Navegando por Assunto "Anemia falciforme"

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Acesso aberto (Open Access)
Doença como experiência: as relações entre vulnerabilidade social e corpo doente enquanto fenômeno biocultural no estado do Pará
(Universidade Federal do Pará, 2012-06-11) SILVA, Ariana Kelly Leandra Silva da; SILVA, Hilton Pereira da; http://lattes.cnpq.br/3917171307194821
This study examines the biosocial representation of individuals with Sickle Cell Anemia (SCA) in Pará State, Brazil, considering that this as a biocultural phenomenon, involving evolutionary, genetic, environmental, socioeconomic and cultural aspects of their daily life. The research deals with the sociability and the health issues of forty people with SCA, representing 10% of the States' cases, contacted in the Fundação Hemopa, Belém, the reference center for blood disorders in the State. The research encompasses their routine situation of social vulnerability, their perceptions of Health and Disease, treatments (Western and Folk Medicine), diagnostic, stigmas, prejudices, taboos and difficulties of access and accessibility to the services of SUS (the Brazilian National Health System). A comprehensive qualitative methodology and content analysis were used to understand the experience of persons who live daily with the instability and complexity of the disease. The personal experience of disease was uncovered though the formal conversations/interviews about the origins of the biological heritage, social relations, family entanglements and extra-familial dimensions of the individuals' in question, focusing on the evolution of SCA, especially considering the physical and psychological pain and other health complications experienced by the study's participants. The habitus in relation to their life ways is a category which includes the perception of the ethnic/racial nature of SCA, still perceived as "a disease that comes from the black people" with all its associated taboos. I conclude suggesting that many impacts of SCA are linked to the Social Determinants of Health and that there are important differences in relation to the susceptibilities of the persons, with many biosocial layers that require greater awareness by the political, clinical and primary care institutions responsible for the care of the affected citizens.
Acesso aberto (Open Access)
Importância da avaliação da hemoglobina fetal na clínica da anemia falciforme
(2008-04) RIBEIRO, Rita de Cassia Mousinho; CARDOSO, Greice de Lemos; SOUSA, Ítallo Esteves Lacerda de; MARTINS, Priscila Kelly Cavalcante
Sickle cell disease is one of the commonest and most studied genetic diseases in the world. Caused by a mutation of the β gene, it changes the molecular structure of hemoglobin. Abnormal Hb S molecules suffer polymerization physiologically provoked by a low oxygen tension, acidosis and dehydration. As a result, red blood cells take on a sickle cell form, which causes microvascular occlusion with varying consequences. The objective of this study was to review the importance of fetal hemoglobin in the clinical assessment of sickle cell disease patients. It has been shown that the association of high levels of fetal hemoglobin with sickle cell disease is favorable in hematological terms. In this interaction, F cells have low Hb S concentrations and thus inhibit Hb S polymerization and the morphological alteration of red blood cells. Treatment with hydroxyurea resulting in an increased fetal hemoglobin expression brings about a significant improvement in the patient's clinical state. Thus, fetal hemoglobin constitutes the greatest inhibitor of desoxi-Hb S polymerization and avoids the morphological alteration of red blood cells, chronic hemolytic anemia, painful microvascular occlusive crises, bone infarction and necrosis of several organs thereby improving the clinical outcome and the patients' life expectancy.
Acesso aberto (Open Access)
Inherited hemoglobin disorders in an Afro-Amazonian community: Saracura
(2012-07) CARDOSO, Greice de Lemos; TAKANASHI, Silvania Yukiko Lins; GUERREIRO, João Farias
The most common hemoglobinopathies, viz, hemoglobins S and C, and α-and β-thalassemias, were investigated through the molecular screening of 116 subjects from the community of Saracura, comprising fugitive African slaves from farms of the municipality of Santarém, in the west of Pará State, Brazilian Amazon. The observed frequency of the HBB*S gene (0.9%) was significantly lower than that encountered in other Afro-derived communities in the region. Concomitantly, the absence of the HBB*C allele has been reported for most of the Afro-Amazonian communities thus far studied. As remnant populations of quilombos are generally small, the heterogeneous distribution of HBB*S and HBB*C alleles among them is probably due to genetic drift and/or founder effect. The observed frequency of 3.7 kb deletion in Saracura (8.5%) was consistent with the African origin of the population, with a certain degree of local differentiation and admixture with individuals of Caucasian ancestry, placed in evidence by the occurrence of - -(MED) deletion (1.2%), a common mutation in Mediterranean regions. As regards f-thalassemia, among the seven different mutations found in Saracura, three βºand two β+ mutations were of Mediterranean origin, and two β+ of African. Thus, only 28% of the local β-thalassemia mutations found in Saracura were of African origin.
Acesso aberto (Open Access)
Manifestações retinianas em pacientes portadores de anemia de células falciformes
(Universidade Federal do Pará, 2011-10) ALMEIDA SOBRINHO, Edmundo Frota de; SARAIVA FILHO, João Carlos de Pina; SILVA, James Nunes da; SILVA, Ana Paula Siqueira da; LIMA, Rafaela Cavalcante de
The World Health Organization counts that more than 5% of the world's population carry some type of hemoglobinopathy. Among them we find the sickle cell aneamia that presents its main harmful effect on the peripheral vasculature. In the retina, the falciform lesions have a well defined physiopathology and classification. To identify the retinal manifestations caused by the falciform aneamia in patients directed to the Bettina Ferro de Souza Hospital from the Hemocenter of the State of Pará – HEMOPA, in the months of September through December of 2006. METHODS: Ophthalmologic examination was performed in the Department of Ophthalmology of the Bettina Ferro de Souza Hospital. and fifty patients with sickle cell aneamia (SS or SC) followed by the department of falciform aneamia of the Hematologia e Hemoterapia do Pará - HEMOPA have been randomly selected and submitted to interview in order to register their: sex; age; color; genotype; ocular alterations; medicines taken. Ophthalmologic examination was performed in the Department of Ophthalmology of the Bettina Ferro de Souza Hospital. It consisted of: evaluation of the acuity of vision with and without correction, indirect biomicroscopy, indirect binocular ophthalmoscopy, and, in case the latter presented alterations, a complementary study witangiofluoresceinography would be carried through. RESULTS: The outcomes have been registered in research protocol and later submitted to the statistic analysis using the Qui-square test, adopting, as level of significance, p<0,05. Eighty-eight percent of the patients did not present any falciform retinal sign, 3% presented peripheral vascular occlusion; in 2%, pigmented plate was shown, and 7% presented injuries which were not compatible with the falciform disease; there was a proportionality of 50% for males and females; there was a higher predominance (38%) of people aged from 11 and 15; 74% had SS genotype and 26% SC genotype; in relation to the use of medicines there was a higher prevalence of ocular alterations in patients who had made use of the folic acid isolated, with 5%, in contrast with those who had used the hidroxyurea association and folic acid, when all (27%) presented normal fundoscopic examination; all the patients (29%) that showed fetal hemoglobin rate above 10% had retina without alterations. CONCLUSION: Few cases of retinal signs have been observed in the studied group, however this research does not diminish the importance of early and periodic ophthalmologic examination, since falciform retinopathy is largely registered and its complications can lead to amaurosis.