The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study

<p>Abstract</p> <p>Background</p> <p>Available iron chelation regimes in thalassaemia may achieve different changes in cardiac and hepatic iron as assessed by MR. The aim of this study was to assess the efficacy of four available iron chelator regimes in 232 thalassaemia major patients by assessing the rate of change in repeated measurements of cardiac and hepatic MR.</p> <p>Results</p> <p>For the heart, deferiprone and the combination of deferiprone and deferoxamine significantly reduced cardiac iron at all levels of iron loading. As patients were on deferasirox for a shorter time, a second analysis ("Initial interval analysis") assessing the change between the first two recorded MR results for both cardiac and hepatic iron (minimum interval 12 months) was made. Combination therapy achieved the most rapid fall in cardiac iron load at all levels and deferiprone alone was significantly effective with moderate and mild iron load. In the liver, deferasirox effected significant falls in iron load and combination therapy resulted in the most rapid decline.</p> <p>Conclusion</p> <p>With the knowledge of the efficacy of the different available regimes and the specific iron load in the heart and the liver, appropriate tailoring of chelation therapy should allow clearance of iron. Combination therapy is best in reducing both cardiac and hepatic iron, while monotherapy with deferiprone or deferasirox are effective in the heart and liver respectively. The outcomes of this study may be useful to physicians as to the chelation they should prescribe according to the levels of iron load found in the heart and liver by MR.</p

Do Children and Adolescents with Overweight or Obesity Adhere to the National Food-Based Dietary Guidelines in Greece?

Childhood obesity increases the risk for metabolic disorders, but is also related to nutritional deficiencies, such as anemia and hypovitaminosis D. Although children/adolescents with overweight/obesity may have higher energy intake, their diet quality and diversity may be low. The present study aimed to evaluate the consumption of foods against the national food-based dietary guidelines in children and adolescents with overweight or obesity in Greece. Sociodemographic, anthropometric and lifestyle data were collected from a sample of 1467 children 2–18 years old (962 obese, 505 overweight, 51.2% females) in 2014–2017. The results of this study show that the consumption of dairy products, fruit, vegetables, legumes and fish by children/adolescents with overweight or obesity was lower than the national recommendations (ranging from a minimum of 39.5% for fish, to a maximum of 75.5% for cereal/potato/rice). Only the consumption of meat/poultry was found to exceed the national recommendation (estimated coverage of 131.3%). Moreover, a large proportion of participants regularly consumed various unhealthy foods/beverages. The present findings indicate that the majority of children/adolescents with overweight/obesity do not comply with the national food-based dietary guidelines in Greece. The implementation of new strategies to promote healthy diets among children/adolescents with overweight/obesity are urgently required

Lifestyle Changes and Determinants of Children’s and Adolescents’ Body Weight Increase during the First COVID-19 Lockdown in Greece: The COV-EAT Study

Previous studies showed that the coronavirus disease 2019 (COVID-19) lockdown imposed changes in adults’ lifestyle behaviors; however, there is limited information regarding the effects on youth. The COV-EAT study aimed to report changes in children’s and adolescents’ lifestyle habits during the first COVID-19 lockdown and explore potential associations between changes of participants’ lifestyle behaviors and body weight. An online survey among 397 children/adolescents and their parents across 63 municipalities in Greece was conducted in April–May 2020. Parents self-reported changes of their children’s lifestyle habits and body weight, as well as sociodemographic data of their family. The present study shows that during the lockdown, children’s/adolescents’ sleep duration and screen time increased, while their physical activity decreased. Their consumption of fruits and fresh fruit juices, vegetables, dairy products, pasta, sweets, total snacks, and breakfast increased, while fast-food consumption decreased. Body weight increased in 35% of children/adolescents. A multiple regression analysis showed that the body weight increase was associated with increased consumption of breakfast, salty snacks, and total snacks and with decreased physical activity. The COV-EAT study revealed changes in children’s and adolescents’ lifestyle behaviors during the first COVID-19 lockdown in Greece. Effective strategies are needed to prevent excessive body weight gain in future COVID-19 lockdowns

Natural and vaccine-induced immunity to Neisseria meningitidis serogroup C in asplenic patients with beta-thalassemia

Natural and vaccine-induced immunity to meningococcus C (MenC) was evaluated in asplenic adolescents and adults with beta-thalassemia. At baseline 19.3% of patients and 22.8% age-matched controls had positive (&gt; 2 mu g/ml) naturally acquired Men C- specific IgG antibodies; patients had a lower probability of having protective SBA compared to controls (OR =21, p = 0.012). MenC conjugate vaccine (MCC) induced protective IgG concentrations in 63% of patients and 90.1% of controls. SBA increased significantly post vaccination and there were no differences between patients and controls; however patients had significantly lower IgG concentrations post vaccination compared to controls (4.52 vs 10.94 mu g/ml. p &lt; 0.001, respectively). A second dose of MCC given to 11 patients who had received MCC in the past induced higher IgG compared to primary response (p =, 0.001). Naturally- and vaccine-induced immunity to MenC is impaired in asplenic beta-thalassemics; a second dose of MCC improves vaccine immunogenicity and is essential for their optimal protection. (C) 2011 Elsevier Ltd. All rights reserved

CARDIAC MAGNETIC RESONANCE IN TRANSFUSION DEPENDENT THALASSAEMIA: ASSESSMENT OF IRON LOAD AND RELATION TO LEFT VENTRICULAR EJECTION FRACTION

International audienceCardiac Magnetic Resonance (CMR) has replaced all other surrogate measurements in the determination of transfusional cardiac iron overload in patients with thalassaemia major. We aimed to determine the diagnostic value of CMR T2* with respect to cardiac dysfunction (CD) as determined by CMR-derived left ventricular ejection fraction (LVEF). Cardiac T2* values and LVEF measured by CMR were recorded in 303 patients with thalassaemia major, at the time of their first CMR. T2* was correlated with LVEF (regression coefficient: 0.57, p 8ms and ≤14 ms and reduced to 9.1% in patients with T2* between 14-20 ms. As the probability of CD is progressively, and not suddenly, reduced with increasing values of T2*, CMR has a limited diagnostic value for cardiac dysfunction (ROC Analysis, AUC = 0.68). Patients with cardiac T2* ≤ 8 ms require careful and intensive management. This risk decreases with increasing values of T2* but even in mildly loaded patients the probability of impaired LVEF is not negligible

Age, beta thalassaemia trait, and iron-deficient anaemia significantly affect reticulocyte indices in pre-school children

Reticulocyte indices are easy to obtain, low cost parameters and have gained interest in the field of diagnosing anaemias of childhood. We assessed distribution, age and gender variation, relation to indices of iron metabolism and diagnostic performance of reticulocyte haemoglobin content (CHr), percentage of microcytic reticulocytes (micro_r), percentage of hypochromic reticulocytes (hypo_r), and percentage of reticulocytes with low CHr (low_CHr) in 386 pre-school children classified in four groups: healthy, iron deficiency (ID), iron deficiency anaemia (IDA), and beta-thalassaemia carriers (beta-thal). Age had a positive effect in CHr (Spearman’s rho = 0.21) and a negative effect in hypo_r (Spearman’s rho = -0.2) in healthy children. CHr and low_CHr were related to ferritin in the IDA group (Spearman’s rho 0.55 and -0.53, respectively). In the beta-thal group, HbA(2) is strongly related to all reticulocyte indices. micro_r and CHr performed best in discriminating between IDA and beta-thal heterozygosity (ROC analysis, area under the curve (AUC): 0.76 and 0.74, respectively). CHr achieved the best AUC (0.58) in identifying ID among children without anaemia. Age, IDA and beta-thal significantly affect reticulocyte indices. CHr and micro_r may have a role as screening tools in discriminating between IDA and beta-thal heterozygosity

Increased Serum Concentrations of High Mobility Group Box 1 (HMGB1) Protein in Children with Autism Spectrum Disorder

High mobility group box 1 protein (HMGB1) has been suggested to be involved in the immune dysfunction and inflammation reported in autism spectrum disorder (ASD). We aimed to assess HMGB1 serum concentrations (SCs) in high-functioning ASD children compared to typically developing (TD) controls and to explore their associations with the autism spectrum quotient (AQ), the empathy quotient (EQ), and the systemizing quotient (SQ). The study involved 42 ASD children and 38 TD children, all-male, aged between 6.1 and 13.3 years old. HMGB1 SCs were measured by enzyme-linked immunosorbent assay (ELISA). Groups were comparable regarding age, general IQ, birth weight, and maternal age at birth. ASD children showed significantly higher HMGB1 SCs compared to TD children (1.25 ± 0.84 ng/mL versus 1.13 ± 0.79 ng/mL, respectively, p = 0.039). The Spearman’s rho revealed that HMGB1 SCs were positively correlated with the AQ attention to detail subscale (rs = 0.46, p = 0.045) and with the SQ total score (rs = 0.42, p = 0.04) in the ASD group. These results show that HMGB1 serum concentrations are altered in ASD children, and suggest that inflammatory processes mediated by HMGB1 may be associated with specific cognitive features observed in ASD