Disease relevance of Anemia, Sickle Cell

• Alleles associated with sickle cell anemia, beta-thalassemia and deficiency in glucose-6-phosphate dehydrogenase have a protective effect against malaria and may have been retained by positive selection in areas of endemic malaria [1].
• In three patients with sickle-cell anemia who had been disabled by recurrent painful crises, sustained dilutional hyponatremia was induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in combination with a high fluid intake [2].
• Thus, CLT and MIC can reverse dehydration and K+ loss of SAD 1 mouse erythrocytes in vitro and in vivo, further supporting the potential utility of these drugs in the treatment of sickle cell anemia [3].
• Clomiphene-responsive hypogonadism in sickle cell anemia [4].
• The currently available polyvalent polysaccharide vaccine induces antibody production among immunologically competent recipients against the 14 serotypes responsible for 80% of pneumococcal bacteremia in the efficacious in clinical trials with healthy young men in epidemic conditions and in patient with sickle cell anemia [5].

Psychiatry related information on Anemia, Sickle Cell

• Five chronic conditions were selected to illustrate patterns of service use: asthma, attention deficit disorder, diabetes, epilepsy, and sickle cell anemia [6].

High impact information on Anemia, Sickle Cell

• Sickle cell anemia (SCA) is a paradigmatic single gene disorder caused by homozygosity with respect to a unique mutation at the beta-globin locus [7].
• In patients with sickle cell anemia, regardless of clinical status, the circulating endothelial cells were predominantly microvascular in origin (CD36-positive), and most of the cells expressed four markers of endothelial-cell activation: intercellular adhesion molecule 1, vascular-cell adhesion molecule 1, E-selectin, and P-selectin [8].
• Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia [9].
• We treated four patients who were receiving hydroxyurea for sickle cell disease (three who were homozygous for sickle cell anemia and one with sickle beta zero-thalassemia) with escalating doses of intravenous erythropoietin for seven weeks, along with oral iron sulfate [10].
• Stimulation of F-cell production in patients with sickle-cell anemia treated with cytarabine or hydroxyurea [11].

Chemical compound and disease context of Anemia, Sickle Cell

• The long-term safety of hydroxyurea in patients with sickle cell anemia is uncertain [9].
• To investigate the mechanism of pharmacologic stimulation of fetal hemoglobin in sickle-cell anemia (hemoglobin S disease), we treated two patients with homozygous disease with various doses of cytarabine (also known as Ara-C) or hydroxyurea and evaluated the effects of each treatment on F-reticulocyte production and on hemopoiesis [11].
• We treated five subjects who have sickle cell anemia with oral clotrimazole, a specific Gardos channel inhibitor [12].
• We concentrated ISC's from patients with sickle cell anemia and compared the morphology and protein composition of ghosts and Triton-extracted ghost residues prepared from these ISC's with similar preparations of reversibly sickable cells and normal cells [13].
• Hepatic bilirubin UDP-glucuronyltransferase in patients with sickle cell anemia [14].

Biological context of Anemia, Sickle Cell

• Structural analysis of the 5' flanking region of the beta-globin gene in African sickle cell anemia patients: further evidence for three origins of the sickle cell mutation in Africa [15].
• In sickle cell anemia, different fetal hemoglobin levels are associated with distinct beta-globin gene haplotypes [16].
• Production of F cells in sickle cell anemia: regulation by a genetic locus or loci separate from the beta-globin gene cluster [17].
• When sera from normal subjects and patients with a range of hematologic disorders were examined by Western blotting, a protein with a molecular mass of 34 Kd was detected in sera from patients with enhanced erythropoiesis including sickle cell anemia, thalassemia, and megaloblastic anemia [18].
• In addition, the known ability of hydroxyurea to ease the pain of sickle cell anemia patients may be the result of vasodilation by the drug-derived nitric oxide [19].

Anatomical context of Anemia, Sickle Cell

• Each had a 2--3 fold higher concentration of beta-globin mRNA precursor than that found in control bone marrow cells from patients with sickle cell anemia [20].
• Neutrophil zinc levels in patients with sickle cell anemia correlated significantly with height and weight, and with serum testosterone in men [21].
• 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 [22].
• In patients with sickle cell anemia, up to 25% of reticulocytes express the alpha 4 beta 1-integrin complex [23].
• The abnormal adherence of sickle red blood cells (SS RBC) to vascular endothelium may play an important role in vasoocclusion in sickle cell anemia [24].

Gene context of Anemia, Sickle Cell

• We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS [25].
• Integrin alpha 4 beta 1 and glycoprotein IV (CD36) are expressed on circulating reticulocytes in sickle cell anemia [26].
• Further studies demonstrated an inverse correlation between the binding affinity of the BP1 protein for the distal beta-globin silencer sequence and the severity of sickle cell anemia, suggesting a possible role for BP1 in determining the production of hemoglobin S [27].
• Natural coagulation inhibitors (protein C, protein S, antithrombin) in patients with sickle cell anemia in a steady state [28].
• These data suggest that TR2/TR4 forms the core of a larger DRED complex that represses embryonic and fetal globin transcription in definitive erythroid cells, and therefore that inhibition of its activity might be an attractive intervention point for treating sickle cell anemia [29].

Analytical, diagnostic and therapeutic context of Anemia, Sickle Cell

• Here we report that oral administration of HU for the treatment of sickle cell anemia produced detectable nitrosyl hemoglobin [30].
• Increased bone marrow blood flow in sickle cell anemia demonstrated by thallium-201 and Tc-99m human albumin microspheres [31].
• METHODS: A 110-bp fragment of the beta-globin gene including the sickle cell anemia locus (A17T) was amplified by PCR in the presence of either the saturating DNA dye, LCGreen Plus, or SYBR Green I [32].
• Long-term follow-up and booster immunization with polyvalent pneumococcal polysaccharide in patients with sickle cell anemia [33].
• Prevention of secondary stroke and resolution of transfusional iron overload in children with sickle cell anemia using hydroxyurea and phlebotomy [34].

References