Understanding Pathophysiology, 6e
Understanding Pathophysiology, 6e
6th Edition
ISBN: 9780323354097
Author: Sue E. Huether RN PhD, Kathryn L. McCance RN PhD
Publisher: Elsevier Science
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Chapter 22.1, Problem 1QC
Summary Introduction

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.

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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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