Hematological Conditions of Childhood



Hematological Conditions of Childhood









IRON-DEFICIENCY ANEMIA

Anemia is the most common blood disorder in children. Anemia can be defined as a reduction in the total number of erythrocytes in the circulating blood or a reduction in the quality or quantity of hemoglobin. There are two classifications for anemia based on either morphology or etiology. Morphological classification is based on size and hemoglobin content. Etiological classification is based on the premise that anemia develops because of a decrease in the production of erythrocytes or an increase in the destruction of erythrocytes. Examples of anemia with a decreased production of red blood cells are iron-deficiency anemia and aplastic anemia. Examples of anemia with an increased destruction of red blood cells are sickle cell anemia and thalassemia.


Pathophysiology

Iron-deficiency anemia is most often caused by an inadequate dietary intake of iron. Newborns have an iron reserve that lasts for up to 12 weeks. As this iron store becomes depleted, the amount of iron in formula or foods must be increased. Iron-deficiency anemia may also be caused by blood loss. In the small child this could be due to gastrointestinal lesion, parasitic infestation, or hemorrhagic disease.

Iron is required to produce hemoglobin in the body that ultimately affects the production of red blood cells. Less oxygen reaches the cells and tissues, causing tissue hypoxia and related symptoms. Symptoms in mild anemia may not be detectable to the parent until moderate anemia presents itself. Symptoms such as general irritability, decreased activity tolerance, and weakness would be signs of moderate anemia. Pallor, anorexia, tachycardia, and murmurs would be signs of severe anemia. Long-term complications of cognitive impairment have been found in children and adolescents with long-standing iron-deficiency anemia.

Levels of anemia are as follows:












Mild iron-deficiency anemia


Hemoglobin 9.5-11 g/dL


Moderate anemia


Hemoglobin 8-9.4 g/dL


Severe anemia


Hemoglobin < 8 g/dL




Treatment

The treatment for iron-deficiency anemia is aimed at correcting the underlying problem and increasing depleted iron stores. Dietary supplementation can be recommended from iron-enriched formula to foods that are high in iron. Iron supplementation may also be given orally or parenterally. Vitamin C may be added to enhance absorption.


SICKLE CELL DISEASE

Hemoglobinopathy is the umbrella term for a number of inherited disorders of the red blood cell (erythrocytes) due to the abnormalities in the protein hemoglobin. Sickle cell disease (SCD), and the older term sickle cell anemia (Hb SS), is the most common hemoglobinopathy variant seen worldwide, is associated with severity of illness, and primarily affects people of African and southwest Asian decent. Sickle cell anemia is a homozygous condition resulting from inheriting a sickle cell gene (Hb S) from both parents. There are several hundred hemoglobin variants. Other common heterozygous hemoglobinopathy disorders include the Hb S gene interacting with Hb C, Hb D, and Hb E; α- and β-thalassemia (primarily affecting people of central and southeast Asian and Mediterranean decent), and other variant combinations. SCD is a genetic mutation of the hemoglobin β gene thought to have come about as a protection against malaria in people living in equatorial climates. The severity of the disease varies in individuals.


Sickle cell trait (Hb AS) is the heterozygous carrier state where one receives a normal hemoglobin gene (Hb A) from one parent and an abnormal variant gene from the other parent. Sickle cell trait may be very common in many of these ethnic groups, and although patients with sickle cell trait are relatively healthy, they may show signs of disease as they age or may experience symptoms under extraordinary conditions such as exhaustion from vigorous exercise, dehydration, exposed to high altitudes, or deep-sea diving pressures.




Pathophysiology

Fetal hemoglobin (Hb F) at birth declines after the first month of life and is replaced by normal adult hemoglobin (Hb A) during the first year in people unaffected by SCD. SCD (Hb SS) is an autosomal recessive disease where the genetic defect is a single point mutation of the sixth amino acid position on the β chain of the hemoglobin molecule, substituting valine for glutamic acid. Stressors on the body such as an infection, fever, emotional concerns, fatigue, and cold or hot weather result in red blood cell hemolysis (cell death) and, if persistent, anemia. Vasoocclusive crises or “sickling” or “sickle cell crisis” occur when the membrane of the red blood cells becomes hard, sticky, and sickle-shaped, causing clumping of the red blood cells in small vessels, blockage and interruption in blood flow, pain episodes, and progressive and irreversible cell and organ damage if not medically managed.

Hematological biochemical changes may be caused by deoxygenation of the blood, dehydration increasing intracellular hemoglobin concentrations, pH changes to acidosis, and fever. Inflammation in the endothelium of the vascular walls and overactivity of the clotting system producing fibrin clots occur during a vasoocclusive crisis, further impeding blood flow and causing tissue ischemia.

Complications from SCD and vasoocclusive events are varied and often associated with age and access to quality primary and emergency health care. Young children may present with

Oct 17, 2016 | Posted by in NURSING | Comments Off on Hematological Conditions of Childhood

Full access? Get Clinical Tree

Get Clinical Tree app for offline access