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Hbss Sickle Cell Questions And Answers With Verified Solutions 100% Correct!!!

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Hbss Sickle Cell Questions And Answers With Verified Solutions 100% Correct!!!

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  • August 12, 2024
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  • 2024/2025
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  • Hbss
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Hbss Sickle Cell Questions And
Answers With Verified Solutions 100%
Correct!!!
Sickle Cell Syndromes

Definition - ANSWER✔✔ Group of hereditary disorders characterized by the
presence of sickle cell hemoglobin (HbS) in red blood cells


Sickle Cell Trait (SCT) - ANSWER✔✔ heterozygous inheritance of one normal cell
gene and one sickle cell hemoglobin gene (HbAS)


Individuals usually asymptomatic


Sickle Cell Disease (SCD) - ANSWER✔✔ heterozygous or homozygous inheritance


Most common genotypes - ANSWER✔✔ HbSS
HbSC
HbSβ+-thal


What is a primary difference between SCT and SCD in tropical regions? -
ANSWER✔✔ SCT offers partial protection against malaria


Etiology *not impt for test* - ANSWER✔✔ Normal hemoglobin (HbA) composed
of two α chains and two β chains


Defects in β chains responsible for sickling:

, HbS: valine substituted for glutamic acid
Hemoglobin C (HbC): lysine for glutamic acid


HbS—increases hydrophobicity


Inheritance - ANSWER✔✔ Sickle Cell Anemia (SCA)—form of SCD that occurs
when patient has both genes encoding for HbS
Risk of offspring inheriting disease depends upon carrier status of parents


β-thalassemia can be present at the same time as HbS
HbSS and HbSβ° do not have normal β-globin production
More severe disease


Pathophysiology

Normal adult RBCs contain - ANSWER✔✔ HbA
HbA₂
HbF (fetal hemoglobin)
Contains α₂γ₂ instead of α₂β₂
Switch to β production occurs just before birth
Increased production—anemia, HSCT, chemotherapy


Pathophysiology

RBC size and shape - ANSWER✔✔ H₂0 and Hb content determine MCHC (mean Hb
Conc)
Passive and active transport—regulate intracellular cation and volume contents

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