Iron incorporation in red blood cells of pediatric sickle cell anemia: a stable isotope pilot investigation.

BACKGROUND/OBJECTIVES: Sickle cell anemia (SCA) is marked by hypoxia, inflammation, and secondary iron overload (IO), which potentially modulate hepcidin, the pivotal hormone governing iron homeostasis. The aim was to evaluate the iron incorporation in red blood cells (RBC) in SCA pediatric patients, considering the presence or absence of IO. SUBJECTS/METHODS: SCA children (n = 12; SCAtotal) ingested an oral stable iron isotope (57Fe) and iron incorporation in RBC was measured after 14 days. Patients with ≥1000 ng/mL serum ferritin were considered to present IO (SCAio+; n = 4) while the others were classified as being without IO (SCAio-; n = 8). Liver iron concentration (LIC) was determined by Magnetic Resonance Imaging (MRI) T2* method. RESULTS: The SCAio+ group had lower iron incorporation (mean ± SD: 0.166 ± 0.04 mg; 3.33 ± 0.757%) than SCAio- patients (0.746 ± 0.303 mg; 14.9 ± 6.05%) (p = 0.024). Hepcidin was not different between groups. Iron incorporation was inversely associated with serum ferritin level (SCAtotal group: r = -0.775, p = 0.041; SCAio- group: r = -0.982; p = 0.018) and sickle hemoglobin (HbS) presented positive correlation with iron incorporation (r = 0.991; p = 0.009) in SCAio- group. LIC was positively associated with ferritin (SCAtotal: r = 0.921; p = 0.026) and C reactive protein (SCAio+: r = 0.999; p = 0.020). CONCLUSION: SCAio+ group had lower iron incorporation in RBC than SCAio- group, suggesting that they may not need to reduce their intake of iron-rich food, as usually recommended. Conversely, a high percentage of HbS may indirectly exacerbate hypoxia and seems to increase iron incorporation in RBC. TRIAL REGISTRATION: This trial was registered at www.ensaiosclinicos.gov.br . Identifier RBR-4b7v8pt.

The effect of food and nutrients on iron overload: what do we know so far?

There has been no established food and nutrition guidance for diseases characterized by the presence of iron overload (IOL) yet. Hepcidin is a hormone that diminishes iron bioavailability. Its levels increase in response to increased iron stores. Hence, IOL conditions could hypothetically trigger a self-regulatory mechanism for the reduction of the intestinal absorption of iron. In addition, some food substances may modulate intestinal iron absorption and may be useful in the dietary management of patients with IOL. This scoping review aimed to systematize studies that support dietary prescriptions for IOL patients. It was carried out according to the method proposed by the Joanna Briggs Institute and the preferred reporting items for systematic reviews and meta-analyses (PRISMA). Although the need to restrict iron in the diet of individuals with hemochromatosis is quite clear, there is a consensus that IOL diminishes the rate of iron absorption. Reduced iron absorption is also present and has been reported in some diseases with transfusion IOL, in which serum hepcidin is usually high. The consumption of polyphenols and 6-shogaol seems to reduce iron absorption or serum ferritin concentration, while procyanidins do not cause any changes. Vitamin C deficiency is often found in IOL patients. However, vitamin C supplementation and alcohol consumption should be avoided not only because they increase iron absorption, but also because they provoke toxic oxidative reactions when the iron is excessive. Dietary approaches must consider the differences in the pathophysiology and treatment of IOL diseases.

Nutritional status of human T-lymphotropic virus 1 patients: A retrospective study.

BACKGROUND & AIMS: The nutritional status of people with human T-lymphotropic virus (HTLV-1) infection has been poorly described because it involves a neglected disease. The few studies that have been conducted mostly involve people with neurologic consequences and the possible clinical evolutions of the disease. The aim of this study was to describe the nutritional status of patients with HTLV-1, including those with associated myelopathy/tropical spastic paraparesis, and to evaluate food security in these patients. METHODS: A retrospective observational study was conducted in people with HTLV-1 admitted to a referral hospital. We collected data from 17 medical records, including anthropometric data (i.e., body mass index, mid-upper arm circumference, triceps skinfold, and mid-arm muscle circumference), laboratory test results (i.e., haemoglobin, haematocrit, albumin, globulin, iron fixation capacity, and iron), the Subjective Global Assessment (SGA) method, and food security (Brazilian Food Insecurity Scale) data. The data were analysed using the R-project software. To evaluate possible associations between the outcomes and predictors (age at hospitalisation, food security, presence of children <18 years of age living in the household, income, schooling, ANSG, BMI, difference between ideal weight and hospitalisation, TSF, MUAC ICU days, hospitalisation outcome, rehospitalisation in the first year after discharge, interval between readmissions, death, associated conditions, constipation upon admission), we used Kruskal-Wallis, Mann-Whitney, Fisher's exact, chi-square tests with continuity correction, and Spearman's correlation coefficient. Hypothesis tests were considered statistically significant when p ≤ 0.05. RESULTS: The mean age of the patients was 57 (52-60) years. The patients were predominantly women (59%) and had an income lower than the local minimum wage with at least 6 years of schooling (52.3%). Only 18.2% of patients were eutrophic according to their BMI and 23.5% of patients were malnourished based on the SGA method. Patients predominantly had food security (64.7%) and good intestinal functions (64.7%) during their hospital stay. CONCLUSION: Despite having a limited number of patients in this study, HTLV-1 patients admitted to hospital are at high risk of malnutrition based on the scores from the SGA method.

Food insecurity and social support in families of children with sickle-cell disease / Insegurança alimentar e apoio social em famílias de crianças com doença falciforme

Abstract Objective: To examine the associations between food insecurity (IA) and social support in families of children with sickle-cell disease (DF). Methods: This cross-sectional study in families of 190 children from five to nine years old in follow-up at a hematology referral hospital in Rio de Janeiro State. IA was measured using the Brazilian food insecurity scale and social support was measured using the Brazilian version of the MOS social support survey instrument. The relation between IA and social support was analyzed by way of a multinomial logistic model. Results: There was IA in 62.2% of the families, in moderate and severe form in, respectively, 11.1% and 7.9% of cases. In families of children with DF, levels of mild and severe food insecurity (IALe and IAGr) were found to relate inversely to levels of social support in the following dimensions: informational support (IALe OR = 0.98; 95% CI 0.96-0.99 and IAGr OR = 0.95; 95% CI 0.92-0.98); social interaction (IALe OR = 0.98; 95% CI 0.96-0.99 and IAGr OR = 0.96; 95% CI 0.93-0.99) and tangible social support (IALe OR = 0.97; 95% CI 0.96-0.99 and IAGr OR = 0.97; 95% CI 0.94-0.99). Conclusion: Considering the positive effects of social support on IA, public policies should be encouraged to assure food and nutritional security and social assistance for care for people with DF. Also, social support groups for people with DF should be strengthened.

Resumo Objetivo: Avaliar as associações entre insegurança alimentar (IA) e apoio social em famílias de crianças com doença falciforme (DF). Métodos: Estudo transversal feito com as famílias de 190 crianças, de cinco a nove anos, atendidas em um hospital de referência em hematologia no Estado do Rio de Janeiro. A IA foi medida com a escala brasileira de insegurança alimentar e o apoio social foi medido com a versão brasileira do instrumento de pesquisa de apoio social MOS. A relação entre IA e o apoio social foi analisada por meio de um modelo logístico multinomial. Resultados: Havia IA em 62,2% das famílias, nas formas moderada e grave em 11,1% e 7,9% dos casos, respectivamente. Em famílias de crianças com DF, os níveis de insegurança alimentar leve e grave (IAL e IAG) apresentaram relação inversa com os níveis de apoio social nas seguintes dimensões: apoio informativo (IAL OR = 0,98; IC 95%: 0,96-0,99 e IAG OR = 0,95; IC 95%: 0,92-0,98); interação social (IAL OR = 0,98; IC 95%: 0,96-0,99 e IAG OR = 0,96; IC 95%: 0,93-0,99) e material (apoio social tangível) (IAL OR = 0,97; IC 95%: 0,96-0,99 e IAG OR = 0,97; 95% CI 0,94-0,99). Conclusão: Considerando os efeitos positivos do apoio social na IA, as políticas públicas devem ser encorajadas a garantir segurança alimentar e nutricional e assistência social para o atendimento de pessoas com DF. Além disso, os grupos de apoio social para pessoas com DF devem ser fortalecidos.

Serum Hepcidin Concentration in Individuals with Sickle Cell Anemia: Basis for the Dietary Recommendation of Iron.

Dietary iron requirements in patients with sickle cell disease (SCD) remain unclear. SCD is a neglected hemoglobinopathy characterized by intense erythropoietic activity and anemia. Hepcidin is the hormone mainly responsible for iron homeostasis and intestinal absorption. Intense erythropoietic activity and anemia may reduce hepcidin transcription. By contrast, iron overload and inflammation may induce it. Studies on SCD have not evaluated the role of hepcidin in the presence and absence of iron overload. We aimed to compare serum hepcidin concentrations among individuals with sickle cell anemia, with or without iron overload, and those without the disease. Markers of iron metabolism and erythropoietic activity such as hepcidin, ferritin, and growth differentiation factor 15 were evaluated. Three groups participated in the study: the control group, comprised of individuals without SCD (C); those with the disease but without iron overload (SCDw); and those with the disease and iron overload (SCDio). Results showed that hepcidin concentration was higher in the SCDio > C > SCDw group. These data suggest that the dietary iron intake of the SCDio group should not be reduced as higher hepcidin concentrations may reduce the intestinal absorption of iron.

Sobrecrescimento bacteriano no intestino delgado, absorçäo e tolerância à lactose e deficiência de ferro em pré-escolares de duas creches da cidade do Rio de Janeiro / Small bowel bacterial overgrowth, lactose absortion and tolerance and iron deficiency in childrn of two preschools in the city of the Rio de Janeiro

Objetivos: Investigar a ocorrência de sobrecrescimento bacteriano no intestino delgado; pesquisar a absorçao e a tolerância à lactose; avaliar a ocorrência de deficiência de ferro e estudar a relaçao entre má absorçao de lactose, sobrecrescimento bacteriano no intestino delgado e deficiência de ferro corporal. Metodologia: Este estudo foi desenvolvido com 104 pré-escolares de duas creches públicas da Cidade do Rio de Janeiro. Foram realizadas avaliaçao antropométrica, teste do hidrogênio no ar expirado com administraçao de lactulose e lactose e avaliaçao bioquímica. O teste com lactulose foi realizado com oferta de 10 gramas. As amostras de ar expirado foram coletadas em jejum, 15, 30, 45, 60, 90, 120, 150 e 180 minutos. O ponto de corte para o diagnóstico de sobrecrescimento bacteriano no intestino delgado foi o incremento na concentraçao de hidrogênio maior ou igual a 20 ppm acima do valor basal em jejum, nas amostras coletadas aos 15 e/ou 30 minutos, ou valor de hidrogênio na amostra basal 3 42 ppm. O teste com lactose foi realizado após jejum de 12 horas, com oferta de 2 gramas/kg de peso corporal em soluçao aquosa a 10 por cento. O ponto de corte para má absorçao da lactose foi o aumento da concentraçao de hidrogênio igual ou acima de 20 ppm em relaçao ao valor de jejum, em qualquer amostra de ar expirado coletada até 180 minutos. A identificaçao da deficiência de ferro foi realizada com o emprego do hemograma completo, ferritina, ferro sérico e capacidade total de ligaçao do ferro. Feito também parasitológico e sangue oculto nas fezes. Resultados: O estado nutricional esteve adequado em 100 (96,2 por cento) crianças. Duas crianças (1,9 por cento) apresentaram baixo peso e outras duas (1,9 por cento) baixa estatura. O parasitológico foi positivo em 24 (26,1 por cento) crianças e a presença de infestaçao mista, em 3 (3,3 por cento). O teste respiratório com lactulose identificou 9 (9,1 por cento) crianças nao produtoras de hidrogênio e 10 (10,1 por cento) com sobrecrescimento bacteriano. Houve associaçao significante entre parasitose intestinal e sobrecrescimento bacteriano. 43 crianças (47,8 por cento) apresentaram má absorçao da lactose e cinco foram intolerantes à lactose. Nao houve diferença significante entre o consumo de leite dos maus absorvedores de lactose é 'os de absorçao normal. Os exames bioquímicos de sangue revelaram que 16 (15,4 por cento) crianças eram anêmicas (hemoglobina < 11,0 g/dL) e 19 (35,9 por cento) apresentavam reserva...(au)