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