Sickle Cell Quiz: Crisis Management & Pathophysiology

1. Which amino acid substitution in the β-globin chain characterizes sickle cell hemoglobin (HbS)?

Glutamic acid to valine at position 6

Lysine to leucine at position 12

Aspartate to alanine at position 3

Histidine to proline at position 8

2. Sickle cell anemia is inherited as which pattern?

Autosomal dominant

Autosomal recessive

X-linked recessive

Mitochondrial inheritance

Submit

4. Which of the following is a major trigger for vaso-occlusive crises in sickle cell disease?

Hypoxemia and dehydration

Hypercalcemia

Hyperglycemia

Hypertension

5. Acute chest syndrome in sickle cell disease is characterized by which clinical findings?

Fever, chest pain, and new pulmonary infiltrate

Hypercalcemia and bone pain only

Elevated liver enzymes without respiratory symptoms

Isolated splenic infarction

6. Hemoglobin electrophoresis in sickle cell anemia shows which pattern?

Predominant HbS with little or no HbA

Only HbA and HbF

Equal amounts of HbS and HbA

Predominantly HbA2

Submit

8. Which medication is a first-line agent for reducing vaso-occlusive crisis frequency?

Hydroxyurea

Ibuprofen

Penicillin V

Folic acid supplementation

Submit

10. Which complication results from repeated splenic infarctions and functional asplenia in sickle cell disease?

Increased susceptibility to encapsulated bacteria infections

Hypersplenia and thrombocytosis

Chronic hepatomegaly

Portal hypertension

11. Fetal hemoglobin (HbF) inhibits polymerization of HbS. True or False?

True

False

12. What is the primary goal of management during a vaso-occlusive crisis?

Pain control, hydration, and oxygenation

Immediate blood transfusion

Splenectomy

High-dose antibiotics

13. Chronic organ damage in sickle cell disease most commonly affects which systems?

Renal, cardiac, and pulmonary systems

Gastrointestinal and endocrine only

Skeletal system exclusively

Dermatologic and sensory systems

Submit

15. Which of the following is NOT a typical feature of sickle cell trait (AS genotype)?

Severe hemolytic anemia

Usually asymptomatic

Mild hemoglobinuria under stress

Normal life expectancy

Sickle Cell Quiz: Can You Manage a Crisis?

1. Which amino acid substitution in the β-globin chain characterizes sickle cell hemoglobin (HbS)?

2.

What first name or nickname would you like us to use?

2. Sickle cell anemia is inherited as which pattern?

3. Under low oxygen conditions, polymerization of HbS leads to the formation of ____.

4. Which of the following is a major trigger for vaso-occlusive crises in sickle cell disease?

5. Acute chest syndrome in sickle cell disease is characterized by which clinical findings?

6. Hemoglobin electrophoresis in sickle cell anemia shows which pattern?

7. The ____ spleen is a common complication of sickle cell disease in adults.

8. Which medication is a first-line agent for reducing vaso-occlusive crisis frequency?

9. Painful crisis in sickle cell disease is primarily caused by ____.

10. Which complication results from repeated splenic infarctions and functional asplenia in sickle cell disease?

11. Fetal hemoglobin (HbF) inhibits polymerization of HbS. True or False?

12. What is the primary goal of management during a vaso-occlusive crisis?

13. Chronic organ damage in sickle cell disease most commonly affects which systems?

14. Newborn screening for sickle cell disease uses ____ to detect abnormal hemoglobins.

15. Which of the following is NOT a typical feature of sickle cell trait (AS genotype)?