1. ANSWER A. Anemia is defined as a decreased number of erythrocytes (red blood cells). Leukopenia is a decreased number of leukocytes (white blood cells). Thrombocytopenia is a decreased number of platelets. Lastly, granulocytopenia is a decreased number of granulocytes (a type of white blood cells).
2. ANSWER C. Anemia stems from a decreased number of red blood cells and the resulting deficiency in oxygen and body tissues. Clotting factors, such as factor VIII, relate to the body’s ability to form blood clots and aren’t related to anemia, not is carbon dioxide of T antibodies.
3. ANSWER C. The precursor to the RBC is the stem cell. B cells, macrophages, and T cells and lymphocytes, not RBC precursors.
4. ANSWER A. Mild anemia usually has no clinical signs. Palpitations, SOB, and pallor are all associated with severe anemia.
5. ANSWER B. In aplastic anemia, the most likely diagnostic findings are decreased levels of all the cellular elements of the blood (pancytopenia). T-helper cell production doesn’t decrease in aplastic anemia. Reed-Sternberg cells and lymph node enlargement occur with Hodgkin’s disease.
6. ANSWER D. Preferably, ferrous gluconate should be taken on an empty stomach. Ferrous gluconae should not be taken with antacids, milk, or whole-grain cereals because these foods reduce iron absorption.
7. ANSWER C. Hemophilia A results from a deficiency of factor VIII. Sickle cell disease is caused by a defective hemoglobin molecule. Christmas disease, also called hemophilia B, results in a factor IX deficiency.
8. ANSWER C. Characteristic sickle cells tend to cause “log jams” in capillaries. This results in poor circulation to local tissues, leading to ischemia and necrosis. The basic defect in sickle cell disease is an abnormality in the structure of RBCs. The erythrocytes are sickle-shaped, rough in texture, and rigid. Sickle cell disease is an inherited disease, not an autoimmune reaction. Elevated serum bilirubin concentrations are associated with jaundice, not sickle cell disease.
9. ANSWER D. Sickle cell disease is an inherited disease that is present at birth. However, 60% to 80% of a newborns hemoglobin is fetal hemoglobin, which has a structure different from that of hemoglobin S or hemoglobin A. Sickle cell symptoms usually occur about 4 months after birth, when hemoglobin S begins to replace the fetal hemoglobin. The gene for sickle cell disease is transmitted at the time of conception, not passed through the placenta. Some hemoglobin S is produced by the fetus near term. The fetus produces all its own hemoglobin from the earliest production in the first trimester. Passive immunity conferred by maternal antibodies is not related to sickle cell disease, but this transmission of antibodies is important to protect the infant from various infections during early infancy.
10. ANSWER C. For the child in a sickle cell crisis, pain is the priority nursing diagnosis because the sickled cells clump and obstruct the blood vessels, leading to occlusive and subsequent tissue ischemia. Although ineffective coping may be important, it is not the priority. Decreased cardiac output is not a problem with this type of vasoocclusive crisis. Typically, a sickle cell crisis can be precipitated by a fluid volume deficit or dehydration.
11. ANSWER B. Children with iron-deficiency anemia are more susceptible to infection because of marked decreases in bone marrow functioning with microcytosis.
12. ANSWERS A, B. Toddlers should have between 2 and 3 cups of milk per day and 8 ounces of juice per day. If they have more than that, then they are probably not eating enough other foods, including iron-rich foods that have the needed nutrients.
13. ANSWER B. Potato, peas, chicken, green vegetables, and rice cereal contain significant amounts of iron and therefore would be recommended. Milk and yellow vegetables are not good iron sources. Rice by itself also is not a good source of iron.
14. ANSWER D. PTT measures the activity of thromboplastin, which is dependent on intrinsic clotting factors. In hemophilia, the intrinsic clotting factor VIII (antihemiphilic factor) is deficient, resulting in a prolonged PTT. Bleeding time reflects platelet function; the tourniquet test measures vasoconstriction and platelet function; and the clot retraction test measures capillary fragility. All of these are unaffected in people with hemophilia.
15. ANSWER A. Bleeding into the joints in the child with hemophilia leads to pain and tenderness, resulting in restricted movement. Therefore, an early sign of hemarthrosis would be the child’s reluctance to move a body part. If the bleeding into the joint continues, the area becomes hot, swollen, and immobile—not cool, pale, and clammy. Ecchymosis formation around a joint would be difficult to assess. Instability of a long bone on passive movement is not associated with joint hemarthrosis.
16. ANSWER A. Because factor VIII concentrate is derived from large pools of human plasma, the risk of hepatitis is always present. Clinical manifestations of hepatitis include yellowing of the skin, mucous membranes, and sclera. Use of factor VIII concentrate is not associated with constipation, abdominal distention, or puffiness around the eyes.
17. ANSWER C. A diagnosis is established based on a complete blood count, examination for sickled red blood cells in the peripheral smear, and hemoglobin electrophoresis. Laboratory studies will show decreased hemoglobin and hematocrit levels and a decreased platelet count, and increased reticulocyte count, and the presence of nucleated red blood cells. Increased reticulocyte counts occur in children with sickle cell disease because the life span of their sickled red blood cells is shortened.
18. ANSWER C. Pain crisis may be precipitated by infection, dehydration, hypoxia, trauma, or physical or emotional stress. The mother of a child with sickle cell disease should encourage fluid intake of 1 ½ to 2 times the daily requirement to prevent dehydration.
19. ANSWER D. The results of a CBC in children with iron deficiency anemia will show decreased hemoglobin levels and microcytic and hypochromic red blood cells. The red blood cell count is decreased. The reticulocyte count is usually normal or slightly elevated.
20. ANSWER D. Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers. Hemophilia is inherited in a recessive manner via a genetic defect on the X-chromosome. Hemophilia A results from a deficiency of factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX.