To explain: The reason behind clinical manifestations of sickle cell anemia not appearing until the infant is at least 6 months old.
Concept introduction: Sickle cell disease belongs to the group of disorders which is characterized by the production of abnormal hemoglobin S (Hb S) due to the genetic mutation in which one amino acid is replaced by another. The Hb S or sickle hemoglobin dehydrates and deoxygenates by stretching and solidifying the erythrocyte into an elongated sickle shape.
Explanation of Solution
The clinical manifestations of sickle cell anemia does not appear until the infant is at least 6 months old. Hb F is the hemoglobin that is present in higher concentration during fetal development. As the concentration of Hb F decreases, the concentrations of hemoglobin S increases during post natal development. Sickle cell anemia is due to mutation of the gene that codes for Hb S and not Hb F. It takes six months after birth for the entire Hb F to be replaced with Hb S. Therefore, until Hb F is totally replaced by Hb S, the clinical manifestations of sickle cell anemia does not appear.
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Understanding Pathophysiology, 6e
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