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What is the most abundant protein in serum? Albumin 4g/dL What is the most abundant protein in the body? Hemoglobin, 14g/dL blood, very soluble!!! Is globin chain synthesis balanced? Yes. What triggers a shift from gamma to beta chains of hemoblogin? Birth. What is the Bohr effect? In acidic environments, like tissues, decreased affinity for oxygen. How does 2,3 DPG affect the oxyhemoglobin disassociation curve? Increased levels ill lead to a right shift, unload oxygen. Does the open or closed hemoglobin state bind better to oxygen? Open. And oxygen opens, so hemoglobin displays cooperativity. Do sickle cell patients have nucleated reds on smear? Yes, because retics so high, and because patients are asplenic. What happens to HbS? It is not as soluble in deoxygenated states. Where is hemolysis in SCD? Extravascular. What contributes to sickle cell symptoms besides hemolytic anemia? (1) Free heme scavenge NO leads to vasoconstriction and inflammation, (2) Exposure of negatively charged phospholipids on external membrane, causing thrombotic events. What induces sickle cell crisis? Hypoxemia, acidosis, dehydration, and infection. How does hyroxyurea work? Increases amount of HbF. Do patients with SCD get penicillin prophylaxis? Yes. Do patients with sickle cell trait have problems? They have some infarctions, including renal medulla, lose the ability to concentrate urine (?), and are susceptible to crisis in high altitudes or dehydrated states. How many alpha genes does one have on each chromosome 16? 2! So, to get alpha-thalassemia, one has to have damage to 3. What causes symptoms of thalassemia? Excess alpha chains are not very soluble. What is Hb delta/A2 a sign of? Beta-Thalassemia. What are target cells a sign of? Thalassemia, less intercellular Hb, relatively more membrane. Is there hemolysis in thalassemia? Yes, intramedullary and extravascular due to damage to RBCs. Retics elevated, but there is partial bone marrow failure. What are Heinz bodies? Precipitated Hb within red cells. What is hydrops fetalis? Alpha-thalassemia of all four genes means that babies have to rely on embryonic Hb can capture oxygen well, but cannot release, and so is not compatible with life. Which is more severe, Alpha-thalassemia, or Beta-Thalassemia? Beta-thalassemia, because alpha-thalassemia is less soluble.