Coordenação de Aperfeiçoamento de Pessoal de Nível Superior / Searching to trace the clinical profile, epidemiologist, evolution and laboratorial of the internments of patients with Sickle Cell Disease (SCD); to identify factors that can be related with the sprouting and evolution of Acute Chest Syndrome (ACS), as well as; to evaluate if the average saturation of oxygen through the oximetry of pulse is diagnosis or prognostic of ACS; a study of coorte with 168 carrying patients was carried through of SCD. During all the year of 2006, 168 patients with SCD had been studied, had predominance of the males (51.8%) and of the age band between 5 and 15 years (53.3%), 81 (48.2%) patient ones had suffered at least a hospital internment, 34 (41.9%) had diagnosis of ACS in at least an internment and 47 (58.1%) had other causes internment as intense painful and anemia crisis. When we compare the ambulatorial accompaniment of the patients who had interned with of that they had not interned in 2006, perceive that the splenomegaly presence was more common between the patients whom they had interned (27.3%), with significance statistics (p = 0, 0006). The average saturation of oxygen through the oximetry of pulse also was significantly different (p = 0, 0092) between the two groups, a time that the patients who had suffered internment had gotten one better performance (95.6%) against 94,1 % of the patients who had not interned in 2006. When analyzing the variable hemoglobin and white cell count, we do not find differences significant. In relation to the patients with ACS, the fever complaint was more prevalent (70.6%), whereas the pain complaint more was related by the patients with other causes of internment (93.3%). With regard to the age band, it enters the older than 15 years, we notice minors indices (6.1%) of internment for ACS (p= 0, 04). It did not have significant difference with regard to the hemoglobin and the saturation of oxygen through the oximetry of pulse when compared the out results with the ones of the hospital admission. The total white globule counting was significantly superior in the occasion of the internment (p=0,001). One concludes that the average saturation of oxygen by pulse oximetry was not capable to identify the patients who would come to be interned with ACS. / Buscando traçar o perfil clínico, epidemiológico, evolutivo e laboratorial das internações de pacientes com Anemia Falciforme (AF); identificar fatores que possam estar relacionados com surgimento e evolução de Síndrome Torácica Aguda (STA), bem como; avaliar se a saturação média de oxigênio através da oximetria de pulso é diagnóstica ou prognóstica em relação a STA; foi realizado um estudo de coorte com 168 pacientes portadores de AF. Houve predomínio do gênero masculino (51,8%) e da faixa etária entre 5 e 15 anos (53,3%), 81(48,2%) pacientes sofreram pelo menos uma internação hospitalar, 34 (41,9%) tiveram diagnóstico de STA em pelo menos uma internação e 47 (58,1%) tiveram outras causas internação como crise dolorosa e anemia intensa. Quando comparamos o acompanhamento ambulatorial dos pacientes que internaram com o dos que não internaram em 2006, percebemos que a presença de esplenomegalia foi mais comum entre os pacientes que internaram (27,3%), com significância estatística (p = 0,0006). A saturação média de oxigênio através da oximetria de pulso também foi significativamente diferente (p = 0,0092) entre os dois grupos, uma vez que os pacientes que sofreram internação obtiveram um melhor desempenho (95,6%) contra 94,1% dos pacientes que não internaram em 2006. Ao analisarmos as variáveis hemoglobina e leucometria, não encontramos diferença estatisticamente significativa. Em relação aos pacientes com STA, a queixa de febre foi mais prevalente (70,6%), enquanto que a queixa de dor foi mais referida pelos pacientes com outras causas de internação (93,3%). Com relação à faixa etária, entre os maiores de 15 anos, notamos menores índices (6,1%) de internação por STA (p= 0,04). Não houve diferença estatisticamente significante com relação à hemoglobina e a saturação de oxigênio através da oximetria de pulso quando comparados os resultados ambulatoriais com os da admissão hospitalar. A contagem total de glóbulos brancos foi significativamente superior na ocasião da internação (p=0,001). Conclui-se que a saturação transcutânea média de oxigênio não foi capaz de identificar os pacientes que viriam a ser internados com STA.

No description available.

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 t 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.