimentos e de solidariedades, rompendo desta maneira uma fronteira : em outros momentos históricos, pareciam ser os meios tradicionais de comunicação o único canal de oferta de temas e conteúdos ao debate público. Com relatos de participantes no perfil da Associação, busca-se eminentemente um empoderamento dos portadores da doença focalizada, estimulando, com palavras e imagens a construção de contextos de interatividades, a disseminação de informações sobre os contextos de tratamentos e as redes de solidariedades à doença.

Objetivos: descrever o perfil clínico-epidemiológico e assistencial dos pacientes pediátricos com doença falciforme. Métodos: estudo descritivo da totalidade (48) de crianças e adolescentes portadores de doença falciforme, atendidos em hospital de referência da rede pública da Paraíba. As informações obtidas em prontuários e entrevistas foram analisadas no programa Epi-Info versão 7.2, foram construídas tabelas de frequência para as variáveis categóricas, e calculadas medidas de tendência central e de dispersão para variáveis referentes a idade e internações. Resultados: a idade variou de 15 meses a 19 anos(mediana 8,6 anos); pardos e negros (91,7%); 81,3% da classe social D-E; 48% dos responsáveis referiram menos de nove anos de estudo; 70,9% residiam fora da cidade de referência. Diagnóstico tardio em 93,8%; 87,5% com seguimento ambulatorial irregular e 62,5% com calendário vacinal incompleto/desatu-alizado. Ocorreram 226 internações; crises álgicas foram as causas mais comum (55,7%). Cada paciente foi internado, em média, 5,2 vezes no período; a mediana do total de dias de internações foi 28. Não houve óbitos. Complicações crônicas mais frequentes: cardíacas (56,2%) e hepatobiliares (54,3%). Conclusões: a doença falciforme é uma condição clínica negligenciada no Nordeste Brasileiro, onde não há adequado cumprimento da política de atenção destinada aos pacientes. [ABSTRACT FROM AUTHOR]

Copyright of Jornal de Pediatria is the property of Sociedade Brasileira de Pediatria and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Objective Evaluate the Neonatal Screening Program of the Health Secretariat of the State of Santa Catarina for sickle-cell disease, from January 2003 to December 2012, regarding program coverage and disease frequency. Methods Descriptive, observational, cross-sectional study with retrospective data collection. The variables analyzed were: number of live births in the State of Santa Catarina; number of screened children; number of children diagnosed with sickle-cell trait and sickle-cell disease; type of sickle-cell disease diagnosed; age at the time of sample collection, ethnicity/skin color, gender, and origin of children with sickle-cell disease. Descriptive measures and frequency tables were used for data analysis. Results During the study period, there were 848,833 live births and 730,412 samples were screened by the program, resulting in a coverage of 86.0%. There were 6173 samples positive for sickle-cell trait and 39 for sickle-cell disease. Among children with sickle-cell disease, the median age at the time of sample collection was 6 days. Regarding the ethnicity/skin color, 25 (64.1%) children were white, seven were black, and seven others were not specified. The Midwest and the Highland (Planalto Serrano) of Santa Catarina were the regions with the highest incidence of sickle-cell disease. Conclusion Coverage by the Neonatal Screening Program of Santa Catarina is good, but did not demonstrate an improvement trend over the years. The frequency of sickle-cell disease is low and lower than in the North, Northeast, and Midwest regions. The median age in days at the time of collection is older than the age recommended by the Ministry of Health.

Abstract Objective To determine eight parameters of oxidative stress markers in erythrocytes from children with sickle cell disease and compare with the same parameters in erythrocytes from healthy children, since oxidative stress plays an important role in the pathophysiology of sickle cell disease and because this disease is a serious public health problem in many countries. Methods Blood samples were obtained from 45 children with sickle cell disease (21 males and 24 females with a mean age of 9 years; range: 3–13 years) and 280 blood samples were obtained from children without hemoglobinopathies (137 males and 143 females with a mean age of 10 years; range: 8–11 years), as a control group. All blood samples were analyzed for methemoglobin, reduced glutathione, thiobarbituric acid reactive substances, percentage of hemolysis, reactive oxygen species, and activity of the enzymes glucose 6-phosphate dehydrogenase, superoxide dismutase, and catalase. Data were analyzed using Student's t-test and were expressed as the mean ± standard deviation. A p-value of

Sickle cell anemia is a hereditary disease characterized by the change in red blood cells, making them look like a half moon. The research aimed to show the socioeconomic conditions of families with sickle cell disease living in the Negros do Riacho quilombola community, in the municipality of Currais Novos, Rio Grande do Norte. A quantitative quali approach was adopted. Data were collected through semi-structured interviews conducted at the participants' home, after the signing of the ICF. The results showed that the proportion of women is higher than that of men. Those who are between the ages of 26 and 35 are 38.8%. Most are married, have primary school and earn up to a minimum wage. The water consumed does not receive any treatment. It comes exclusively from well. As there is no public collection of garbage, it is deposited in the open and only 5.6%, incinerated. Only 11.2% of the patients have already done or do some type of treatment. It is concluded that there is a relation between socioeconomic conditions and the incidence and prevalence of sickle cell anemia. Financial and logistic support is suggested for the development of studies / diagnoses in this community, alerting the authorities to intervene continuously and systematically in the prevention, treatment and control of sickle cell anemia.

OBJECTIVE: To evaluate the frequency of infectious complications in children with sickle cell disease (SCD) after surgical splenectomy for acute splenic sequestration crisis. METHODS: Retrospective cohort of children with SCD who were born after 2002 and were regularly monitored until July 2013. Patients were divided into two groups: cases (children with SCD who underwent surgical splenectomy after an episode of splenic sequestration) and controls (children with SCD who did not have splenic sequestration and surgical procedures), in order to compare the frequency of invasive infections (sepsis, meningitis, bacteremia with positive blood cultures, acute chest syndrome and/or pneumonia) by data collected from medical records. Data were analyzed by descriptive statistical analysis. RESULTS: 44 patients were included in the case group. The mean age at the time of splenectomy was 2.6 years (1-6.9 years) and the mean postoperative length of follow-up was 6.1 years (3.8-9.9 years). The control group consisted of 69 patients with a mean age at the initial follow-up visit of 5.6 months (1-49 months) and a mean length of follow-up of 7.2 years (4-10.3 years).All children received pneumococcal conjugate vaccine. No significant difference was observed between groups in relation to infections during the follow-up. CONCLUSIONS: Surgical splenectomy in children with sickle cell disease that had splenic sequestration did not affect the frequency of infectious complications during 6 years of clinical follow-up.

Objetivo Relatar um caso de coexistência de lúpus eritematoso sistêmico (LES) e doença falciforme (DF) com revisão da literatura sobre o tema. Metodologia Relato de caso e pesquisa da associação entre LES e DF na literatura, através de artigos científicos nas bases de dados de ciências da saúde, como LILACS, MEDLINE/Pubmed e Scielo, até maio de 2012. Descritores utilizados: 1. anemia falciforme; 2. doença falciforme; 3. lúpus eritematoso sistêmico; 4. hemoglobinopatias. Resultados Os autores descrevem a associação de LES e hemoglobinopatia SS em paciente do sexo feminino, oito anos, apresentando manifestações articulares, hematológicas e neuropsiquiátricas durante a sua evolução clínica. Na literatura são descritos 45 casos de associação entre LES e DF, sendo a maioria em mulheres (78%) adultas (62,2%), apresentando fenótipo SS em 78% dos casos e com manifestações clínicas variadas. Comparando com a nossa paciente, manifestações articulares, hematológicas e neuropsiquiátricas, estiveram presentes em 76%, 36% e 27% dos casos, respectivamente. Conclusões LES e DF são doenças crônicas que apresentam diversos achados clínicos e laboratoriais em comum, implicando em dificuldades diagnósticas e na correta condução terapêutica dessas doenças. A associação entre essas enfermidades não é comum, mas está descrita na literatura, por isso é importante que médicos que cuidam dessas enfermidades estejam atentos para tal possibilidade.

Sickle cell disease results from a genetic mutation that causes chronic hemolytic anemia with severe clinical symptoms. The diagnosis of this hemoglobinopathy was included in Ministry of Health Ordinance No. 822/11 in the National Neonatal Screening Program (PNTN) and the methodology established by said program is isoelectric focusing electrophoresis (FIE) or High Performance Liquid Chromatography (HPLC). The survival rate is low. The medical records from January 2017 to July of the same year were analyzed and the following findings were observed: the main sequelae was splenectomy. The main complaints were fever and pain, in the hemograms the main findings were erythropenia, hypochromia, poikilocytosis, anisocytosis, polychromasia and mild leukocytosis. [ABSTRACT FROM AUTHOR]

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O presente estudo teve como objetivo conhecer e analisar a história de mulheres negras com doença falciforme e compreender como a subjetividade destas é construída e confrontada a partir dos sentidos subjetivos associados aos marcadores de gênero, raça e classe social. Para entender os sentidos e configurações subjetivas utilizamos entrevistas semi abertas com questões semi-estruturadas que foram exploradas através da análise crítica do discurso. As participantes foram 9 mulheres negras acompanhadas pelo Centro de Referência da Anemia Falciforme do Hospital de Clínicas de Porto Alegre. A participação das mulheres nesse estudo exploratório de delineamento qualitativo estava condicionada à auto identificação como negra e ao diagnóstico de doença falciforme. Os resultados mostraram que as mulheres negras percebem a sua condição de saúde como uma doença que estreita os laços entre seu pertencimento racial e as suas construções de gênero e de classe. O discurso das mulheres negras com doença falciforme transcendeu à complexidade da doença crônica, transformando-a e construindo possibilidades que as potencializam como mulheres, como mães e como sujeitos sociais. Sobre os sentidos subjetivos elas assinalaram que a interseccionalidade de raça e classe social intensifica a cegueira pública da mulher negra e das questões relacionadas à população negra e isto provoca um sentimento de invisibilidade. Com relação à construção de gênero, as mulheres negras incorporaram o autocuidado como uma dimensão que minimiza o impacto subjetivo da enfermidade. Ao mesmo tempo, o estudo legitima o sentido subjetivo da maternidade na história das mulheres negras, ser mãe foi uma experiência incontestável que deu “cor” às suas vidas.

Sickle Cell Disease (SCD) is a chronic inherited disorder, characterized by the presence of abnormal erythrocytes containing S hemoglobin (HbS) that leads to diverse clinical complications and special nutritional needs. This article aimed to describe food and nutrition education actions for people with SCD. This is a report of the experience with educational practices developed by a Reference Center and performed at a hematology ambulatory in the university hospital from 2010 to 2012 that aimed to promote adequate and healthy eating habits. Data were obtained by consulting the educational practice notes; 171 people participated in 15 educational practices. The subjects were grouped into four main themes: General recommendations for people with sickle cell disease; Increased nutrient requirements; Strategies to reduce salt and sodium intake; and Iron content and availability in foods. Educational practices allowed for the reflection and guidance on appropriate feeding practices and for the findingof doubts on dietary recommendationsfor SCD through people's own reports. The space in the waiting room promoted the exchange of knowledge between the professionals who conducted the practices and participants. However, it was noted as weaknesses, the focus centered on the nutrients, the difficulty of adopting methodological strategies more targeted for age and the lack of inclusion of a multidisciplinary team. The experience presented has important reflections on the need for food, and food and nutrition education is common tool to healthcare professionals throughout the network of assistance to people with sickle cell disease. DOI: http://dx.doi.org/10.12957/demetra.2014.10539

A equidade é um princípio de justiça e igualdade social. Seu conceito tem sido empregado pela saúde pública brasileira visando atender indivíduos diferentes, conforme suas necessidades. Sua implantação é difícil e complexa; envolve o comprometimento dos profissionais e da organização do sistema de saúde. A investigação da doença falciforme na população é resultado da luta social em prol de um tratamento igualitário e justo, firmado em 2001, por meio do Programa Nacional de Triagem Neonatal (PNTN). O estudo pretende reconhecer o PNTN para a doença falciforme como uma ação que respeita o princípio de equidade no sistema de saúde brasileiro. Constatou-se que a meta do programa é identificar, tratar e prevenir as complicações da patologia precocemente, acompanhando e orientando o indivíduo portador, família e sociedade, independente de suas características físicas e condição social. Uma representação da força social em prol de uma saúde pública justa, que conserve a justiça como um pilar do sistema.