-HEREDITARY-SPHEROCY_37786

-HEREDITARY-SPHEROCY_37786 - HEREDITARY SPHEROCYTOSIS A...

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HEREDITARY SPHEROCYTOSIS A congenital, largely autosomal dominant hemolytic disorder caused by a defect in the red cell membrane, due to an abnormality in one of the membrane proteins. Most common inherited red cell disorder (1/5000 in Northern European populations ). Symptoms are mild. Include slight jaundice and elevated serum bilirubin. Anemia mild or absent because of compensating red cell production in bone marrow. Red cells display: Microspherocytosis : small round cells Osmotic fragility : as measured by in vitro lysis of red cells suspended in saline solutions. Cells have a decreased surface-volume ratio and tolerate less swelling in hypotonic medium. Hence, they have greater susceptibility to rupture.
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HEREDITARY SPHEROCYTOSIS Osmotic Fragility
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I. RED CELL MEMBRANE Erythrocytes can be lysed to obtain membranous particles, termed erythrocyte ghosts. The red cell membrane consists of a fluid lipid bilayer in which are embedded integral proteins and loosely associated peripheral proteins . All proteins can be solubilized in a detergent-containing solution and separated by gel electrophoresis. At least 12 bands can be visualized using a protein stain (e.g. Coomassie Blue) and some (glycoproteins) also stain with a reagent used to detect
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-HEREDITARY-SPHEROCY_37786 - HEREDITARY SPHEROCYTOSIS A...

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