HEREDITARY SPHEROCYTOSIS - PATHOLOGY скачать в хорошем качестве

HEREDITARY SPHEROCYTOSIS - PATHOLOGY

📌 𝐅𝐨𝐥𝐥𝐨𝐰 𝐨𝐧 𝐈𝐧𝐬𝐭𝐚𝐠𝐫𝐚𝐦:-   / drgbhanuprakash   📌𝗝𝗼𝗶𝗻 𝗢𝘂𝗿 𝗧𝗲𝗹𝗲𝗴𝗿𝗮𝗺 𝗖𝗵𝗮𝗻𝗻𝗲𝗹 𝗛𝗲𝗿𝗲:- https://t.me/bhanuprakashdr 📌𝗦𝘂𝗯𝘀𝗰𝗿𝗶𝗯𝗲 𝗧𝗼 𝗠𝘆 𝗠𝗮𝗶𝗹𝗶𝗻𝗴 𝗟𝗶𝘀𝘁:- https://linktr.ee/DrGBhanuprakash Hereditary Spherocytosis Hereditary Spherocytosis is an autosomal dominant defect in the genes encoding red blood cell (RBC) proteins leading to the creation of red blood cells that are sphere-shaped. Defective proteins implicated in this disease often include those such as spectrin and ankyrin. The defect is due to a loss of RBC membrane surface area without a decrease in RBC volume, which imposes a spherical shape on affected cells. This causes affected cells to get stuck in the narrow passages of the splenic cords leading to their destruction in the spleen (hence extravascular hemolysis). The incidence of disease is about 200 per million in those of northern European populations and less common in other parts of the world. Hereditary spherocytosis can present with hemolytic anemia, jaundice, and splenomegaly. Jaundice is usually most intense in the neonatal period as most cases are detected soon after birth. Splenomegaly and gallstone formation (especially as patient ages) are common in most patients with hereditary spherocytosis. The presenting hemolytic anemia can range from mild-severe depending upon the patient. Hereditary spherocytosis is diagnosed with a variety of laboratory findings. Notably, a peripheral blood smear will show spherocytes (small cells that have lost their central pallor). CBC will show anemia and reticulocytosis. The mean corpuscular hemoglobin concentration (MCHC) will be elevated reflecting the membrane loss and red cell dehydration. The osmotic fragility test demonstrates red blood cells with increased membrane fragility. The cells are suspended in a graded series of hypotonic solutions; spherocytes do not withstand swelling because of theirshape thus rupture sooner than normal red blood cells. Complications experienced by patients with hereditary spherocytosis include occasional hemolytic crises, leg ulcers, priaprism, and hypertrophic cardiomyopathy. Treatment of choice for hereditary spherocytosis is splenectomy. Supportive care can consist of folic acid, blood transfusions (especially in infants), and administration of erythropoietin. Splenectomy can eliminate or at least minimize anemia in patients with spherocytosis. The bilirubin and reticulocytosis should also fall to normal levels post procedure. #Hereditaryspherocytosis #hereditaryspherocytosispathology #Hereditaryspherocytosisgenetics #Hereditaryspherocytosistreatment