When the clinical manifestations of 272 patients with sickle cell anemia are compared with their level of fetal hemoglobin (HbF), the results suggest that there may be a threshold above which HbF is effective in ameliorating the morbidity of this disease. The age of entry of these SS patients into the study ranged from birth to 56 yr; the average length of follow-up was 11 yr for a total of 3,0...

To study whether the culture time at which the burst populations mature influences the expression of fetal hemoglobin in bursts, we measured hemoglobin synthesis in cohorts of fully hemoglobinized erythroid bursts maturing sequentially in cultures of adult peripheral blood BFU-Es. In 13 of 15 experiments, a decline in gamma/gamma + beta ratio was noted as the culture time advanced. On the avera...

Pharmacologic stimulation of fetal hemoglobin (HbF) expression may be a promising approach for the treatment of beta-thalassemia. In this study, we have investigated the HbF-inducing activity and molecular mechanisms of specific histone deacetylase (HDAC) inhibitors in human K562 erythroleukemia cells. Apicidin was the most potent agent compared with other HDAC inhibitors (trichostatin A, MS-27...

In utero exposure to bisphenol A (BPA) increases mammary cancer susceptibility in offspring. High-fat diet is widely believed to be a risk factor of breast cancer. The objective of this study was to determine whether maternal exposure to BPA in addition to high-butterfat (HBF) intake during pregnancy further influences carcinogen-induced mammary cancer risk in offspring, and its dose-response c...

BACKGROUND FMH quantification is necessary to calculate an individual dose of prophylactic anti-RhD immunoglobulin and to diagnose fetal anaemia causes. We encountered a healthy woman with a numerous RBCs containing fetal haemoglobin (HbF). AIMS To investigate the cause of this sign and the correct evaluation of fetal RBCs in maternal circulation. MATERIALS AND METHODS Patients samples and ...

Sickle cell disease (SCD) results from a point mutation in the β-globin gene forming hemoglobin S (HbS), which polymerizes in deoxygenated erythrocytes, triggering recurrent painful vaso-occlusive crises and chronic hemolytic anemia. Reactivation of fetal Hb (HbF) expression ameliorates these symptoms of SCD. Nuclear factor (erythroid derived-2)-like 2 (Nrf2) is a transcription factor that trig...

Circulating 14-day erythroid progenitors (BFU-E) from 28 sickle cell anemia (SS) patients with hemoglobin F (HbF) levels ranging from 2% to 16% were studied to determine their sensitivity to [3H] thymidine kill and burst-promoting activity (BPA)-like factor production. We find that the proportion of BFU-E sensitive to 3H-dT kill, and hence active in DNA synthesis, was inversely correlated with ...

When hemolytic anemia was induced in 26 baboons (Papio cynocephalus), aged 7-22 mo, they increased their production of fetal hemoglobin (HbF). Although the resulting reduction in hematocrits and increases of reticulocyte counts were similar in all stressed animals there was marked variability in the maximal rates of HbF synthesis. The maximal levels of HbF attained appeared to fall into three s...

Percentage fetal haemoglobins (% HbF) were measured in 7081 newborns at the University Hospital, Nottingham, between 1990 and 1992 to assess the effects of a range of factors, and to determine if there was an association between % HbF at birth and risk of SIDS (sudden infant death syndrome). Data from the obstetric clinical records were analysed by multiple linear regression to establish the ma...