Chapter 30 - The Child with Hematologic or Immunologic Dysfunction Flashcards
An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. These manifestations are most suggestive of what complication?
a.
Air embolism
b.
Allergic reaction
c.
Hemolytic reaction
d.
Circulatory overload
ANS: D
The signs of circulatory overload include distended neck veins, hypertension, crackles, a dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema are signs and symptoms of allergic reactions. Hemolytic reactions are characterized by chills, shaking, fever, pain at infusion site, nausea, vomiting, tightness in chest, flank pain, red or black urine, and progressive signs of shock and renal failure.
What statement is descriptive of most cases of hemophilia?
a.
X-linked recessive deficiency of platelets causing prolonged bleeding
b.
X-linked recessive inherited disorder in which a blood clotting factor is deficient
c.
Autosomal dominant deficiency of a factor involved in the blood-clotting reaction
d.
Y-linked recessive inherited disorder in which the red blood cells become moon shaped
ANS: B
The inheritance pattern in 80% of all the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency (hemophilia A, or classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas disease). The disorder involves coagulation factors, not platelets. The disorder does not involve red blood cells or the Y chromosome.
The nurse is planning care for an adolescent with acquired immunodeficiency syndrome. What is the priority nursing goal?
a.
Prevent infection.
b.
Prevent secondary cancers.
c.
Identify source of infection.
d.
Restore immunologic defenses.
ANS: A
As a result of the immunocompromise that is associated with human immunodeficiency virus (HIV) infection, the prevention of infection is paramount. Although certain precautions are justified in limiting exposure to infection, these must be balanced with the concern for the child’s normal developmental needs. Preventing secondary cancers is not currently possible. Case finding is not a priority nursing goal in planning care for an individual. Current drug therapy is affecting the disease progression; although not a cure, these drugs can suppress viral replication, preventing further deterioration but not actually restoring immunologic defenses.
Nursing strategies to improve the growth and development of the child with human immunodeficiency virus (HIV) infection should include what?
a.
Provide only those foods that the child feels like eating.
b.
Fortify foods with nutritional supplements to maximize quality of intake.
c.
Weigh the child and measure height and muscle mass on a daily basis.
d.
Provide high-fat and high-calorie meals and snacks to meet body requirements for growth.
ANS: B
HIV infection often leads to marked failure to thrive and multiple nutritional deficiencies. Nutritional management may be difficult because of recurrent illness, diarrhea, and other physical problems. The nurse should implement intensive nutritional interventions if the child’s growth begins to slow or weight begins to decrease. Fortifying foods with nutritional supplements will maximize quality of intake. The child does not need to be weighed daily, and high-fat meals and snacks should not be encouraged.
The nurse is teaching a parent of an infant to limit the amount of formula to encourage the intake of iron-rich food. What amount should the nurse teach to the parent?
a.
500 ml
b.
750 ml
c.
1000 ml
d.
1250 ml
ANS: C
The nurse should teach the parent to limit the amount of formula to no more than 1 1/day to encourage intake of iron-rich solid foods.
The nurse is caring for a child with hemophilia A. The child’s activity is as tolerated. What activity is contraindicated for this child?
a.
Ambulating to the cafeteria
b.
Active range of motion
c.
Ambulating to the playroom
d.
Passive range of motion exercises
ANS: D
Passive range of motion exercises should never be part of an exercise regimen after an acute episode because the joint capsule could easily be stretched and bleeding could recur. Active range of motion exercises are best so that the patient can gauge his or her own pain tolerance. The child can ambulate to the playroom or the cafeteria.
The nurse is preparing a community outreach program about the prevention of iron-deficiency anemia in infants. What statement should the nurse include in the program?
a.
Whole milk can be introduced into the infant’s diet in small amounts at 6 months.
b.
Iron supplements cannot be given until the infant is older than 1 year of age.
c.
Iron-fortified cereal should be introduced to the infant at 2 months of age.
d.
Breast milk or iron-fortified formula should be used for the first 12 months.
ANS: D
Prevention, the primary goal in iron-deficiency anemia, is achieved through optimal nutrition and appropriate iron supplements. The American Academy of Pediatrics recommends feeding an infant only breast milk or iron-fortified formula for the first 12 months of life. Whole cow’s milk should not be introduced until after 12 months, iron supplements can be given during the first year of life, and iron-fortified cereals should not be introduced until the infant is 4 to 6 months old
What information should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations?
a.
Give with meals.
b.
Stop immediately if nausea and vomiting occur.
c.
Adequate dosage will turn the stools a tarry green color.
d.
Allow preparation to mix with saliva and bathe the teeth before swallowing.
ANS: C
The nurse should prepare the mother for the anticipated change in the child’s stools. If the iron dose is adequate, the stools will become a tarry green color. A lack of color change may indicate insufficient iron. The iron should be given in two divided doses between meals when the presence of free hydrochloric acid is greatest. Iron is absorbed best in an acidic environment. Vomiting and diarrhea may occur with iron administration. If these occur, the iron should be given with meals, and the dosage reduced and gradually increased as the child develops tolerance. Liquid preparations of iron stain the teeth; they should be administered through a straw and the mouth rinsed after administration.
In which condition are all the formed elements of the blood simultaneously depressed?
a.
Aplastic anemia
b.
Sickle cell anemia
c.
Thalassemia major
d.
Iron deficiency anemia
ANS: A
Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Sickle cell anemia is a hemoglobinopathy in which normal adult hemoglobin is partly or completely replaced by abnormal sickled hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin chains. Iron-deficiency anemia results in a decreased amount of circulating red cells.
What medication is classified as an antiretroviral?
a.
Dapsone (Aczone)
b.
Pentamidine (Pentam)
c.
Didanosine (Videx)
d.
Trimethoprim–sulfamethoxazole (Bactrim)
ANS: C
Classes of antiretroviral agents include nucleoside reverse transcriptase inhibitors (e.g., zidovudine, didanosine, stavudine, lamivudine, abacavir), nonnucleoside reverse transcriptase inhibitors (e.g., nevirapine, delavirdine, efavirenz), and protease inhibitors (e.g., indinavir, saquinavir, ritonavir, nelfinavir, amprenavir, lopinavir, ritonavir). Dapsone, pentamidine, and Bactrim are anti-infectives.
What condition precipitates polycythemia?
a.
Dehydration
b.
Severe infections
c.
Immunosuppression
d.
Prolonged tissue hypoxia
ANS: D
Oxygen transport depends on both the number of circulating RBCs and the amount of normal hemoglobin in the cell. This explains why polycythemia (increase in the number of erythrocytes) occurs in conditions characterized by prolonged tissue hypoxia, such as cyanotic heart defects. Dehydration, severe infections, or immunosuppression will not precipitate polycythemia.
A child with sickle cell disease is in a vasoocclusive crisis. What nonpharmacologic pain intervention should the nurse plan?
a.
Exercise as a distraction
b.
Heat to the affected area
c.
Elevation of the extremity
d.
Cold compresses to the affected area
ANS: B
Frequently, heat to the affected area is soothing. Cold compresses are not applied to the area because doing so enhances vasoconstriction and occlusion. Bed rest is usually well tolerated during a crisis, although the actual rest obtained depends a great deal on pain alleviation and the use of organized schedules of nursing care. Although the objective of bed rest is to minimize oxygen consumption, some activity, particularly passive range of motion exercises, is beneficial to promote circulation. Usually the best course is to let children determine their activity tolerance. Elevating the extremity will not help in sickle cell disease.
The nurse is administering a unit of blood to a child. What are signs and symptoms of a transfusion reaction? (Select all that apply.)
a.
Chills
b.
Shaking
c.
Flank pain
d.
Hypothermia
e.
Sudden severe headache
ANS: A, B, C, E
Signs and symptoms of a transfusion reaction include chills, shaking, flank pain, and sudden severe headache. Hyperthermia, not hypothermia, occurs.
The nurse is preparing to admit a 4-year-old child with chronic benign neutropenia. What clinical features of chronic benign neutropenia should the nurse recognize? (Select all that apply.)
a.
Gingivitis is present.
b.
Anemia is not present.
c.
Monocytosis is present.
d.
It has an autosomal recessive pattern.
e.
Treatment is by bone marrow transplantation.
ANS: A, B, C
The clinical features of chronic benign neutropenia include gingivitis, no anemia, and monocytosis. It is not inherited, and because it is benign, it does not require treatment except antibiotics as indicated.
The school nurse is informed that a child with human immunodeficiency virus (HIV) infection will be attending school soon. What is an important nursing intervention to include in the plan of care?
a.
Carefully follow universal precautions.
b.
Inform the parents of the other children.
c.
Determine how the child became infected.
d.
Reassure other children that they will not become infected.
ANS: A
Universal precautions are necessary to prevent further transmission of the disease. Informing the parents of the other children would violate the child’s right to privacy. It is not within the role of the school nurse to determine how the child became infected. Reassuring other children that they will not become infected violates the child’s privacy. General health classes can discuss prevention of HIV transmission.
In anticipation of the admission of a child with hereditary spherocytosis (HS) who is experiencing an aplastic crisis, what action should the nurse plan?
a.
Secure an isolation room.
b.
Prepare for a transfusion of packed red blood cells.
c.
Anticipate preoperative preparation for a splenectomy.
d.
Gather equipment and medication for treatment of shock.
ANS: B
In hereditary spherocytosis, aplastic crisis results in a sudden cessation of RBC production by the bone marrow. Hemoglobin and hematocrit values drop rapidly, which results in severe anemia. Transfusion support may be needed, and close monitoring of the child’s cardiovascular status is necessary. The nurse should prepare for a transfusion of packed red blood cells initially. An isolation room is not needed, splenectomy would not be done at this time, and the child will not be in shock.
The clinic nurse is evaluating causes for iron deficiency due to impaired iron absorption. What should the nurse recognize as causes for iron deficiency due to impaired iron absorption? (Select all that apply.)
a.
Gastric acidity
b.
Chronic diarrhea
c.
Lactose intolerance
d.
Absence of phosphates
e.
Inflammatory bowel disease
ANS: B, C, E
Causes for iron deficiency due to impaired iron absorption include chronic diarrhea, lactose intolerance, and inflammatory bowel disease. Gastric alkalinity, not acidity, and the presence, not absence, of phosphates can be causes of impaired iron absorption
For children who do not have a matched sibling bone marrow donor, the therapeutic management of aplastic anemia includes what intervention?
a.
Antibiotics
b.
Antiretroviral drugs
c.
Iron supplementation
d.
Immunosuppressive therapy
ANS: D
It is thought that aplastic anemia may be an autoimmune disease. Immunosuppressive therapy, including antilymphocyte globulin, antithymocyte globulin, cyclosporine, granulocyte colony-stimulating factor, and methylprednisone, has greatly improved the prognosis for patients with aplastic anemia. Antibiotics are not indicated as the management. They may be indicated for infections. Antiretroviral drugs and iron supplementation are not part of the therapy.
What physiologic defect is responsible for causing anemia?
a.
Increased blood viscosity
b.
Depressed hematopoietic system
c.
Presence of abnormal hemoglobin
d.
Decreased oxygen-carrying capacity of blood
ANS: D
Anemia is a condition in which the number of red blood cells or hemoglobin concentration is reduced below the normal values for age. This results in a decreased oxygen-carrying capacity of blood. Increased blood viscosity is usually a function of too many cells or of dehydration, not of anemia. A depressed hematopoietic system or abnormal hemoglobin can contribute to anemia, but the definition depends on the decreased oxygen-carrying capacity of the blood.
What explanation provides the rationale for why iron-deficiency anemia is common during infancy?
a.
Cow’s milk is a poor source of iron.
b.
Iron cannot be stored during fetal development.
c.
Fetal iron stores are depleted by 1 month of age.
d.
Dietary iron cannot be started until 12 months of age.
ANS: A
Children between the ages of 12 and 36 months are at risk for anemia because cow’s milk is a major component of their diet, and it is a poor source of iron. Iron is stored during fetal development, but the amount stored depends on maternal iron stores. Fetal iron stores are usually depleted by ages 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life.
The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor?
a.
Hemoglobin
b.
Tissue hypoxia
c.
Reticulocyte count
d.
Number of RBCs
ANS: B
Hemoglobin does not directly control RBC production. If there is insufficient hemoglobin to adequately oxygenate the tissue, then erythropoietin may be released. When tissue hypoxia occurs, the kidneys release erythropoietin into the bloodstream. This stimulates the marrow to produce new RBCs. Reticulocytes are immature RBCs. The “retic” count can be used to monitor hematopoiesis. The number of RBCs does not directly control production. In congenital cardiac disorders with mixed blood flow or decreased pulmonary blood flow, RBC production continues secondary to tissue hypoxia
What immunoglobulin pattern does the nurse expect in a child recently diagnosed with Wiskott-Aldrich syndrome?
a.
Diminished levels of IgG
b.
Diminished levels of IgA
c.
Diminished levels of IgM
d.
Diminished levels of IgE
ANS: C
The level of IgM is diminished early in the course of the disease, but levels of IgG, IgA, and IgE may be elevated initially.
