Question

background information about Hereditary Spherocytosis about 3 pages if possible

shera O introduction, topic, and role. (Online: Week 4, 20 points) Med math Pre-test Homework: 3 to 4-page Literature review (background information of your Final paper on disease topic assigned week 1 (100 points: Analytical Perspective of Inquiry) DUE Sunday, September 8, 11:59 pm. (NO Cbstract)

Answer 1

HEREDITARY SPHEROCYTOSIS;
HEREDITARY SPHEROCYTOSIS > Inherited disorders & Mainly due to defects in the Intrinsic system of red skeleton membione That render Red cells spheroid less deformable and easily to splenic sequestration and finally got destauet. - autosomal dominant inheritance a) Prevalonu high in northern Europe. Pathogenesis & There is loss of spectrun, ankyrin and band 3.1 protein is RBC membiare Therefore The red blood cells could not maintain the shape and change into the sphere, microvesicles Normal RBC'S Biconcave rauge surface area . central pallor e can maneuver Through splenie sinusoids Sphenocytes a circulau smaller surfau area No central pallor cannot mareuver, get stuck is splenic Sinusoids I cannot expand is hypotonic solution. Thay will burst, → can expand is hypotonic solution, because it biconcane

Primary membrane skeletal defect 1. membrone stability → membrane loss a spheroapte decrease sulau to Volume rectio decrease adeformability i splenic Trapping Erythrostasis & Colucose VPH phagoutosis, entra Vascular hemolysis morphology ar a small, dark-staining and cells lacking The central zore of pallor nutie Splonie macrophag clinical features & Diagnosts history, clinical ů based only on The pmily teatroes of the patients Jaundice I Sometime anemia, splenomegaly and gall stors also present I lab Test Both Memoglobin livel, and are decreased due to anomia Hematocrit

MCHC - mean corpusular hemoglobis. concertation is increased because its The only anemia with the high Miche » Peripheral smear There is numerous Spherocytes with no central pallar - osmoter fragility test when red blood call in hypotonic solution Normally the red blood cells should take by their osmosis because ite hypertonie dside relative to outsidh & Ph should expord That is normal but is spherocyte, cannok Expand Finally is bust Treatment a - No cure because genetic clefact causing defective protein is membiare of RBC - But we can manage by doing EPO, stimulate the bone marrow to produce more red blood cells - Folate &tion for building the red bood cells but we waik until boch puberty becaus spleer & lymph Digan dore boya • anti-pneumococcal vaccire cloing The spleer nemoved because spleen protect against capsulated organism a splenectomy meer & lymph segar
Hereditary Spherocytosis is one of the inherited disorder and it's pathogenesis present in red blood cell membrane,with clinical feature of anemia,jaundice,gall stone and splenomegaly.There is no cure ,only manage and control the disease to be done.