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Medical Knowledge Assessment: Sickle Cell Disease Quiz

Challenge Your Understanding of Red Blood Disorders

Difficulty: Moderate
Questions: 20
Learning OutcomesStudy Material
Colorful paper art promoting quiz on Medical Knowledge Assessment Sickle Cell Disease

Ready to challenge your medical knowledge on sickle cell disease? This interactive Sickle Cell Disease Knowledge Quiz offers 15 multiple-choice questions covering genetics, pathophysiology, and management. Ideal for medical students, educators, and healthcare professionals aiming to improve understanding of red blood cell disorders. It's fully customizable, so you can modify questions in our quizzes editor to suit your needs. Start testing your expertise today and gain valuable insights with the Medical Knowledge Assessment Quiz suite.

Which type of mutation in the HBB gene leads to sickle cell disease?
Missense point mutation
Splice-site mutation
Nonsense mutation
Frameshift deletion
Sickle cell disease arises from a single missense point mutation in the beta globin gene substituting glutamic acid with valine. Other mutations such as nonsense or frameshift do not cause the specific amino acid change seen in HbS.
What is the inheritance pattern of sickle cell disease?
X-linked recessive
Autosomal recessive
Mitochondrial inheritance
Autosomal dominant
Sickle cell disease follows an autosomal recessive inheritance pattern, requiring two copies of the mutant HBB allele. Carriers with one allele generally have trait but not full disease symptoms.
On which chromosome is the beta globin gene located?
Chromosome 11
Chromosome 16
Chromosome 6
Chromosome 22
The beta globin gene cluster is located on chromosome 11. Other globin genes, such as alpha globin, reside on different chromosomes like 16.
Which finding on a peripheral blood smear is characteristic of sickle cell disease?
Macro-ovalocytes
Schistocytes
Target cells
Sickled red blood cells
Sickle cell disease is characterized by sickled red blood cells visible under hypoxic conditions due to hemoglobin polymerization. Other shapes like target cells are seen in different hemoglobinopathies.
What is the average lifespan of a sickled red blood cell?
60 - 90 days
30 - 45 days
120 days
10 - 20 days
Sickled red blood cells are destroyed prematurely, surviving on average 10 - 20 days compared to 120 days for normal red cells. This shortened lifespan contributes to hemolytic anemia.
Which medication is used to increase fetal hemoglobin and reduce vaso-occlusive crises?
Aspirin
Hydroxyurea
Dipyridamole
Folic acid
Hydroxyurea increases production of fetal hemoglobin (HbF), which inhibits polymerization of sickle hemoglobin and reduces the frequency of pain crises. Other options do not have this effect.
What is the recommended prophylactic antibiotic in young children with sickle cell disease?
Cephalexin
Azithromycin
Amoxicillin
Penicillin
Daily penicillin prophylaxis in young children with sickle cell disease reduces risk of invasive pneumococcal infection due to functional asplenia. Other antibiotics are not standard prophylaxis.
Which event most directly triggers a vaso-occlusive pain crisis?
Red blood cell polymerization under hypoxia
Bacterial sepsis
Complement activation
Immune complex deposition
Hypoxia induces polymerization of sickle hemoglobin, distorting red blood cells and obstructing microvasculature, which directly causes vaso-occlusive pain crises. The other mechanisms do not initiate the crisis.
Patients with repeated splenic infarctions are at increased risk of infection by which organism?
Cytomegalovirus
Streptococcus pneumoniae
Pseudomonas aeruginosa
Epstein-Barr virus
Functional asplenia from repeated splenic infarctions in sickle cell disease increases susceptibility to encapsulated bacteria such as Streptococcus pneumoniae. Viral pathogens listed are less directly related to asplenia.
Which laboratory marker is most indicative of ongoing hemolysis in sickle cell disease?
Elevated lactate dehydrogenase
Elevated amylase
Elevated creatine kinase
Elevated troponin
Elevated lactate dehydrogenase (LDH) is a sensitive marker of hemolysis as it is released from destroyed red blood cells. The other enzymes are not markers of red cell destruction.
What type of gallstones are commonly seen in sickle cell patients?
Pigment stones
Struvite stones
Cholesterol stones
Calcium oxalate stones
Chronic hemolysis in sickle cell disease increases bilirubin turnover, leading to formation of pigment gallstones. Cholesterol stones and the others have different etiologies.
Which diagnostic test confirms sickle cell trait or disease?
Direct Coombs test
Flow cytometry for CD55
Osmotic fragility test
Hemoglobin electrophoresis
Hemoglobin electrophoresis separates and quantifies hemoglobin variants, confirming presence of HbS in trait or disease. Osmotic fragility and Coombs tests assess other red cell disorders.
A patient presents with chest pain, fever, and pulmonary infiltrates on x-ray. Which complication is this?
Priapism
Acute chest syndrome
Leg ulcer
Deep vein thrombosis
Acute chest syndrome is characterized by new pulmonary infiltrates, chest pain, and fever in sickle cell disease. The other options do not present with these respiratory findings.
What is the primary mechanism of acute splenic sequestration in sickle cell disease?
Autoimmune destruction of red cells
Bacterial infiltration of splenic tissue
Disseminated intravascular coagulation
Rapid trapping of sickled cells in the spleen
Acute splenic sequestration occurs when large numbers of sickled red blood cells become trapped in the spleen, causing sudden enlargement and hypovolemia. Autoimmune processes and DIC are not the main cause.
Which clinical indication warrants emergent exchange transfusion in sickle cell disease?
Routine annual checkup
Chronic anemia without symptoms
Stable leg ulcers
Acute stroke
Acute stroke in sickle cell disease requires emergent exchange transfusion to rapidly reduce sickled cells and prevent further ischemic damage. The other situations do not require this urgent intervention.
Gene editing approaches using CRISPR for sickle cell disease often target which regulatory element to increase fetal hemoglobin?
Alpha globin locus
Gamma chain intron
BCL11A enhancer
HBB promoter
CRISPR strategies frequently disrupt the BCL11A enhancer in erythroid cells, de-repressing fetal hemoglobin production to ameliorate sickling. The other elements are not primary targets for this therapy.
Dactylitis in infants with sickle cell disease is caused by which pathophysiological process?
Immune-mediated synovitis
Viral myositis
Septic arthritis
Bone marrow infarction in small bones
Infant dactylitis represents vaso-occlusive infarction of the bone marrow in small bones of the hands and feet, causing painful swelling. Infectious and immune causes affect different tissues.
Which laboratory test may give a false negative result for sickling in a newborn due to high fetal hemoglobin levels?
Osmotic fragility test
Sodium metabisulfite sickling test
Direct antiglobulin test
Heinz body preparation
The sodium metabisulfite sickling test relies on deoxygenation to induce sickling, but high levels of HbF in newborns inhibit polymerization, causing false negatives. Other tests do not assess sickling directly.
Endothelial upregulation of which adhesion molecule contributes to vaso-occlusion in sickle cell disease?
P-selectin
MHC class I
CD4
CD8
P-selectin on activated endothelium binds sickled cells and leukocytes, promoting vaso-occlusion. CD4 and CD8 are T-cell markers and MHC class I is a general antigen-presenting molecule.
Which patient education point is most critical to prevent complications in sickle cell disease?
Ensure hydration and stay up to date on vaccinations
Avoid all fruits to reduce sugar load
Engage in high-impact sports regularly
Skip prophylactic antibiotics once infections resolve
Proper hydration reduces sickling risk and immunizations protect against encapsulated organisms in functionally asplenic patients. Avoiding fruits or high-impact sports is not advised, nor is discontinuing prophylaxis prematurely.
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Learning Outcomes

  1. Analyse sickle cell disease pathophysiology
  2. Identify genetic inheritance patterns of hemoglobin S
  3. Evaluate common clinical complications and crises
  4. Apply principles of disease management and prevention
  5. Interpret laboratory and diagnostic findings in SCD
  6. Delineate patient education strategies for effective care

Cheat Sheet

  1. Genetic Basis of Sickle Cell Disease - Dive into the heart of sickle cell disease by discovering how a tiny mutation in the HBB gene leads to the creation of hemoglobin S. This oddball hemoglobin turns red blood cells into rigid, crescent-shaped warriors that can block blood flow and cause all sorts of mischief. Getting the genetics down is your first step toward mastering SCD! GeneReviews®: Sickle Cell Disease Genetics
  2. Inheritance Pattern - Sickle cell disease follows an autosomal recessive pattern, so you need two copies of the mutated gene - one from each parent - to show symptoms. If you inherit just one copy, you'll be a carrier with the sickle cell trait, usually symptom-free but still important to know about. Understanding inheritance helps you predict risks and plan ahead! GeneReviews®: Sickle Cell Disease Inheritance
  3. Common Clinical Manifestations - Watch out for anemia that leaves you feeling tired, sudden pain crises like mini volcanic eruptions, and pesky swelling in your hands and feet. Frequent infections, delayed growth in kids, and vision hiccups can also tag along for the ride. Spotting these signs early means faster relief and better management! Mayo Clinic: Symptoms & Causes
  4. Potential Complications - Don't let SCD sneak up on you - strokes, acute chest syndrome, organ damage, and pulmonary hypertension are possible curveballs. Learning to recognize warning flags can help you dodge serious outcomes and keep life on track. Knowledge truly is your superpower in preventing complications! Mayo Clinic: Complications Overview
  5. Diagnostic Methods - Getting an accurate diagnosis is as simple as a blood test that checks for hemoglobin S, and newborn screening programs catch SCD early for prompt care. Early detection means earlier management, so you can stay one step ahead of potential crises. It's like giving your body an early warning system! Mayo Clinic: Diagnosis & Treatment
  6. Treatment Options - From the trusty hydroxyurea that reduces pain episodes to blood transfusions that boost healthy cells, there's an arsenal of therapies to explore. And for those who aim for a cure, bone marrow transplants can be life-changing - though they come with their own set of considerations. Staying informed about these treatments can dramatically improve patient outcomes. Mayo Clinic: Treatment Strategies
  7. Importance of Hydration - Think of your bloodstream like a river - if it dries up, the boats (your red blood cells) can't sail smoothly. Dehydration can trigger painful sickle cell crises, so sipping water regularly keeps your cells happy and flowing freely. It's a simple habit with powerful protective effects! Mayo Clinic: Self-Care Tips
  8. Role of Vaccinations - With SCD, infections are unwanted party crashers that can turn nasty fast, so staying up to date on pneumococcal and meningococcal vaccines is essential. These shots help your immune system keep germ invaders at bay, reducing risky hospital visits. Prevention is always better (and more fun) than cure! Mayo Clinic: Vaccination Guidelines
  9. Regular Medical Check-Ups - Routine clinic visits are your chance to track progress, spot any new issues, and tweak treatments like a pro. Think of your healthcare team as co-pilots on your SCD journey - frequent check-ins keep the trip smooth and safe. Consistency is key to long-term success! Mayo Clinic: Ongoing Care
  10. Patient Education and Support - Knowledge is power, and sharing it with patients and families boosts confidence and self-management skills. Support groups, counseling, and educational resources create a community that understands your daily wins and challenges. When everyone's on the same page, life with SCD becomes a team effort! Mayo Clinic: Educational Resources
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