Podcast
Questions and Answers
What is the primary mechanism behind painful vaso-occlusive crises in sickle cell disease?
What is the primary mechanism behind painful vaso-occlusive crises in sickle cell disease?
- Aggregation of platelets leading to thrombus formation in small blood vessels.
- Inflammation of the blood vessel walls due to autoimmune response.
- Sickling of red blood cells causing obstruction in small blood vessels. (correct)
- Increased production of white blood cells causing hyperviscosity of blood.
Which of the following best describes the variability in clinical presentation of Hb S?
Which of the following best describes the variability in clinical presentation of Hb S?
- All patients experience equally severe crises throughout their lives.
- Most patients develop severe crises in infancy but improve with age.
- Some patients may live normal lives with no crises, while others experience severe complications from infancy. (correct)
- The severity of crises is directly proportional to the amount of Hb S present in the blood.
How does the duration of a typical painful vaso-occlusive crisis directly impact the affected tissues?
How does the duration of a typical painful vaso-occlusive crisis directly impact the affected tissues?
- The length of the crisis does not affect the severity of tissue damage as it depends on individual patient factors.
- Extended crises exacerbate tissue damage due to prolonged oxygen deprivation and infarction. (correct)
- The duration only affects the intensity of the pain experienced, not the extent of tissue damage.
- Prolonged crises allow the tissues to adapt to reduced oxygen supply, minimizing damage.
Why does sickle cell primarily affect small blood vessels, leading to vaso-occlusive crises?
Why does sickle cell primarily affect small blood vessels, leading to vaso-occlusive crises?
What is the underlying pathological process that connects vaso-occlusion with organ infarction in sickle cell disease?
What is the underlying pathological process that connects vaso-occlusion with organ infarction in sickle cell disease?
Flashcards
Clinical Presentation of Hb S
Clinical Presentation of Hb S
Describes how the disease shows itself in patients; it varies widely.
Vaso-occlusive Crisis
Vaso-occlusive Crisis
Severe pain caused by sickle cells blocking small blood vessels, leading to tissue damage and organ infarction.
Consequences of Blocked Vessels
Consequences of Blocked Vessels
Vaso-occlusive crises lead to tissue damage and organ infarction because of blocked blood flow.
Hand-Foot Syndrome
Hand-Foot Syndrome
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Crisis Duration
Crisis Duration
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Study Notes
- Clinical presentation of Hb S varies among patients.
- Some patients live normal lives without crises.
- Others experience severe crises, even as infants, and may die in early childhood or as young adults.
Types of Crises
- Can include painful vaso-occlusive crises.
- These crises are typically associated with severe pain.
- Sickle cells cause vaso-occlusive crises in small vessels.
- This results in tissue damage and organ infarction.
- Hand-foot syndrome is an example.
- Painful vaso-occlusive crises last from 4-6 days or possibly weeks.
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Description
The clinical presentation of Hb S varies, with some patients living normal lives and others experiencing severe crises. These crises, include vaso-occlusive events causing tissue damage and organ infarction. Hand-foot syndrome is one example of painful vaso-occlusive crises, lasting days or weeks.
