Nutritional Status, Nutrient Intake, and Food Diversity Among Children With Sickle Cell Anemia.

Children with sickle cell anemia (SCA) often exhibit nutritional deficiencies and are at high risk of dying before the age of 5 years. Ensuring adequate nutrition is a critical part of health care for such children. This study aimed to investigate the association between nutritional status, nutrient intake, and food diversity in children with SCA. A descriptive cross-sectional study was conducted on 74 children with SCA, between 24 and 71 months of age. Anthropometric measurements, food and nutrients consumption were determined. The prevalence of low weight, stunting, and overweight/obesity were 16.2%, 35.1%, and 16.2%, respectively. Mean folic acid intake was low (49.05%±51.22%), whereas the intakes of protein (426.71%±171.93%), retinol (292.97%±403.88%), phosphorus (204.55%±151.35%), magnesium (233.02%±151.14%), iron (250.76%±165.81%), and zinc (243.21%±148.40%) were high. The dietary phosphorus/protein ratio was high for 31.1% of the children, and 44.6% of the children had low dietary diversity score. No correlation was found between food diversity, nutrient adequacy, and nutritional status. Despite the adequacy of the intake of most micronutrients, diet quality was inadequate, constituting mainly ultraprocessed foods. Knowing the food consumption pattern of these children enables a more resolute nutritional intervention.

Omega 3 (n-3) fatty acids down-regulate nuclear factor-kappa B (NF-κB) gene and blood cell adhesion molecule expression in patients with homozygous sickle cell disease.

Chronic inflammation and reduced blood levels of omega-3 fatty acids (n-3) are known characteristics of sickle cell disease (SCD).The anti-inflammatory properties of n-3 fatty acids are well recognized. Omega-3 treated (n = 24), hydroxyurea (HU) treated (n = 18), and n-3 untreated (n=21) homozygous SCD patients (HbSS) and healthy (HbAA) controls (n = 25) matched for age (5-16 years), gender and socioeconomic status were studied. According to age (5-10) or (11-16) years, two or three capsules containing 277.8 mg docosahexaenoic (DHA) and 39.0mg eicosapentaenoic (EPA) or high oleic acid placebo (41%) were assigned to n-3 treated and n-3 untreated groups, respectively. Hydroxyurea treated group was on dosage more than 20 mg/kg/day. The effect of supplementation on systemic and blood cell markers of inflammation was investigated. The n-3 treated group had higher levels of DHA and EPA (p < 0.001) and lower white blood cell count and monocyte integrin (p < 0.05) compared with the n-3 untreated. No difference was detected between the two groups regarding C-reactive protein, granulocytes integrin and selectin, plasma tumour necrosis factor-α and interleukin-10. The n-3 treated group had lowered nuclear factor-kappa B (NF-κB) gene expression compared to n-3 untreated and HU treated groups (p < 0.05). This study provides evidence that supplementation with n-3 fatty acids may ameliorate inflammation and blood cell adhesion in patients with SCD.

Pilot study of omega-3 fatty acid supplements in sickle cell disease.

In a previous retrospective study, it was observed that the greater the amounts of the omega-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in the blood, the lesser the number of complications of sickle cell disease (SCD) and the higher the steady state haemoglobin level. SCD causes ischaemia-reperfusion injury and inflammation; which can be ameliorated by a metabolite of DHA that down-regulates expression of pro-inflammatory genes. The objectives of this prospective pilot study were to evaluate the effects of DHA and EPA supplements in SCD, and test the hypothesis that these effects are mediated partly by reducing inflammation. Oral DHA and EPA supplements were given to 16 SCD patients for 6 months. We then compared pre- and post-supplementation values of number of crisis, steady state Hb, plasma unconjugated bilirubin and three indices of inflammation: plasma interleukin-6, blood neutrophil and platelet counts. There was a significant reduction in the plasma level of unconjugated bilirubin, and the number of sickle cell crisis; but not in the markers of inflammation. The pilot data suggest that DHA and EPA supplements reduce the number of crisis and steady state haemolysis in SCD; but provide no evidence that these effects are mediated by reducing inflammation.

Potential utility of full-spectrum antioxidant therapy, citrulline, and dietary nitrate in the management of sickle cell disease.

There is considerable evidence that oxidative stress and a loss of nitric oxide bioactivity are key mediators of the vasculopathies associated with sickle cell disease. A comprehensive nutraceutical strategy for mitigating the contribution of oxidative stress to pathogenesis - dubbed "full-spectrum antioxidant therapy" - may have utility in this syndrome. This strategy entails concurrent administration of phycocyanobilin - a phytochemical richly supplied by spirulina, shown to inhibit NADPH oxidase in a manner analogous to its chemical relatives biliverdin and bilirubin; high-dose folate - recently shown to quench peroxynitrite-derived radicals and restore coupling of NO synthase; N-acetylcysteine - for boosting intracellular glutathione levels; and a phase 2 inducer such as lipoic acid - to further promote glutathione synthesis while increasing expression of antioxidant enzymes. Suboptimal endothelial arginine levels, reflecting increased plasma arginase activity and elevated ADMA, contribute to the loss of NO bioactivity in sickle cell disease; supplementation with the arginine precursor citrulline may ameliorate this defect. Increased intakes of plant-derived nitrate have the potential to diminish the quenching of NO by plasma hemoglobin in sickle cell patients, while boosting systemic NO production independent of NO synthase activity. In addition to the well-documented utility of hydroxyurea - possibly a suboptimal strategy for life-long therapy owing to its mutagenic activity - rational pharmaceutical options for managing sickle cell disease include pentoxifylline and phosphodiesterase 5 inhibitors such as sildenafil.

Acompanhamento nutricional de criança portadora de anemia falciforme na Rede de Atenção Básica à Saúde / Nutritional follow-up of children with sickle cell anemia treated in a Primary Care Unit

OBJETIVO: Relatar estudo de caso de atendimento nutricional a criança com diagnóstico de anemia falciforme. DESCRIÇÃO DO CASO: Realizaram-se quatro atendimentos nutricionais pela equipe do Internato de Nutrição da Universidade do Estado do Rio de Janeiro (Uerj) no período de julho a setembro de 2005 a criança de 1 a 4 meses, feminina, negra, com anemia falciforme. Na avaliação do estado nutricional e do ganho de peso, empregaram-se os seguintes indicadores antropométricos: comprimento/idade, peso/idade e peso/comprimento, e um indicador de impacto nutricional. A análise dietética compreendeu a avaliação da ingestão energética e de macronutrientes observada na primeira consulta após 30 dias de intervenção. COMENTÁRIOS: Ao longo do período analisado, a baixa estatura para a idade (z=−1,32) evoluiu para adequação (z=0,87), enquanto o peso em relação ao comprimento manteve-se inadequado (z=−2,53). O ganho de peso foi 50% inferior ao incremento esperado. O consumo energético inicialmente inadequado (60% das recomendações) alcançou, após 30 dias, 117%. A Estratégia em Saúde da Família vem sendo recomendada como importante ferramenta para monitorar as condições nutricionais, bem como para melhorar a atenção prestada. Entretanto, considerando o modelo de atenção primária local, observa-se haver necessidade de capacitação, especialmente no que tange às peculiaridades inerentes à condição de nutrição e de saúde dos portadores de anemia falciforme.

OBJECTIVE: To report the nutritional follow-up of a black baby girl, one year and four months old, with homozygous sickle cell anemia. CASE DESCRIPTION: From July-September 2005, the infant attended four nutritional appointments at the Nutrition Internship Program from the State University of Rio de Janeiro, Rio de Janeiro, Brazil. The nutritional status was evaluated by the anthropometric indexes: length/age, weight/age and weight/length, and by one indicator of nutritional impact. Analysis of the energy dietary intakes and macronutrients ingestion was performed in two opportunities: at the first nutritional evaluation and 30 days after the first intervention. COMMENTS: At the first nutritional evaluation, the child presented low stature/age (z=−1,32), that became adequate during the follow-up (z=0,87). The low weight/length (z=−2,53) was similar at the first and final evaluations. The weight gain during the follow-up period was only 50% of the expected increment. The energy dietary intake was 66% of the recommended energy intake at the first appointment and 117% after 30 days. The Family Health Strategy is recommended as an important tool to monitor nutritional status and improve nutritional care. There is a need of developing professional skills regarding nutritional care of patients with sickle cell anemia.

Manifestações bucais da anemia falciforme e suas implicações no atendimento odontológico / Buccal manifestations of sickle cell anemia and their implications in the dentistry services

A anemia das células falciformes é uma hemoglobinopatia genética de alta prevalência no Brasil. Essa doença é caracterizada pelo afoiçamento dos eritrócitos, o que gera quadros de isquemia e infarto tecidual, comprometendo vários órgãos, inclusive a região dento-maxilo-facial. Nesse estudo, foram investigados os aspectos gerais e bucais de 8 pacientes com anemia falciforme residentes em datas-MG. Os achados bucais mais frequentes foram: palidez de mucosa, hipomaturação em esmalte e dentina e grosseiro trabeculado ósseo, sendo que todos os pacientes relataram história prévia de pneumonia. O principal objetivo desse estudo é esclarecer e demostrar profissionais da odontologia quais as alterações bucais presentes em indivíduos falcêmicos para a realização de um tratamento eficaz frente às peculiaridades dessa doença.

Folate supplementation and twinning in patients with sickle cell disease.

Patients with sickle cell disease routinely take folic acid daily as a supplement to maintain effective erythropoiesis. One of the controversial effects of folic acid is its effect on twin gestation rates. In this report, we present our experience in patients with sickle cell disease and twin pregnancy. Our data show that twin pregnancies seem to be associated with folate supplementation.

Arginine supplementation improves rotorod performance in sickle transgenic mice.

Patients with sickle cell disease (SCD) have been shown to have impaired visual-motor speed and coordination. Sensorimotor deficits in mice can be investigated by motor coordination tests that require whole body movements such as the rotorod. A sickle transgenic mouse model (S+S-Antilles) that expresses human alpha, human beta(S) and human beta(S-Antilles), is homozygous for the mouse beta(major) deletion, and has low plasma arginine was compared to control C57BL/6J mice and S+S-Antilles mice supplemented with 5% arginine on the rotorod. The rotorod consists of a 2.5 cm diameter, grooved rod turning at constant acceleration, requiring postural adjustments on the part of the mice to maintain equilibrium. C57BL mice on Purina mouse chow had an average latency to fall of S+S-Antilles mice on Purina mouse chow had an average of 127+/-56 s S+S-Antilles mice after 5% arginine supplementation had a mean latency of Arginine may improve rotorod performance in sickle transgenic mice by increasing NO synthesis thereby improving vasodilatation and blood flow with reversal of ischemia in brain and/or muscle. In conclusion, impaired rotorod performance in sickle transgenic mice presents an opportunity to apply this simple task to provide an efficient method to screen some types of therapeutic regimens for efficacy in SCD.

Supplementation of patients with homozygous sickle cell disease with zinc, alpha-tocopherol, vitamin C, soybean oil, and fish oil.

Thirteen patients (aged 0.7-17.9 y) with homozygous sickle cell disease were supplemented with alpha-tocopherol, vitamin C, zinc, and soybean oil (suppl 1; for 8 mo) and alpha-tocopherol, vitamin C, and fish oil (suppl 2; for 7 mo). Urinary zinc (suppl 1), plasma vitamin C, plasma cholesterol ester and erythrocyte (RBC) omega 3 fatty acids (suppl 2), and plasma and RBC alpha-tocopherol (suppl 1 and 2) increased. Suppl 1 decreased irreversibly sickled cells by 37.5%, decreased RBC protoporphyrin and urinary porphyrins, and increased the RBC total fatty acid-cholesterol ratio. Suppl 2 decreased plasma triglycerides, further increased the RBC alpha-tocopherol, moderately increased the RBC double-bond index, but decreased the RBC total fatty acid-cholesterol ratio. Zinc, copper, and porphyrins showed prolonged changes. The supplements did not change hemoglobin concentrations, RBC age (reticulocytes, polyamines), or number of aplastic and vasoocclusive crises. Zinc reduces irreversibly sickled cells. Augmentation of RBC antioxidant status by alpha-tocopherol and vitamin C and incorporation of omega 3 fatty acids into RBCs do not affect hemolytic component. Effects on vasoocclusive component are unclear.