Abstract Purpose: To analyze the serum levels of nitric oxide and correlate them with the levels of thiobarbituric acid reactive substances (TBARS) in liver, brain and spinal cord of animals using L-NAME and treated with hydroxyurea. Methods: Eighteen male albino Wistar rats were divided into three groups. NG-nitro-L-arginine methyl ester (L-NAME) was intraperitoneally administered to induce oxidative stress. TBARS and plasma nitric oxide levels were analyzed in all groups. Histopathology of the liver and vascular tissue was performed. Results: Statistically significant differences were seen in liver, brain and spinal cord TBARS levels. Conclusions: Following the use of L-NAME, hepatic tissue increased the number of Kupffer cells as oxidative stress and inflammatory response increased. The use of L-NAME caused an increase in lipid peroxidation products and, consequently, in oxidative stress in animals. Hydroxyurea doses of 35 mg / kg / day reduced TBARS values in liver, brain and spinal cord.

RESUMO Objetivo: Identificar as evidências disponíveis na literatura sobre qualidade de vida relacionada à saúde em adultos com doença falciforme. Método: revisão integrativa nas bases de dados MEDLINE, CUMED, LILACS e SciELO, de artigos provenientes de estudos desenvolvidos nesta temática, publicados entre 2005 a 2015, em inglês, português ou espanhol. Resultados: foram incluídos 22 artigos, sendo utilizadas seis escalas para avaliar os escores de qualidade de vida relacionada à saúde: três genéricas e três específicas. Nenhuma escala específica para adultos com doença falciforme foi adaptada para o português brasileiro até o momento. Pacientes acometidos por crises dolorosas frequentes, com baixa adesão ao tratamento, apresentaram comprometimento da qualidade de vida. Conclusão: Os estudos selecionados evidenciaram que os pacientes com doença falciforme têm piores escores do que a população em geral. Esses indicadores deveriam instrumentalizar o enfermeiro na proposição de intervenções e estratégias assistenciais e socioeducativas, com vistas a melhorar a qualidade de vida desta clientela.

ABSTRACT Objective: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. Method: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. Results: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Conclusion: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.

RESUMEN Objetivo: Identificar las evidencias disponibles en la literatura sobre cualidad de vida relacionada a la salud en adultos con enfermedad falciforme. Método: revisión integradora en las bases de datos MEDLINE,CUMED,LILACS y SciELO, de artículos provenientes de estudios desarrollados en esta temática, publicados entre 2005 y 2015, en inglés, portugués o español. Resultados: fueron incluidos 22 artículos, siendo utilizadas seis escalas para evaluar las puntuaciones de cualidad de vida relacionada a la salud: tres genéricas y tres específicas. Ninguna escala específica para adultos con enfermedad falciforme fue adaptada para el portugués brasileño hasta el momento. Pacientes acometidos por crisis dolorosas frecuentes, con baja adhesión al tratamiento, presentaron comprometimiento de la cualidad de vida. Conclusión: Los estudios seleccionados evidenciaron que los pacientes con enfermedad falciforme tienen peores puntuaciones que la población en general. Esos indicadores deberían instrumentalizar el enfermero en la proposición de intervenciones y estrategias asistenciales y socioeducativas, con vistas a mejorar la cualidad de vida de esta clientela.

ABSTRACT Objective: To identify the available evidence in the literature on health-related quality of life in adults with sickle cell disease. Method: integrative review of MEDLINE, CUMED, LILACS and SciELO databases, from articles developed in this area, published between 2005 and 2015, in English, Portuguese or Spanish. Results: 22 articles were included, six scales were used to evaluate health-related quality of life scores: three generic and three specific. No specific scale for adults with sickle cell disease has been adapted to Brazilian Portuguese so far. Patients affected by frequent painful crises, with low adherence to treatment, had a compromised quality of life. Conclusion: Selected studies have shown that patients with sickle cell disease have worse scores than the general population. These indicators should be instrumental to the nurse in the proposal of interventions and strategies of assistance and socio-educational, with a view to improving the quality of life of this clientele.

RESUMEN Objetivo: Identificar las evidencias disponibles en la literatura sobre cualidad de vida relacionada a la salud en adultos con enfermedad falciforme. Método: revisión integradora en las bases de datos MEDLINE,CUMED,LILACS y SciELO, de artículos provenientes de estudios desarrollados en esta temática, publicados entre 2005 y 2015, en inglés, portugués o español. Resultados: fueron incluidos 22 artículos, siendo utilizadas seis escalas para evaluar las puntuaciones de cualidad de vida relacionada a la salud: tres genéricas y tres específicas. Ninguna escala específica para adultos con enfermedad falciforme fue adaptada para el portugués brasileño hasta el momento. Pacientes acometidos por crisis dolorosas frecuentes, con baja adhesión al tratamiento, presentaron comprometimiento de la cualidad de vida. Conclusión: Los estudios seleccionados evidenciaron que los pacientes con enfermedad falciforme tienen peores puntuaciones que la población en general. Esos indicadores deberían instrumentalizar el enfermero en la proposición de intervenciones y estrategias asistenciales y socioeducativas, con vistas a mejorar la cualidad de vida de esta clientela.

RESUMO Objetivo: Identificar as evidências disponíveis na literatura sobre qualidade de vida relacionada à saúde em adultos com doença falciforme. Método: revisão integrativa nas bases de dados MEDLINE, CUMED, LILACS e SciELO, de artigos provenientes de estudos desenvolvidos nesta temática, publicados entre 2005 a 2015, em inglês, português ou espanhol. Resultados: foram incluídos 22 artigos, sendo utilizadas seis escalas para avaliar os escores de qualidade de vida relacionada à saúde: três genéricas e três específicas. Nenhuma escala específica para adultos com doença falciforme foi adaptada para o português brasileiro até o momento. Pacientes acometidos por crises dolorosas frequentes, com baixa adesão ao tratamento, apresentaram comprometimento da qualidade de vida. Conclusão: Os estudos selecionados evidenciaram que os pacientes com doença falciforme têm piores escores do que a população em geral. Esses indicadores deveriam instrumentalizar o enfermeiro na proposição de intervenções e estratégias assistenciais e socioeducativas, com vistas a melhorar a qualidade de vida desta clientela.

Background: In 2001, the Brazilian Ministry of Health added hemoglobinopathies to the National Neonatal Screening Program to be implemented in three steps. In order to meet the proposed goals, it is crucial to establish periodic assessments of this program with the aim of monitoring its implementation. Objective: To assess the scope and the efficiency of the stages of the National Newborn Screening Program in identifying hemoglobin S. Methods: A cross-sectional study was developed with the results of the heel prick test using the high performance liquid chromatography method for babies born in Mato Grosso do Sul from 2006 to 2010. The following variables were investigated: year, number of live births, total screening, coverage ratio, prevalence, time between the child birth and the blood collection; age at diagnosis; age at the time of the first consultation; and time between the diagnosis and the first appointment. Results: Over the five years of the study, the mean coverage rate was 91.77%.The prevalences of hemoglobin FAS and hemoglobin FS were 1.65% and 0.011%, respectively. Blood samples from 43.48% of children were collected from the second to the seventh day. The age at diagnosis was within the first 28 days in 87.80% of the screened children. The lowest mean indices for the first consultation and the time between the diagnosis and the first appointment (58.8 and 46.4 days, respectively) occurred in 2010. Conclusions: The scope of the National Neonatal Screening Program for hemoglobinopathies is good, with a large number of individuals being tested. Efficiency is a suitable indicator to assess the program steps. Three points are recommended: the training of the individuals involved, studies to assess the assistance provided to the affected child, and genetic counseling to the mothers.