Navegando por Assunto "Hidroxiuréia"

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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)
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.