Hb S Clinical Presentation and Crises

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Questions and Answers

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?

  • 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?

  • 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?

<p>Sickled red blood cells are more likely to become trapped and cause obstructions in the narrow diameter of small vessels. (A)</p> Signup and view all the answers

What is the underlying pathological process that connects vaso-occlusion with organ infarction in sickle cell disease?

<p>Reduced blood flow and oxygen supply to the organs due to vascular blockages. (A)</p> Signup and view all the answers

Flashcards

Clinical Presentation of Hb S

Describes how the disease shows itself in patients; it varies widely.

Vaso-occlusive Crisis

Severe pain caused by sickle cells blocking small blood vessels, leading to tissue damage and organ infarction.

Consequences of Blocked Vessels

Vaso-occlusive crises lead to tissue damage and organ infarction because of blocked blood flow.

Hand-Foot Syndrome

A painful condition in infants and young children due to vaso-occlusion in the hands and feet.

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Crisis Duration

Vaso-occlusive crises can last for several days to weeks.

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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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