Anemia, Sickle Cell: A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.Sickle Cell Trait: The condition of being heterozygous for hemoglobin S.Anemia: A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.Hemoglobin, Sickle: An abnormal hemoglobin resulting from the substitution of valine for glutamic acid at position 6 of the beta chain of the globin moiety. The heterozygous state results in sickle cell trait, the homozygous in sickle cell anemia.Erythrocytes, Abnormal: Oxygen-carrying RED BLOOD CELLS in mammalian blood that are abnormal in structure or function.Antisickling Agents: Agents used to prevent or reverse the pathological events leading to sickling of erythrocytes in sickle cell conditions.Hemoglobin SC Disease: One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia.Fetal Hemoglobin: The major component of hemoglobin in the fetus. This HEMOGLOBIN has two alpha and two gamma polypeptide subunits in comparison to normal adult hemoglobin, which has two alpha and two beta polypeptide subunits. Fetal hemoglobin concentrations can be elevated (usually above 0.5%) in children and adults affected by LEUKEMIA and several types of ANEMIA.Anemia, Hemolytic: A condition of inadequate circulating red blood cells (ANEMIA) or insufficient HEMOGLOBIN due to premature destruction of red blood cells (ERYTHROCYTES).Anemia, Aplastic: A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements.Hemoglobins: The oxygen-carrying proteins of ERYTHROCYTES. They are found in all vertebrates and some invertebrates. The number of globin subunits in the hemoglobin quaternary structure differs between species. Structures range from monomeric to a variety of multimeric arrangements.Fanconi Anemia: Congenital disorder affecting all bone marrow elements, resulting in ANEMIA; LEUKOPENIA; and THROMBOPENIA, and associated with cardiac, renal, and limb malformations as well as dermal pigmentary changes. Spontaneous CHROMOSOME BREAKAGE is a feature of this disease along with predisposition to LEUKEMIA. There are at least 7 complementation groups in Fanconi anemia: FANCA, FANCB, FANCC, FANCD1, FANCD2, FANCE, FANCF, FANCG, and FANCL. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=227650, August 20, 2004)Hydroxyurea: An antineoplastic agent that inhibits DNA synthesis through the inhibition of ribonucleoside diphosphate reductase.Thalassemia: A group of hereditary hemolytic anemias in which there is decreased synthesis of one or more hemoglobin polypeptide chains. There are several genetic types with clinical pictures ranging from barely detectable hematologic abnormality to severe and fatal anemia.Anemia, Hemolytic, Autoimmune: Acquired hemolytic anemia due to the presence of AUTOANTIBODIES which agglutinate or lyse the patient's own RED BLOOD CELLS.Anemia, Hypochromic: Anemia characterized by a decrease in the ratio of the weight of hemoglobin to the volume of the erythrocyte, i.e., the mean corpuscular hemoglobin concentration is less than normal. The individual cells contain less hemoglobin than they could have under optimal conditions. Hypochromic anemia may be caused by iron deficiency from a low iron intake, diminished iron absorption, or excessive iron loss. It can also be caused by infections or other diseases, therapeutic drugs, lead poisoning, and other conditions. (Stedman, 25th ed; from Miale, Laboratory Medicine: Hematology, 6th ed, p393)beta-Thalassemia: A disorder characterized by reduced synthesis of the beta chains of hemoglobin. There is retardation of hemoglobin A synthesis in the heterozygous form (thalassemia minor), which is asymptomatic, while in the homozygous form (thalassemia major, Cooley's anemia, Mediterranean anemia, erythroblastic anemia), which can result in severe complications and even death, hemoglobin A synthesis is absent.Erythrocytes: Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.Anemia, Macrocytic: Anemia characterized by larger than normal erythrocytes, increased mean corpuscular volume (MCV) and increased mean corpuscular hemoglobin (MCH).Anemia, Pernicious: A megaloblastic anemia occurring in children but more commonly in later life, characterized by histamine-fast achlorhydria, in which the laboratory and clinical manifestations are based on malabsorption of vitamin B 12 due to a failure of the gastric mucosa to secrete adequate and potent intrinsic factor. (Dorland, 27th ed)Hemoglobinopathies: A group of inherited disorders characterized by structural alterations within the hemoglobin molecule.Jamaica: An island in the Greater Antilles in the West Indies. Its capital is Kingston. It was discovered in 1494 by Columbus and was a Spanish colony 1509-1655 until captured by the English. Its flourishing slave trade was abolished in the 19th century. It was a British colony 1655-1958 and a territory of the West Indies Federation 1958-62. It achieved full independence in 1962. The name is from the Arawak Xaymaca, rich in springs or land of springs. (From Webster's New Geographical Dictionary, 1988, p564 & Room, Brewer's Dictionary of Names, 1992, p267)Acute Chest Syndrome: Respiratory syndrome characterized by the appearance of a new pulmonary infiltrate on chest x-ray, accompanied by symptoms of fever, cough, chest pain, tachypnea, or DYSPNEA, often seen in patients with SICKLE CELL ANEMIA. Multiple factors (e.g., infection, and pulmonary FAT EMBOLISM) may contribute to the development of the syndrome.alpha-Thalassemia: A disorder characterized by reduced synthesis of the alpha chains of hemoglobin. The severity of this condition can vary from mild anemia to death, depending on the number of genes deleted.Blood Transfusion: The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed)Hemoglobin C Disease: A disease characterized by compensated hemolysis with a normal hemoglobin level or a mild to moderate anemia. There may be intermittent abdominal discomfort, splenomegaly, and slight jaundice.Anemia, Sideroblastic: Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.Hemolysis: The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.Hemoglobin A: Normal adult human hemoglobin. The globin moiety consists of two alpha and two beta chains.Anemia, Megaloblastic: A disorder characterized by the presence of ANEMIA, abnormally large red blood cells (megalocytes or macrocytes), and MEGALOBLASTS.Erythrocyte Count: The number of RED BLOOD CELLS per unit volume in a sample of venous BLOOD.Infectious Anemia Virus, Equine: A species of LENTIVIRUS, subgenus equine lentiviruses (LENTIVIRUSES, EQUINE), causing acute and chronic infection in horses. It is transmitted mechanically by biting flies, mosquitoes, and midges, and iatrogenically through unsterilized equipment. Chronic infection often consists of acute episodes with remissions.Anemia, Refractory: A severe sometimes chronic anemia, usually macrocytic in type, that does not respond to ordinary antianemic therapy.Anemia, Hemolytic, Congenital: Hemolytic anemia due to various intrinsic defects of the erythrocyte.Erythrocyte Indices: ERYTHROCYTE size and HEMOGLOBIN content or concentration, usually derived from ERYTHROCYTE COUNT; BLOOD hemoglobin concentration; and HEMATOCRIT. The indices include the mean corpuscular volume (MCV), the mean corpuscular hemoglobin (MCH), and the mean corpuscular hemoglobin concentration (MCHC).Hemoglobins, Abnormal: Hemoglobins characterized by structural alterations within the molecule. The alteration can be either absence, addition or substitution of one or more amino acids in the globin part of the molecule at selected positions in the polypeptide chains.Erythrocyte Deformability: Ability of ERYTHROCYTES to change shape as they pass through narrow spaces, such as the microvasculature.Hematocrit: The volume of packed RED BLOOD CELLS in a blood specimen. The volume is measured by centrifugation in a tube with graduated markings, or with automated blood cell counters. It is an indicator of erythrocyte status in disease. For example, ANEMIA shows a low value; POLYCYTHEMIA, a high value.Exchange Transfusion, Whole Blood: Repetitive withdrawal of small amounts of blood and replacement with donor blood until a large proportion of the blood volume has been exchanged. Used in treatment of fetal erythroblastosis, hepatic coma, sickle cell anemia, disseminated intravascular coagulation, septicemia, burns, thrombotic thrombopenic purpura, and fulminant malaria.Hemoglobin C: A commonly occurring abnormal hemoglobin in which lysine replaces a glutamic acid residue at the sixth position of the beta chains. It results in reduced plasticity of erythrocytes.Leg Ulcer: Ulceration of the skin and underlying structures of the lower extremity. About 90% of the cases are due to venous insufficiency (VARICOSE ULCER), 5% to arterial disease, and the remaining 5% to other causes.Globins: A superfamily of proteins containing the globin fold which is composed of 6-8 alpha helices arranged in a characterstic HEME enclosing structure.Pregnancy Complications, Hematologic: The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS.Erythrocyte Aging: The senescence of RED BLOOD CELLS. Lacking the organelles that make protein synthesis possible, the mature erythrocyte is incapable of self-repair, reproduction, and carrying out certain functions performed by other cells. This limits the average life span of an erythrocyte to 120 days.Erythropoietin: Glycoprotein hormone, secreted chiefly by the KIDNEY in the adult and the LIVER in the FETUS, that acts on erythroid stem cells of the BONE MARROW to stimulate proliferation and differentiation.Iron: A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.Erythrocyte Transfusion: The transfer of erythrocytes from a donor to a recipient or reinfusion to the donor.Equine Infectious Anemia: Viral disease of horses caused by the equine infectious anemia virus (EIAV; INFECTIOUS ANEMIA VIRUS, EQUINE). It is characterized by intermittent fever, weakness, and anemia. Chronic infection consists of acute episodes with remissions.Reticulocytes: Immature ERYTHROCYTES. In humans, these are ERYTHROID CELLS that have just undergone extrusion of their CELL NUCLEUS. They still contain some organelles that gradually decrease in number as the cells mature. RIBOSOMES are last to disappear. Certain staining techniques cause components of the ribosomes to precipitate into characteristic "reticulum" (not the same as the ENDOPLASMIC RETICULUM), hence the name reticulocytes.Pain: An unpleasant sensation induced by noxious stimuli which are detected by NERVE ENDINGS of NOCICEPTIVE NEURONS.Splenic Infarction: Insufficiency of arterial or venous blood supply to the spleen due to emboli, thrombi, vascular torsion, or pressure that produces a macroscopic area of necrosis. (From Stedman, 25th ed)Erythrocyte Membrane: The semi-permeable outer structure of a red blood cell. It is known as a red cell 'ghost' after HEMOLYSIS.Hemoglobinometry: Measurement of hemoglobin concentration in blood.Chicken anemia virus: The type species of GYROVIRUS, a small, non-enveloped DNA virus originally isolated from contaminated vaccines in Japan. It causes chicken infectious anemia and may possibly play a key role in hemorrhagic anemia syndrome, anemia dermatitis, and blue wing disease.Anemia, Dyserythropoietic, Congenital: A familial disorder characterized by ANEMIA with multinuclear ERYTHROBLASTS, karyorrhexis, asynchrony of nuclear and cytoplasmic maturation, and various nuclear abnormalities of bone marrow erythrocyte precursors (ERYTHROID PRECURSOR CELLS). Type II is the most common of the 3 types; it is often referred to as HEMPAS, based on the Hereditary Erythroblast Multinuclearity with Positive Acidified Serum test.Erythropoiesis: The production of red blood cells (ERYTHROCYTES). In humans, erythrocytes are produced by the YOLK SAC in the first trimester; by the liver in the second trimester; by the BONE MARROW in the third trimester and after birth. In normal individuals, the erythrocyte count in the peripheral blood remains relatively constant implying a balance between the rate of erythrocyte production and rate of destruction.Anemia, Diamond-Blackfan: A rare congenital hypoplastic anemia that usually presents early in infancy. The disease is characterized by a moderate to severe macrocytic anemia, occasional neutropenia or thrombocytosis, a normocellular bone marrow with erythroid hypoplasia, and an increased risk of developing leukemia. (Curr Opin Hematol 2000 Mar;7(2):85-94)Vascular Diseases: Pathological processes involving any of the BLOOD VESSELS in the cardiac or peripheral circulation. They include diseases of ARTERIES; VEINS; and rest of the vasculature system in the body.Blood Viscosity: The internal resistance of the BLOOD to shear forces. The in vitro measure of whole blood viscosity is of limited clinical utility because it bears little relationship to the actual viscosity within the circulation, but an increase in the viscosity of circulating blood can contribute to morbidity in patients suffering from disorders such as SICKLE CELL ANEMIA and POLYCYTHEMIA.Homozygote: An individual in which both alleles at a given locus are identical.Fanconi Anemia Complementation Group Proteins: A diverse group of proteins whose genetic MUTATIONS have been associated with the chromosomal instability syndrome FANCONI ANEMIA. Many of these proteins play important roles in protecting CELLS against OXIDATIVE STRESS.Iron Overload: An excessive accumulation of iron in the body due to a greater than normal absorption of iron from the gastrointestinal tract or from parenteral injection. This may arise from idiopathic hemochromatosis, excessive iron intake, chronic alcoholism, certain types of refractory anemia, or transfusional hemosiderosis. (From Churchill's Illustrated Medical Dictionary, 1989)Ferritins: Iron-containing proteins that are widely distributed in animals, plants, and microorganisms. Their major function is to store IRON in a nontoxic bioavailable form. Each ferritin molecule consists of ferric iron in a hollow protein shell (APOFERRITINS) made of 24 subunits of various sequences depending on the species and tissue types.Oxygen: An element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.Erythrocyte Aggregation: The formation of clumps of RED BLOOD CELLS under low or non-flow conditions, resulting from the attraction forces between the red blood cells. The cells adhere to each other in rouleaux aggregates. Slight mechanical force, such as occurs in the circulation, is enough to disperse these aggregates. Stronger or weaker than normal aggregation may result from a variety of effects in the ERYTHROCYTE MEMBRANE or in BLOOD PLASMA. The degree of aggregation is affected by ERYTHROCYTE DEFORMABILITY, erythrocyte membrane sialylation, masking of negative surface charge by plasma proteins, etc. BLOOD VISCOSITY and the ERYTHROCYTE SEDIMENTATION RATE are affected by the amount of erythrocyte aggregation and are parameters used to measure the aggregation.gamma-Globins: Members of the beta-globin family. In humans, two non-allelic types of gamma-globin - A gamma and G gamma are encoded in the beta-globin gene cluster on CHROMOSOME 11. Two gamma-globin chains combine with two ZETA-GLOBIN chains to form the embryonic hemoglobin Portland. Fetal HEMOGLOBIN F is formed from two gamma-globin chains combined with two ALPHA-GLOBIN chains.Nigeria: A republic in western Africa, south of NIGER between BENIN and CAMEROON. Its capital is Abuja.Reticulocyte Count: The number of RETICULOCYTES per unit volume of BLOOD. The values are expressed as a percentage of the ERYTHROCYTE COUNT or in the form of an index ("corrected reticulocyte index"), which attempts to account for the number of circulating erythrocytes.Oxyhemoglobins: A compound formed by the combination of hemoglobin and oxygen. It is a complex in which the oxygen is bound directly to the iron without causing a change from the ferrous to the ferric state.Hemorheology: The deformation and flow behavior of BLOOD and its elements i.e., PLASMA; ERYTHROCYTES; WHITE BLOOD CELLS; and BLOOD PLATELETS.beta-Globins: Members of the beta-globin family. In humans, they are encoded in a gene cluster on CHROMOSOME 11. They include epsilon-globin, gamma-globin, delta-globin and beta-globin. There is also a pseudogene of beta (theta-beta) in the gene cluster. Adult HEMOGLOBIN is comprised of two ALPHA-GLOBIN chains and two beta-globin chains.Dimethyl Adipimidate: Bifunctional cross-linking agent that links covalently free amino groups of proteins or polypeptides, including those in cell membranes. It is used as reagent or fixative in immunohistochemistry and is a proposed antisickling agent.Anemia, Neonatal: The mildest form of erythroblastosis fetalis in which anemia is the chief manifestation.Prevalence: The total number of cases of a given disease in a specified population at a designated time. It is differentiated from INCIDENCE, which refers to the number of new cases in the population at a given time.Splenic DiseasesLutheran Blood-Group System: A complex blood group system having pairs of alternate antigens and amorphic genes, but also subject to a dominant independently segregating repressor.Hematinics: Agents which improve the quality of the blood, increasing the hemoglobin level and the number of erythrocytes. They are used in the treatment of anemias.Anemia, Refractory, with Excess of Blasts: Chronic refractory anemia with granulocytopenia, and/or thrombocytopenia. Myeloblasts and progranulocytes constitute 5 to 40 percent of the nucleated marrow cells.Kidney Papillary Necrosis: A complication of kidney diseases characterized by cell death involving KIDNEY PAPILLA in the KIDNEY MEDULLA. Damages to this area may hinder the kidney to concentrate urine resulting in POLYURIA. Sloughed off necrotic tissue may block KIDNEY PELVIS or URETER. Necrosis of multiple renal papillae can lead to KIDNEY FAILURE.Glucosephosphate Dehydrogenase Deficiency: A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.Priapism: A prolonged painful erection that may lasts hours and is not associated with sexual activity. It is seen in patients with SICKLE CELL ANEMIA, advanced malignancy, spinal trauma; and certain drug treatments.Blood Cell Count: The number of LEUKOCYTES and ERYTHROCYTES per unit volume in a sample of venous BLOOD. A complete blood count (CBC) also includes measurement of the HEMOGLOBIN; HEMATOCRIT; and ERYTHROCYTE INDICES.Erythrocyte Inclusions: Pathologic inclusions occurring in erythrocytes.Osmotic Fragility: RED BLOOD CELL sensitivity to change in OSMOTIC PRESSURE. When exposed to a hypotonic concentration of sodium in a solution, red cells take in more water, swell until the capacity of the cell membrane is exceeded, and burst.2,3-Diphosphoglycerate: A highly anionic organic phosphate which is present in human red blood cells at about the same molar ratio as hemoglobin. It binds to deoxyhemoglobin but not the oxygenated form, therefore diminishing the oxygen affinity of hemoglobin. This is essential in enabling hemoglobin to unload oxygen in tissue capillaries. It is also an intermediate in the conversion of 3-phosphoglycerate to 2-phosphoglycerate by phosphoglycerate mutase (EC 5.4.2.1). (From Stryer Biochemistry, 4th ed, p160; Enzyme Nomenclature, 1992, p508)Neonatal Screening: The identification of selected parameters in newborn infants by various tests, examinations, or other procedures. Screening may be performed by clinical or laboratory measures. A screening test is designed to sort out healthy neonates (INFANT, NEWBORN) from those not well, but the screening test is not intended as a diagnostic device, rather instead as epidemiologic.Fanconi Anemia Complementation Group C Protein: A Fanconi anemia complementation group protein that regulates the activities of CYTOCHROME P450 REDUCTASE and GLUTATHIONE S-TRANSFERASE. It is found predominately in the CYTOPLASM, but moves to the CELL NUCLEUS in response to FANCE PROTEIN.Fanconi Anemia Complementation Group D2 Protein: A Fanconi anemia complementation group protein that undergoes mono-ubiquitination by FANCL PROTEIN in response to DNA DAMAGE. Also, in response to IONIZING RADIATION it can undergo PHOSPHORYLATION by ataxia telangiectasia mutated protein. Modified FANCD2 interacts with BRCA2 PROTEIN in a stable complex with CHROMATIN, and it is involved in DNA REPAIR by homologous RECOMBINATION.Retinal DiseasesFanconi Anemia Complementation Group A Protein: A Fanconi anemia complementation group protein that is the most commonly mutated protein in FANCONI ANEMIA. It undergoes PHOSPHORYLATION by PROTEIN KINASE B and forms a complex with FANCC PROTEIN in the CELL NUCLEUS.Hemoglobin A2: An adult hemoglobin component normally present in hemolysates from human erythrocytes in concentrations of about 3%. The hemoglobin is composed of two alpha chains and two delta chains. The percentage of HbA2 varies in some hematologic disorders, but is about double in beta-thalassemia.Hyphema: Bleeding in the anterior chamber of the eye.Splenectomy: Surgical procedure involving either partial or entire removal of the spleen.Infant, Newborn: An infant during the first month after birth.Hypertension, Pulmonary: Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.Premarital Examinations: Medical tests taken by couples planning to be married in order to determine presence of genetic and contagious diseases.Heinz Bodies: Abnormal intracellular inclusions, composed of denatured hemoglobin, found on the membrane of red blood cells. They are seen in thalassemias, enzymopathies, hemoglobinopathies, and after splenectomy.Splenomegaly: Enlargement of the spleen.Risk Factors: An aspect of personal behavior or lifestyle, environmental exposure, or inborn or inherited characteristic, which, on the basis of epidemiologic evidence, is known to be associated with a health-related condition considered important to prevent.MethemoglobinDiphosphoglyceric AcidsEnuresis: Involuntary discharge of URINE after expected age of completed development of urinary control. This can happen during the daytime (DIURNAL ENURESIS) while one is awake or during sleep (NOCTURNAL ENURESIS). Enuresis can be in children or in adults (as persistent primary enuresis and secondary adult-onset enuresis).Mediterranean Islands: Scattered islands in the Mediterranean Sea. The chief islands are the Balearic Islands (belong to Spain; Majorca and Minorca are among these), Corsica (belongs to France), Crete (belongs to Greece), CYPRUS (a republic), the Cyclades, Dodecanese and Ionian Islands (belong to Greece), MALTA (a republic), Sardinia and SICILY (belong to Italy). (From Webster's New Geographical Dictionary, 1988, p747)Spherocytosis, Hereditary: A group of familial congenital hemolytic anemias characterized by numerous abnormally shaped erythrocytes which are generally spheroidal. The erythrocytes have increased osmotic fragility and are abnormally permeable to sodium ions.Prospective Studies: Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.Anemia, Hemolytic, Congenital Nonspherocytic: Any one of a group of congenital hemolytic anemias in which there is no abnormal hemoglobin or spherocytosis and in which there is a defect of glycolysis in the erythrocyte. Common causes include deficiencies in GLUCOSE-6-PHOSPHATE ISOMERASE; PYRUVATE KINASE; and GLUCOSE-6-PHOSPHATE DEHYDROGENASE.Pallor: A clinical manifestation consisting of an unnatural paleness of the skin.Retrospective Studies: Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.Hematologic Tests: Tests used in the analysis of the hemic system.Hemoglobinuria: The presence of free HEMOGLOBIN in the URINE, indicating hemolysis of ERYTHROCYTES within the vascular system. After saturating the hemoglobin-binding proteins (HAPTOGLOBINS), free hemoglobin begins to appear in the urine.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.Tricuspid Valve Insufficiency: Backflow of blood from the RIGHT VENTRICLE into the RIGHT ATRIUM due to imperfect closure of the TRICUSPID VALVE.

Polymer structure and solubility of deoxyhemoglobin S in the presence of high concentrations of volume-excluding 70-kDa dextran. Effects of non-s hemoglobins and inhibitors. (1/2332)

Earlier observations indicated that volume exclusion by admixed non-hemoglobin macromolecules lowered the polymer solubility ("Csat") of deoxyhemoglobin (Hb) S, presumably by increasing its activity. In view of the potential usefulness of these observations for in vitro studies of sickling-related polymerization, we examined the ultrastructure, solubility behavior, and phase distributions of deoxygenated mixtures of Hb S with 70-kDa dextran, a relatively inert, low ionic strength space-filling macromolecule. Increasing admixture of dextran progressively lowered the Csat of deoxyHb S. With 12 g/dl dextran, a 5-fold decrease in apparent Csat ("dextran-Csat") was obtained together with acceptable sensitivity and proportionality with the standard Csat when assessing the effects of non-S Hb admixtures (A, C, and F) or polymerization inhibitors (alkylureas or phenylalanine). The volume fraction of dextran excluding Hb was 70-75% of total deoxyHb-dextran (12 g/dl) volumes. Electron microscopy showed polymer fibers and fiber-to-crystal transitions indistinguishable from those formed without dextran. Thus when Hb quantities are limited, as with genetically engineered recombinant Hbs or transgenic sickle mice, the dextran-Csat provides convenient and reliable screening of effects of Hb S modifications on polymerization under near-physiological conditions, avoiding problems of high ionic strength.  (+info)

Sustained induction of fetal hemoglobin by pulse butyrate therapy in sickle cell disease. (2/2332)

High levels of fetal hemoglobin (Hb F) protect from many of the complications of sickle cell disease and lead to improved survival. Butyrate and other short chain fatty acids were previously shown to increase Hb F production in erythroid cells in vitro and in animal models in vivo. However, butyrates are also known to inhibit the proliferation of many cell types, including erythroid cells. Experience with the use of butyrate in animal models and in early clinical trials demonstrated that the Hb F response may be lost after prolonged administration of high doses of butyrate. We hypothesized that this loss of response may be a result of the antiproliferative effects of butyrate. We designed a regimen consisting of intermittent or pulse therapy in which butyrate was administered for 4 days followed by 10 to 24 days with no drug exposure. This pulse regimen induced fetal globin gene expression in 9 of 11 patients. The mean Hb F in this group increased from 7.2% to 21.0% (P <.002) after intermittent butyrate therapy for a mean duration of 29.9 weeks. This was associated with a parallel increase in the number of F cells and F reticulocytes. The total hemoglobin levels also increased from a mean of 7.8 g/dL to a mean of 8.8 g/dL (P <.006). The increased levels of Hb F were sustained in all responders, including 1 patient who has been on pulse butyrate therapy for more than 28 months. This regimen, which resulted in a marked and sustained increase in Hb F levels in more than two thirds of the adult sickle cell patients enrolled in this study, was well tolerated without adverse side effects. These encouraging results require confirmation along with an appropriate evaluation of clinical outcomes in a larger number of patients with sickle cell disease.  (+info)

In vivo blood flow abnormalities in the transgenic knockout sickle cell mouse. (3/2332)

The accepted importance of circulatory impairment to sickle cell anemia remains to be verified by in vivo experimentation. Intravital microscopy studies of blood flow in patients are limited to circulations that can be viewed noninvasively and are restricted from deliberate perturbations of the circulation. Further knowledge of sickle blood flow abnormalities has awaited an animal model of human sickle cell disease. We compared blood flow in the mucosal-intestinal microvessels of normal mice with that in transgenic knockout sickle cell mice that have erythrocytes containing only human hemoglobin S and that exhibit a degree of hemolytic anemia and pathological complications similar to the human disease. In sickle cell mice, in addition to seeing blood flow abnormalities such as sludging in all microvessels, we detected decreased blood flow velocity in venules of all diameters. Flow responses to hyperoxia in both normal and sickle cell mice were dramatic, but opposite: Hyperoxia promptly slowed or halted flow in normal mice but markedly enhanced flow in sickle cell mice. Intravital microscopic studies of this murine model provide important insights into sickle cell blood flow abnormalities and suggest that this model can be used to evaluate the causes of abnormal flow and new approaches to therapy of sickle cell disease.  (+info)

Candida dubliniensis candidemia in patients with chemotherapy-induced neutropenia and bone marrow transplantation. (4/2332)

The recently described species Candida dubliniensis has been recovered primarily from superficial oral candidiasis in HIV-infected patients. No clinically documented invasive infections were reported until now in this patient group or in other immunocompromised patients. We report three cases of candidemia due to this newly emerging Candida species in HIV-negative patients with chemotherapy-induced immunosuppression and bone marrow transplantation.  (+info)

Development of viral vectors for gene therapy of beta-chain hemoglobinopathies: optimization of a gamma-globin gene expression cassette. (5/2332)

Progress toward gene therapy of beta-chain hemoglobinopathies has been limited in part by poor expression of globin genes in virus vectors. To derive an optimal expression cassette, we systematically analyzed the sequence requirements and relative strengths of the Agamma- and beta-globin promoters, the activities of various erythroid-specific enhancers, and the importance of flanking and intronic sequences. Expression was analyzed by RNase protection after stable plasmid transfection of the murine erythroleukemia cell line, MEL585. Promoter truncation studies showed that the Agamma-globin promoter could be deleted to -159 without affecting expression, while deleting the beta-globin promoter to -127 actually increased expression compared with longer fragments. Expression from the optimal beta-globin gene promoter was consistently higher than that from the optimal Agamma-globin promoter, regardless of the enhancer used. Enhancers tested included a 2.5-kb composite of the beta-globin locus control region (termed a muLCR), a combination of the HS2 and HS3 core elements of the LCR, and the HS-40 core element of the alpha-globin locus. All three enhancers increased expression from the beta-globin gene to roughly the same extent, while the HS-40 element was notably less effective with the Agamma-globin gene. However, the HS-40 element was able to efficiently enhance expression of a Agamma-globin gene linked to the beta-globin promoter. Inclusion of extended 3' sequences from either the beta-globin or the Agamma-globin genes had no significant effect on expression. A 714-bp internal deletion of Agamma-globin intron 2 unexpectedly increased expression more than twofold. With the combination of a -127 beta-globin promoter, an Agamma-globin gene with the internal deletion of intron 2, and a single copy of the HS-40 enhancer, gamma-globin expression averaged 166% of murine alpha-globin mRNA per copy in six pools and 105% in nine clones. When placed in a retrovirus vector, this cassette was also expressed at high levels in MEL585 cells (averaging 75% of murine alpha-globin mRNA per copy) without reducing virus titers. However, recombined provirus or aberrant splicing was observed in 5 of 12 clones, indicating a significant degree of genetic instability. Taken together, these data demonstrate the development of an optimal expression cassette for gamma-globin capable of efficient expression in a retrovirus vector and form the basis for further refinement of vectors containing this cassette.  (+info)

Osteonecrosis of the hip in sickle-cell disease associated with tuberculous arthritis. A review of 15 cases. (6/2332)

We report a study of 15 cases of tuberculous hips with sickle-cell disease who presented during 1991-1993. Although the osteonecrosis was long-standing, biopsy was nearly always required to reveal the more recent tuberculous infection. Management consisted of 6 months of anti-tuberculous chemotherapy with appropriate palliative surgery 5-8 weeks after the start of drug treatment. The operative techniques which we used are described. The results were good both post-operatively, and in 12 patients followed-up at an average of 3 years. We recommend this combined management for the treatment of secondary tuberculous infections of hips previously damaged by sickle-cell disease.  (+info)

Large cerebral vessel disease in sickle cell anaemia. (7/2332)

An 18 year old male with documented sickle cell disease was admitted to the hospital for the final time in coma. Cerebral angiography revealed multiple stenotic lesions of the large cerebral vessels. The pathology of this large vessel involvement is demonstrated and the potential contribution of large as opposed to small cerebral vessel disease in the neurological manifestations of sickle cell anaemia is discussed.  (+info)

Perceived stress factors and coping mechanisms among mothers of children with sickle cell disease in western Nigeria. (8/2332)

While many studies have looked at the stressful effects of chronic illness of those who suffer such conditions, less is known about the effects on caregivers, especially in developing countries. Mothers in particular must bear the brunt of care and stress for children who have sickle cell disease (SCD). A sample of 200 mothers attending six SCD clinics in both public and private hospitals in the Ibadan-Ibarapa Health Zone of Oyo State, Nigeria, were interviewed. Stress levels were measured using an instrument comprised of stressors listed by mothers themselves in focus group discussions that preceded the survey. Higher levels of stress were associated with less educated and older women, as well as non-married women and those in polygamous households. Stress levels were also greater when there was more than one child with SCD in the family and when the index child was of school age. Coping mechanisms varied according to the category of stressor. Financial stress and disease factors were met with confrontation while family sources of stress were either complained about, accepted or avoided. Knowledge of the different types of mothers who experience more stress and of their preferred coping mechanisms can be useful in designing clinic-based counseling.  (+info)

TY - JOUR. T1 - Impact of mannose-binding protein gene polymorphisms in omani sickle cell disease patients. AU - Zachariah, Mathew. AU - Al Zadjali, Shoaib. AU - Bashir, Wafa. AU - Al Ambusaidi, Rahma. AU - Misquith, Rhea. AU - Wali, Yasser. AU - Pathare, Anil. PY - 2016. Y1 - 2016. N2 - Objectives: Our aim was to study mannose-binding protein (MBP) polymorphisms in exonic and promoter region and correlate it with associated infections and vasoocculsive (VOC) episodes in sickle cell disease (SCD) patients since MBP plays an important role in innate immunity by activating the complement system. Methods: We studied the genetic polymorphisms in the Exon 1 (alleles A/O) and promoter region (alleles Y/X; H/L, P/Q) of the MBL2 gene, in SCD patients as an increased incidence of infections is seen in these patients. A PCR-based, targeted genomic DNA sequencing of MBL2 was used to study 68 SCD Omani patients and 44 controls (healthy voluntary blood donors). Results: In SCD patients, the frequency of the ...
TY - JOUR. T1 - A prospective study of soft-tissue ultrasonography in sickle cell disease patients with suspected osteomyelitis. AU - William, Ranjan R.. AU - Hussein, Samir S.. AU - Jeans, William D.. AU - Wali, Yasser A.. AU - Lamki, Zakia A.. PY - 2000. Y1 - 2000. N2 - Aim: A prospective study was done to assess the accuracy of soft tissue ultrasonography in patients with sickle cell disease (SCD) presenting with suspected osteomyelitis. Materials and Methods: Thirty-one SCD patients had soft tissue ultrasonography on 38 occasions (18 men, 13 women; mean age 8.2 years). The initial ultrasonographic signs and diagnosis were compared with the final clinical diagnosis, which was based on clinical progress and scintigraphy. Result: The overall sensitivity of ultrasound in diagnosing osteomyelitis was 74% with a specificity of 63%. The principal ultrasonographic finding of subperiosteal fluid was present in 14 (74%) patients with osteomyelitis and seven (37%) patients without infection. A finding ...
Treatment of Sickle cell anemia is an inherited form of anemia - a condition in which there arent enough healthy red blood cells to carry oxygen throughout your body, Under normal circumstances, your red blood cells are flexible and round, and they move easily through your blood vessels to carry oxygen to all parts of your body. In people with sickle cell anemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons, These irregular-shaped blood cells die prematurely, resulting in a chronic shortage of red blood cells. Plus, they can get stuck when traveling through small blood vessels, which can slow or block blood flow and oxygen to certain parts of the body. This produces pain and can lead to the serious complications of sickle cell anemia, Theres no cure for most people with sickle cell anemia. However, treatments can relieve pain and prevent further problems associated with sickle cell anemia, Sickle Cell Anemia, Sickle Cell Disease, Sickle Cell Anemia Symptoms
E most common type is known as sickle cell anaemia (SCA). Bies and children age 2 and. Wever, knowledge of sickle? Alth experts have long believed that sickle cell gene variants. Ckle cell disease (SCD) encompasses a group of hemoglobinopathies characterized by a single amino acid substitution in the ß globin chain. Ckle Cell News for September. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. Wever, knowledge of sickle. Sickle cell disease (SCD) is a group of blood disorders typically inherited from a persons parents. The Sickle Cell Disease Process. Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin S (HbS) (see the. Sickle cell trait is a benign carrier condition, usually with none of the symptoms of sickle cell anemia or other sickle cell diseases. RESEARCH PAGE. INTRODUCTION. Ndmark Article in New England Journal of ...
TY - JOUR. T1 - Thrombotic thrombacytopenic purpura in a patient with sickle cell crisis. AU - Bolaños-Meade,J.. AU - Keung,Y. K.. AU - López-Arvizu,C.. AU - Florendo,R.. AU - Cobos,E.. PY - 1999/12. Y1 - 1999/12. N2 - The combination of sickle cell disease crisis and thrombotic thrombocytopenic purpura has been described only a few times. Here we present the case of a patient with a hemolytic crisis due to sickle cell disease complicated by thrombotic thrombocytopenic purpura. We also review the cases previously reported and compare and contrast them, highlighting diagnostic challenges.. AB - The combination of sickle cell disease crisis and thrombotic thrombocytopenic purpura has been described only a few times. Here we present the case of a patient with a hemolytic crisis due to sickle cell disease complicated by thrombotic thrombocytopenic purpura. We also review the cases previously reported and compare and contrast them, highlighting diagnostic challenges.. KW - Hemolysis. KW - Sickle ...
Background: Sickle cell anaemia is an autosomal recessive disorder that arises due to the substitution of glutamic acid with valine. This occurs at position 6 of the haemoglobin b chain, resulting in the synthesis of abnormal haemoglobin and the consequent production of the characteristic sickled red blood cells. Studies have documented several alterations in lipid homeostasis in this population. Both hyper and hypolipidaemias are known to be associated with increased morbidity and mortality and it is therefore imperative to evaluate their relationships with sickle cell anaemia. Aim: The aim of this study was to establish baseline serum lipid levels in sickle cell anaemia patients in LASUTH and correlate this with severity scores in the patients. Subjects and Methods: Serum Total cholesterol (TC), Triglycerides (TG), Low-density lipoproteins (LDL), High-density lipoproteins (HDL) and Very low-density lipoproteins (VLDL) were measured in 57 Haemoglobin SS (HbSS) patients in steady state. All ...
TY - JOUR. T1 - Sickle Cell Crisis Complicated by Synthetic Cannabinoid Abuse. T2 - A Case Report. AU - Zheng, Crystal Y.. AU - Minniti, Caterina P.. AU - Chaitowitz, Mark H.. PY - 2016/3/4. Y1 - 2016/3/4. N2 - We describe a case of delirium occurring in a hospitalized sickle cell patient. Following admission for a typical pain crisis, the patient continued to report unrelieved pain with marked agitation for several days, despite escalating doses of opioid analgesia, and ultimately required intubation following development of acute chest syndrome (ACS). After some delay, it was discovered that he had been using a synthetic cannabinoid (K2) which may have precipitated his pain crisis and, with hindsight, explained his prolonged period of delirium. Delayed recognition was due to multiple factors, notably the absence of an index of suspicion for this novel drug, the presence of alternate explanations for the patients altered mental status, and the fact that reliable laboratory screening for ...
Sickle cell anemia is an autosomal recessive disorder and the most common genetic disease affecting African-Americans. Approximately 0.15% of African-Americans are homozygous for sickle cell disease, and 8% have sickle cell trait. Acute pain crisis, acute chest syndrome (ACS), and secondary pulmonary hypertension are common complications of sickle cell anemia. Mortality rates of sickle cell patients with pulmonary hypertension are significantly increased as compared to patients without pulmonary hypertension. Recent studies report up to 40% mortality at 22 months after detection of elevated pulmonary artery pressures in sickle cell patients. Furthermore, pulmonary hypertension is thought to occur in up to 30% of clinic patients with sickle cell anemia.. This study is designed to determine the prevalence and prognosis of secondary pulmonary hypertension in adult patients with sickle cell anemia, and to determine whether genetic polymorphisms in candidate genes contribute to its development or ...
Background and Objective: Inflammatory markers that influence bone pain crisis (BPC) and other complications of sickle cell anaemia (SCA) are numerous and play various roles. This study determined the plasma levels of tumour necrosis factor (TNF) - ?, interleukin - 8 (IL-8), and endothelin - 1 (ET-1) in adult SCA patients during BPC and in steady state. In addition, the plasma levels of these cytokines were correlated with the severity of BPC of the patients.. Methods and Materials: Sixty adult SCA patients (30 during BPC and 30 during steady state) and 30 haemoglobin A controls were enrolled for this cross-sectional study. The severity of BPC was assessed clinically, and questionnaires were filled. Plasma levels of TNF- ?, IL-8 and ET-1 were quantified by ELISA, and haematological parameters were determined using a 5-part auto-analyzer. Plasma levels were correlated with the severity of bone pain crisis. Results were considered statistically significant if p,0.05.. Results: Plasma TNF-?, IL-8, ...
A new research study has determined that body measurements (anthropometric variables) of children with sickle cell anemia are usually smaller than healthy children.. The research study, "Body mass index and other anthropometric variables in children with sickle cell anemia," was published in Pakistan Journal of Medical Sciences.. Sickle cell anemia is a frequently inherited hematological disease among people of African descent and common in Nigeria. Symptoms of the disease are many and include chronic haemolytic anemia, musculoskeletal anomalies, infections and some growth problems.. According the report, abnormal growth occurs often in children with sickle cell anemia with nutrition playing a key role in anthropometric status, illness severity and body composition. For example, energy supply is known to cause a constant disturbance in children with sickle cell anemia which influences body mass index (BMI), which can be due to high metabolic rates, inefficient absorption and raised ...
This study will examine the long-term safety and efficacy of Deferasirox in patients with sickle cell disease and iron overload from repeated blood tran
Hello Dr. Keller, Recently at our facility (mid-sized jail), we have been having more difficulty managing some of our Sickle Cell Disease patients. I am looking for input from you and / or your readers regarding strategies for managing Sickle Cell patients. The problem as I see it is this: Sickling can be brought on by stressful and/or uncomfortable conditions; temperature variations, hydration issues, and physical or emotional stressful conditions - ALL of which occur routinely in a jail environment. At my facility, I typically allow for more comfort measures when dealing with my Sickle Cell Disease patients. I give an extra mat and extra blanket for comfort and warmth. I give them a small pitcher to fill with water to encourage good hydration. I start off with Tylenol/Motrin for mild everyday pain. We typically avoid opiates in our facility, but allow for opiate medication when it is believed patients are in moderate discomfort. I keep a close eye on things that would suggest infection - heart ...
Read about the positive impact a multidisciplinary team has on the mortality rates of pregnant women with sickle cell disease in low-resource countries.
The study compared the efficacy of two treatments for the potentially life-threatening problem of iron overload caused by chronic transfusion therapy. The transfusions are used to guard against additional strokes in young sickle cell anemia patients. The trial, known as SWiTCH or Stroke with Transfusion Changing to Hydroxyurea, was halted in May after an interim safety review determined the alternative therapy was not significantly better than the standard treatment at reducing iron buildup and was associated with an increased stroke risk ...
At Virginia Commonwealth University (VCU), researchers sought to shed light on the biopsychosocial and spiritual effects of taking prescribed opioids to treat noncancer pain.
Reduction of frequency of pain in clinical trial with association of arginine and hydroxyurea in sickle cell anemias patients in Brazil, Renata Eleutà ©rio
Primary objective:. To evaluate the efficacy of oral L-glutamine as a therapy for sickle cell anemia and sickle ß0-thalassemia as evaluated by the number of occurrences of sickle cell crises.. Secondary objectives:. To assess the effect of oral L-glutamine on: (a) frequency of hospitalizations for sickle cell pain; (b) frequency of emergency room/medical facility visits for sickle cell pain; and (c) hematological parameters (hemoglobin, hematocrit, and reticulocyte count); and to assess the safety of L-glutamine as a therapy for sickle cell anemia as evaluated by adverse events, laboratory parameters, and vital signs.. Methodology:. This was a 2:1 randomized, double-blind, placebo-controlled, parallel-group, multicenter study in patients with sickle cell anemia and sickle ß0-thalassemia who were at least 5 years old. Informed consent was obtained up to four weeks prior to Week 0 (Baseline). Screening procedures were performed anytime between the date of consent and Week 0, as long as all ...
Background: Sickle cell anaemia (SCA) is a major problem in Uganda; it is estimated to contribute about 15% of paediatric admissions and 10% mortality in children at Mulago hospital. Fetal haemoglobin (HbF) level is one of the most important factors influencing the clinical course in SCA. Populations with high levels of HbF like those in Saudi Arabia have been described as having a mild clinical course with fewer complications. Disease modifying drugs can induce the Hb F levels and modify the presentation of SCA. However, in Uganda, there is no documentation on HbF levels and the correlation to clinical features and disease severity in patients with SCA. Objective: The aims of this study were to document fetal haemoglobin levels and describe the correlation between these levels and disease severity in patients with SCA in Mulago Hospital. Design: This study had two designs: a cross sectional survey to document fetal haemoglobin levels in children with SCA attending Mulago hospital and a ...
The Dana-Farber/Boston Children's Sickle Cell Disease Program treats children and young adults with all types of sickle cell disease, including sickle cell anemia. Learn More.
Results: The study showed that the frequency of SCD was found in Hosa tribe with frequency of 57% Followed by Falata, Burno, four, Masalet , Tama and Messeria with frequencies of 18%,9%7%,5%,3% 1% respectively .Geographic distribution showed that the highest frequencies of SCD were found in tribes originated from outside Sudan (Hosa , Falata ,Tama, Burno) with percentage of 87 % &the lowest were found in tribes originated from Kurdufan state (Messeria) with frequency of (1%). Also the study showed that no case was recorded from tribes of North , South and East of Sudan. The result showed that the percentage distribution of patients according to Hb type was 53% for AS and 47% for SS ...
Of a cohort of 308 children with homozygous sickle cell disease diagnosed at birth, 89 experienced 132 clinically significant attacks of acute splenic sequestration (ASS) over a 10-year period. The age at first attack ranged from 3 months to 6 years. Survival curve analysis of the interval until first attack indicated a cumulative probability of 0.225 by 2 years, and 0.265 by 3 years, and 0.297 by 5 years of age. Thirteen events were fatal, 11 during the first attack, and all before transfusion could be instituted. Recurrences occurred in 49% of survivors of the first attacks, and there were diminishing intervals between subsequent events. Respiratory symptoms were associated with 52 of 132 events, but bacterial isolates on blood culture were less frequent, and ASS was not prevented by pneumococcal vaccine or penicillin prophylaxis. A high fetal hemoglobin level protected against attacks of ASS. A parental education program aimed at early diagnosis of ASS was followed by an increase in the incidence
TY - JOUR. T1 - Fetal hemoglobin in sickle cell anemia. T2 - Genetic determinants of response to hydroxyurea. AU - Ma, Q.. AU - Wyszynski, D. F.. AU - Farrell, J. J.. AU - Kutlar, Abdullah. AU - Farrer, L. A.. AU - Baldwin, C. T.. AU - Steinberg, M. H.. PY - 2007/12/1. Y1 - 2007/12/1. N2 - The increase in fetal hemoglobin (HbF) in response to hydroxyurea (HU) varies among patients with sickle cell anemia. Twenty-nine candidate genes within loci previously reported to be linked to HbF level (6q22.3-q23.2, 8q11-q12 and Xp22.2-p22.3), involved in metabolism of HU and related to erythroid progenitor proliferation were studied in 137 sickle cell anemia patients treated with HU. Three-hundred and twenty tagging single nucleotide polymorphisms (SNPs) for genotyping were selected based on HapMap data. Multiple linear regression and the nonlinear regression Random Forest method were used to investigate the association between SNPs and the change in HbF level after 2 years of treatment with HU. Both ...
Patients with sickle cell disease need certain treatment and follow-up even when not having a painful crisis. Supplementation with folic acid , an essential element in producing cells, is required because of the rapid red blood cell turnover. Bone marrow transplants are currently the only potential cure for sickle cell anemia. In this treatment the patients bone marrow (which makes the sickled red blood cells) is replaced with bone marrow from another individual without sickle cell disease. However, it is difficult to find the right bone marrow donor, and the drugs needed to make the transplant possible are highly toxic. During a sickle crisis, certain therapies may be necessary. Gene therapy (replacing the Hemoglobin S with a normal Hemoglobin A) may be the ideal treatment, but it has proven to be very difficult in humans Pain management. Pain is a common problem with sickle cell anemia. Some patients get the relief they need from over-the-counter medication,. Others need stronger painkillers. ...
The National Heart, Lung, and Blood Institute (NHLBI) announced today a treatment that reduces the rate of stroke (cerebral infarction) in children with sickle cell anemia. Strokes occur in approximately 10% of children with sickle cell anemia. These events can be very debilitating, leading to physical and neuro-psychological impairment which can affect motor skills, school performance, and overall quality of life. The treatment, periodic red blood cell transfusions to maintain the level of hemoglobin S (HbS) below 30%, reduced the rate of cerebral infarction by 90% in children found to be at increased risk by virtue of having elevated transcranial doppler velocities.. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) proposed to reduce first-time stroke in children with sickle cell anemia by 70% by the administration of prophylactic transfusion therapy. The study design was based on the clinical observation that if hemoglobin S (HbS) levels are maintained at or below 30% in children who ...
Background: Hydroxyurea is an antimetabolite that minimizes pain and prolongs survival in patients with sickle cell anemia (1). It is not widely prescribed because of concerns about late effects, including cancer (2), and its leukemogenic risk is extrapolated from its reported risk in myeloproliferative disorders (3). Few cases of leukemia in patients with sickle cell anemia have been described, and only half of them report cytogenetics (4). Acute myelogenous leukemia (AML) in patients with sickle cell anemia receiving hydroxyurea treatment is exceptionally rare, but data on its true incidence are insufficient (1, 2). Whether AML in hydroxyurea-treated patients with sickle cell anemia is coincidental or related to therapy remains an unanswered question (2) ...
A stem cell transplant, also called a bone marrow transplant, includes changing bone marrow affected by sickle cell anemia with healthy bone marrow from a donor. Since of the risks connected with a stem cell transplant, the procedure is suggested only for individuals who have considerable symptoms and problems from sickle cell anemia.. If a donor is found, the infected bone marrow in the individual with sickle cell anemia wases initially diminished with radiation or chemotherapy. Healthy stem cells from the donor are filtered from the blood.. The healthy stem cells are injected intravenously into the bloodstream of the person with sickle cell anemia, where they migrate to the bone marrow cavities and begin generating new members cells. The procedure needs a prolonged hospital stay. After the transplant, youll get drugs to assist avoid rejection of the contributed stem cells.. A stem cell transplant brings risks. Theres a possibility that your body may decline the transplant, resulting in ...
INTRODUCTION. Sickle cell anemia is a genetic disorder characterized by a mutant type of hemoglobin, called hemoglobin S (HbS), that causes the sickling of red blood cells.1 Sickle cell anemia is the most common hereditary disease in Brazil and worldwide.2-5 In Brazil, it is estimated that more than two million people carry the gene for HbS, and over 8,000 individuals have the severe form (SS). Approximately 700,000 new cases of sickle-cell disease occur annually.6 The disease is prevalent among African descendents.6,7. Sickle cell anemia, although treatable, is a chronic incurable disease involving medical, dental, genetic, and psychosocial factors.7,8 Dentists play an important role in preventing complications and improving the quality of life of patients with sickle cell disease9 because these patients are more susceptible to infections and periodontal disease.10 These patients are also at a higher risk of developing dental caries because of the high prevalence of dental opacities (changes in ...
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What is the main genetic abnormality in sickle cell disease? Describe why patients with sickle cell disease are at risk of vascular crises.. Sickle cell disease includes all conditions associated with hemolytic anemia and vaso-occlusive pain. Over 20% of people in equatorial Africa are heterozygous for the sickle gene, as it is believed to offer protection against malaria. The pathophysiology arises from an amino acid switch in the 6th position (11th chromosome) from glutamic acid to valine.. When hemoglobin S encounters a deoxygenated state, the hemoglobin is less soluble and the Hb S tetramers aggregate or polymerize. The sickled RBCs adhere to the vascular surface, causing occlusion and an increased hypercoagulable state.. Not surprisingly, sickle cell disease patients have the potential for many co-morbidities:. ...
Vaso-occlusive crisis-sickle cell pain related crisis-is the primary cause for hospital visits for sickle cell patients. Although the etiology of vaso-occlusion is not well understood, increased expression of P-selectin in endothelial cells and platelets contributes to the pathogenesis. Crizanlizumab, a monoclonal antibody directed toward P-selection, blocks its interaction with other cells and may reduce the risk of sickle cell pain crises. In a phase-2, multi-center, placebo-controlled trial, 198 patients were randomized in a 1:1:1 ratio to receive either high dose crizanlizumab (5.0 mg per kg of body weight), low dose crizanlizumab (2.5 mg per kg) or a placebo drug during the one year study. Patients with sickle cell disease who were taking high doses of crizanlizumab had a median rate of 1.63 pain crises compared to 2.98 among those taking the placebo (45.3% lower rate, P=0.01). Furthermore, the median time to first crisis was longer for patients taking high dose crizanlizumab, and these ...
Another candidate for Novartis future blockbuster club is here. Now armed with an early FDA approval for Adakveo, the Swiss drugmaker will launch the first targeted therapy to prevent painful vaso-occlusive crises in sickle cell disease patients.
Cholelithiasis is a common clinical condition in patients with sickle cell disease and there are conflicting reports on laboratory indices useful in predicting those patients who are likely to have gallstones. There is however lack of similar studies from Kenya. We therefore studied the role of clinical (Body Mass Index), haematological (reticulocyte count, haemoglobin level), and biochemical (serum bilirubin: direct and indirect, serum alkaline phosphatase, serum transaminase) indices in predicting sickle cell anaemia patients likely to develop gallstones. A cross sectional descriptive study was conducted from October 1993 to December 1994 on consecutive male and female patients of all ages with homozygous sickle cell disease (HbSS) confirmed by cellulose acetate paper electrophoresis. A total of 64 patients aged between three and 37 years were recruited into the study. They were classified into two groups: stone formers and non-formers. The difference in the two groups with respect to ...
To diagnose sickle cell anemia, doctors use a special blood test called a hemoglobin electrophoresis (pronounced: eh-lek-tro-fuh-ree-sis) to look for sickle hemoglobin in a persons blood.. It is possible for some people to be ill enough to die from the disease (although most young people with sickle cell anemia dont die). Doctors can provide treatments that help prevent complications from the disease, though. Folic acid, a vitamin that helps the body produce new red blood cells, is often prescribed for teens with sickle cell anemia. Pain medications help relieve the symptoms of crises. And kids and teens who have sickle cell disease should take penicillin or other antibiotics to help prevent infections. Drinking lots of fluids and avoiding extreme cold or heat can help prevent crises.. Some crises can be managed at home with pain medicines, rest, and extra fluids. But if a crisis is especially intense, a teen may need to go to the hospital for intravenous (IV) fluids and stronger pain ...
Everyone has two copies of the haemoglobin gene in every cell in their body (apart from eggs and sperm). They get one from their mother and one from their father. When eggs and sperm are made, only one of the two genes goes into each egg or sperm cell. This is so that when the egg and sperm come together to make a new baby this new person has two genes in every cell in their body as well. The genes the baby gets will therefore depend on the genes carried in its parents.. Sickle Cell Anaemia is called a recessive condition because you must have two copies of the sickle haemoglobin gene to have the disorder. Sickle haemoglobin is often shortened to S or HbS. If you have only one copy of the sickle haemoglobin along with one copy of the more usual haemoglobin (A or HbA) you are said to have Sickle Cell Trait. This is not an illness but means that you "carry" the gene and can pass it on to you children. If your partner also has Sickle Cell Trait or Sickle Cell Anaemia your children could get Sickle ...
Sickle cell anemia is an inherited disorder of erythrocytes characterized by chronic anemia, recurrent pain, susceptibility to infections, chronic and recurrent organ dysfunction, and diminished life-expectancy. Even among patients having documented homozygous sickle cell anemia (Hb SS), there exists an astonishing degree of variability in clinical severity. Some die from strokes as young children, others live well into their seventies. Some seek medical attention for pain weekly, others go years without pain. The absolute nature of current therapeutic measures for sickle cell disease (SCD), e. g. termination of pregnancy and bone marrow transplantation (BMT), emphasizes the importance of pretherapeutic predictions of mild and severe disease before the nature of the disease has been declared. In this regard, we propose to use polymerase chain reaction (PCR)-based methods to detect sequence variations that are related to disease severity - alpha-globin genotypes, beta-cluster restriction fragment ...
TY - JOUR. T1 - Activated monocytes in sickle cell disease. T2 - Potential role in the activation of vascular endothelium and vaso-occlusion. AU - Belcher, J. D.. AU - Marker, P. H.. AU - Weber, J. P.. AU - Hebbel, R. P.. AU - Vercellotti, G. M.. PY - 2000/10/1. Y1 - 2000/10/1. N2 - Sickle cell anemia is characterized by painful vaso-occlusive crises. It is hypothesized that monocytes are activated in sickle cell disease and can enhance vaso-occlusion by activating endothelium. To test this hypothesis, human umbilical vein endothelial cells (HUVEC) and human microvascular endothelial cells (MVEC) with sickle and normal mononuclear leukocytes were incubated, and endothelial activation was measured. Endothelial cells incubated with sickle mononuclear leukocytes were more activated than those incubated with normal mononuclear leukocytes, as judged by the increased endothelial expression of adhesion molecules and tissue factor and the adhesion of polymorphonuclear leukocytes (PMNL). Monocytes, not ...
Review question We reviewed the evidence about the effect of giving vitamin D supplements to people with sickle cell disease.. Background Sickle cell disease is an inherited red blood cell disorder affecting millions of people worldwide. In sickle cell disease, the red blood cells become crescent-shaped and hard so that they block small blood vessels resulting in a lack of oxygen supplied to tissues and organs. This blockage causes episodes of pain, short-term and long-term organ damage, acute chest syndrome and stroke. Sickle cell disease can also lead to bone complications in both the short and long term. Pain and musculoskeletal complications are the most common reasons for people with sickle cell disease seeking medical treatment; even though they do not greatly contribute to mortality, they remain an important cause of illness in the short and long term.. Vitamin D deficiency is common in people with sickle cell disease regardless of age and season. Since vitamin D regulates calcium levels ...
Eighteen eyes of 9 consecutive patients with a median age of 41 years (range: 19-54) with electrophoretic confirmation of sickle cell disease were included and analyzed. A complete ophthalmologic examination was performed, including fundus examination, FA (Spectralis HRA+OCT, Heidelberg Engineering, Heidelberg, Germany), and OCTA (RTVue XR Avanti, Optovue Inc, Fremont, California, USA). Nine eyes of five healthy subjects were also analyzed with OCTA to serve as a control group. Results : OCTA demonstrated microvascular abnormalities in the perifoveal region of the macula in all eyes, whereas FA appeared normal in 9/18 eyes (50%). Most capillary abnormalities were located in the temporal juxtafoveal region and involved both the superficial and the deep capillary plexuses. The non-flow area (foveal avascular zone) was significantly larger in sickle cell disease patients than in the control group, both in the superficial and the deep capillary plexuses (P , 0.0001). The perifoveal vessel density ...
Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1) ...
Sickle cell anaemia (SCA) is a major chronic health problem in Uganda. In patients with SCA, the level of foetal haemoglobin (HbF) has been found to be important in influencing the clinical course of the disease. Thus populations with high levels of HbF like those in Saudi Arabia have been described as having a milder clinical course with fewer complications as compared to populations with lower levels. Disease modifying drugs can increase the Hb F levels and modify the presentation of SCA. This was a cross sectional study in which we determined foetal haemoglobin levels and examined the relationship between HbF levels and disease severity in SCA patients in Mulago Hospital, Kampala, Uganda. We consecutively enrolled 216 children aged 1 year to 18 years with SCA attending the Sickle Cell Clinic at Mulago Hospital whose guardians had given consent. The history included age at onset of initial symptoms and diagnosis, number of hospitalisations and blood transfusions and other complications of SCA
Our sickle cell disease and hemoglobinopathies program is part of the Manhattan Comprehensive Sickle Cell Center. This is an NIH-funded program that brings together in the same institution clinical and basic research projects along with clinical and psychosocial services to serve the patients from our community better. We have a team of dedicated physicians, nurses, and social workers who specialize in sickle cell disease, providing a comprehensive approach to the management of this disease and helping the patients deal with its ravishing effects on their daily lives.. A large number of our patients participate in cutting-edge clinical trials that investigate the use of agents that stimulate fetal hemoglobin (e.g., butyrate and hydroxyurea) in the treatment of sickle cell disease and b-thalassemia. We also have several laboratory-based research projects on sickle cell disease. One of these projects is focused on the investigation of the role of adhesion in the pathophysiology of sickle cell ...
This page includes the following topics and synonyms: Sickle Cell Anemia with Splenic Sequestration, Splenic Sequestration in Sickle Cell Anemia, Splenic Sequestration.
This page includes the following topics and synonyms: Sickle Cell Anemia with Splenic Sequestration, Splenic Sequestration in Sickle Cell Anemia, Splenic Sequestration.
Treatment depends on the level of pain and how long it lasts. Sometimes, non-prescription, or over-the-counter, pain relievers such as ibuprofen can help. Other times, a person needs stronger pain relief medicine that is prescribed or given by a doctor. Some painful episodes may need IV therapy for fluids and powerful pain medicines, such as morphine, to ease the pain.. You can prepare for a crisis in advance by creating a pain management plan with your doctor. This plan should include not only the types of medicines you can take at home but also other actions you can take at home to relieve pain. Also, your plan helps you know when to call your doctor or go to a hospital.. For more information, see Sickle Cell Disease: Pain Management. ...
TY - JOUR. T1 - Delayed methotrexate clearance in a patient with sickle cell anemia and osteosarcoma. AU - Mantadakis, Elpis. AU - Rogers, Zora R.. AU - Smith, Angela K.. AU - Quigley, Raymond. AU - Ratliff, Arleen F.. AU - Kamen, Barton A.. PY - 1999/3/1. Y1 - 1999/3/1. N2 - A 15-year-old girl with homozygous sickle cell anemia (HbSS) and osteosarcoma is described. Delayed clearance of methotrexate (MTX) after the second course of high-dose MTX (HDMTX) led to the development of renal and hepatic toxicities. Rescue was accomplished with high-dose leucovorin, intravenous carboxypeptidase G2, and thymidine. Although the renal and hepatic abnormalities resolved, focal tonic-clonic seizures developed, accompanied by abnormal brain imaging. Four weeks after this episode, all clinical and biochemical abnormalities resolved. Preexistent end-organ damage associated with HbSS may compromise the ability to deliver high-dose chemotherapy with curative intent in patients with malignant disease.. AB - A ...
Boy Cured of Sickle Cell Anemia. 9:06 PM EST; December 13, 1999; Atlanta, GA (AP) -- A 13-year-old boy who underwent an experimental blood-cell transfusion was declared cured of sickle cell anemia Monday. Doctors had replaced the bone marrow of Keone Penn with stem cells from the umbilical cord of an unrelated infant in the hopes that the new cells would produce healthy marrow, which in turn produces blood cells.. "Im talking, my heart is beating and my brain is working," Keone said in a statement from Childrens Healthcare of Atlanta. "I made it." The transplant was performed December 11, 1998, and was believed to be the first time unrelated cord blood has been used to treat sickle cell anemia -- an inherited, crippling and sometimes lethal disease prevalent among blacks. Doctors had said that they would wait a year to see if the stem cells were creating healthy blood before declaring Keone cured. "The cord blood cells are now fully operational, making all healthy blood cells. ... We see no ...
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TY - JOUR. T1 - Vaso-Occlusive Episodes in Older Children with Sickle Cell Disease. T2 - Emergency Department Management and Pain Assessment. AU - Frei-Jones, Melissa J.. AU - Baxter, Amy L.. AU - Rogers, Zora R.. AU - Buchanan, George R.. PY - 2008/2/1. Y1 - 2008/2/1. N2 - Objective: To describe emergency department (ED) management of older children with sickle cell disease (SCD) experiencing a vaso-occlusive episode (VOE) and factors associated with disposition and ED return. Study design: We retrospectively reviewed ED visits of children age ≥8 years with SCD over the course of 1 year. Data were collected from the electronic medical record and the SCD database. Results: VOE was diagnosed 279 times in 105 patients; 45 of the patients had 1 ED visit, 25 had 2 ED visits, and 16 had ≥5 ED visits. The overall admission rate was 178/279 (64%), 166 on the first ED visit and 12 on a return visit within 72 hours. Use of home opioids, duration of VOE, and hemoglobin concentration were not ...
This past summer, a Columbus woman suffering from sickle cell disease said she would have died had it not been for a lifesaving blood donor.
Sickle cell disease is inherited. It is the result of a genetic mutation that causes hemoglobin cells to be defective. This mutation is thought to have originated in areas of the world where malaria was common, since people with sickle trait do not get malaria. The sickle trait actually offers some protection from the parasite that causes malaria, which is carried by mosquitoes. Malaria is most often seen in Africa and in the Mediterranean area of Europe.. Sickle cell disease primarily affects those of African descent and Hispanics of Caribbean ancestry, but the trait has also been found in those with Middle Eastern, Indian, Latin American, Native American, and Mediterranean heritage. In the US, it has been estimated that 1,000 children are born each year with sickle cell disease. One in twelve African-Americans has sickle cell trait.. A baby will be born with sickle cell disease only if two sickle cell genes are inherited - one from the mother and one from the father. A person who has only one ...
Sickle-cell[edit]. Main article: Sickle-cell anemia. See also: Sickle-cell trait and Evolutionary_baggage § Sickle-Cell and ... While malaria is still affecting the regular cells (2), the ratio of sickle to regular cells is 50/50 due to sickle cell anemia ... Sickle cell - The gene for HbS associated with sickle-cell is today distributed widely throughout sub-Saharan Africa, the ... HbAS: Heterozygous sickle-cell hemoglobin; HbAA: normal hemoglobin; HbSS: homozygous sickle-cell hemoglobin. [65] ...
Sickle-cell anaemia[change , change source]. Such a balance is seen more simply in sickle-cell anaemia, which is found mostly ... Another example is sickle-cell anaemia.. In order to be classified as such, morphs must occupy the same habitat at the same ... The sickle-cell variant survives in the population because the heterozygote is resistant to malaria and the malarial parasite ... The sickle-cell and Haemoglobin C genes in some African populations. Ann. Human Genet. 21, 67-89. ...
... results in the disease known as Sickle Cell Anemia. Sickle-Cell Anemia is an autosomal recessive disorder that affects 1 in 500 ... Sickle-cell anemia is caused by a point mutation in the β-globin chain of hemoglobin, causing the hydrophilic amino acid ... "Anemia, Sickle Cell". Genes and Disease. Bethesda MD: National Center for Biotechnology Information. 1998. NBK22183. Clancy S ( ... These sickle-shaped cells cannot carry nearly as much oxygen as normal red blood cells and they get caught more easily in the ...
Sickle cell anemia. Toxins, including ifosfamide (more commonly causing pRTA than dRTA), toluene, lithium carbonate and ... Cell Biol. 37 (6): 1151-61. doi:10.1016/j.biocel.2005.01.002. PMID 15778079. Buckalew VM Jr (1989). "Nephrolithiasis in renal ... Distal RTA is characterized by a failure of acid secretion by the alpha intercalated cells of the cortical collecting duct of ... 1997). "Familial distal renal tubular acidosis is associated with mutations in the red cell anion exchanger (Band 3, AE1) gene ...
... sickle-cell anaemia; and the mosquito transmission of Venezuelan Equine Encephalomeyelitis Virus in Trinidad. But he will best ...
Sickle Cell Anemia ...thalassemia == Blood disorders Hematologists Hematology topics http://www.austincc.edu/mlt/clin1/ ... Anemias (lack of red blood cells or hemoglobin) Hematological malignancies Coagulopathies (disorders of bleeding and ... It involves treating diseases that affect the production of blood and its components, such as blood cells, hemoglobin, blood ... Blood Venous blood Venipuncture Hematopoiesis Blood tests Cord blood Red blood cells Erythropoiesis Erythropoietin Iron ...
A well-studied case is that of sickle cell anemia in humans, a hereditary disease that damages red blood cells. Sickle cell ... The sickle-cell and Haemoglobin C genes in some African populations. Ann. Human Genet. 21, 67-89. Sickle cell anemia. 2009. ... A person who inherits the sickle cell gene from one parent and a normal hemoglobin allele (HgbA) from the other, has a normal ... Malaria versus sickle-cell trait distributions Frequency-dependent selection occurs when the fitness of a phenotype is ...
"FDA Approves Immucor's PreciseType HEA Test to Screen for Sickle Cell Trait". Sickle Cell Anemia News. Retrieved 22 November ... Through its work with cord blood, stem cells and sickle cell treatments, NYBC is a leader in precision medicine, which takes ... The PreciseType HEA test screens blood donors for sickle cell trait (SCT), an inherited blood disorder that affects 1 million ... "FDA approves Immucor's PreciseType® HEA Test to be used for screening blood donors for Sickle Cell Trait (SCT)". Nasdaq. 21 ...
Sickle-cell anemiaEdit. Sickle-cell anemia (SCA) is a genetic disorder caused by the presence of two incompletely recessive ... Possible advantage of being heterozygous for sickle cell anemia disease (A) vs. normal blood cell response (B) when infected ... If effective sickle-cell anemia treatments become available to the same degree, allele frequencies should remain at their ... A well-established case of heterozygote advantage is that of the gene involved in sickle cell anaemia. ...
Luzzatto L (2012). "Sickle cell anaemia and malaria". Mediterr J Hematol Infect Dis. 4 (1): e2012065. doi:10.4084/MJHID. ... are resistant to malaria and develop minimal effects of the anaemia. Sickle cell disease is closely related to another mutant ... The most common is HbS, which causes sickle cell disease. HbS is produced by a point mutation in HBB in which the codon GAG is ... Mutations in the gene produce several variants of the proteins which are implicated with genetic disorders such as sickle-cell ...
Miles has sickle cell anemia. http://www.fyi.legis.state.tx.us/fyiwebdocs/PDF/house/dist146/m1.pdf "Simpson Withdraws, Straus ...
Sickle cell anemia is the most common genetic disorder among African Americans in the United States. While approximately 8% are ... doi:10.1111/j.1745-7599.2004.tb00426.x. B., G. (October 5, 1956). "Malaria and Sickle-Cell Anemia". Science. 124: 619-624. doi: ... The test looks at a person's DNA, which is taken from cells in a blood sample or from cells that are gently scraped from inside ...
Mason VR: Sickle cell anemia. JAMA 1922;79:1318-1320. Frank Capra. The Name Above the Title. Macmillan. New York. 1971. p. 174 ... As a medical resident at Hopkins in 1922 Mason gave the disease sickle cell anemia its name. When motion picture director Frank ...
Sickle cell anaemia. Variable degrees of hemolysis and intermittent episodes of vascular occlusion resulting in tissue ischemia ... Cell-free fetal DNA (cffDNA) testing is a non-invasive (for the fetus) test. It is performed on a sample of venous blood from ... For example, a medical procedure called a buccal smear uses a small brush or cotton swab to collect a sample of cells from the ... Alternatively, a small amount of saline mouthwash may be swished in the mouth to collect the cells. The sample is sent to a ...
Sickle cell anemia. In the event of a positive fecal occult blood test, the next step in the workup is a form of visualization ... Iron deficiency anemia[edit]. An extensive literature has examined the clinical value of FOBT in iron deficiency anemia. ... If colon cancer is suspected in an individual (such as in someone with an unexplained anemia) fecal occult blood tests may not ... St John DJ, Young GP (April 1978). "Evaluation of radiochromium blood loss studies in unexplained iron-deficiency anaemia". ...
Sickle-cell anemia is also considered a recessive condition, but heterozygous carriers have increased resistance to malaria in ... Williams T. N.; Obaro S. K. (2011). "Sickle cell disease and malaria morbidity: a tale with two tails". Trends in Parasitology ... Examples of this type of disorder are Albinism, Medium-chain acyl-CoA dehydrogenase deficiency, cystic fibrosis, sickle-cell ... LHCGR (Luteinizing hormone insensitivity, Leydig cell hypoplasia, Male-limited precocious puberty). *FSHR (Follicle-stimulating ...
This was introduced in November 1949, with the seminal paper, "Sickle Cell Anemia, a Molecular Disease", in Science magazine, ... "Sickle Cell Anemia, a Molecular Disease". Science, 25 November 1949, vol. 110, no. 2865, pp. 543-548. http://www.mayo.edu/ ...
Working with populations in which sickle cell anemia was endemic, in 1966, KCH physicians Drs. Margaret G. Robinson and R. ... "Pneumococcal Meningitis in Sickle-Cell Anemia". New England Journal of Medicine. 274: 1006-1008. doi:10.1056/ ... These results proved paramount in establishing the practice of vaccinating patients suffering from sickle-cell anemia against ... Janet Watson observed a high incidence of pneumococcal meningitis in sickle cell patients - a similar rate to that of post- ...
His success with sickle cell anemia led Pauling to speculate that a number of other diseases, including mental illnesses such ... It was the first proof of a human disease caused by an abnormal protein, and sickle cell anemia became the first disease ... Pauling, L.; Itano, H. A.; Singer, S. J.; Wells, I. C. (25 November 1949). "Sickle Cell Anemia, a Molecular Disease". Science. ... In November 1949, Pauling, Harvey Itano, S. J. Singer and Ibert Wells published "Sickle Cell Anemia, a Molecular Disease" in ...
"Sickle Cell Anemia, a Molecular Disease". Science, 25 November 1949, vol. 110, no. 2865, pp. 543-548. BJ Strasser, Perspectives ... In November 1949, with the seminal paper, "Sickle Cell Anemia, a Molecular Disease", in Science magazine, Linus Pauling, Harvey ... "Sickle Cell Anemia, a Molecular Disease"] Science, 19 November 1999, vol. 286, no.5444, pp. 1488 - 1490. RJ Williams (1956) ... The concept of the distribution of medicine to each individual cell just as oxygen would be an example of the practice of ...
While at Caltech, Itano joined the lab of Linus Pauling and began working on sickle cell anemia, a genetic disease that Pauling ... recognizing his sickle cell work. Pauling, Linus; Harvey A. Itano; S. J. Singer; Ibert C. Wells (1949-11-01). "Sickle Cell ... Ingram, V. M. (1956-10-13). "A Specific Chemical Difference Between the Globins of Normal Human and Sickle-Cell Anaemia ... Pauling was convinced that sickle cell disease was caused by defective hemoglobin, and set Itano to find out what made sickle ...
Pauling, Linus; Harvey A. Itano; S. J. Singer; Ibert C. Wells (1949). "Sickle Cell Anemia, a Molecular Disease". Science. 110 ( ... which are defined as any microscopic organism that comprises either a single cell (unicellular), cell clusters or no cell at ... This section contains a list of works on cell biology, the study of cells - their physiological properties, their structure, ... 2001). Landmark papers in cell biology : selected research articles celebrating forty years of the American Society for Cell ...
One example is sickle cell anemia. It is due to a mutation in the hemoglobin gene leading to sickle shape formation of red ... The alternative homozygote, which does not carry the sickle cell disease allele, is susceptible to infection by Plasmodium. As ... CS1 maint: Multiple names: authors list (link) "What Is Sickle Cell Disease?". National Heart, Lung, and Blood Institute. June ... Hence, homozygote and heterozygote genotypes for the sickle-cell disease allele show malaria resistance, while the homozygote ...
Doctors Taliaferro and Huck discovered a latent form of sickle cell anemia. Their study on sickle cell anemia was the first of ... Beginning in 1920, doctors at Johns Hopkins Hospital conducted research on sickle cell anemia, or sickle cell disease. Although ... There is a lack of phenotypic expression of Ho-2 in terms of sickle cell, so a person with sickle cell and hemoglobin Hopkins-2 ... There were, however, no sickled cells found in the blood and they had no symptoms relating to sickle cell. There was also a ...
... vascular complications due to sickle cell anemia; other cardiovascular diseases including heart attack, stroke, and peripheral ... cell movement) of native T cells; and impairs the adhesion of dendritic cells to T cells thereby inhibiting dendritic cell- ... Foulon I, Bachir D, Galacteros F, Maclouf J (1993). "Increased in vivo production of thromboxane in patients with sickle cell ... Both isoforms stimulate cells in part by activating the Gq family of G proteins. In at least certain cell types, however, TPα ...
... sickle cell anemia; sepsis; congestive heart failure; chronic obstructive pulmonary disease; and complications of devices, ... T-cell count drops below 200).[76] The Medicaid eligibility policy contrasts with the Journal of the American Medical ... or in certain patients commencing at an even higher T-cell count. Due to the high costs associated with HIV medications, many ... Association (JAMA) guidelines which recommend therapy for all patients with T-cell counts of 350 or less, ...
Sickle cell trait is a benign carrier condition, usually with none of the symptoms of sickle cell anemia or other sickle cell ... Sickle cell trait is a benign carrier condition, usually with none of the symptoms of sickle cell anemia or other sickle cell ... Sickle cell trait is a benign carrier condition, usually with none of the symptoms of sickle cell anemia or other sickle cell ... Sickle cell trait is a benign carrier condition, usually with none of the symptoms of sickle cell anemia or other sickle cell ...
Sickle Cell Anemia, Sickle Cell Disease, Sickle Cell Anemia Symptoms, Cause Of Sickle Cell Anemia, Definition Of Sickle Cell ... Genetics Of Sickle Cell Anemia, Sickle Cell Anemia Complications, Sickle Cell Anemia Emedicine, Sickle Cell Anemia Pain, Sickle ... Sickle Cell Anemia Disease, Sickle Cell Anemia Information, Sickle Cell Anemia Inheritance, Sickle Cell Anemia Test, Sickle ... Sickle Cell Anemia Diagnosis, Treatment Of Sickle Cell Anemia, Cure For Sickle Cell Anemia, Prevention Of Sickle Cell Anemia, ...
Levine, L. A. and Guss, S. P. Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia-associated ... The reticulocyte count is often elevated in men with sickle cell anemia. Hemoglobin electrophoresis identifies the presence of ... Short period of administration of diethylstilbestrol in stuttering priapism in sickle cell anemia. Am J Hematol, 69: 297, 2002. ... In such cases, screening for sickle cell disease or trait should be performed by either the Sickledex test or examination of a ...
Background: Sickle cell anaemia is an autosomal recessive disorder that arises due to the substitution of glutamic acid with ... Aim: The aim of this study was to establish baseline serum lipid levels in sickle cell anaemia patients in LASUTH and correlate ... LIPID PROFILE AND DISEASE SEVERITY IN SICKLE CELL DISEASE PATIENTS IN LAGOS STATE. NIGERIA * Uche Ebele ... Keywords: sickle cell disease, serum total cholesterol, low-density lipoproteins, high-density lipoproteins, triglycerides, ...
Pharmacokinetics and bioequivalence of a liquid formulation of hydroxyurea in children with sickle cell anemia., Jeremie H. ... The Influence of Age on the Diagnostic Performance of White Blood Cell Count and Absolute Neutrophil Count in Suspected ... Quantification of Dihydroxyacetone Phosphate (DHAP) in Human Red Blood Cells by HPLC-TripleTOF 5600™ Mass Spectrometer., Shuang ... Hematologic outcomes after total splenectomy and partial splenectomy for congenital hemolytic anemia., Brian R Englum, Jennifer ...
Sickle-cell anaemia.. Br Med J 1973; 1 doi: https://doi.org/10.1136/bmj.1.5851.488-a (Published 24 February 1973) Cite this as ...
Hemoglobin and Sickle Cell Anemia - Oregon State University Library Sickle Cell Anemia, a Molecular Disease - reproduction of ... "Sickle Cell Anemia, a Molecular Disease" is a 1949 scientific paper by Linus Pauling, Harvey A. Itano, Seymour J. Singer and ... Pauling, Linus; Harvey A. Itano; S. J. Singer; Ibert C. Wells (1949-11-01). "Sickle Cell Anemia, a Molecular Disease". Science ... Strasser, Bruno J. (1999-11-19). "Perspectives: Molecular Medicine: "Sickle Cell Anemia, a Molecular Disease"". Science. 286 ( ...
Anemia Anemia is a common blood disorder that affects your red blood cells. Learn about the types, causes, treatments, and ...
What are common sickle cell complications? Well go over all of them, from acute chest syndrome to vision loss. Learn how ... Understanding sickle cell anemia. Sickle cell anemia (SCA), also known as sickle cell disease, is an inherited red blood cell ( ... A sickle cell test is a blood test used to determine if you have sickle cell disease (SCD) or sickle cell trait. People with ... Learn everything you need to know about sickle cell trait (SCT) and how SCT differs from sickle cell disease. ...
... or sickle cell anemia) causes your body to produce abnormally shaped red blood cells. Learn about symptoms and treatment. ... If you are born with one sickle cell gene, its called sickle cell trait. People with sickle cell trait are generally healthy, ... Sickle Cell Anemia Disease (For Kids) (Nemours Foundation) * Sickle Cell Disease (For Parents) (Nemours Foundation) Also in ... What is sickle cell disease (SCD)?. Sickle cell disease (SCD) is a group of inherited red blood cell disorders. If you have SCD ...
The following organizations are good resources for information on sickle cell anemia: ... The following organizations are good resources for information on sickle cell anemia:. *American Sickle Cell Anemia Association ... Sickle Cell Disease Association of America -- www.sicklecelldisease.org. *Centers for Disease Control and Prevention -- www.cdc ... US National Library of Medicine, Genetics Home Reference -- ghr.nlm.nih.gov/condition/sickle-cell-disease. ...
Sickle-cell Anaemia in Africa. Br Med J 1952; 2 doi: https://doi.org/10.1136/bmj.2.4791.996-b (Published 01 November 1952) Cite ...
To Promote the issues surrounding sickle cell disease, because these issues are a political hot bed. Medical genetics is the ... A painful time for Sickle Cell sufferers. The number of Sickle Cell anaemia sufferers in Northampton has tripled since 2004. ... Sickle Cell. A pain "episode" or "crisis" is the most common symptom of sickle cell disease, and the top reason that people ... Sickle cell disease is as common as cystic fibrosis, yet less is known about the severe complications that can lead to death in ...
Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, ... Sickle Cell Anemia) and Sickle Cell Anemia What to Read Next on Medscape. Related Conditions and Diseases. * Anemia ... Proliferative sickle cell retinopathy. Sickle cell retinopathy is believed to be vaso-occlusion of peripheral arterioles of the ... Sickle Cell Anemia Differential Diagnoses. Updated: Sep 04, 2018 * Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa ...
Functional asplenia in sickle-cell anemia.. Pearson HA, Spencer RP, Cornelius EA. ... Anemia, Sickle Cell/complications. *Anemia, Sickle Cell/genetics. *Anemia, Sickle Cell/physiopathology* ...
Researchers discover how carriers of the sickle-cell anaemia gene are protected from malaria. ... Sickle-Cell Anemia Mystery Is Solved. Researchers discover how carriers of the sickle-cell anaemia gene are protected from ... Enter the sickle-cell factor. In red blood cells containing the aberrant sickle-cell haemoglobin, Lanzer and his team observed ... In people with two copies of the S mutation, they deform into a half-moon shape--the 'sickle cells' that give the ...
Is hydroxyurea treatment associated with lower medical costs for young children with sickle cell anemia? Learn more. ... What is Sickle Cell Disease (SCD)?. A group of inherited red blood cell disorders in which the red blood cells become hard and ... The total cost of health care for young children 1-3 years of age with sickle cell anemia who received HU treatment was reduced ... Average Medicaid reimbursements for hospital stays for US children with sickle cell anemia in 2009 were used to calculate the ...
sickle cell hemoglobin. OAHI - obstructive apnea hypopnea index. OSAS - obstructive sleep apnea syndrome. SCA - sickle cell ... Obstructive Sleep Apnea and Sickle Cell Anemia. Carol L. Rosen, Michael R. Debaun, Robert C. Strunk, Susan Redline, Sinziana ... Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108(9):2923-2927pmid: ... Pulse oximetry in sickle cell anemia. Crit Care Med. 2001;29(9):1803-1806pmid:11546990. ...
Symptoms of sickle cell anemia include bacterial infections, arthritis, leg ulcers, fatigue, and lung and heart injury. Sickle ... sickle cell disease) is a blood disease that shortens life expectancy. It is caused by an inherited abnormal hemoglobin that ... cell treatment is focused on managing and preventing the worst symptoms of the disease. ... Picture of Sickle Cell Red Blood Cell. Sickling of the red blood cells in patients with sickle cell anemia results in cells of ...
16 at the Tydings Park Yacht Basin was sponsored by The Association for the Prevention of Sickle Cell Anemia Inc. of Harford ... The Sickle Cell Anemia 2 Mile Run/Walk-A-Thon Sunday, Sept. ... Sickle Cell Anemia 2 Mile Run/Walk. Bobby Parker for The Record ... The Sickle Cell Anemia 2 Mile Run/Walk-A-Thon Sunday, Sept. 16 at the Tydings Park Yacht Basin was sponsored by The Association ... The Sickle Cell Anemia 2 Mile Run/Walk-A-Thon Sunday, Sept. 16 at the Tydings Park Yacht Basin was sponsored by The Association ...
I decided to see a doc and he took a blood sample from me, results came and turns out I have anemia and my iron is critically ... There are different types of anemia. Some are hereditary, and some are acquired. Have you considered going back to the doctor ...
Sickle cell disease causes red blood cells to be sickle-shaped. Read on to learn about risk factors, symptoms, and more. ... Red blood cells are normally shaped like discs, which allows them to travel through blood vessels. ... What is sickle cell anemia?. Sickle cell anemia, or sickle cell disease (SCD), is a genetic disease of the red blood cells ( ... Sickle Cell Test. A sickle cell test is a blood test used to determine if you have sickle cell disease (SCD) or sickle cell ...
Normal blood cells are disk-shaped with an indentation in the ... Sickle cell anemia is a blood disorder that causes abnormally ... Treatment of Sickle Cell Anemia. Most cases of sickle cell anemia cannot be cured, but blood and marrow stem cell transplants ... Sickle-Cell Anemia News. Sickle cell anemia is a blood disorder that causes abnormally shaped red blood cells. Normal blood ... But with sickle cell anemia, the body produces red blood cells that are shaped like a sickle, or crescent. These cells dont ...
... , Sickle Cell Trait, Sickle Cell Disease, Sickle Cell Disorder, Sickle Hemoglobin. ... Anemias, Sickle Cell, Sickle Cell Anemia, Sickle Cell Anemias, Sickle-cell anemia, Sickle-cell anemia, unspecified, Disease, ... anemia hemolytic sickle cell anemia, sickle cell anemia, Sickle cell anaemia NOS, Sickle cell anaemia unsp type, Sickle cell ... syndrome sickle cell, Anaemia;sickle cell, anemia cell disorder sickle, anemia sickle cell, anemia sickle celled, sickle cell ...
... but will have sickle cell trait. People who have sickle cell trait dont have sickle cell anemia and usually dont have any ... Sickle Cell Anemia. Sickle cell anemia is an inherited blood disorder that mostly affects people of African ancestry, but also ... What Is Sickle Cell Anemia?. Sickle cell anemia is a blood disorder that affects hemoglobin (pronounced: hee-muh-glow-bin), the ... People with sickle cell anemia have inherited two sickle cell genes, one from each parent. A child who has inherited the sickle ...
  • All agreed further work is required to determine if the damage done to the white matter from sickle cell to see if it was preventable or reversible ( Stotesburg et al, 2018) . (emaxhealth.com)
  • Couple that with world-renowned diagnostic experts and the most advanced diagnostic techniques to enable physicians to detect sickle cell anemia as early as possible, and patients are experiencing improved outcomes, faster responses to treatment and fewer side effects. (osu.edu)
  • The patient will add a drop of blood, to a plastic microfluidic chip that is pre-loaded with reagents required to detect sickle cell anemia. (indiavision.com)
  • LVMH has been actively involved in the fight against sickle cell anemia since 2011, providing support for teams at Robert Debré Hospital in Paris. (lvmh.com)
  • The event raises funds for the fight against sickle cell anemia, a concrete example of the Group's commitment to social responsibility. (lvmh.com)