Sickle-cell anaemia
Introduction to Sickle-Cell Anaemia
Welcome to the Sickle-Cell Anaemia subsection of the Haematology for the MSRA course.
This section is designed to provide comprehensive and structured learning materials to help you understand and master the topic of Sickle-Cell Anaemia for the MSRA.
Resources for Sickle-Cell Anaemia:
- Sickle-Cell Anaemia Revision Notes for MSRA: Detailed and concise notes covering causes, symptoms, diagnosis, and treatment based on UK NICE guidelines. Use these notes to get a thorough understanding of the condition.
- Sickle-Cell Anaemia Flashcards for MSRA: Interactive flashcards to help you retain key facts and concepts related to sickle-cell anaemia. Perfect for quick revision sessions and reinforcing knowledge.
- Sickle-Cell Anaemia Accordion Q&A Notes for the MSRA: A question-and-answer style resource designed to enhance active learning and test your understanding. Ideal for quick coverage of weak areas.
- Sickle-Cell Anaemia Rapid Fire Quiz for MSRA: A fast-paced quiz to evaluate your recall and understanding of sickle-cell anaemia. Use it to assess your progress and readiness for more advanced material.
We encourage you to use these resources as part of your revision plan, and explore related topics within the Haematology for the MSRA course.
Key Points about Sickle-Cell Anaemia:
- Definition: Sickle-cell anaemia is an inherited blood disorder characterized by the production of abnormal hemoglobin (HbS), causing red blood cells to become rigid and shaped like a sickle, leading to blockages in small blood vessels and decreased oxygen delivery.
- Causes:
- Autosomal recessive inheritance of the sickle hemoglobin gene (HbS).
- Both parents must carry the sickle-cell trait (HbAS) for a child to inherit the condition.
- Symptoms:
- Painful episodes (sickle cell crises) due to blocked blood flow.
- Fatigue, pallor, and jaundice.
- Increased risk of infections due to splenic dysfunction.
- Delayed growth and development in children.
- Diagnosis:
- Newborn screening (heel prick test) to detect abnormal hemoglobin.
- Hemoglobin electrophoresis to confirm the presence of HbS.
- Blood tests revealing anemia and sickle-shaped red blood cells on blood smear.
- Management:
- Pain management during sickle cell crises with analgesics and hydration.
- Regular blood transfusions in severe cases to reduce sickle cell formation.
- Hydroxyurea to increase fetal hemoglobin (HbF) levels, reducing sickling.
- Vaccinations and prophylactic antibiotics to prevent infections.
- Complications:
- Stroke, acute chest syndrome, and organ damage from repeated sickling episodes.
- Gallstones due to chronic hemolysis.
- Avascular necrosis of the hip or shoulder joints.
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