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Sickle Cell Disease Overview

Learn about the introduction, history, epidemiology, pathophysiology, laboratory features, clinical course, sickle cell crisis, and treatment modalities of sickle cell disease.

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@VivaciousVoice
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Questions and Answers

What is the characteristic shape of red blood cells in sickle cell disorders?

Sickle shape

What is the severe form of sickle cell disorder known as?

Sickle cell anaemia

Which type of sickle cell disorder is the heterozygous form of Hb S known as?

Sickle cell trait

Who provided the first written account of sickle cell disease in 1874?

<p>Africanus Norton</p> Signup and view all the answers

In which year was the first case of sickle cell disease reported by Drs James Herrick and Ernest Irons?

<p>1910</p> Signup and view all the answers

What is the state in which red cells exposed to deoxygenation polymerize and deform into a sickle shape called?

<p>Sickling state</p> Signup and view all the answers

What did Linus Pauling and colleagues demonstrate in 1949?

<p>Molecular differences between various types of hemoglobin</p> Signup and view all the answers

In what region is Sickle cell anaemia more prevalent?

<p>Sub-Saharan Africa</p> Signup and view all the answers

What did Dr. Alan Raper suggest in 1949?

<p>The survival advantage of the Sickle Cell trait</p> Signup and view all the answers

What is the estimated carrier rate of Sickle cell disease in Nigeria?

<p>1 in 4 persons</p> Signup and view all the answers

What is the mechanism proposed for the overt protection enjoyed in the carrier state of Sickle cell disease?

<p>Selective sickling of parasitized red cells resulting in more efficient removal by cells of the monocyte-macrophage system</p> Signup and view all the answers

What did Hahn and Gillespie show in 1927?

<p>Relationship between low oxygen tension and low pH</p> Signup and view all the answers

Where is Hb C most prevalent?

<p>Northern Ghana and Burkina Faso</p> Signup and view all the answers

What did Sydenstricker and Huck note in 1923?

<p>'Latent sicklers' among relatives of diagnosed patients</p> Signup and view all the answers

What was demonstrated by Hunt and Ingram in the late 1950s?

<p>The sequence of the globin peptide</p> Signup and view all the answers

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