We have studied the cellular and molecular responses to long-term hydroxyurea (HU) treatment in 29 severely affected young patients with sickle cell disease (mean age, 10.9 6 4.1 years). Patients received HU at 20 mg/kg/d on 4 consecutive days per week initially, with a monthly escalated dose avoiding marrow-toxicity (mean steady-state dose, 34.2 6 4.6 mg/kg/d) for 12 to 36 months (mean duratio...

Mechanisms underlying fetal hemoglobin (HbF) reactivation in stress erythropoiesis have not been fully elucidated. We suggested that a key role is played by kit ligand (KL). Because glucocorticoids (GCs) mediate stress erythropoiesis, we explored their capacity to potentiate the stimulatory effect of KL on HbF reactivation, as evaluated in unilineage erythropoietic culture of purified adult pro...

Background and Objectives. We report on two families in which the β0-thalassemia mutation IVS2+1G→A occurs either in the homozygous or compound heterozygous condition with other β-thalassemia determinants. In the first family the proband, homozygous for the IVS2+1 determinant, is asymptomatic and was detected by chance during a screening program for β-thalassemia. In the second family, the prob...

Arabinoxylans were prepared from different hull-less barley milling fractions (bran, shorts and flour). The yields of hull-less bran arabinoxylan (HBB-AX), shorts arabinoxylan (HBS-AX) and flour arabinoxylan (HBF-AX) were 8.42%, 4.08% and 2.13% respectively. Sugar composition analysis showed that arabinose and xylose were the main sugars. HBF-AX had the highest Ara/Xyl ratio, followed by HBS-AX...

Although hematopoietic stem cell transplantation and gene therapy have the potential to cure β-thalassemia and sickle cell disease, they are not currently available to most people with these diseases. In the near term, pharmacologic induction of fetal hemoglobin (HbF) may offer the best possibility for safe, effective, and widely available therapy. In an effort to define new pathways for target...

Currently, bone marrow transplantation is the only curative treatment for β-thalassemia and sickle cell disease. In rare cases, sustained and full fetal hemoglobin production was observed in patients after failure of bone marrow transplantation. This rendered the patients transfusion-free, despite genetic disease and transplant rejection. The mechanisms underlying this phenomenon remain unexplo...

Sickle cell disease (SCD) comprises a group of genetic disorders in before one year of age in SCA and the adhesion of reticulocytes to the which the red blood cells (RBCs) produce abnormal sickle haemoglobin (HbS) that can polymerise when oxygen concentrations are low. The clinical manifestations of SCD are numerous, and vary from patient to patient, but recurrent vaso-occlusive processes can c...

hepatic dysfunction is a frequent manifestation in patients with sickle cell anemia. it is usually a multifactorial process. a rare benign form of extreme hyperbilirubinemia, presumably due to intrahepatic sickling, may be the cause. we report a 9 year old girl with sickle-thalassemia hemoglo binopathy, presenting with profound jaundice. sickle cell disease is often mild in the iranian populati...

The downlink of a reconfigurable intelligent surface (RIS)-aided multi-user (MU) millimeter wave (mmWave) multiple-input multiple-output (MIMO) system relying on non-diagonal RIS (NDRIS) phase shift matrix is considered. A max-min fairness (MMF) problem formulated under the total transmit power constraint while employing joint active hybrid beamforming (HBF) both at BS as well each user equipme...