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Norsworthy, William Ludy 1948. "Life Stress and Incidence of Pediatric Sickle Cell Anemia Pain Crises." Thesis, North Texas State University, 1985. https://digital.library.unt.edu/ark:/67531/metadc331499/.
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This study investigated the relationship between stress and pain crisis incidence in pediatric Sickle Cell Anemia (SCA). It was hypothesized that SCA children were exposed to higher levels of stress than healthy children. It was also hypothesized that a significant positive correlation existed between level of stress and pain crisis incidence both within and between years. The sample consisted of 20 Black elementary school children with SCA. There were 12 female and 8 male children. The period of investigation included the calendar years 1983 and 1984. Pain crisis incidence was determined through parent interviews and verified by a review of medical records.
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Okoye, C. D. "Relevance of sickle cell anaemia and its protection against malaria in african children." Thesis, Sumy State University, 2017. http://essuir.sumdu.edu.ua/handle/123456789/61237.
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Sickle cell anaemia, an autosomal recessive disease caused by a single point mutation in nucleobase sequence of chromosome 11 with substitution of glutamic acid by valine and formation of HbS. Malaria on the other hand is an infection caused by a parasite (Plasmodium sp) that is transmitted to humans by female anopheles mosquito and is prevalent in tropical and subtropical regions of Africa due to increased rainfall, constant high temperatures and high humidity.
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Tarazi, Reem A. "Neuropsychological functioning in preschool-aged children with sickle cell disease : the role of illness-related and psychosocial factors /." Click for resource, 2004. http://dspace.library.drexel.edu/handle/1860/289.
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Anderson, Ericka Lisle Barakat Lamia. "A prospective examination of neuropsychological functioning in preschool-age children with sickle cell disease and its association with psychosocial factors /." Philadelphia, Pa. : Drexel University, 2005. http://dspace.library.drexel.edu/handle/1860/610.
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Mackey, Michelle Noble. "Understanding Parents' Disease-Managing Strategies for Children With Sickle Cell Disease." ScholarWorks, 2019. https://scholarworks.waldenu.edu/dissertations/6610.
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Sickle cell disease (SCD) is one of the most difficult and stressful chronic diseases for parents of afflicted children to manage. Managing SCD can be traumatic for parents particularly if they have no specific coping strategies for managing the disease or ensuring the child visits the doctor as scheduled. The use of certain coping strategies may affect the parents' and patients' perceptions of the illness and influence their decisions regarding treatment, which can have a lasting impact on their lives. Effective parental strategies such as positive thinking can aid in disease management, but there is limited research on the coping strategies used by parents of children with SCD specifically. The purpose of this phenomenological study, which was guided by Thompson and Gustafson's transactional stress and coping model, was to describe parents' coping strategies in managing their young child's SCD as it relates to use of health services. Data collection included one-to-one, open-ended interviews with 10 parents of children with SCD. Colaizzi's method of phenomenological data analysis was used to identify themes. Five themes emerged from data analysis and they are: parental methods of coping with SCD, participants' understanding of SCD, SCD family and support, managing SCD with hydration and medication, and experience accessing healthcare. These results indicated the participants' coping strategies varied according to their individual situations. Insight from this study could lead to positive social change by helping to identify specific coping strategies parents can use to better manage their child's disease and effectively access available health services.
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Sousa, Eulange de. "Crianças e adolescentes portadores de anomia falciforme: os significados das relações estabelecidas com os profissionais no âmbito dos serviços de saúde." Universidade Federal de Goiás, 2014. http://repositorio.bc.ufg.br/tede/handle/tede/5374.
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Previous issue date: 2014-08-29<br>Fundação de Amparo à Pesquisa do Estado de Goiás - FAPEG<br>Sickle cell anemia is an inherited disease considered a public health problem
in Brazil. In Brazil, it is estimated that there are more than 2 million people affected
by some form of this anemia, with 3.500 new cases occurring every year. This
dissertation aims to understand the meanings present in the relations established
between children and adolescents with sickle cell anemia and professionals within
the health services. The methodological choice of this study was the Social Strategic
Qualitative Research and it took place at a Teaching Hospital. Data were collected
through semi-structured interviews with 3 children, 5 adolescents and 9 health
professionals. Data were analyzed through the content analysis technique. From the
interviews with children and adolescents, the following categories emerged: the
disease, the professionals, and the treatment in other services. From the interviews
with health professionals, the following categories emerged: child, adolescent,
mother-family, professionals from other services, the disease, and what to do. The
research conducted indicated that it is necessary to engage children and adolescents
in decisions related to their health-disease process. It also indicated that, in order to
achieve it, the health professionals training have to include elements that enable
professionals to consider children and adolescents as protagonists in their healthdisease
process. In addition, it demonstrated the occurrence of institutional violence
in the health treatment that patients with sickle cell anemia receive, as they have
their autonomy, their subjectivity and their speech prevented and annulled.<br>A Anemia falciforme é uma doença hereditária considerada um problema de
saúde pública no Brasil. Estima-se que existem, no Brasil, mais de 2 milhões de
pessoas afetadas por alguma forma desta anemia, com uma ocorrência de 3.500
novos caso por ano. Este trabalho tem por objetivo compreender os significados
presentes nas relações estabelecidas entre as crianças e adolescentes portadores
de anemia falciforme e os profissionais no âmbito dos serviços de saúde. Esta
pesquisa foi realizada tendo como opção metodológica a Pesquisa Social
Estratégica de abordagem qualitativa, e como local de estudo optou-se por um
hospital de ensino. Os dados foram obtidos por meio de entrevistas semiestruturadas
com três crianças, cinco adolescentes e nove profissionais de saúde.
Os dados foram analisados pela técnica de análise de conteúdo. A partir das
entrevistas com as crianças e adolescentes, emergiram as seguintes categorias:
doença, os profissionais e o atendimento em outros serviços. Das entrevistas com
os profissionais, emergiram as categorias: criança, adolescente, mãe–família,
profissionais de outros serviços, a doença e o que fazer. O estudo realizado indicou
que é necessário envolver as crianças e adolescentes nas decisões relativas a seu
processo saúde-doença e que a formação de profissionais de saúde deve incluir
elementos que os instrumentalizem no sentido de considerar crianças e
adolescentes como protagonistas em seu processo saúde-doença. Indicou também
a ocorrência de violência institucional no atendimento que os portadores de anemia
falciforme recebem, pois têm sua autonomia, sua subjetividade e sua fala impedidas
e anuladas.
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Chun, Kathryn Malia. "Adolescent older siblings of children with Sickle Cell Disease : parent-child interaction, "parentification," and peer relationships /." Connect to CIFA website:, 2005. http://ourworld.compuserve.com/homepages/pdwerner/cifa1.htm.
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Menezes, Adeline Soraya de Oliveira da Paz [UNIFESP]. "Qualidade de vida em portadores de doença falciforme." Universidade Federal de São Paulo (UNIFESP), 2011. http://repositorio.unifesp.br/handle/11600/9996.
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Previous issue date: 2011-11-24<br>Objetivos. 1) Avaliar a qualidade de vida relacionada à saúde (QVRS) de crianças e adolescentes com doença falciforme assistidas em um Hemocentro de referência e 2) Mensurar a QVRS dos respectivos familiares. Métodos. Amostra de conveniência de 100 pacientes portadores de doença falciforme (64 do sexo feminino e 34 do sexo masculino), divididos em três subgrupos conforme a faixa etária: de 5 a 7 (n=18), de 8 a 12 (n=32) e de 13 a 18 (n=20) e respectivos pais. O grupo controle foi composto por 50 crianças e adolescentes aparentemente saudáveis de uma escola pública local, também divididos nos mesmos três subgrupos de idade e seus respectivos cuidadores. Foi aplicado o questionário genérico “Pediatric Quality of Life Inventory” (PedsQL) versão 4.0 às crianças e aos adolescentes de ambos os grupos; aos familiares foi aplicando o questionário genérico Medical Outcomes Study 36 – Item Short-Form Health Survey (SF-36). As respostas obtidas foram linearmente transformadas em um escore e comparadas. Resultados. Os escores dos pacientes foram significativamente mais baixos do que os escores do grupo controle (p < 0,0001) em todos os 4 aspectos estudados (capacidade física, emocional, social e atividade escolar). Com relação ao SF-36, aplicados aos pais, observamos que os escores foram mais baixos, sendo as perdas de qualidade de vida mais significativas (superiores a 50%) às relacionadas aos aspectos sócioemocionais, à saúde mental, limitação por aspectos físicos e ao estado geral de saúde. Conclusão. A doença falciforme compromete a qualidade de vida das crianças, dos adolescentes e das respectivas famílias. Os pacientes percebem restrições nos aspectos emocional, social/familiar e físico dentre outros.<br>Objective. 1) To evaluate the quality of life in children and adolescents with sickle cell disease attending the Blood Center reference. 2) To evaluate the quality of life of relatives of these patients. Method. We selected 100 patients (64 female, 34 male) with sickle cell disease that were divided into three subgroups with age: 5 to 7 (n = 18), 8-12 (n = 32) and 13 to 18 (n = 20), and their parents. The control group was 50 healthy children and adolescents from a public school local, also divided into the same three subgroups of age and their caregivers. The Questionnaire Pediatric Quality of life Inventory - PedsQL version 4.0 was applied in both groups - children and adolescents, in the family was applied the generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36). The answers were linearly transformed into a score and compared. Results: The scores of patients were significantly lower than the scores of the control group (p <0.0001) in all four areas studied (physical, emotional, social and school activities). In the version for parents was the same in almost all respects, with the loss of quality of life more meaningful (more than 50%) were related to the socio-emotional, mental health, limited by the physical appearance and general state of health. Conclusion: Sickle cell disease affects the quality of life of children, adolescents and their families. Patients perceive restrictions in the emotional, social / family and physical and others.<br>TEDE<br>BV UNIFESP: Teses e dissertações
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Gumiero, Ana Paula dos Santos. "Litiase biliar na doença falciforme : descrição das caracteristicas clinicas em crianças." [s.n.], 2008. http://repositorio.unicamp.br/jspui/handle/REPOSIP/309095.
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Previous issue date: 2008<br>Resumo: Introdução- A anemia falciforme (AF) causa hemólise crônica e acelerada que é reconhecida como um fator de risco para desenvolvimento de colelitíase. Esta complicação pode ocorrer em mais de 50% da população adulta com AF. A colecistectomia é a conduta consensual para pacientes sintomáticos, mas nos assintomáticos a sua indicação é controversa. Objetivos- Relatar a freqüência de colelitíase em pacientes com AF e descrever a opção de conduta médica em caso de diagnóstico desta complicação, numa coorte de pacientes seguidos num centro terciário de hematologia pediátrica. Pacientes e métodos- Estudo descritivo e retrospectivo, com revisão dos prontuários de 225 pacientes e registro dos dados relacionados à evolução clínica. Resultados- A freqüência cumulativa de colelitíase foi de 44,9%, sendo que metade dos pacientes eram assintomáticos. As idades médias no diagnóstico de colelitíase e no momento do tratamento cirúrgico foram, respectivamente, 12,5 anos (DP = 5 anos) e 14 anos (DP = 5,4 anos). A prevalência de colelitíase foi maior nos pacientes com fenótipos SS e Sß Talassemia, comparada à prevalência nos pacientes com fenótipo SC (?2= 0,001). Entre os pacientes sintomáticos (50%), a dor abdominal inespecífica foi o único sintoma ou o sintoma mais freqüentemente referido. Entre pacientes que realizaram colecistectomia (N = 44), 39 tiveram melhora ou resolução de seus sintomas após o procedimento. Durante o seguimento clínico dos pacientes assintomáticos e não tratados cirurgicamente (7 anos ± 4,8 anos), nenhuma criança apresentou complicação relacionada a colelitíase. Conclusões- A freqüência de colelitíase na população estudada foi de 44,9%. Um terço dos pacientes foi diagnosticado antes dos 10 anos de idade. Pacientes com fenótipos SS e Sß associaram-se à maior freqüência de litíase biliar. Os pacientes assintomáticos e não operados não apresentaram complicações num período de 7 anos de seguimento<br>Abstract: Background- Sickle cell disease (SCD) causes chronic and recurrent hemolysis which is a recognized risk factor for cholelithiasis. This complication occurs in 50% of adults with SCD. Surgery is the consensual therapy in symptomatic patients, but the surgical approach is still controversial in asymptomatic individuals. Aims- To describe the frequency and the outcome of children with SCD complicated with gallstones followed up in a tertiary pediatric hematology center. Patients and methods- In a retrospective and descriptive study, 225 charts were reviewed and data regarding patient¿s outcome were recorded. Results- The prevalence of cholelithiasis was 44,9% and half of the patients had no symptom. The mean age diagnosis of cholelithiasis and surgical treatment were 12.5 years (SD= 5) and 14 years (SD= 5.4), respectively. The prevalence of cholelithiasis was higher in patients with SS and Sß thalassemia when compared to patients with SC disease (?2 = 0.001). In 50% of symptomatic patients, recurrent abdominal pain was the single or predominant symptom. Thirty-nine of 44 operated patients reported symptom relieve after surgical procedure. Asymptomatic non-operated individuals were followed up for 7 years (SD = 4.8), and none of them presented complications related to cholelithiasis during this period. Conclusions- Cholelithiasis frequency in studied children was 44,9%. One-third of patients were diagnosed before 10 years of age. Patients with the SS or Sß phenotype showed higher frequency of cholelithiasis. About 50% of patients with gallstones had no symptom and most of them did not undergo surgery and did not present complications during a 7-year follow-up period<br>Mestrado<br>Pediatria<br>Mestre em Saude da Criança e do Adolescente
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Ribera, Melissa Chaves Vieira. "Modulação autonômica cardíaca em crianças e adolescentes com anemia falciforme." Universidade de São Paulo, 2017. http://www.teses.usp.br/teses/disponiveis/6/6136/tde-17072017-165426/.
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Introdução - Alterações cardíacas na anemia falciforme (AF) são frequentes e iniciam-se precocemente. Há evidências de que exista também disfunção na regulação do sistema nervoso autônomo o que pode contribuir com eventos de morbidade. Objetivos Avaliar a modulação autonômica cardíaca por meio da variabilidade da frequência cardíaca em crianças e adolescentes com anemia falciforme. Método - Estudo analítico no qual foi realizada uma comparação da variabilidade da frequência cardíaca em 45 crianças e adolescentes, menores de 20 anos, com anemia falciforme, com um grupo controle pareado um a um por idade e sexo. A frequência cardíaca foi obtida pelo frequencímetro de pulso e analisada, batimento a batimento. Estes pacientes são usuários do ambulatório de hematologia pediátrica do Sistema Único de Saúde. Esta pesquisa está em consonância com a resolução 466/2012 do Ministério da Saúde. Resultados - Observamos diferença significativa nos índices do domínio da frequência (VLF, LF, HF e LF/HF). Estas diferenças não foram observadas nos pacientes em uso de hidroxiureia. Conclusão - Existe uma disfunção autonômica na AF que ocorre desde a infância, podendo estar relacionada a uma menor modulação do simpático e uma maior modulação do parassimpático. Esta diferença não foi observada em pacientes em uso de hidroxiureia<br>Introduction - Cardiac changes in sickle cell disease (AF) are frequent and begin early. There is evidence that there is also dysfunction in the regulation of the autonomic nervous system, which may contribute to morbidity events. Objectives - To evaluate the autonomic cardiac modulation by heart rate variability in children and adolescents with sickle cell anemia. Method - An analytical study comparing the heart rate variability of 45 children and adolescents, younger than 20 years, with sickle cell anemia, with a control group matched one by one by age and sex. The heart rate was obtained by pulse frequency and analyzed, beat by beat. These patients are attending the pediatric hematology outpatient of the National Health System. Results - We observed a significant difference in the frequency domain indexes (VLF, LF, HF and LF / HF). The results of this study are in agreement with resolution 466/2012 of the Ministry of Health of Brazil. These differences were not observed in patients taking hydroxyurea. Conclusion - There is an autonomic dysfunction in AF that occurs from childhood, and may be related to a lower modulation of the sympathetic and greater modulation of the parasympathetic. This difference was not observed in patients taking hydroxyurea
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Barbosa, Silvia Maria de Macedo. "Estudo da influência de fatores ambientais sobre o desencadeamento de crise álgica em crianças e adolescentes portadores de anemia falciforme na cidade de São Paulo." Universidade de São Paulo, 2006. http://www.teses.usp.br/teses/disponiveis/5/5144/tde-19042007-154059/.
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Medicina da Universidade de São Paulo. Objetivo: Esse estudo foi desenvolvido para avaliar o impacto dos poluentes do ar na morbidade nas crianças e adolescentes portadoras de anemia falciforme. Métodos: Foram utilizadas as associações entre as concentrações diárias entre as concentrações dos poluentes do ar (PM10, SO2, NO2, CO, e O3) e os atendimentos no Pronto Socorro de anemia falciforme (CID 10 - D57) no período de setembro de 1999 até dezembro de 2004. Foi utilizado o desenho de case - crossover com dias de exposição de referencia escolhidos utilizando-se uma abordagem estratificada por tempo onde as exposições no dia índice foram comparadas a exposições com dias do mesmo mês com o mesmo valor de temperatura como o caso índice, controlando-se para o dia da semana. Estação do ano, clima e covariantes variáveis lentas foram controladas por pareamento. Também foram adotados modelos onde as exposições do caso índice foram comparadas às exposições para o mesmo dia da semana ou a cada três dias. Os efeitos estimados também foram estratificados para as duas causas principais de atendimento no pronto socorro de pacientes falciformes, dor e infecção respiratória. Resultados: As variações de interquartile aumentaram nas médias móveis de PM10 (25.9 mg/m3), NO2 (64.8 mg/m3), SO2 (8.1 mg/m3), CO (1.2 ppm), e O3 (57.23 mg/m3) foram associadas com aumento de 19.2% (95% CI: 11.2 - 27.8), 15.6% (95% CI: 6.3 - 25.8), 14.5% (95% CI: 6.6 - 23.0), 15,1% (95% CI: 7.7 -23.1), e 10% (95% CI: 1,3 - 18,6) nos atendimentos totais dos pacientes falciformes no pronto socorro, respectivamente. Na análise estratificada para dor, que é a manifestação cardinal dos processos de vaso-oclusão, os aumentos nos atendimentos dos pacientes falciformes com dor (23.1%, 95%CI: 11.0 - 36.5) devido a um aumento na variação interquartile da média móvel de 3 dias do PM10 foi 43% maior que nos pacientes falciformes sem dor. Conclusão: esse estudo mostra que o efeito da poluição do ar na saúde da população pediátrica não se restringe às doenças respiratórias. Também , entre os pacientes falciformes a manifestação principal dos efeitos adversos foi associada com o processo inflamatório dos vasos, reproduzindo resultados já observados entre pacientes adultos e idosos saudáveis e não saudáveis. Finalmente, esse estudo mostrou que os efeitos da poluição do ar na saúde ainda não esta definitivamente estimados e outros resultados serão observados em mais investigações.<br>Objective: This study was developed to assess the impact of air pollutants on sickle cell morbidity in children and adolescents. Methods we examined the associations between daily air pollutants concentrations (PM10, SO2, NO2, CO, and O3) and sickle cell (ICD10th revision: D57) emergency room visits, from September 1999 to December 2004. We applied a case-crossover design with referent exposure days chosen using the time-stratified approach such that exposures on the case day were compared to exposures on days of the same month with the same value of temperature as the case day, controlling for day of the week. Season, weather, and slowly varying covariates were controlled for by matching. We also adopted models where exposures on the case day were compared to exposures on the same day of the week or on every third day. Effects estimates were also stratified by the two main causes of sickle cell emergency room visits, pain and respiratory infections. Results: interquartile range increases of the 3-day moving averages of PM10 (25.9 mg/m3), NO2 (64.8 mg/m3), SO2 (8.1 mg/m3), CO (1.2 ppm), and O3 (57.23 mg/m3) were associated with increases of 19.2% (95% CI: 11.2 - 27.8), 15.6% (95% CI: 6.3 - 25.8), 14.5% (95% CI: 6.6 - 23.0), 15,1% (95% CI: 7.7 - 23.1), and 10% (95% CI: 1,3 - 18,6) in total sickle cell emergency room visits, respectively. When the analyses were stratified by pain, the main clinical manifestation of vase-occlusion process, increases in visits of sickle cell patients with pain (23.1%, 95%CI:11.0 -36.5) due to an interquartile range increase of the 3-day moving averages of PM10 were 43% higher than in sickle cell patients without pain. Conclusions: this study found that the effects of air pollutants on children\'s health are not limited to respiratory diseases. Also, among sickle cell patients, the main manifestation of adverse effect was associated with inflammatory process of vases, reproducing results already observed among healthy and non-healthy adults and elderly people. Finally, this study showed that the burden of air pollution on health has not been definitively estimated and other outcomes deserve further investigation.
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Saleh, A. W. "Modulation of fetal hemoglobin in sickle cell anemia." Maastricht : Maastricht : Universiteit Maastricht ; University Library, Maastricht University [Host], 1998. http://arno.unimaas.nl/show.cgi?fid=8498.
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Winn, Vaniethia R. "The psychosocial adjustment of adolescents with Sickle Cell Anemia." DigitalCommons@Robert W. Woodruff Library, Atlanta University Center, 1994. http://digitalcommons.auctr.edu/dissertations/381.
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The overall objective of this study is to investigate the psychosocial problems experienced by adolescents with sickle cell anemia. To attain this objective, the following issues were addressed by the researcher: (a) peer relationships; (b) independence; (c) stress; and (d) self-concept. A descriptive research design was used in the study. A questionnaire developed by the researcher was utilized and administered via telephone conversation/interview to 13 adolescents with sickle cell anemia located in the Metropolitan Atlanta area. The results of the study indicated that the participants diagnosed with sickle cell anemia maintain good peer relationships, experienced high levels of stress, have low self-concept and, have not achieved an appropriate level of independence with regard to a sense of personal freedom.
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Yeboah, Sybil A. Parise Leslie V. "Do CD40L and CD40 contribute to sickle cell anemia?" Chapel Hill, N.C. : University of North Carolina at Chapel Hill, 2008. http://dc.lib.unc.edu/u?/etd,1640.
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Thesis (M.S.)--University of North Carolina at Chapel Hill, 2008.<br>Title from electronic title page (viewed Sep. 16, 2008). "... in partial fulfillment of the requirements for the degree of Master of Science in the Department of Biochemistry." Discipline: Biochemistry and Biophysics; Department/School: Medicine.
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Brown, Marena Dessette. "Sickle cell-endothelial interactions : modulation of cell adhesion molecule expression." Diss., Georgia Institute of Technology, 1995. http://hdl.handle.net/1853/11306.
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Weng, Weijun Ferrone Frank A. "Universal metastability of sickle hemoglobin polymerization /." Philadelphia, Pa. : Drexel University, 2008. http://hdl.handle.net/1860/2832.
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Reed, Caroline. "Increasing Hydroxyurea Adherence for Pediatric Patients With Sickle Cell Anemia." Thesis, Walden University, 2016. http://pqdtopen.proquest.com/#viewpdf?dispub=10141603.
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<p> Sickle cell disease is a disabling chronic autosomal recessive blood disease characterized by abnormal hemoglobin, pain crises, and frequent emergency department visits. Adherence to hydroxyurea therapy has been shown to improve these patient outcomes. Guided by the theory of comfort, the purpose of this project was to determine if an educational intervention would increase adherence to hydroxyurea therapy in pediatric patients between 2 and 17 years of age recruited from an urban university hospital hematology clinic. The RE-AIM model was used to support the translation of evidence and the change process. An educational video produced by AFLAC was viewed by patients’ parents 4 weeks after enrollment into this pretest/posttest design project. A total of 22 African-American parent participants completed the 8-item Morisky Medication Adherence Scale at baseline and again at 8 weeks to assess hydroxyurea adherence. The Short Test of Functional Health Literacy in Adults tool was used to assess parents’ health learning needs; all parents met the adequate literacy level at baseline. Using <i>t</i> test statistics, no statistically significant differences were found pretest to posttest on the Morisky Medication Adherence Scale scores, mean corpuscular hemoglobin, and fetal hemoglobin percentages. Wilcoxon Signed Rank tests showed no significant differences in emergency room visits nor number of pain crisis. Although no significant changes emerged in short-term hematologic findings, emergency room visits, and pain crises, social change in the health care setting was promoted by confirming parents were able to understand education and a high level of hydroxyurea adherence was maintained; literature indicated that long-term adherence to hydroxyurea limits severe attacks.</p>
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Moxley, Kristan Michelle. "Impact of Carrier Screening on Pregnant Women’s Knowledge of Sickle Cell Anemia." Case Western Reserve University School of Graduate Studies / OhioLINK, 2008. http://rave.ohiolink.edu/etdc/view?acc_num=case1201280917.
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Zakharov, Mikhail N. Ferrone Frank A. "A microrheological study of sickle hemoglobin polymerization /." Philadelphia, Pa. : Drexel University, 2009. http://hdl.handle.net/1860/3075.
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Mogor, Odinaka P., and Odinaka P. Mogor. "Sickle Cell Anemia: The Effects of Hydroxyurea on Red Blood Cell Polymerization Reversal." Thesis, The University of Arizona, 2017. http://hdl.handle.net/10150/626380.
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Sickle cell anemia – the most common form of sickle cell disease (SCD) – is a debilitating condition that presents with health complications ranging from severe pain due to vaso-occlusion and acute chest syndrome, to a reduced ability to sufficiently transport oxygen through the body due to accelerated hemolysis of red blood cells. The underlying cause of SCD is a genetic mutation in which valine is replaced by glutamic acid at the sixth position of the β-globin chain, leading to the production of an abnormal sickle hemoglobin (hemoglobin S). This abnormal protein within the red blood cells have an increased propensity to polymerize into long fibers when deoxygenated, decreasing red-cell deformability while increasing the rate of cell death by damaging the cell membrane. These sickled red blood cells can then clump together to occlude blood vessels causing pain and organ damage. The impact of SCD has led to the evaluation of different treatment modalities in attempt to cure or ameliorate the manifestations of the disease. This thesis is intended to review the underlying disease process, elucidate the treatment modalities that have been previously proposed, and highlight the effect of hydroxyurea as the primary approved treatment of sickle cell anemia due to its ability to reverse the polymerization of already sickled cells, and increase the levels of fetal hemoglobin (HbF). The increase of HbF levels is a major goal of treatment that can achieved by increasing the expression of gamma-globin genes, therefore we can hypothesize that upregulating the activity of enzyme Demethylase will reverse the methylation of the CpG islands and reverse the binding of Histone deacetylase allowing the transcription of already silenced genes to resume.
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Al-Mukhaini, Zainab Ali Mohammad. "Psychosocial impact of sickle cell disorder on children with sickle cell disorder in Oman." Thesis, University of Leeds, 2006. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.436431.
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Moxley, Kristan Michelle. "Impact of carrier screening on pregnant women's knowledge of sickle cell anemia." Cleveland, Ohio : Case Western Reserve University, 2008. http://rave.ohiolink.edu/etdc/view?acc%5Fnum=case1201280917.
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Wagner, Matthew Christian. "Histamine as a Potential Initiator of Sickle Pain crisis by Mediation of Sickle Erythrocyte Adherence in a Shear-Dependent Manner." Diss., Georgia Institute of Technology, 2006. http://hdl.handle.net/1853/14478.
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The genetic disorder sickle cell anemia causes hemolytic anemia and sickle pain crisis, episodes of microvascular occlusion resulting in painful ischemic tissue damage. Pain crisis is thought to occur when sickle erythrocytes adhere in the post-capillary venule, partially occluding the vessel. The resulting slowed blood flow causes more extensive cell adherence and entrapment of rigid, deoxygenated erythrocytes until the vessel is entirely occluded. It was hypothesized that the inflammatory mediators histamine and tumor necrosis factor-, factors known to cause endothelial expression of adhesive ligands, might significantly increase sickle erythrocyte adhesion, and thus be capable of initiating sickle pain crisis. It was also hypothesized that the perfusion shear stress environment of the endothelium, known to be oscillatory and reduced in sickle cell patients, was a significant mediating factor of sickle cell adhesion. An in-vitro flow chamber using cultured endothelial cells and erythrocytes from blood samples of sickle cell anemic patients was used to quantify sickle erythrocyte adherence to stimulated and unstimulated endothelial cells under shear stresses from 1.0 to 0.1 dyne/cm2. Results showed that both endothelial stimulation and reduction of the perfusion shear stress increased sickle erythrocyte adherence. In combination, the use of inflammatory stimulation with reduced shear stress resulted in further increased adhesion, but only when above the range of 0.1 V 0.2 or 0.4 dyne/cm2, depending on the inflammatory mediator. Adhesion below this level of shear is not significantly increased by endothelial stimulation. The mechanism by which histamine mediates adhesion was investigated, and found to involve the endothelial H2 and H4 receptors and expression of the P-selectin ligand. These data suggest that irregular flow, typical of sickle microvasculature, may act in conjunction with the pro-inflammatory state of sickle vasculature and the histaminergic nature of some pain treatments to initiate or propagate sickle vaso-occlusion. Findings concerning histamine, tumor necrosis factor-alpha, and shear stress effects on adherence are discussed in relation to their possible applicability to patient health, future studies are outlined to confirm the relation of in vitro data to in vivo patient condition, and proposals are made for applying these methodologies to other potential mediators of sickle erythrocyte adhesion.
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Okoye, Chukwuebuka D., and Ayodeji Segun Bamisile. "Peculiarities of sickle cell anemia in patients with malaria in Africa." Thesis, Sumy State University, 2016. http://essuir.sumdu.edu.ua/handle/123456789/47876.
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Background. Sickle cell anemia is an autosomal recessive disease caused by a single point mutation in nucleobase sequence of chromosome 11 with substitution of glutamic acid by valine and formation of HbS is widespread in mainly African countries with prevalence of 20% - 30% in Cameroon, Republic of Congo, Gabon, Ghana, Nigeria and 45% in Uganda. Malaria on the other hand is an infection caused by a parasite (Plasmodium sp.) that is transmitted to humans by female anopheles mosquito and is prevalent in tropical and subtropical regions of Africa due to increased rainfall, constant high temperatures and high humidity.
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Gbotosho, Oluwabukola Temitope. "Novel approaches to diagnosis, prognosis and pathogenesis of sickle cell disease." Thesis, University of Cambridge, 2015. https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.708915.
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Gavlak, J. C. D. "Sleep in children with sickle cell disease." Thesis, University College London (University of London), 2016. http://discovery.ucl.ac.uk/1528622/.
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BACKGROUND: Sickle Cell Anaemia (SCA) or homozygosity for the sickle haemoglobin gene (HbSS) is the most common genetic condition in the UK. A high prevalence of Sleep Disordered Breathing (SDB) in SCA is widely accepted but there is a lack of unselected population based studies. As Polysomnography (PSG) is expensive, screening for SDB using a robust tool, e.g. the Delta 12 (Δ12) index and 3% Oxygen Desaturation Indices (ODI) calculated from home pulse oximetry, should be validated. Elevated Cerebral Blood Flow Velocity (CBFV) is a predictor of stroke in SCA, and may be associated with SDB. METHODS AND RESULTS: Prevalence of OSA was assessed and compared with the general paediatric population from Polysomnography (SCA n=195) analysed using the American Academy of Sleep Medicine (AASM) criteria. Interobserver reliability between two observers was excellent. At all ages, the prevalence of OSA was higher in SCA. OSA prevalence was highest in young children, with 50% of 4-6 year olds having an obstructive apnoea hyopnoea index (OAHI) of >1. A zero inflated model was fitted for prediction of OAHI from Δ12 or ODI; for predicting OAHI zero or >1, 3% ODI alone had the best fit. In 83 London patients, OSA was associated with elevated CBFV. Mean SpO2, ODI and CAI were predictors of basilar velocity independent of age. In the final model, mean SpO2 remained a predictor (B-3.1, beta -0.41, t -4.2, p < 0.0005) independent of age (B- 2.9, beta -0,453, t -4.7, p < 0.0005). CONCLUSION: Despite the difficulties in comparing across populations, there is a higher prevalence of OSA in unselected children with SCA than in typically developing children with no SDB symptoms, screening with overnight pulse oximetry to select those with OAHI>1 is feasible, and this may be important as there is an association between OSA and basilar CBFV in this population.
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Montes, Richard Anthony O. "Physico-chemical modulation of sickle adherence : a different in vitro model." Diss., Georgia Institute of Technology, 1999. http://hdl.handle.net/1853/11021.
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Brittain, Henri A. "Adherence of sickle red cells to human microvascular endothelial cells : a role for plasma, von Willebrand factor, and platelet thrombospondin." Diss., Georgia Institute of Technology, 1992. http://hdl.handle.net/1853/12091.
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Tudor-Tangeman, Jessie F. E. "An Ethical Analysis of The Black Panther Party and The United States Government’s Sickle Cell Anemia Initiatives." The Ohio State University, 2020. http://rave.ohiolink.edu/etdc/view?acc_num=osu1587119362677946.
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Wu, Li-Chen. "Correction of sickle cell disease by homologous recombination." Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2008. https://www.mhsl.uab.edu/dt/2008p/wu.pdf.
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Cytlak, Urszula Malgorzata. "Phosphatidylserine exposure in red blood cells from patients with sickle cell disease." Thesis, University of Cambridge, 2015. https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.708601.
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Smolinski, Paula A. "Biophysical analysis of receptor mediated erythrocyte adherence in sickle cell anemia : involvement of infection and hemodynamics." Diss., Georgia Institute of Technology, 1996. http://hdl.handle.net/1853/10092.
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Almeida, Camila Bononi de. "Caracterização das fosfodiesterases (PDE) de GMPc nas celulas de linhagem eritroide e efeitos de drogas inibidoras de PDE na produção de hemoglobina fetal." [s.n.], 2006. http://repositorio.unicamp.br/jspui/handle/REPOSIP/311506.
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Orientador: Nicola Amanda Conran Zorzetto<br>Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas<br>Made available in DSpace on 2018-08-10T04:12:18Z (GMT). No. of bitstreams: 1
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Previous issue date: 2006<br>Resumo: A anemia falciforme é um distúrbio genético causado por uma mutação de ponto (Ácido Glutâmico-Valina) que produz uma hemoglobina S. Esta, quando desoxigenada, polimeriza causando deformação dos eritrócitos que se agregam às paredes da micro circulação culminando em crises vaso-oclusivas. Existem algumas moléculas de adesão que colaboram no bloqueio da circulação aderindo eritrócitos, células brancas e plaquetas à parede dos túbulos sanguíneos. A hidroxiuréia (HU) vem sendo utilizada no tratamento da doença devido à sua habilidade em aumentar a produção de hemoglobina fetal (HbF). No entanto, nem todos os pacientes respondem positivamente ao tratamento com HU. Estudos mostram que a HU quando degradada, produz óxido nítrico (NO), o qual ativa a enzima guanilato ciclase solúvel (GCs). Esta, por sua vez, transforma GTP em GMPc que atua como segundo mensageiro ativando provavelmente a transcrição do gene ?-globina aumentando assim, a concentração de HbF e melhorando o quadro clínico dos pacientes com essa doença. Por isso, o NO tornou-se fonte de grandes estudos, uma vez que é um importante vasodilatador. O objetivo desse trabalho foi elucidar qual família de fosfodiesterase (PDE) atua na degradação do GMPc das células de linhagem eritróide e se as drogas inibidoras dessas PDEs seriam capazes de aumentar a produção de ?-globina e conseqüentemente HbF em células eritróides de cultura. Para isso foram realizadas culturas de células K562 tratadas com diferentes drogas inibidoras de PDE (vinpocetina, sildenafil, dipiridamol e zaprinast) e com estimulador de guanilato ciclase, BAY41-2272. Observamos que os níveis de GMPc aumentaram principalmente nas células tratadas com 10µg/ml de dipiridamol e em alguns pontos de coletas com 10 e 50µM de vinpocetina. O mesmo não foi observado nas células tratadas com sildenafil e zaprinast. No entanto, todas as células tratadas com as diferentes concentrações de BAY41-2272 apresentaram aumento na concentração de GMPc. Com relação à expressão gênica relativa de ?-globina, observamos que as drogas dipiridamol, vinpocetina, sildenafil e zaprinast não foram capazes de provocar um aumento da expressão. Diferentemente, BAY41-2272 estimulou um aumento da expressão de ?-globina nas células K562 tratadas com 60 e 300nM após 48 horas de cultura. Além disso, estudamos a expressão das PDEs 1, 5 e 9 em células hematopoéticas e diferentes tecidos. Observamos que PDE9 é significativamente mais expressa em células K562, reticulócitos e neutrófilos. Além disso, observamos também que a expressão dessa PDE é significativamente maior nos reticulócitos e neutrófilos de paciente com anemia falciforme. Já naqueles que se encontravam sob terapia com HU, a expressão da PDE9 nos reticulócitos reverteu aos níveis do controle diferentemente dos neutrófilos, cuja expressão de PDE9 não se alterou entre os pacientes com ou sem terapia com HU. A expressão da PDE9 em outros tecidos (T98G, cólon, ovário, mama, fígado, pele, baço, testículo, útero e linfonodo) é significativamente menor do que nestas células hematopoéticas. Assim sendo, como aumento nos níveis celulares de GMPc pode ser importante tanto para a produção de HbF em células eritróides como para inibir a adesão de leucócitos, bloquear a ação da PDE9 pode representar uma nova estratégica célula-especifica para o tratamento da anemia falciforme<br>Abstract: Sickle cell disease (SCD) is a systemic disease with a multifaceted pathophysiology; the polymerisation of the sickle haemoglobin molecule (HbS), when deoxygenated, has many consequences that include haemolysis, inflammation, dysregulated nitric oxide (NO) homeostasis and oxidative abnormalities. Agents, such as hydroxyurea (HU), that increase levels of fetal hemoglobin (HbF), with consequent inhibition of HbS polymerization, and that may counteract other aspects of the disease's pathophysiology characterize important therapies for the disorder. Whilst HU therapy successfully augments HbF levels in many patients, not all patients respond to treatment. Studies demonstrate that HU, when degraded, produces nitric oxide (NO), in turn activating the enzyme, soluble guanylate cyclase (GC) in erythroid lineage cells. GC converts GTP to cGMP, which acts as a second messenger for NO, possibly activating the transcription of the ?-globin gene and, consequently, increasing the concentration of HbF. Due to evidence that NO bioavailability may be decreased in SCD individuals, recent research into SCD has focused on the role of NO in the disease, particularly since this gas is an important vasodilator. The objective of this study was to investigate which family(ies) of phosphodiesterases (PDE) may mediate the degradation of cGMP in erythroid lineage cells and whether drugs that inhibit these PDEs are capable of augmenting the production of ?-globin and, consequently, HbF in erythroid cells in culture. For this, cultures of K562 erythroleukemic cells were treated with different PDE inhibitors; vinpocetine, sildenafil citrate, dipyridamole and zaprinast. In addition, cultures were also treated with the guanylate cyclase stimulator, BAY41-2272. Increased levels of cGMP were observed principally in cells treated with 10µg/ml dipyridamole and at some time-points in the presence of 10 and 50µM vinpocetine. No changes in cGMP levels were observed in cells treated with sildenafil nor with zaprinast. However, cells treated with BAY 41-2272 demonstrated augmented cGMP levels at all concentrations used. With regard to relative ?-globin gene expression, dipyridamole, vinpocetine, sildenafil and zaprinast did not increase ?-globin gene expression; in contrast, BAY 41-2272 (60 and 300nM) stimulated increased ?-globin expression after 48h of culture. The gene expressions of PDEs 1, 5 and 9 were also studied in different hematopoietic cell types and other tissues. PDE9 is expressed, at significantly higher levels, in K562 cells (erythroleukemic cells), reticulocytes and neutrophils than in numerous other cell types tested (T98G, colon, mammary, ovary, testicle, liver, skin, spleen, uterus, limphonode). Furthermore, the expression of this PDE was even higher in the reticulocytes and neutrophils of patients with SCD. In patients on HU therapy, the expression of PDE9 in reticulocytes was reversed back to levels similar to those seen in controls. In contrast, PDE9 expression in neutrophils was similar in patients on or of HU therapy. Thus, since the increase in intracellular levels of cGMP may be important for the production of HbF in erythroid cells and also to inhibit leukocyte adhesion, the inhibition of the action of PDE9 may represent a novel drug target for cell-specific treatment of SCD<br>Mestrado<br>Ciencias Basicas<br>Mestre em Clinica Medica
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Sylvester, Karl Peter. "Pulmonary function abnormalities in children with sickle cell disease." Thesis, King's College London (University of London), 2005. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.424442.
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Bandeen, Timothy C. "Effects of sickle cell disease on growth of the craniofacial complexes. /." View the abstract Download the full-text PDF version View the full-text HTML version, 2005. http://etd.utmem.edu/ABSTRACTS/2005%5F001%5Fbandeen%5Findex.html.
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Thesis (M.S.)--University of Tennessee Health Sciences Center, 2005.<br>Spine title: Effects of sickle cell disease on growth of the craniofacial complexes. Appendices: leaves 162-414 Bibliography: leaves 145-161.
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McNaull, Stewart A. "Analysis of sickle erythrocyte adherence to endothelium in confined flow channels." Diss., Georgia Institute of Technology, 1999. http://hdl.handle.net/1853/11019.
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Siano, James P. "In vitro studies of intercellular interactions with pulmonary microvascular endothelium : involvement of cell adhesion, inflammatory mediators, and endothelial injury in sickle cell-related acute pulmonary complications." Diss., Georgia Institute of Technology, 1998. http://hdl.handle.net/1853/10154.
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Brown, Lola A. "The Effects of Sickle Erythrocytes on Endothelial Permeability." Thesis, Georgia Institute of Technology, 2005. http://hdl.handle.net/1853/6960.
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Sickle cell anemia is a hematological disorder that is caused by a single point mutation in the beta-globin chain of hemoglobin. It results in several complications related to the small and large vessels in patients with the disease. Large vessel complications include cerebral infarcts, which are observed in children under ten years old. The mechanism behind this complication is not completely understood.
It is the goal of this project to begin to understand the role sickle erythrocytes may play in causing endothelial dysfunction as a precursor to sickle related complications. The hypothesis of this work is that exposure of large vessel endothelium to sickle erythrocytes causes an increase in endothelial permeability through loosening of adherens junctions.
In the first goal of this work, bovine aortic endothelial cells (BAECs) are grown on coverslips and exposed to sickle erythrocytes for 5 minutes and either immediately fixed or incubated in 30 minutes and then fixed. Immunofluorescent studies labeling VE cadherin show changes in VE cadherin dynamics, suggesting sickle erythrocytes may be involved in this observation.
Next, BAECs were grown on transwell inserts and exposed to sickle erythrocytes for 5 minutes. The erythrocytes are washed off and the BAEC are incubated with 10,000 MW dextran conjugated to lucifer yellow or FITC-BSA or to determine BAEC permeability. When dextran is used as the test molecule, endothelial permeability did not show a significant change from baseline. However, when BSA is used as the test molecule, increases in endothelial permeability are observed. Explanations into the differences between the transport mechanisms of the two molecules are discussed.
These experiments show changes in VE cadherin localization due to sickle erythrocyte exposure. This may cause increases in endothelial permeability and an experimental model and preliminary studies are performed. This study provides potential mechanisms to explain the changes in VE cadherin localization and provide suggestions for further studies to test the effect of sickle erythrocytes on endothelial permeability. This work provides a strong foundation for continuing studies on the effects of sickle erythrocytes on endothelial dysfunction within the confines of sickle related complications.
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Stewart, Kai Anika Djenaba. "An examination of African American college students' knowledge and attitudes regarding sickle cell disease and sickle cell disease carrier testing a mixed methods study /." Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2007. https://www.mhsl.uab.edu/dt/2008r/stewart.pdf.
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Dimovski, Aleksandar Jovo. "Factors affecting the fetal hemoglobin levels in patients with sickle cell anemia and thalassemia." [Maastricht : Maastricht : Rijksuniversiteit Limburg] ; University Library, Maastricht University [Host], 1993. http://arno.unimaas.nl/show.cgi?fid=6583.
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Bezerra, Marcos Andre Cavalcanti. "Determinação das propriedades adesivas e funcionais em globulos vermelhos, neutrofilos e plaquetas de pacientes com hemoglobinopatia SC, S/Beta talassemia e talassemia intermediaria." [s.n.], 2009. http://repositorio.unicamp.br/jspui/handle/REPOSIP/309318.
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Orientador: Fernando Ferreira Costa<br>Tese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias Medicas<br>Made available in DSpace on 2018-08-13T14:17:07Z (GMT). No. of bitstreams: 1
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Previous issue date: 2009<br>Resumo: O estado inflamatório crônico que ocorre na doença falciforme (DF) é decorrente
de diversos fatores que se interligam e se retroalimentam, formando um ciclo inflamatório
permanente. Estudos prévios sobre as propriedades adesivas e funcionais das células são
restritos à forma homozigota do gene da HbS (SS). No entanto, trabalhos que avaliem essas
características celulares, que possam contribuir para o esclarecimento da heterogeneidade
clínica em outros grupos de DF e talassemia intermediária (TI), são escassos na literatura.
O objetivo desse trabalho foi avaliar a capacidade adesiva de células vermelhas, neutrófilos
e plaquetas, quimiotaxia dos neutrófilos, os níveis plasmáticos das citocinas inflamatórias e
o stress oxidativo na DF e ß-TI. Dessa maneira foram selecionados pacientes com DF: SS
(n=20), SC (n=20), 3 grupos com S/ß talassemia - S/ß-IVS-I-6 (T->C) (n=17), S/ß-IVS-I-5
(G->C) (n=16), S/ß-Códon39 (C??T) (n=12) - e TI homozigotos para a mutação IVS-I-6
(T->C) (n=20), acompanhados na Fundação HEMOPE. As técnicas utilizadas no
desenvolvimento desse estudo foram: ensaio de quimiotaxia (ChemoTx) para avaliação da
capacidade migratória dos neutrófilos, adesão estática para determinação da adesão basal
das células, citometria de fluxo para avaliar a produção de ROS e a expressão de moléculas
de adesão, e ELISA para determinar a atividade da superóxido dismutase (SOD) e a
dosagem das citocinas. Para avaliação de nossos resultados, todos os grupos de pacientes
foram comparados ao grupo controle AA: a adesão de neutrófilos e plaquetas mostrou-se
significativamente aumentada em todos dos grupos de DF; somente os grupos SS e SC
apresentaram capacidade quimiotática significativamente aumentada, assim como a adesão
de células vermelhas e a expressão das moléculas de adesão CD36 e CD49d. Os níveis
plasmáticos das citocinas IL1-ß, IL-6, IL-8 e TNF-a mostraram-se significativamente
aumentados no grupo SS, e nos demais grupos de DF apresentaram uma distribuição
heterogênea. Observamos um aumento significativo na produção de ROS em células
vermelhas, mononucleares e neutrófilos de todos os grupos de DF, exceto para os grupos
S/ß nas células vermelhas. Além disso, a atividade plasmática da SOD mostrou-se reduzida
nos grupos SS, SC e S/ß-Cd39. A adesão dos neutrófilos, células vermelhas e plaquetas foi
significativamente maior nos pacientes com TI, assim como a expressão das moléculas
CD36 e CD49d nas células vermelhas e a capacidade quimiotática dos neutrófilos. Nesses pacientes, a produção de ROS e os níveis plasmáticos das citocinas foram
significativamente aumentados, enquanto que a atividade enzimática da SOD foi
significativamente reduzida. Apesar da doença SC possuir uma clínica mais branda, nossos
dados sugerem que neutrófilos, glóbulos vermelhos e plaquetas desses pacientes possuem
características adesivas e quimiotáticas semelhantes às encontradas nas células de pacientes
com AF. Além disso, nossos resultados sugerem que quanto maiores os níveis de HbA na
S/ß talassemia, menor a adesão de neutrófilos, células vermelhas e plaquetas, podendo
explicar as diferenças clínicas encontradas nesses pacientes em função do genótipo. É
possível que o aumento da aderência, da capacidade quimiotática, da produção de ROS, das
citocinas e diminuição do mecanismo antioxidante, observados neste estudo, contribuam
nas complicações clínicas encontradas na ß-TI, tais como hipertensão pulmonar e úlceras
de perna. Estudos adicionais podem contribuir para o entendimento das diferenças na
apresentação clínica desses pacientes.<br>Abstract: The chronic inflammatory state that occurs in sickle cell disease (SCD) is due to
several factors that are interlinked and feeds back to a permanent inflammatory cycle.
Previous studies about the adhesive properties and functional cells are restricted to the
homozygous form of the HbS gene (SS). However, studies that assess the cellular
characteristics that may contribute to the clarification of clinical heterogeneity in other
groups of SCD and thalassemia intermedia (TI), are scarce in the literature. The aim of this
study was to evaluate the adhesive properties of red blood cells (RBC), platelets and
neutrophils (NS), NS chemotaxis, inflammatory cytokine plasma levels and oxidative stress
in SCD and ß-TI patients. Thus, we selected patients with different SCD genotypes: SS
(n=20), SC (n=20), three groups of HbS/ß thalassemia - HbS/ß39 (C->T) (n=12), HbS/IVSI-
5 (G->C) (n=16), and HbS/IVS-I-6 (T->C) (n=17) - and patients with homozygous
thalassemia intermedia IVS-I-6 (T->C) - followed regularly at the Fundação HEMOPE -
Brazil. The techniques used in the development of this study were: chemotaxis assay
(ChemoTx) to evaluate the migratory ability of neutrophils, basal adhesion was compared
using static adhesion assays, flow cytometry to assess the production of ROS and
expression of adhesion molecules, and ELISA to determine the superoxide dismutase
(SOD) activity and cytokine plasma levels. For evaluation of our results, all groups of
patients were compared with the AA control group: the neutrophil and platelet adhesions
were significantly increased in all groups of SCD, only the SS and SC groups showed
significant increase in the chemotactic ability, as well as the RBC adhesion and the
expression of adhesion molecules CD36 and CD49d. All the SCD groups investigated
showed an increase in IL-6 plasma levels. IL1-ß levels were significantly higher in the
S/ß-IVS-I-5 (G??C), S/ß-Cd39 and SS groups. Plasma levels of IL-8 were increased only
in the SS and SC groups, however TNF-a levels were significantly higher in SS and the
three groups with HbS-ß thalassemia. NS and mononuclear cell ROS production was
significantly increased in all SCD groups, however red blood ROS production was higher
only in the SS and SC groups. Moreover, the SOD plasma activity was shown to be reduced
in groups SS, SC and S/ß-Cd39. The NS, RBC and platelets adhesion was significantly
higher in TI patients, as well as the expression of molecules CD36 and CD49d in RBC and chemotactic ability of NS. In these patients, the ROS production and cytokines plasma
levels were significantly increased, while SOD plasma activity was significantly reduced.
Although SC disease has a milder clinical manifestations, our data suggest that
NS, RBC and platelets of these patients have chemotactic and adhesive characteristics
similar to those found in cells of patients with SS. Moreover, our results suggest that the
higher levels of HbA in S/ß thalassemia reduce the NS, RBC and platelets adhesion and
may explain the clinical differences found in these patients, according to genotype. It is
possible that the increase in the cell adherence, chemotactic capacity, ROS production, of
cytokines levels and the reduction in antioxidant mechanism, observed in this study,
contribute to several clinical complications of ß-TI, such as pulmonary hypertension and
leg ulcers. Further studies may contribute to our understanding of the differences in the
clinical presentation of these patients.<br>Doutorado<br>Medicina Experimental<br>Doutor em Fisiopatologia Medica
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Sherrill, Amy Whiteman. "Influence of sickle erythrocytes on arterial endothelial elongation and alignment in response to shear stress." Thesis, Georgia Institute of Technology, 1995. http://hdl.handle.net/1853/11151.
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Kumar, Anjali. "In vitro studies of receptor-mediated adherence of sickle erythrocytes under flow to vascular endothelium : role of inflammatory mediators." Diss., Georgia Institute of Technology, 1995. http://hdl.handle.net/1853/11764.
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Moreira, Juliane Almeida. "Impacto dos haplÃtipos do gene ΒS sobre os marcadores de hemÃlise em pacientes com anemia falciforme em estado basal." Universidade Federal do CearÃ, 2013. http://www.teses.ufc.br/tde_busca/arquivo.php?codArquivo=12359.
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CoordenaÃÃo de AperfeÃoamento de Pessoal de NÃvel Superior<br>A anemia falciforme (AF) Ã uma doenÃa hereditÃria resultante de uma mutaÃÃo pontual (GAG  GTG) no cÃdon do gene da βS - globina, levando a uma substituiÃÃo de Ãcido glutÃmico por valina na sexta posiÃÃo da cadeia polipeptÃdica, gerando uma hemoglobina (Hb) anormal denominada de HbS, em homozigose. A AF se caracteriza por anemia hemolÃtica crÃnica associada a mÃltiplos eventos tais como processo inflamatÃrio crÃnico, aumento do estresse oxidativo, dano endotelial, diminuiÃÃo da biodisponibilidade do Ãxido nÃtrico (NO), ativaÃÃo da coagulaÃÃo, dentre outros. Os biomarcadores de hemÃlise tais como: contagem de reticulÃcitos, lactato desidrogenase (LDH), Ãcido Ãrico e arginase I sÃo fundamentais na avaliaÃÃo do grau da hemÃlise, principalmente, de natureza intravascular contribuindo com o monitoramento da anemia nesses pacientes. O presente estudo teve como objetivo avaliar o impacto dos haplÃtipos do gene βS sobre os marcadores de hemÃlise em adultos com AF em estado basal, acompanhados no ambulatÃrio do serviÃo de hematologia no Hospital UniversitÃrio Walter CantÃdio (HUWC). Um total de 50 pacientes adultos com AF foi selecionado, com diagnÃstico confirmado por estudo molecular. Os pacientes se encontravam em uso de hidroxiurÃia (HU), dose variando de 500 mg a 1,5 g/kg/dia por no mÃnimo seis meses. Um grupo controle foi elaborado, sendo constituÃdo por 20 indivÃduos supostamente saudÃveis. Foram coletados 10 mL de sangue venoso em tubo de coleta a vÃcuo, contendo o anticoagulante EDTA (etileno-diamino-tetracÃtico), para a realizaÃÃo da contagem de reticulÃcitos e 6 mL de sangue venoso em tubo de coleta a vÃcuo contendo gel separador, sem anticoagulante, para as dosagens sÃricas de LDH , Ãcido Ãrico e arginase I. As variÃveis idade, sexo, dosagem e tempo de uso do medicamento, concentraÃÃo da Hb e da hemoglobina fetal (HbF) foram obtidas nos prontuÃrios mÃdicos no momento da realizaÃÃo do estudo. As anÃlises estatÃsticas foram realizadas no programa GraphPad Prism (versÃo 5.0) e o nÃvel de significÃncia estabelecido foi p < 0,05. Foi verificado aumento significante nos nÃveis de reticulÃcitos, LDH, Ãcido Ãrico e arginase I nos pacientes com AF em relaÃÃo ao grupo controle (p < 0,05). Foi observada diferenÃa significativa na Hb nos grupos Bantu/Benin em relaÃÃo aos demais haplÃtipos do cluster da βs-globina. Os nÃveis de HbF apresentaram uma tendÃncia a aumento no haplÃtipo Benin/Benin em relaÃÃo aos demais. Foi verificada uma tendÃncia no aumento de LDH no genÃtipo Bantu/Bantu em comparaÃÃo com os demais haplÃtipos. Foi observada diferenÃa significativa da arginase I entre os grupos Bantu/Bantu vs Bantu/Benin e Bantu/Bantu vs Benin/Benin. Os resultados do presente estudo reforÃam a hipÃtese de que a arginase I possa ser utilizada como possÃvel indicador de gravidade uma vez que a mesma foi associada ao haplÃtipo Bantu.
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Lockamy, Virginia Lee. "The search for the mechanism of nitric oxide release in hydroxyurea therapy /." Electronic thesis, 2004. http://etd.wfu.edu/theses/available/etd-05182004-115414/.
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Amos, Amanda Owings. "Regulation of cytokine-induced adhesion molecule expression and sickle erythrocyte adhesion to microvascular endothelial cells by intracellular adenosine 3',5'-cyclic monophosphate and nitric oxide." Diss., Available online, Georgia Institute of Technology, 2006, 2006. http://etd.gatech.edu/theses/available/etd-04052006-104355/.
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Thesis (Ph. D.)--Chemical and Biomolecular Engineering, Georgia Institute of Technology, 2006.<br>Dr. Peter A. Lane, Committee Member ; Dr. Larry V. McIntire, Committee Member ; Dr. Ronald W. Rousseau, Committee Member ; Dr. James R. Eckman, Committee Member ; Dr. Timothy M. Wick, Committee Chair.
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Santiago, Thiago de Melo. "Estudo de propriedades mecânicas e morfológicas de hemácias em indivíduos portadores de anemia falciforme por microscopia de força atômica." reponame:Repositório Institucional da UFC, 2012. http://www.repositorio.ufc.br/handle/riufc/13627.
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SANTIAGO, Thiago de Melo. Estudo de propriedades mecânicas e morfológicas de hemácias em indivíduos portadores de anemia falciforme por microscopia de força atômica. 2012. 117 f. Dissertação (Mestrado em Física) - Programa de Pós-Graduação em Física, Departamento de Física, Centro de Ciências, Universidade Federal do Ceará, Fortaleza, 2012.<br>Submitted by Edvander Pires (edvanderpires@gmail.com) on 2015-10-16T20:53:51Z
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Previous issue date: 2012<br>Nesse trabalho, propomos o uso da Microscopia de Força Atômica para a investigação das propriedades mecânicas e morfológicas dos eritrócitos de pacientes com anemia falciforme, visando propor que parâmetros físicos tais como curvatura, rugosidade, elasticidade e viscosidade, possam ser utilizados na investigação laboratorial dessa doença. Em cada etapa experimental, foram medidas as propriedades mecânicas de cinco células por indivíduo. Nessa pesquisa analisamos o sangue de cinco pacientes em tratamento no Centro de Hematologia e Hemoterapia do Estado do Ceará (HEMOCE) e, como controle, cinco doadores sadios voluntários. Os resultados indicam a diminuição da rugosidade da superfície citoplasmática da membrana eritrocitária dos doadores com a doença. A análise de elasticidade das hemácias dos pacientes com anemia indica que módulo de Young, em média, é cerca de três vezes maior do que o módulo encontrado nas hemácias de doadores sadios. A análise da histerese de curvas de forças sobre os eritrócitos revela que as hemácias contendo a hemoglobina anormal dissipam menos energia e, portanto, são mais viscosas que as hemácias advindas de doadores sadios.
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Silva, Lilianne Brito da. "CaracterizaÃÃo clÃnica, hematolÃgica e molecular dos pacientes com anemia falciforme em Fortaleza, CearÃ." Universidade Federal do CearÃ, 2009. http://www.teses.ufc.br/tde_busca/arquivo.php?codArquivo=3637.
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Conselho Nacional de Desenvolvimento CientÃfico e TecnolÃgico<br>IntroduÃÃo: A anemia falciforme à o resultado de uma mutaÃÃo pontual (GAGGTG) no cÃdon do gene da globina β, conduzindo a uma substituiÃÃo de Ãcido glutÃmico por valina na sexta posiÃÃo da cadeia polipeptÃdica. A anemia falciforme apresenta manifestaÃÃes clÃnicas heterogÃneas, que podem ser relacionadas ao tipo de haplÃtipo associado ao gene da HbS e aos nÃveis de HbF. Objetivo: CaracterizaÃÃo clÃnica, hematolÃgica e molecular dos pacientes com anemia falciforme em Fortaleza, CearÃ. Metodologia: Foram analisados 47 pacientes com anemia falciforme, adultos e de ambos os sexos. A determinaÃÃo dos valores hematolÃgicos foi realizada em contador automÃtico de cÃlulas sangÃÃneas; a determinaÃÃo da presenÃa de HbSS foi realizada por eletroforese em pH alcalino em fitas de acetato de celulose e por eletroforese de diferenciaÃÃo em Ãgar-fostato pH 6.2; os nÃveis de HbF foram determinados pela tÃcnica da desnaturaÃÃo alcalina; e a anÃlise dos haplÃtipos da mutaÃÃo ÃS foi realizada por meio da tÃcnica da reaÃÃo em cadeia mediada pela polimerase para polimorfismo dos comprimentos dos fragmentos de restriÃÃo (PCR-RFLP). As anÃlises estatÃsticas foram desenvolvidas no programa GraphPad Prism (versÃo 5.0) e o nÃvel de significÃncia estabelecido foi p < 0,05. Resultados: A distribuiÃÃo dos haplÃtipos do gene da βS-globina, 63% do tipo Bantu, 25% do tipo Benin e 12% do tipo AtÃpico, està em conformidade com a observada para a populaÃÃo brasileira, em que o haplÃtipo Bantu à o mais prevalente, seguido pelo Benin e Senegal. NÃo houve diferenÃa significativa entre o presente estudo e os resultados encontrados no Rio de Janeiro, Porto Alegre, Campinas e RibeirÃo Preto; porÃm uma diferenÃa significativa foi observada quando o estudo foi comparado aos resultados obtidos em Salvador, BelÃm, Amazonas e por outros pesquisadores no CearÃ. A distribuiÃÃo das freqÃÃncias dos haplÃtipos do gene da βS-globina nos diferentes estudos està condizente com a histÃria da formaÃÃo da populaÃÃo brasileira, exceto nos resultados do estudo anterior realizado no CearÃ, que obteve o haplÃtipo Benin com maior prevalÃncia. Conforme os dados histÃricos sobre as origens da populaÃÃo negra trazida ao estado do CearÃ, o haplÃtipo Bantu seria o mais prevalente. Na comparaÃÃo entre os haplÃtipos e as caracterÃsticas hematolÃgicas estudadas, apenas os valores de HbF e Ht apresentaram diferenÃa estatisticamente significativa. Os nÃveis de HbF foram maiores no haplÃtipo Benin, seguido do haplÃtipo Bantu, o que està em conformidade com os dados da literatura. Foi demonstrada maior presenÃa de crises vaso-oclusivas e episÃdios de pneumonia no haplÃtipo Benin/AtÃpico do que no haplÃtipo Bantu/AtÃpico; e maior presenÃa de crises de infecÃÃo urinÃria no haplÃtipo Benin/AtÃpico do que no haplÃtipo Benin/Benin. NÃo houve diferenÃa estatisticamente significativa entre os haplÃtipos Bantu/Bantu e Benin/Benin em relaÃÃo Ãs complicaÃÃes clÃnicas, entretanto foi observado que o haplÃtipo Bantu/Bantu tem uma maior freqÃÃncia em todos os eventos clÃnicos estudados quando comparado ao Benin/Benin. Dentre os resultados foi demonstrada uma tendÃncia de menor nÃmero de pacientes com crises vasos-oclusivas e Ãlceras de perna com o aumento dos nÃveis de HbF. NÃo houve diferenÃa estatisticamente significativa na comparaÃÃo entre os nÃveis de HbF e os valores de HemÃcias, Hemoglobina, HematÃcrito, LeucÃcitos e Plaquetas. ConclusÃes: A determinaÃÃo dos haplÃtipos da anemia falciforme à de grande importÃncia nÃo sà para o acompanhamento e prognÃstico dos pacientes, como tambÃm como ferramenta para estudos antropolÃgicos que contribuam no esclarecimento da origem dos africanos que tanto contribuÃram na formaÃÃo etnolÃgica, econÃmica, cultural e social do Brasil.<br>Introduction: The sickle cell anemia is the result of a point mutation in the β-globin gene, leading to a substitution of glutamic acid by valine at the sixth position of the polypeptide chain. The sickle cell anemia presents heterogeneous clinical manifestations, which may be related to the type of haplotype associated with the gene for HbS and HbF levels. Objective: Clinical characterization, molecular and haematological patients with sickle cell anemia in Fortaleza, CearÃ. Methods: We analyzed 47 patients with sickle cell anemia, adults of both sexes. The determination of hematological values was performed on blood cells automated meter; the determination of the presence of HbSS was performed by alkaline hemoglobin electrophoresis on cellulose acetate tapes and by differentiation electrophoresis on agar-phosphate pH 6.2; the levels of HbF were determined by alkali denaturation technique; and the analysis of the haplotypes of the ÃS mutation was done by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Statistical analysis was developed in the program GraphPad Prism (version 5.0) and the level of significance was set p <0.05. Results: The distribution of the haplotypes of βS-globin gene - 63% of the Bantu type, 25% of the Benin type and 12% atypical â was in conformity with that observed for the entire Brazilian population, in which the Bantu haplotype is most prevalent, followed by the Benin and Senegal. There was no significant difference between the results found in this study and those found for the cities of Rio de Janeiro, Porto Alegre, Campinas and RibeirÃo Preto; but there was a significant difference with the results obtained for the cities of Salvador and BelÃm and the state of Amazonas, and by other researchers in CearÃ. The distribution of haplotype frequencies of the βS-globin gene in the different studies is in line with the history of the formation of the Brazilian population, except for the results of a previous study carried out in CearÃ, in which the Benin haplotype was found to be most prevalent. According to the historical information on the origins of the slave population brought to CearÃ, the Bantu haplotype should be the most prevalent. In the comparison between the haplotypes and the haematological characteristics studied, only the values of HbF and Ht showed statistically significant difference. The levels of HbF were higher in the Benin haplotype, followed by the Bantu haplotype, which is in accordance with the literature. Was demonstrated greater presence of painful episodes and episodes of pneumonia in Benin haplotype/Atypical haplotype than in Bantu/Atypical and increased presence of urinary infection crises in Benin haplotype/Atypical haplotype than in Benin/Benin. There was no statistically significant difference between the haplotypes Bantu/Bantu and Benin/Benin for clinical complications, however it was observed that the haplotype Bantu/Bantu has a higher frequency in all studied clinical events when compared to Benin/Benin. Among the results was shown a trend of fewer patients with painful episodes and of leg ulcers with increased levels of HbF. There was no statistically significant difference in the comparison between the levels of HbF and the values of red blood cells, hemoglobin, hematocrit, leukocytes and platelets. Conclusions: The determination of haplotypes of sickle cell anemia is of great importance not only for monitoring and prognosis of patients, but also as a tool for anthropological studies which help in clarifying the origin of Africans who have contributed so much in training ethnological, economic, cultural and social Brazil.
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Shimauti, Eliana Litsuko Tomimatsu [UNESP]. "Capacidade antioxidante na Hb S: influência de talassemia alfa, haplótipos do gene βS globina e melatonina". Universidade Estadual Paulista (UNESP), 2011. http://hdl.handle.net/11449/102743.
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shimauti_elt_dr_sjrp.pdf: 703318 bytes, checksum: bf063f504644666407e22683da45d271 (MD5)<br>A anemia falciforme (Hb SS), caracterizada por estados de inflamação crônica e eventos de agressão isquêmica e reperfusão pode causar danos oxidativos às macromoléculas biológicas. Os genes epistáticos e os haplótipos do gene da globina βS são possíveis moduladores de variabilidade fenotípica na Hb SS. A melatonina pode atenuar o estresse oxidativo e abrandar a morbidade. O objetivo deste foi analisar a influência de talassemia alfa (-α3.7), haplótipos do gene βS e melatonina na capacidade antioxidante total e expressão fenotípica, em indivíduos com Hb SS e Hb AS. Para avaliar o nível de melatonina sérica foram selecionadas 15 amostras de pacientes com Hb SS e 24 com Hb AA da região noroeste do estado do Paraná (NOPR). A triagem das hemoglobinas foram realizadas por métodos eletroforéticos e cromatográfico, e a caracterização genotípica da Hb S por meio de PCR- RFLP e PCR-AE. As espécies reativas de ácido tiobarbitúrico (TBARS), e a capacidade antioxidante total em equivalência ao Trolox (TEAC) foram determinadas por meio da espectrofotometria. A melatonina foi estimada por meio de HPLC. O estado redox foi analisado selecionando amostras de 68 indivíduos com Hb SS, 53 com Hb AS e 149 com Hb AA, das regiões sul e sudeste do Brasil. Para analisar a influência dos genes epistáticos sobre a TEAC, melatonina e expressão fenotípica, foram selecionadas 17 amostras com Hb SS, 30 com Hb AS, e 30 com Hb AA, da NOPR. O critério para elegebilidade foi ser não fumantes não gestantes, não etilistas e estar na fase estável para os indivíduos com HbSS. Os haplótipos do gene βS e a talassemia -α3.7 foram identificados por meio de PCR-RFLP e PCR multiplex, respectivamente. Os níveis séricos de melatonina apresentaram-se reduzidos nos indivíduos com Hb SS (P<0,001), e os TBARS e TEAC elevados quando comparados a grupo controle (P<0,001 e P<0,01 respectivamente)...<br>Sickle cell disease (Hb SS), characterized by chronic inflammation and ischemic damage episodes and reperfusion, comprises excessive production of free radicals which can cause oxidative damage to biologic macromolecules. The epistatic genes and the polymorphism of the βS globin gene region are possible modulators of phenotypical variability of sickle cell disease in Hb SS. Melatonin can diminish both the oxidative stress and morbidity. The objective of this study was to analyze the influence of -α-3.7 thalassemia and from βS gene haplotypes on total anti-oxidant capacity, melatonin serum levels and phenotypical expression in individuals with Hb SS and Hb AS. To evaluate melatonin serum level 15 samples with HbSS and 24 with Hb AA from the northwest region of Paraná state (NOPR). The hemoglobin screenings were performed by electrophoretic and chromatographic methods and genotypic characterization of Hb S by PCR- RFLP and PCR-AE. The concentrations of thiobarbituric acid reactive substances (TBARS), used as markers of oxidative stress and total antioxidant capacity in equivalence to Trolox (TEAC), were determined by spectrophotometry. Melatonin was estimated by HPLC. The redox state was analyzed through selecting 68 individuals with Hb SS, 53 with Hb AS and 149 with Hb AA from Brazil’s south and southeast regions. To analyze the influence of epistatic genes on TEAC, melatonin and phenotypical expression, there were selected 17 samples with Hb SS, 30 with Hb AS, and 30 with Hb AA from NOPR. The βS gene haplotypes and -α-3.7 thalassemia were identified through RFLP-PCR and multiplex PCR, respectively. The melatonin serum levels appeared significantly reduced in individuals with Hb SS (P<0.001); and the TBARS and TEAC appeared significantly increased when compared to the control group (P<0.001 and P<0.01, respectively). The correlation between TBARS and TEAC (r=0.51; P=0.04)... (Complete abstract click electronic access below)
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Shimauti, Eliana Litsuko Tomimatsu. "Capacidade antioxidante na Hb S : influência de talassemia alfa, haplótipos do gene βS globina e melatonina /". São José do Rio Preto : [s.n.], 2011. http://hdl.handle.net/11449/102743.
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Resumo: A anemia falciforme (Hb SS), caracterizada por estados de inflamação crônica e eventos de agressão isquêmica e reperfusão pode causar danos oxidativos às macromoléculas biológicas. Os genes epistáticos e os haplótipos do gene da globina βS são possíveis moduladores de variabilidade fenotípica na Hb SS. A melatonina pode atenuar o estresse oxidativo e abrandar a morbidade. O objetivo deste foi analisar a influência de talassemia alfa (-α3.7), haplótipos do gene βS e melatonina na capacidade antioxidante total e expressão fenotípica, em indivíduos com Hb SS e Hb AS. Para avaliar o nível de melatonina sérica foram selecionadas 15 amostras de pacientes com Hb SS e 24 com Hb AA da região noroeste do estado do Paraná (NOPR). A triagem das hemoglobinas foram realizadas por métodos eletroforéticos e cromatográfico, e a caracterização genotípica da Hb S por meio de PCR- RFLP e PCR-AE. As espécies reativas de ácido tiobarbitúrico (TBARS), e a capacidade antioxidante total em equivalência ao Trolox (TEAC) foram determinadas por meio da espectrofotometria. A melatonina foi estimada por meio de HPLC. O estado redox foi analisado selecionando amostras de 68 indivíduos com Hb SS, 53 com Hb AS e 149 com Hb AA, das regiões sul e sudeste do Brasil. Para analisar a influência dos genes epistáticos sobre a TEAC, melatonina e expressão fenotípica, foram selecionadas 17 amostras com Hb SS, 30 com Hb AS, e 30 com Hb AA, da NOPR. O critério para elegebilidade foi ser não fumantes não gestantes, não etilistas e estar na fase estável para os indivíduos com HbSS. Os haplótipos do gene βS e a talassemia -α3.7 foram identificados por meio de PCR-RFLP e PCR multiplex, respectivamente. Os níveis séricos de melatonina apresentaram-se reduzidos nos indivíduos com Hb SS (P<0,001), e os TBARS e TEAC elevados quando comparados a grupo controle (P<0,001 e P<0,01 respectivamente)... (Resumo completo, clicar acesso eletrônico abaixo)<br>Abstract: Sickle cell disease (Hb SS), characterized by chronic inflammation and ischemic damage episodes and reperfusion, comprises excessive production of free radicals which can cause oxidative damage to biologic macromolecules. The epistatic genes and the polymorphism of the βS globin gene region are possible modulators of phenotypical variability of sickle cell disease in Hb SS. Melatonin can diminish both the oxidative stress and morbidity. The objective of this study was to analyze the influence of -α-3.7 thalassemia and from βS gene haplotypes on total anti-oxidant capacity, melatonin serum levels and phenotypical expression in individuals with Hb SS and Hb AS. To evaluate melatonin serum level 15 samples with HbSS and 24 with Hb AA from the northwest region of Paraná state (NOPR). The hemoglobin screenings were performed by electrophoretic and chromatographic methods and genotypic characterization of Hb S by PCR- RFLP and PCR-AE. The concentrations of thiobarbituric acid reactive substances (TBARS), used as markers of oxidative stress and total antioxidant capacity in equivalence to Trolox (TEAC), were determined by spectrophotometry. Melatonin was estimated by HPLC. The redox state was analyzed through selecting 68 individuals with Hb SS, 53 with Hb AS and 149 with Hb AA from Brazil's south and southeast regions. To analyze the influence of epistatic genes on TEAC, melatonin and phenotypical expression, there were selected 17 samples with Hb SS, 30 with Hb AS, and 30 with Hb AA from NOPR. The βS gene haplotypes and -α-3.7 thalassemia were identified through RFLP-PCR and multiplex PCR, respectively. The melatonin serum levels appeared significantly reduced in individuals with Hb SS (P<0.001); and the TBARS and TEAC appeared significantly increased when compared to the control group (P<0.001 and P<0.01, respectively). The correlation between TBARS and TEAC (r=0.51; P=0.04)... (Complete abstract click electronic access below)<br>Orientador: Claudia Regina Bonini Domingos<br>Coorientador: Eduardo Alves de Almeida<br>Banca: Hermione Elly Melara de Campos Bicudo<br>Banca: Paulo César Naoum<br>Banca: Isaac Lima da Silva Filho<br>Banca: Octavio Ricci Junior<br>Doutor