Chronic osteo-articular changes in patients with sickle cell disease

Abstract Background: Sickle cell disease (SCD) is an autosomal recessive genetic disease in which a mutation occurs in the β-globin chain gene, resulting in abnormal hemoglobin levels. In an environment with reduced oxygen concentration, red blood cells change their conformation, resulting in chronic hemolysis and consequent anemia and vaso-occlusive crises with injuries to several organs, with a significant impairment of the osteoarticular system. This study aimed to verify the chronic osteoarticular alterations and their association with clinical and laboratory characteristics of patients with SCD with a more severe phenotype (SS and Sβ0), on a steady-state fasis. Methods: Fifty-five patients were referred to a medical consultation with a specialized assessment of the locomotor system, followed by laboratory tests and radiographic examinations. Results: In total, 74.5% patients had hemoglobinopathy SS; 67.3% were female; and 78.2% were non-whites. The mean patient age was 30.5 years. Most patients (61.8%) reported up to three crises per year, with a predominance of high-intensity pain (65.5%). Radiographic alterations were present in 80% patients. A total of 140 lesions were identified, most which were located in the spine, femur, and shoulders. Most lesions were osteonecrosis and osteoarthritis and were statistically associated with the non-use of hydroxyurea. Conclusions: There was a high prevalence of chronic osteoarticular alterations, which was statistically associated only with the non-regular use of hydroxyurea.(AU)

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function / Prevalência de priapismo em indivíduos com doença falciforme e suas implicações na função sexual masculina

ABSTRACT Objective To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. Methods This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. Results Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. Conclusion Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.

RESUMO Objetivo Avaliar aspectos epidemiológicos do priapismo em pacientes com doença falciforme e o impacto desses aspectos na função sexual de adultos. Métodos Trata-se de estudo transversal, que incluiu indivíduos com doença falciforme acompanhados em um centro de referência. Os participantes responderam a um questionário estruturado acerca das características sociodemográficas e eventos de priapismo. A função sexual foi avaliada por meio de dois instrumentos validados, a Escala de Rigidez de Ereção e um sobre satisfação com a vida sexual. Resultados Foram entrevistados 64 indivíduos com média de idade de 12 (7-28) anos. A prevalência de priapismo foi de 35,9% (23/64). O episódio mais precoce ocorreu aos 2 anos de idade e o mais tardio, aos 42 anos. A projeção estatística foi de que 71,1% desses sujeitos teriam pelo menos um episódio de priapismo ao longo da vida. Pacientes adultos com episódios de priapismo (10/23) apresentaram função erétil significativamente pior Escala de Rigidez de Ereção de 2 [1-3]; p=0,01 e estavam menos satisfeitos com a vida sexual 3 [3-5]; p=0,02. Conclusão O priapismo manifesta-se desde a infância, e episódios graves estão associados a dano cavernoso, prejuízo na qualidade da ereção e menor satisfação sexual.

Effects of oxidative stress on liver, brain and spinal cord of rats using L-NAME and treated with hydroxyurea. A model of sickle cell complication

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.

Prevalence of enuresis and its impact in quality of life of patients with sickle cell disease

ABSTRACT Introduction Evidence indicates an increase in the prevalence of enuresis in individuals with sickle cell disease. The present study aims to evaluate the prevalence and impact of enuresis on quality of life in individuals with sickle cell disease. Materials and Methods This cross-sectional study evaluated individuals with sickle cell disease followed at a reference clinic, using a questionnaire designed to evaluate the age of complete toilet training, the presence of enuresis and lower urinary tract, and the impact on quality of life of these individuals. Results Fifty children presenting SCD (52% females, mean age ten years) were included in the study. Of those, 34% (17/50) presented as HbSC, 56% with HbSS (28/50), 2% Sα-thalassemia (1/5) and 8% the type of SCD was not determined. The prevalence of enuresis was 42% (21/50), affecting 75% of subjects at five years and about 15% of adolescents at 15 years of age. Enuresis was classified as monosymptomatic in 33.3% (7/21) and nonmonosymptomatic in 66.6% (14/21) of the cases, being primary in all subjects. Nocturia was identified in 24% (12/50), urgency in 20% (10/50) and daytime incontinence 10% (5/50) of the individuals. Enuresis had a significant impact on the quality of life of 67% of the individuals. Conclusion Enuresis was highly prevalent among children with SCD, and continues to be prevalent throughout early adulthood, being more common in males. Primary nonmonosymptomatic enuresis was the most common type, and 2/3 of the study population had a low quality of life.

Características socioeconômicas e nutricionais de crianças e adolescentes com anemia falciforme: uma revisão sistemática / Socioeconomic and nutritional characteristics of children and adolescents with sickle cell anemia: a systematic review

RESUMO Objetivo: Descrever as características socioeconômicas e nutricionais de crianças e adolescentes com anemia falciforme. Fonte de dados: Estudo de revisão sistemática da literatura baseado em artigos publicados em revistas científicas. As buscas foram realizadas na base de dados eletrônica da National Library of Medicine, National Institutes of Health- PubMed.Foram realizadas duas buscas de artigos publicados nos últimos 20anos e sem limitação de idioma. Aprimeira partiu do Descritor em Ciências da Saúde "Anemia Falciforme" associado com "Fatores Socioeconômicos"; e a segunda partiu do descritor "Anemia Falciforme" associado com "Antropometria". Asbuscas foram direcionadas para pesquisas realizadas em seres humanos na faixa etária de 0 a 18anos. Síntese dos dados: A seleção final foi composta por 11artigos referentes às características socioeconômicas e 21 referentes às características nutricionais. Asamostras estudadas foram de crianças e adolescentes de ambos os sexos, com idade de 0 a 18anos e com predomínio de populações negras. Famílias de crianças e adolescentes com anemia falciforme eram predominantemente de baixo nível socioeconômico. Ospais apresentaram níveis educacionais inferiores, quando comparados a pais de crianças e adolescentes saudáveis. Asmedidas corporais (peso e estatura) e os indicadores antropométricos de crianças com anemia falciforme foram frequentemente menores, quando comparados com os dos grupos saudáveis ou das populações de referência. Conclusão: Crianças e adolescentes com anemia falciforme apresentam limitações socioeconômicas e piores condições nutricionais, quando comparados às populações de referência. Essas limitações podem implicar pior crescimento e maior ocorrência de possíveis complicações, que podem prejudicar sua qualidade de vida.

ABSTRACT Objective: To describe the socioeconomic and nutritional characteristics of children and adolescents with sickle cell anemia. Data sources: The present study is a systematic literature review based on published scientific articles. The searches were carried out using the electronic database of the National Library of Medicine, National Institutes of Health- PubMed. Two searches of articles published in the last 20years and without limitation of language were carried out. Thefirst one started from the Medical Subject Headings term "Anemia, Sickle Cell" associated with "Socioeconomic Factors"; and the second started from the term "Anemia, Sickle Cell" associated with "Anthropometry". Thesearches were directed to research conducted on humans in the age group from 0 to 18years. Data synthesis: The final selection was composed by 11 articles on socioeconomic characteristics and 21articles on nutritional characteristics. Allstudies included children and adolescents with sickle cells disease (age range 0-18years), both genders, and most of them of black ethnicity. Families of children and adolescents with sickle cell anemia were of predominantly low socioeconomic status. Parents had lower educational levels when compared to parents of healthy children and adolescents. Body measurements (weight and height) and anthropometric indicators of children with sickle cell anemia were often lower when compared to healthy groups or reference populations. Conclusions: Children and adolescents with sickle cell anemia have socioeconomic limitations and worse nutritional conditions, when compared to reference populations. These limitations may lead to worse growth and greater occurrence of possible complications that can impair their quality of life.

Enfermedad de células falciformes: un diagnóstico para tener presente / Sickle cell disease: a diagnosis to keep in mind

La enfermedad de células falciformes (ECF) es un trastorno genético autosómico recesivo. Es la hemoglobinopatía estructural más frecuente en todo el mundo y se produce por alteración en los genes de la cadena de globina. En Chile, no hay datos sobre la prevalencia de la ECF ya que es considerada una condición muy rara. La incidencia de esta enfermedad ha venido aumentando debido a la migra ción de personas de áreas con mayor prevalencia de ECF. Por esta razón resulta importante conocer y considerar este diagnóstico en un grupo seleccionado de pacientes con anemia, para prevenir y tratar las diferentes complicaciones de la enfermedad. En este artículo se revisan los nuevos aportes en el conocimiento de la fisiopatología, con especial énfasis en aquellas publicaciones de consenso y guías relacionadas al diagnóstico y manejo de esta entidad.

Sickle cell disease (SCD) is an autosomal recessive genetic disorder. It is the most frequent structural hemoglobinopathy worldwide, and it is produced by an alteration in the globin chain genes. In Chile, there is no data on the prevalence of SCD since it is considered a very rare condition. The incidence of this disease has been increasing due to migration of people from areas with greater presence of SCD. It is important to know and consider this diagnosis in a selected group of patients with anemia, in order to prevent and treat the different complications of this disease. This article reviews the most recent information that shows new concepts in the knowledge of the physiopathology, and especially publications of guidelines and consensus in relation to the diagnosis and management of this con dition.

Patients with sickle cell disease are frequently excluded from the benefits of transcranial doppler screening for the risk of stroke despite extensive and compelling evidence / Pacientes com doença falciforme são frequentemente excluídos dos benefícios do screening com doppler transcraniano para avaliação do risco de acidente vascular cerebral, apesar de evidência extensa e convincente

ABSTRACT Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19±5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.

RESUMO Doppler transcraniano (DTC) é um componente estratégico da prevenção primária do acidente vascular cerebral (AVC) em crianças com doença falciforme (DF). Este estudo foi realizado para examinar as características do DTC de crianças com DF em nove centros médicos diferentes no Brasil. Métodos: DTC foi realizado de acordo com o protocolo de Stroke Prevention Trial in Sickle Cell Anemia Protocol (STOP). Resultados: Dos 396 pacientes avaliados, 69,5% eram homozigotos para hemoglobina SS. DTC foi anormal em 4,8%, condicional em 12,6%, inadequado em 5,3% e anormalmente baixo em 1%. As máximas velocidades de fluxo médio foram 121 ± 23,83cm/s e 124 ± 27,21 cm/s nas artérias cerebrais media esquerda e direita, respectivamente. Um total de 28,8% dos pacientes (média de 9,19 ± 5,92 anos) foram avaliados com o DTC pela primeira vez. Conclusões: Pacientes com DF foram avaliados com DTC numa idade considerada avançada, o que representa uma importante oportunidade perdida para a prevenção de AVC nessa população. Uma vez que a triagem com DTC em pacientes com DF é essencial para detectar aqueles com alto risco de AVC, recomenda-se que essa triagem seja amplamente disponível no país.

Modulação autonômica cardíaca em crianças e adolescentes com anemia falciforme / Cardiac autonomic modulation in children and adolescents with sickle cell anemia

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

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

Relationship between ventilation heterogeneity and exercise intolerance in adults with sickle cell anemia

Sickle cell anemia (SCA) causes dysfunction of multiple organs, with pulmonary involvement as a major cause of mortality. Recently, there has been growing interest in the nitrogen single-breath washout (N2SBW) test, which is able to detect ventilation heterogeneity and small airway disease when the results of other pulmonary function tests (PFTs) are still normal. Thus, the objectives of the present study were to assess the heterogeneity in the ventilation distribution in adults with SCA and to determine the association between the ventilation distribution and the clinical, cardiovascular, and radiological findings. This cross-sectional study included 38 adults with SCA who underwent PFTs, echocardiography, computed tomography (CT), and 6-min walk test. To evaluate the ventilation heterogeneity, the patients were categorized according to the phase III slope of the N2SBW (SIIIN2). Compared with adults with lower SIIIN2 values, adults with higher SIIIN2 values showed lower hemoglobin levels (P=0.048), a history of acute chest syndrome (P=0.001), an elevated tricuspid regurgitation velocity (P=0.039), predominance of a reticular pattern in the CT (P=0.002), a shorter 6-min walking distance (6MWD) (P=0.002), and lower peripheral oxygen saturation (SpO2) after exercise (P=0.03). SIIIN2 values correlated significantly with hemoglobin (rs=-0.344; P=0.034), forced vital capacity (rs=-0.671; P<0.0001), diffusing capacity for carbon monoxide (rs=-0.376; P=0.019), 6MWD (rs=-0.554; P=0.0003), and SpO2 after exercise (P=0.040). Heterogeneity in the ventilation distribution is one of the most common pulmonary dysfunctions in adults with SCA. Moreover, relationships exist between ventilation heterogeneity, worsening of pulmonary structural damage, and reduced tolerance for exercise.

Avascular necrosis of the femoral head in sickle cell disease in pediatric patients suffering from hip dysfunction / Osteonecrosis de la cabeza femoral en pacientes con anemia de células falciformes y disfunciones de la cadera en la infancia

ABSTRACT Objective The aim of this study is to verify the prevalence of avascular necrosis (AVN) in pediatric patients with sickle cell anemia and hip dysfunction, and to evaluate the presence of associated risk factors. Method A cross-sectional study was conducted in a group of 92 patients with sickle cell disease and hip dysfunction. Clinical and sociodemographic characteristics were collected, and laboratory variables were evaluated. All the subjects underwent radiographic and clinical evaluation of the hip. The participants were divided into two groups: the "AVN Group" consisting of patients with AVN, and the "Comparison Group" without AVN. Both groups were evaluated in search of factors associated with osteonecrosis of the femoral head. Results 43 (46.7 %) out of 92 individuals presented hip dysfunction, and 13 were diagnosed with AVN (30.2 %). Comparison between groups showed significant differences in time of diagnosis, previous trauma, presence of pain, and mean values of functional scores. Higher percentage rates of fetal hemoglobin, higher platelet counts and lower rates of total hemoglobin were perceived in the Comparison Group. Conclusions Pediatric patients with sickle cell anemia with hip dysfunction present a prevalence of 39.4 % of osteonecrosis of the femoral head. This was associated with a longer time of diagnosis (97 months), previous trauma in 92 % of patients, and a mean Charnley score of 15 points. Also, an association with lower rate of fetal hemoglobin (7.2 versus 11.8) was found, which supports the hypothesis that fetal hemoglobin may function as a protective factor against avascular necrosis.(AU)

RESUMEN Objetivo El objetivo de este estudio es confirmar la prevalencia de necrosis avas-cular (AVN) en pacientes con anemia de células falciformes, así como disfuncio-nes de cadera en la infancia, además de evaluar la presencia de factores de ries-go en estos individuos. Métodos Se realizó un estudio de corte transversal en un grupo de 92 pacientes con enfermedad falciforme y disfunción de cadera. Se hizo una recolección de caracte-rísticas clínicas y sociodemográficas, y se realizó un análisis de las variables encon-tradas en laboratorio. Todos los sujetos se sometieron a evaluación clínica y radio-gráfica de la cadera, y fueron divididos en dos grupos: el "Grupo AVN" que consistía de pacientes con AVN y el "Grupo Comparativo" sin AVN. Se evaluó ambos grupos con el fin de encontrar factores asociados con la osteonecrosis de cabeza femoral. Resultados 43 (46.7 %) de los 92 individuos presentaron disfunción en la cadera y 13 de ellos recibieron un diagnóstico de AVN (30.2 %). La comparación entre los dos grupos mostró diferencias importantes en el tiempo de diagnóstico, trauma previo, presencia de dolor, y media de valores en los puntajes funcionales. El Grupo Com-parativo evidenció valores porcentuales más altos de hemoglobina fetal, así como un conteo de plaquetas más alto y valores menores de hemoglobina total. Conclusiones Entre los pacientes pediátricos con anemia falciforme y con disfun-ción de cadera se presenta una prevalencia de 39.4 % de osteonecrosis de cabeza femoral, lo que está asociado a un tiempo mayor de diagnóstico (97 meses), trau-ma previo en 92 % de los pacientes, y valores de puntaje medio de Chanrley de 15 puntos. También se encontró una asociación con valores menores de hemog-lobina fetal (7.2 versus 11.8), lo cual respalda la hipótesis de que la hemoglobina fetal puede funcionar como un factor de protección contra la necrosis avascular.(AU)

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem? / Função pulmonar em crianças e adolescentes com doença falciforme: temos dado atenção adequada a esse problema?

ABSTRACT Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

RESUMO Objetivo: Avaliar a função pulmonar e a capacidade funcional em crianças e adolescentes com doença falciforme. Métodos: Estudo transversal com 70 crianças e adolescentes com doença falciforme (8-15 anos), submetidos a testes de função respiratória (espirometria) e de capacidade funcional (teste de caminhada de seis minutos). Os resultados da avaliação da função pulmonar foram comparados com variáveis relacionadas à gravidade da doença falciforme e à presença de história de asma e de síndrome torácica aguda. Resultados: Dos 64 pacientes submetidos à espirometria, 15 (23,4%) apresentaram resultados alterados: distúrbio ventilatório restritivo, em 8; (12,5%) e distúrbio respiratório obstrutivo, em 7 (10,9%). Dos 69 pacientes submetidos ao teste de caminhada de seis minutos, 18 (26,1%) apresentaram resultados alterados na distância em % do previsto para a idade, e houve uma queda ≥ 3% na SpO2 em 36 (52,2%) dos pacientes. Não houve associações significativas entre função pulmonar alterada e as outras variáveis analisadas, exceto para hipoxemia e distúrbio ventilatório restritivo. Conclusões: Observou-se uma significativa prevalência de alterações na função pulmonar nesta amostra de crianças e adolescentes com doença falciforme. A elevada prevalência de distúrbios ventilatórios sugere a necessidade de um olhar mais atento à função pulmonar desde a infância nessa população.

Erythrocyte oxidative stress markers in children with sickle cell disease / Marcadores de estresse oxidativo em eritrócitos de crianças com doença falciforme

Abstract Objective To determine eight parameters of oxidative stress markers in erythrocytes from children with sickle cell disease and compare with the same parameters in erythrocytes from healthy children, since oxidative stress plays an important role in the pathophysiology of sickle cell disease and because this disease is a serious public health problem in many countries. Methods Blood samples were obtained from 45 children with sickle cell disease (21 males and 24 females with a mean age of 9 years; range: 3–13 years) and 280 blood samples were obtained from children without hemoglobinopathies (137 males and 143 females with a mean age of 10 years; range: 8–11 years), as a control group. All blood samples were analyzed for methemoglobin, reduced glutathione, thiobarbituric acid reactive substances, percentage of hemolysis, reactive oxygen species, and activity of the enzymes glucose 6-phosphate dehydrogenase, superoxide dismutase, and catalase. Data were analyzed using Student's t-test and were expressed as the mean ± standard deviation. A p-value of <0.05 was considered significant. Results Significant differences were observed between children with sickle cell disease and the control group for the parameters methemoglobin, thiobarbituric acid reactive substances, hemolysis, glucose 6-phosphate dehydrogenase activity, and reactive oxygen species, with higher levels in the patients than in the controls. Conclusions Oxidative stress parameters in children's erythrocytes were determined using simple laboratory methods with small volumes of blood; these biomarkers can be useful to evaluate disease progression and outcomes in patients.

Resumo Objetivo Determinar parâmetros de estresse oxidativo em eritrócitos de crianças com doença falciforme e compará-los com os mesmos parâmetros em eritrócitos de crianças saudáveis, pois o estresse oxidativo desempenha um importante papel na fisiopatologia da doença falciforme, considerada um sério problema de saúde pública em muitos países. Métodos Foram obtidas amostras de sangue de 45 crianças com doença falciforme (21 meninos e 24 meninas com média de 9 anos, variação de 3 a 13) e 280 amostras de sangue de crianças sem hemoglobinopatias (137 meninos e 143 meninas com média de 10 anos, variação de 8 a 11), como grupo controle. Em todas as amostras foram determinados meta-hemoglobina, glutationa reduzida, substâncias reativas ao ácido tiobarbitúrico, porcentagem de hemólise, espécies reativas de oxigênio e atividade das enzimas glucose6-fosfato desidrogenase, superóxido dismutase e catalase. Os dados foram analisados com o teste t de Student e foram expressos como média ± desvio padrão. Um valor de p < 0,05 foi considerado significativo. Resultados Foram observadas diferenças significativas entre as crianças com doença falciforme e o grupo controle para os parâmetros meta-hemoglobina, substâncias reativas ao ácido tiobarbitúrico, porcentagem de hemólise, espécies reativas de oxigênio e atividade da enzima glucose6-fosfato desidrogenase, com níveis aumentados nos pacientes. Conclusões Foi possível determinar parâmetros de estresse oxidativo em eritrócitos de crianças, com técnicas laboratoriais simples e pequenos volumes de sangue. Esses biomarcadores podem ser úteis na avaliação da progressão e dos resultados de tratamentos da doença.

Maternal risk behavior and caries incidence in children with sickle cell disease

The aim of this study was to analyze the incidence of caries, in relation to maternal risk behaviors and clinical conditions representing different levels of sickle cell disease severity. A total of 295 children aged 6 to 60 months participated in this cohort conducted from August 2007 to December 2008. They were diagnosed and monitored by the referral service of the state. Interviews were made with families to identify sociodemographic variables, and an oral exam was performed to determine dental caries. The SRQ (Self Report Questionnaire) scale was used to diagnose the presence of common mental disorders, and the CAGE (Cut down, Annoyed, Guilty and Eye opener) was applied to determine abusive use of alcohol. The absolute and relative frequencies of the variables of interest were analyzed by Chi-square and Mann-Whitney, with a 5% significance level. The incidence variables were analyzed according to the logistic regression model, with a confidence interval of 95%. Caries incidence (1.98; SD = 4.68) was higher in the HbSS genotype. There was a statistically significant association between caries incidence and both abusive use of alcohol (32.43%, RR = 1.99; 1.05-3.78; 95%CI) and common mental disorders (8.77% RR = 0.37; 0.15-0.93; 95%CI). There was also an association between caries incidence and maternal risk behavior, indicating that the care network should be expanded to include patients with sickle cell disease.

Cirurgia oral em paciente com anemia falciforme: o que o cirurgião-dentista precisa saber. Relato de caso / Oral surgery in patient with sickle cell disease: what the dentist need to know. Case report

Anemia falciforme é uma doença hereditária que se caracteriza por um distúrbio sanguíneo que afeta as células vermelhas, resultando, principalmente, em hemoglobinas alteradas. A doença caracteriza-se pela falta de glóbulos vermelhos saudáveis, causando dificuldade em transpor oxigênio para todos os tecidos. As manifestações sistêmicas e na cavidade oral podem ser variadas, sendo importante estar atento aos sinais clínicos e sintomas. Para o tratamento desses pacientes é necessário atenção aos cuidados e riscos a infecções oriundas das técnicas realizadas pelo cirurgião dentista, e, por esta razão, a antibioticoprofilaxia se faz tão necessária juntamente com a relação multidisciplinar. O objetivo deste trabalho foi apresentar um relato de caso de instalação de implante osseointegrado em paciente portadora de anemia falciforme, enfatizando as características da doença e os cuidados que o cirurgião-dentista deve ter no manejo destes pacientes.(AU)

Sickle cell disease is a hereditary disease characterized by a disorder that affects the blood red cells resulting mainly in altered hemoglobin. The disease results in lack of enough healthy red blood cells causing difficulty in transposing oxygen to all tissues. Systemic and bucal manifestations can be varied and it is very important to be aware of the clinical signs and symptoms. In the treatment of these patients it is necessary to pay attention for the risks of arising infections from the techniques performed by dentists and that´s why antibiotic prophylaxis is done as needed along with the multidisciplinary relationship. The objective of this study was a report of case of osseointegrated implant installation in a female patient with sickle cell anemia, emphasizing the characteristics of the disease and the care that the dentist must have in the management of these patients.(AU)

Is sickle cell anemia a risk factor for severe dental malocclusion?

The aim of this study was to investigate possible associations between sickle cell anemia (SCA) and the severity of dental malocclusion (MO). This was a retrospective cohort study of 93 individuals with SCA (G1) and 186 individuals without the disease (G2). SCA patients were randomly selected by a simple draw from patients treated in the Centro de Hematologia e Hemoterapia do Maranhão (HEMOMAR) in northeastern Brazil. Patients aged between 16 and 60 were included after being tested for the hemoglobin S gene. G2 consisted of individuals living in the same residence as the patients. The Dental Aesthetic Index (DAI), as well as some morphological deviations not included in DAI, were used for the orthodontic evaluation of MO. Poisson regression with robust variance adjustment was employed to estimate relative risk (RR). In the multivariate analysis, SCA was associated with moderate (RR = 1.36) and very severe MO (RR = 8.0). SCA is correlated with anterior tooth loss (RR = 1.94), anterior spacing (RR = 1.66), overjet (RR = 1.87), anterior crossbite (RR = 1.94), and open bite (RR = 1.94). Thus, SCA is a risk factor for moderate and very severe MO.

Association between morphometric variables and nocturnal desaturation in sickle-cell anemia / Associação entre variáveis morfométricas e dessaturação noturna na anemia falciforme

OBJECTIVE: to evaluate associations between morphometric variables, cervical circumference (CC), and abdominal circumference (AC) with the presence of nocturnal desaturation in children and adolescents with sickle-cell anemia. METHODS: all patients were submitted to baseline polysomnography, oral cavity measurements (maxillary intermolar distance, mandibular intermolar distance, and overjet), and CC and AC measurements. RESULTS: a total of 85 patients were evaluated. A positive correlation was observed between the height/age Z-score and CC measurement (r = 0.233, p = 0.031). The presence of nocturnal desaturation was associated with CC (59.2± 9.3 vs. 67.5 ± 10.7, p = 0.006) and AC measurements (27.0 ± 2.0 vs. 29.0± 2.1, p = 0.028). There was a negative correlation between desaturation and maxillary intermolar distance (r = -0.365, p = 0.001) and mandibular intermolar distance (r = -0.233, p = 0.037). CONCLUSIONS: the morphometric variables of CC and AC may contribute to raise suspicion of nocturnal desaturation in children and adolescents with sickle-cell anemia. .

OBJETIVO: avaliar associações entre variáveis morfométricas e circunferências cervical (CC) e abdominal (CA) com a presença de dessaturação noturna em crianças e adolescentes com anemia falciforme. MÉTODOS: todos os pacientes foram submetidos à polissonografia basal, medidas da cavidade oral (distância intermolar da maxila, distância intermolar da mandíbula e overje), CC e CA. RESULTADOS: foram avaliados 85 pacientes. Foi observada correlação positiva entre o escore Z altura/idade e a medida da circunferência cervical (r = 0,233 p = 0,031). A presença da dessaturação noturna associou-se com as medidas da circunferência cervical (59,2 ± 9,3 vs 67,5 ± 10,7; p = 0,006) e abdominal (27,0 ± 2,0 vs 29,0 ± 2,1; p = 0,028). Houve correlação negativa entre a dessaturação e a distância entre os segundos molares da maxila (r =-0,365, p = 0,001) e da mandíbula (r = -0,233, p = 0,037). CONCLUSÕES: as variáveis morfométricas e circunferências cervical e abdominal podem contribuir para a suspeita da dessaturação noturna em crianças e adolescentes com anemia falciforme. .