Bone Marrow Failure in Fanconi Anemia: Clinical and Genetic Spectrum in a Cohort of 20 Pediatric Patients

Prognostic refinement in Fanconi anemia (FA) is needed, especially when considering allogeneic hematopoietic stem cell transplantation (HCT). We studied 20 children with FA and bone marrow failure from a single center. According to Hôpital Saint-Louis risk classification for FA, patients were classified in stage A (no or mild cytopenia/dysplasia), B (single non-high-risk cytogenetic abnormality), C (severe cytopenia and/or significant dysplasia and/or high-risk cytogenetic abnormality), and D (myelodysplastic syndrome with excess of blasts/acute myeloid leukemia) in 4, 2, 13, and 0 cases, respectively. Nine patients received androgens +/- steroids, with a response rate of 30%, and 11 patients underwent HCT. Ten-year cumulative incidence (CI) of myelodysplastic syndrome/acute myeloid leukemia and overall survival (OS) were 21.9% and 45.3%, respectively, in the entire cohort, whereas cumulative incidence of transplantation-related mortality and OS were 27% and 63%, respectively, in patients who underwent HCT. Patients with significant dysplasia at diagnosis (stages C and D) had significantly shorter OS post-HCT as compared with patients without dysplasia. All patients in stages C and D at diagnosis or during evolution died from their disease. HCT in recent years was associated with more favorable outcomes. Larger cohorts could validate homogenous reporting of risk and help decision-making, particularly for HCT. © 2019 Wolters Kluwer Health, Inc. All rights reserved

The double burden of obesity and iron deficiency on children and adolescents in Greece: the Healthy Growth Study

Background: Some small cohort studies have noted that obesity co-exists with lower serum iron levels. The present study aimed to examine the association between being overweight and iron deficiency (ID) in a large cohort of Greek children and adolescents. Methods: A representative sample of 2492 primary schoolchildren aged 9-13 years old was examined. Anthropometric, biochemical, clinical, dietary intake and physical activity data were collected. Results: The prevalence of ID and iron deficiency anaemia (IDA) was higher in obese boys and girls compared to their normal-weight peers (P < 0.05). Serum ferritin was higher in obese compared to normal-weight boys (P = 0.024) and higher in obese compared to normal-weight and overweight girls (P = 0.001). By contrast, a negative association was found between transferrin saturation and adiposity in both boys and girls (P = 0.001 and P = 0.005). Furthermore, obese girls had significantly higher fibre intake than normal-weight girls (P = 0.048) and also overweight and obese boys and girls recorded significantly fewer pedometer steps than their normal-weight peers (P < 0.001). Finally, obesity more than doubled the likelihood of ID in both boys (odds ratio = 2.83; 95% confidence inteval = 1.65-4.85) and girls (odds ratio = 2.03; 95% confidence interval = 1.08-3.81) after controlling for certain lifestyle and clinical indices as potential confounders. Conclusions: The present study shows that obese children and adolescents were at greater risk for ID and IDA than their normal-weight peers. Low grade inflammation induced by excessive adiposity may be a reason for the observed low iron levels. This is also strengthened by the elevated serum ferritin levels, comprising an acute phase protein that is plausibly increased in inflammation

COVID-19 among children with cancer in Greece (2020): Results from the Nationwide Registry of Childhood Hematological Malignancies and Solid Tumors (NARECHEM-ST)

[No abstract available