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reference materials

Sickle Cell Anemia

key information

source: Family Practice Notebook

summary/abstract:

Sickle Cell Anemia:
II. Epidemiology
A. Sickle Cell Disease (Sickle Cell Anemia)
Prevalence (U.S.): 100,000 (1 per 365 black or african american descent)

B. Sickle Cell Trait (A/S) Incidence
1. Americans of African Descent: 1 in 12
2. Also Seen in Greeks, Italians, Turks, Saudi Arabians and hispanic patients

III. Pathophysiology

A. Normal Hemoglobin A replaced by Hemoglobin S (Hb S)
Deoxygenated Hemoglobin-S assumes a sickle shape

B. Substitution of Valine for glutamic acid
Occurs at the 6th position of the beta-chain

C. Mechanisms of dysfunction
1. Hypercoagulable
a. Round blood cells
b. Clotting Cascade activation
c. Inflammatory cascade activation
d. Platelet activation

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