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.

Iron absorption in adults with sickle cell anemia: a stable-isotope approach.

PURPOSE: Iron absorption in sickle cell anemia (SCA) remains unclear and studies in adults with SCA are scarce. The aim of this study was to evaluate the iron absorption SCA adults and its association with iron status and hepcidin concentration. METHODS: SCA patients (n = 13; SCAtotal) and control participants (n = 10) ingested an oral stable iron isotope (57Fe). Iron absorption was measured by inductively coupled plasma mass spectrometry (ICP-MS) 14 days after isotope administration. Patients with ≥ 1000 ng/mL serum ferritin were considered to present iron overload (IO) (SCAio+; n = 3) and others classified without IO (SCAio-; n = 10). RESULTS: Iron absorption in the control group ranged from 0.3 to 26.5% (median = 0.9%), while it varied from 0.3 to 5.4% in SCAio+ (median = 0.5%) and from 0.3 to 64.2% in the SCAio- (median = 6.9%). Hepcidin median values were 14.1 ng/mL (3.0-31.9 ng/mL) in SCAio-, 6.2 ng/mL (3.3-7.8 ng/mL) in SCAio + and 6.2 ng/mL (0.6-9.3 ng/mL) in control. Iron absorption was associated with ferritin level (r = - 0.641; p = 0.018) and liver iron concentration (LIC; r = - 0.786; p = 0.036) in the SCAtotal group. CONCLUSION: Our data suggest that SCAio- individuals may be at risk of developing primary IO. Simultaneously, secondary IO may induce physiological adaptation, resulting in reduced iron absorption. Further studies evaluating intestinal iron absorption using larger sample sizes should be conducted to help establish a safe nutrition approach to be adopted and to ensure the security of food-fortifying public policies for these patients. TRIAL REGISTRATION: This trial was registered at www.ensaiosclinicos.gov.br (Identifier RBR-4b7v8pt).

Novel bioanalytical strategy using isotope pattern deconvolution and ICP-QMS for the study of iron incorporation in erythrocytes: An insight to better assessment.

Iron is an essential element for human life and its nutritional status in the human body is directly linked to human health. More than 1015 atoms of iron per second are necessary for the maintenance of haemoglobin formation. To predict iron bioavailability three approaches are normally employed: (a) faecal recovery; (b) plasma appearance; and (c) erythrocyte incorporation (the most used). Isotope Pattern Deconvolution (IPD) is a mathematical tool that allows the isolation of distinct isotope signatures from mixtures of natural abundance and enriched tracers. In this work we propose a novel strategy to assess erythrocyte iron incorporation, based on the use of an iron stable isotope (57Fe) and the IPD concept. This strategy allows direct calculation of the exogenous concentration of 57Fe incorporated into RBCs after supplementation. In this way, to determine the mass of iron incorporated into erythrocytes, the unique prediction that must be made is the blood volume, estimate to reproduce the natural dilution of the tracer (57Fe) in the blood. This novel bioanalytical approach was applied for the measurements of iron incorporation and further iron absorption studies in humans, using a group of twelve healthy participants, that should be further evaluated for the assessment of other chemical elements that could be of health concerns and directly impact society.

Condições clínicas, nutricionais e sociais de crianças com doença falciforme acompanhadas em um centro de referência: estudo descritivo / Clinical, nutitional and family conditions of children with sickle cell disease followed up in a referral hematology center: a descriptive study

Objetivo: Descrever as características clínicas, sociais e nutricionais de crianças com doença falciforme de 5-9 anos. Método: Trata-se de estudo seccional em amostra aleatória de 190 crianças avaliada entre 2015 e 2016, cadastradas em um hospital de referência em hematologia do Rio de Janeiro, Brasil. Resultados: A maioria das crianças estudadas possuía o genótipo Hb SS (65,3%), teve seu diagnóstico na triagem neonatal (91%) e iniciou o acompanhamento médico antes dos seis meses de idade (91,5%). Excesso de peso e baixa estatura foram encontrados em 14,2% e 6,2% das crianças, respectivamente. Em relação às condições sociais da família, a maioria dos pais possuíam companheiros (60,5%), viviam com até meio salário mínimo (55,8%) e as mulheres deixaram seus empregos para acompanhar o tratamento da criança (52,4%). Conclusão: As crianças com doença falciforme foram diagnosticadas precocemente, aproximadamente um sexto delas estavam em excesso de peso e 20% declararam renda de até um quarto de salário mínimo. O fortalecimento da rede de atenção no SUS às pessoas com DF é uma importante medida para mitigar os problemas identificados neste estudo. (AU)

Objective: To describe the clinical, social and nutritional characteristics of children with sickle cell disease aged 5 to 9 years. Method: Cross-sectional study carried out in 2015- 2016 with a random sample of 190 children receiving care at a reference hematology hospital in Rio de Janeiro, Brazil. Results: The majority of the children had the genotype Hb SS (65.3%), were diagnosed with SCD during newborn screening (91%) and began clinical treatment before six months of age (91.5%). Overweight and low stature were found in 4.2% and 6.2% of the children, respectively. With respect to the household socioeconomic conditions, most of the parents had a partner (60.5%), lived with up to half the minimum wage (55.8%), and the women quitted their jobs to take care of their children (52.4%). Conclusion: Children with sickle cell disease were diagnosed early; nearly one sixth of them were overweight; and 20% of the parents had income of up to one fourth of the minimum wage. Strengthening the health care network in SUS for individuals with SCD is of vital importance to mitigate the problems identified in this study. (AU)