NUR 212 Test 2

A nurse obtains health histories when admitting clients to a medical-surgical unit. With which client should the nurse discuss predisposition genetic testing? a. Middle-aged woman whose mother died at age 48 of breast cancer b. Young man who has all the symptoms of rheumatoid arthritis c. Pregnant woman whose father has sickle cell disease d. Middle-aged man of Eastern European Jewish ancestry

ANS: A A client with a family history of breast cancer should be provided information about predisposition testing. Predisposition testing should be discussed with clients who are at high risk of hereditary breast, ovarian, and colorectal cancers so that the client can engage in heightened screening activities or interventions that reduce risk. The client with symptoms of rheumatoid arthritis should be given information about symptomatic diagnostic testing. The client with a familial history of sickle cell disease and the client who is of Eastern European Jewish ancestry should be given information about carrier genetic testing.

A newborn has been diagnosed with congenital adrenal hyperplasia. Which assessment finding should the nurse expect? a. Ambiguous genitalia b. Prenatal growth retardation c. An abnormally large tongue d. Legs and arms significantly shorter than torso

ANS: A A newborn diagnosed with congenital adrenal hyperplasia can have ambiguous genitalia or virilization of female external genitalia caused by elevated androgen levels. Prenatal growth retardation is present with Bloom syndrome. An abnormally large tongue is seen with Beckwith-Wiedemann syndrome. Legs and arms significantly shorter than torso are seen with achondroplasia.

A 5-year-old child is admitted to the hospital in a sickle cell crisis. The child has been alert and oriented but in severe pain. The nurse notes that the child is complaining of a headache and is having unilateral hemiplegia. What action should the nurse implement? a. Notify the health care provider. b. Place the child on bed rest. c. Administer a dose of hydrocodone (Vicodin). d. Start O2 per the hospital's protocol.

ANS: A Any number of neurologic symptoms can indicate a minor cerebral insult, such as headache, aphasia, weakness, convulsions, visual disturbances, or unilateral hemiplegia. Loss of vision is usually the result of progressive retinopathy and retinal detachment. The nurse should notify the health care provider.

19. 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.

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.

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.

A couple has given birth to their first child, a boy with a recessive disorder. The genetic counselor tells them that the risk of recurrence is one in four. Which statement is a correct interpretation of this information? a. The risk factor remains the same for each pregnancy. b. The risk factor will change when they have a second child. c. Because the parents have one affected child, the next three children should be unaffected. d. Because the parents have one affected child, the next child is four times more likely to be affected.

ANS: A Each pregnancy has the same risks for an affected child. Because an odds ratio reflects the risk, this does not change over time. The statement by the genetic counselor refers to a probability. This does not change over time. The statement "Because the parents have one affected child, the next child is four times more likely to be affected" does not reflect autosomal recessive inheritance.

12. The inheritance of which is X-linked recessive? a. Hemophilia A b. Marfan syndrome c. Neurofibromatosis d. Fragile X syndrome

ANS: A Hemophilia A is inherited as an X-linked recessive trait. Marfan syndrome and neurofibromatosis are inherited as autosomal dominant disorders. Fragile X is inherited as an X-linked trait.

What pain medication is contraindicated in children with sickle cell disease (SCD)? a. Meperidine (Demerol) b. Hydrocodone (Vicodin) c. Morphine sulfate d. Ketorolac (Toradol)

ANS: A Meperidine (pethidine [Demerol]) is not recommended. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with SCD are particularly at risk for normeperidine-induced seizures.

What rationale explains why prolonged use of oxygen should be discouraged in a child with anemia? a. Prolonged use of oxygen can decrease erythropoiesis. b. Prolonged use of oxygen can interfere with iron production. c. Prolonged use of oxygen interferes with a child's appetite. d. Prolonged use of oxygen can affect the synthesis of hemoglobin.

ANS: A Oxygen administration is of limited value, because each gram of hemoglobin is able to carry a limited amount of the gas. In addition, prolonged use of supplemental oxygen can decrease erythropoiesis. Prolonged use of oxygen does not interfere with iron production, a child's appetite, or affect the synthesis of hemoglobin.

Parents ask the nurse about the characteristics of autosomal recessive inheritance. Which is characteristic of autosomal recessive inheritance? a. Affected individuals have unaffected parents. b. Affected individuals have one affected parent. c. Affected parents have a 50% chance of having an affected child. d. Affected parents will have unaffected children.

ANS: A Parents who are carriers of a recessive gene are asymptomatic. For a child to be affected, both parents must have a copy of the gene, which is passed to the child. Both parents are asymptomatic but can have affected children. In autosomal recessive inheritance, there is a 25% chance that each pregnancy will result in an affected child. In autosomal dominant inheritance, affected parents can have unaffected children.

Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention? a. Perform a splenectomy. b. Supplement the diet with calcium. c. Institute a maintenance transfusion program. d. Increase intake of iron-rich foods such as meat.

ANS: A Splenectomy corrects the hemolysis that occurs in HS. The splenectomy is generally reserved for children older than age 5 years with symptomatic anemia. Supplementation with calcium does not affect the HS. Additional folic acid can prevent deficiency caused by the rapid cell turnover. A maintenance transfusion program suppresses red blood cell formation. At this time, the risks of transfusion are greater than those of a splenectomy. Iron supplementation does not influence the course of HS.

Which is a birth defect or disorder that occurs as a new case in a family and is not inherited? a. Sporadic b. Polygenic c. Monosomy d. Association

ANS: A Sporadic describes a birth defect previously unidentified in a family. It is not inherited. Polygenic inheritance involves the inheritance of many genes at separate loci whose combined effects produce a given phenotype. Monosomy is an abnormal number of chromosomes whereby the chromosome is represented by a single copy in a somatic cell. A nonrandom cluster of malformations without a specific cause is an association.

A couple expecting their first child has a positive family history for several congenital defects and disorders. The couple tells the nurse that they are opposed to abortion for religious reasons. Which should the nurse consider when counseling the couple? a. The couple should be encouraged to have recommended diagnostic testing. b. The couple needs counseling regarding advantages and disadvantages of pregnancy termination. c. Diagnostic testing is required by law in this situation. d. Diagnostic testing is of limited value if termination of pregnancy is not an option.

ANS: A The benefits of prenatal diagnostic testing extend beyond decisions concerning abortion. If the child has congenital disorders, decisions can be made about fetal surgery if indicated. In addition, if the child is expected to require neonatal intensive care at birth, the mother is encouraged to deliver at a level III neonatal center. The couple is counseled about the advantages and disadvantages of prenatal diagnosis, not pregnancy termination, although the family cannot be forced to have prenatal testing. The information gives the parents time to grieve and plan for their child if congenital disorders are present. If the child is free of defects, then the parents are relieved of a major worry.

A child with hemophilia A is scheduled for surgery. What precautions should the nurse institute with this child? a. Handle the child gently when transferring to a cart. b. Caution the child not to brush his teeth before surgery. c. Use tape sparingly on postoperative dressings. d. Do not administer analgesics before surgery.

ANS: A The goal of prevention of bleeding episodes is directed toward decreasing the risk of injury. The child should be handled carefully when transferring to a cart. Brushing teeth, use of tape, and giving analgesics will not risk a bleeding episode.

A breastfed infant has just been diagnosed with galactosemia. The therapeutic management of this includes which? a. Stop breastfeeding the infant. b. Add amino acids to breast milk. c. Substitute a lactose-containing formula for breast milk. d. Give the appropriate enzyme along with breast milk.

ANS: A The infant with galactosemia is fed a diet free of all milk and lactose-containing foods. This includes breast milk. Soy-protein formula is the formula of choice. Other strategies are being identified.

A school-age child is admitted in vasoocclusive sickle cell crisis (pain episode). The child's care should include which therapeutic interventions? a. Hydration and pain management b. Oxygenation and factor VIII replacement c. Electrolyte replacement and administration of heparin d. Correction of alkalosis and reduction of energy expenditure

ANS: A The management of crises includes adequate hydration, pain management, minimization of energy expenditures, electrolyte replacement, and blood component therapy if indicated. Factor VIII is not indicated in the treatment of vasoocclusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels. Also, prolonged oxygen can reduce bone marrow activity. Heparin is not indicated in the treatment of vasoocclusive sickle cell crisis. Electrolyte replacement should accompany hydration. The acidosis will be corrected as the crisis is treated. Energy expenditure should be minimized to improve oxygen utilization. Acidosis, not alkalosis, results from hypoxia, which also promotes sickling.

A toddler is diagnosed with chronic benign neutropenia. The parents are being taught about caring for their child. What information is important to include? a. Avoid large indoor crowds and people who are ill. b. Parenteral antibiotics are necessary to control disease. c. Frequent rest periods are needed during the daytime. d. List the side effects of corticosteroids used to decrease inflammation.

ANS: A The parents are taught to minimize risk of infection by avoiding crowded areas and individuals who are ill. Parents are also cautioned about when to notify their practitioner and administration of granulocyte colony-stimulating factor, if indicated. Antibiotics are not needed unless the child has an infection. The toddler does not need any additional rest as a result of the neutropenia. Corticosteroids are not indicated.

Turner syndrome is suspected in an adolescent girl with short stature. What causes this? a. Absence of one of the X chromosomes b. Presence of an incomplete Y chromosome c. Precocious puberty in an otherwise healthy child d. Excess production of both androgens and estrogens

ANS: A Turner syndrome is caused by an absence of one of the X chromosomes. Most girls who have this disorder have one X chromosome missing from all cells. No Y chromosome is present in individuals with Turner syndrome. These young women have 45 rather than 46 chromosomes.

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.

9. 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 nurse is caring for a 12-year-old child with b-thalassemia. What clinical manifestations should the nurse expect to observe? (Select all that apply.) a. Anorexia b. Unexplained fever c. Enlarged spleen or liver d. Bronzed, freckled complexion e. Precocious sexual development

ANS: A, B, C, D The clinical manifestations of b-thalassemia include anorexia; unexplained fever; an enlarged spleen or liver; and a bronzed, freckled complexion. There is delayed sexual maturation, not precocious.

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.

3. What are signs and symptoms of anemia? (Select all that apply.) a. Pallor b. Fatigue c. Dilute urine d. Bradycardia e. Muscle weakness

ANS: A, B, E Signs and symptoms of anemia include, pallor, fatigue, and muscle weakness. Tachycardia, not bradycardia, and dark urine, not dilute, are signs and symptoms of anemia.

The clinic nurse is evaluating causes for iron deficiency caused by inadequate supply of iron. What should the nurse recognize as causes for iron deficiency caused by an inadequate iron supply? (Select all that apply.) a. Prematurity b. Slow growth rate c. Excessive milk intake d. Severe iron deficiency in the mother e. Exclusive breastfeeding of infant from birth to 3 months

ANS: A, C, D Causes for iron deficiency caused by an inadequate supply of iron include prematurity, excessive milk intake, and severe iron deficiency in the mother. Rapid growth rate, not slow, and exclusive breastfeeding of infant after 6 months, not from birth to 3 months, can be causes of inadequate supply of iron.

The nurse is teaching nursing students about assessment clues to genetic disorders in the newborn. Which should the nurse include in the teaching session? (Select all that apply.) a. Low-set ears b. Mongolian spots c. Epicanthal folds d. Cephalohematoma e. Forehead prominence

ANS: A, C, E Assessment clues to genetic disorders in the newborn include low-set ears, epicanthal folds, and forehead prominence. Mongolian spots and cephalohematoma are findings in a newborn that are not indicative of a genetic disorder.

Which are signs and symptoms the nurse should assess in the newborn that can indicate an inborn error of metabolism? (Select all that apply.) a. Jaundice b. Strabismus c. Poor feeding d. Acrocyanosis e. Metabolic acidosis

ANS: A, C, E Signs of inborn errors of metabolism include jaundice, poor feeding, and metabolic acidosis. Strabismus and acrocyanosis are normal findings in the newborn.

2. What activity should the school nurse recommend for a child with hemophilia A? (Select all that apply.) a. Golf b. Soccer c. Rugby d. Jogging e. Swimming

ANS: A, D, E Children and adolescents with severe hemophilia can participate in noncontact sports such as swimming, golf, walking, jogging, fishing, and bowling. Contact sports such as football, boxing, hockey, soccer, and rugby are strongly discouraged because the risk of injury outweighs the physical and psychosocial benefits of participating in these sports.

1. The nurse is caring for a 14-year-old child with disseminated intravascular coagulation (DIC). What clinical manifestations should the nurse expect to observe? (Select all that apply.) a. Petechiae b. Chronic diarrhea c. Hepatosplenomegaly d. Bleeding from openings in the skin e. Hypotension f. Purpura

ANS: A, D, E, F Some clinical manifestations of DIC are petechiae, bleeding from openings in the skin, hypotension, and purpura. Hepatosplenomegaly and chronic diarrhea are clinical manifestations of human immunodeficiency virus (HIV) infection in children.

A young child with human immunodeficiency virus (HIV) is receiving several antiretroviral drugs. What is the purpose of these drugs? a. Cure the disease. b. Delay disease progression. c. Prevent spread of infection. d. Treat Pneumocystis carinii pneumonia.

ANS: B Although not a cure, these antiretroviral drugs can suppress viral replication, preventing further deterioration of the immune system, and delay disease progression. At this time, cure is not possible. Antiretroviral drugs do not prevent the spread of the disease. P. carinii prophylaxis is accomplished with antibiotics.

Which genetic term refers to a person who possesses one copy of an affected gene and one copy of an unaffected gene and is clinically unaffected? a. Allele b. Carrier c. Pedigree d. Multifactorial

ANS: B An individual who is a carrier is asymptomatic but possesses a genetic alteration, either in the form of a gene or chromosome change. Alleles are alternative expressions of genes at a different locus. A pedigree is a diagram that describes family relationships, gender, disease, status, or other relevant information about a family. Multifactorial describes a complex interaction of both genetic and environmental factors that produce an effect on the individual.

Care for the child with acute idiopathic thrombocytopenic purpura (ITP) includes which therapeutic intervention? a. Splenectomy b. Intravenous administration of anti-D antibody c. Use of nonsteroidal anti-inflammatory drugs (NSAIDs) d. Helping child participate in sports

ANS: B Anti-D antibody causes an increase in platelet count approximately 48 hours after administration. Splenectomy is reserved for chronic severe ITP not responsive to pharmacologic management. NSAIDs are not used in ITP. Both NSAIDs and aspirin interfere with platelet aggregation. The nurse works with the child and parents to choose quiet activities while the platelet count is below 100,000/mm3.

The nurse is assessing a neonate who was born 1 hour ago to healthy white parents in their early forties. Which finding should be most suggestive of Down syndrome? a. Hypertonia b. Low-set ears c. Micrognathia d. Long, thin fingers and toes

ANS: B Children with Down syndrome have low-set ears. Infants with Down syndrome have hypotonia, not hypertonia. Micrognathia is common in trisomy 16, not Down syndrome. Children with Down syndrome have short hands with broad fingers.

18. Iron overload is a side effect of chronic transfusion therapy. What treatment assists in minimizing this complication? a. Magnetic therapy b. Infusion of deferoxamine c. Hemoglobin electrophoresis d. Washing red blood cells (RBCs) to reduce iron

ANS: B Deferoxamine infusions in combination with vitamin C allow the iron to remain in a more chelatable form. The iron can then be excreted more easily. Use of magnets does not remove additional iron from the body. Hemoglobin electrophoresis is used to confirm the diagnosis of hemoglobinopathies; it does not affect iron overload. Washed RBCs remove white blood cells and other proteins from the unit of blood; they do not affect the iron concentration.

A hospitalized school-age child with phenylketonuria (PKU) is choosing foods from the hospital's menu. Which food choice should the nurse discourage the child from choosing? a. Banana b. Milkshake c. Fruit juice d. Corn on the cob

ANS: B Foods with low phenylalanine levels (e.g., some vegetables [except legumes]; fruits; juices; and some cereals, breads, and starches) must be measured to provide the prescribed amount of phenylalanine. Most high-protein foods, such as meat and dairy products, are either eliminated or restricted to small amounts.

47. 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.

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.

A child has been found to have a deficiency in 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase. Which condition is this child at risk for? a. Increased uric acid b. Hypercholesterolemia c. Increased phenylketones d. Altered oxygen transport

ANS: B HMG-CoA leads to a disruption of metabolic feedback mechanism and accumulation of end product (cholesterol) with the resulting condition of hypercholesterolemia.

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.

Which is characteristic of X-linked recessive inheritance? a. There are no carriers. b. Affected individuals are principally males. c. Affected individuals are principally females. d. Affected individuals will always have affected parents.

ANS: B In X-linked recessive disorders, the affected individuals are usually male. With recessive traits, usually two copies of the gene are needed to produce the effect. Because the male only has one X chromosome, the effect is visible with only one copy of the gene. Females are usually only carriers of X-linked recessive disorders. The X chromosome that does not have the recessive gene will produce the "normal" protein, so the woman will not show evidence of the disorder. The transmission is from mother to son. Usually the mother and father are unaffected.

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 lab results for a child. What recorded hematocrit (Hct) result is considered within the normal range? a. 30% b. 40% c. 50% d. 60%

ANS: B Normal hematocrit (Hct) is 35% to 45%.

The nurse is caring for a school-age child with severe anemia and activity intolerance. What diversional activity should the nurse plan for this child? a. Playing a musical instrument b. Playing board or card games c. Participating in a game of table tennis d. Participating in decorating the hospital room

ANS: B Plan diversional activities that promote rest but prevent boredom and withdrawal. Because short attention span, irritability, and restlessness are common in anemia and increase stress demands on the body, plan appropriate activities such as playing board or card games. Playing a musical instrument, participating in a game of table tennis, or decorating the hospital room would cause undue exertion.

A child with Prader-Willi syndrome has been hospitalized. Which assessment findings does the nurse expect with this syndrome? a. Nonverbal b. Insatiable hunger c. Abnormal, puppetlike gait d. Paroxysms of inappropriate laughter

ANS: B Prader-Willi syndrome is characterized by insatiable hunger that can lead to morbid obesity in childhood. Abnormal, puppetlike gait, paroxysms of inappropriate laughter, and nonverbal are characteristics seen in Angelman syndrome.

What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

ANS: B Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron-deficiency anemia affects red blood cell size and depth of color but does not involve abnormal hemoglobin.

21. 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.

A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse? a. Administer 100% oxygen to relieve hypoxia. b. Notify the practitioner because chest syndrome is suspected. c. Infuse intravenous antibiotics as soon as cultures are obtained. d. Give ordered pain medication to relieve symptoms of pain episode.

ANS: B These are the symptoms of chest syndrome, which is a medical emergency. Notifying the practitioner is the priority action. Oxygen may be indicated; however, it does not reverse the sickling that has occurred. Antibiotics are not indicated initially. Pain medications may be required, but evaluation by the practitioner is the priority.

Which abnormality is a common sex chromosome defect? a. Down syndrome b. Turner syndrome c. Marfan syndrome d. Hemophilia

ANS: B Turner syndrome is caused by an absence of one of the X chromosomes. Down syndrome is caused by trisomy 21 (three copies rather than two copies of chromosome 21). Marfan syndrome is a connective tissue disorder inherited in an autosomal dominant pattern. Hemophilia is a disorder of blood coagulation inherited in an X-linked recessive pattern.

15. A woman, age 43 years, is 6 weeks pregnant. It is important that she be informed of which? a. The need for a therapeutic abortion b. Increased risk for Down syndrome c. Increased risk for Turner syndrome d. The need for an immediate amniocentesis

ANS: B Women who are older than age 35 years at the birth of a single child or 31 years at the birth of twins are advised to have prenatal diagnosis. The risk of having a child with Down syndrome increases with maternal age. There is no indication of a need for a therapeutic abortion at this stage. Turner syndrome is not associated with advanced maternal age. Amniocentesis cannot be done at a gestational age of 6 weeks.

The nurse is preparing to admit a 1-month-old infant with severe congenital neutropenia (Kostmann disease). What clinical features of severe congenital neutropenia should the nurse recognize? (Select all that apply.) a. Anemia is present. b. Neutropenia is present. c. The illness is severe. d. It has a dominant inheritance pattern. e. There are decreased eosinophils in the bone marrow.

ANS: B, C The clinical features of severe congenital neutropenia include anemia and neutropenia, and the illness is severe. It has an autosomal recessive inheritance pattern, and there are increased, not decreased, eosinophils in the bone marrow.

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.

The nurse is reviewing the characteristics of autosomal dominant inheritance. Which are true about these characteristics? (Select all that apply.) a. A carrier state exists. b. The phenotype appears in consecutive generations. c. Males and females are equally likely to be affected. d. Parents who have affected children are usually asymptomatic. e. Children of an affected parent have a 50% chance of being affected.

ANS: B, C, E Characteristics of autosomal dominant inheritance include the phenotype appears in consecutive generations, males and females are equally affected, and children of an affected parent have a 50% chance of being affected. A carrier state and parents who have affected children are usually asymptomatic are characteristic of autosomal recessive inheritance.

The nurse is reviewing the characteristics of autosomal recessive inheritance. Which are true about these characteristics? (Select all that apply.) a. Most affected persons are males. b. Males and females are equally affected. c. All daughters of an affected male are carriers. d. Carrier parents have a 25% chance of producing an affected child. e. Carrier parents have a 50% chance of producing a carrier child in each pregnancy.

ANS: B, D, E Characteristics of autosomal recessive inheritance include males and females are equally affected, carrier parents have a 25% chance of producing an affected child, and carrier parents have a 50% chance of producing a carrier child in each pregnancy. Most affected persons who are males and all daughters of an affected male are carriers are characteristics of X-linked recessive inheritance.

The nurse is interviewing a prenatal client about specific risk factors that are indications for prenatal testing. Which specific risk factors should the nurse note? (Select all that apply.) a. Previous twins b. Inherited disorder c. Previous preterm birth d. Cytomegalovirus infection e. Previous stillbirth or neonatal death

ANS: B, D, E Specific risk factors that are indications for prenatal testing include inherited disorder, cytomegalovirus infection (teratogenic infection), and previous stillbirth or neonatal death. Previous twins or previous preterm birth are not specific risk factors that are indications for prenatal testing.

Which can be directly attributed to a single-gene disorder? (Select all that apply.) a. Cleft lip b. Cystic fibrosis c. Turner syndrome d. Klinefelter syndrome e. Neurofibromatosis

ANS: B, E Cystic fibrosis is a single-gene disorder inherited as an autosomal recessive trait, and neurofibromatosis is a single-gene disorder inherited as an autosomal dominant trait. Cleft lip is classified as a multifactorial disorder in which a genetic susceptibility and appropriate environment appear to play important roles. Turner and Klinefelter syndromes are disorders of sex chromosome number.

The nurse understands that which occurring soon after birth can indicate cystic fibrosis? a. Murmur b. Hypoglycemia c. Meconium ileus d. Muscle weakness

ANS: C A symptom of cystic fibrosis is a meconium ileus soon after birth. A murmur can be a sign of a congenital heart disease. Hypoglycemia can be a sign of Beckwith-Wiedemann syndrome. Muscle weakness can be a sign of myotonic dystrophy.

13. Chromosome analysis of the fetus is usually accomplished through the testing of which? a. Fetal serum b. Maternal urine c. Amniotic fluid d. Maternal serum

ANS: C Amniocentesis is the most common method to retrieve fetal cells for chromosome analysis. Viable fetal cells are sloughed off into the amniotic fluid, and when a sample is taken, they can be cultured and analyzed. It is difficult to obtain a sample of the fetal blood. It is a high-risk situation for the fetus. Fetal cells are not present in the maternal urine or blood.

The nurse is teaching student nurses about newborn screening. Which statement made by the student indicates understanding of the teaching? a. "The newborn screening is not mandatory but voluntary." b. "It is acceptable to 'layer' the blood on the Guthrie paper." c. "The initial specimen should be collected as close to discharge as possible." d. "It is best to collect the specimen before the newborn takes the first feeding."

ANS: C Because of early discharge of newborns, recommendations for screening include collecting the initial specimen as close as possible to discharge. Newborn screening tests are mandatory in all 50 U.S. states. When collecting the specimen, avoid "layering" the blood specimen on the special Guthrie paper. Layering is placing one drop of blood on top of the other or overlapping the specimen. Best results are obtained by collecting the specimen with a pipette from the heel stick and spreading the blood uniformly over the blot paper. The screening test is most reliable if the blood sample is taken after the infant has ingested a source of protein.

A nurse is educating a client about genetic screening. The client asks why red-green color blindness, an X-linked recessive disorder noted in some of her family members, is expressed more frequently in males than females. How should the nurse respond? a. "Females have a decreased penetrance rate for this gene mutation and are therefore less likely to express the trait." b. "Females have two X chromosomes and one is always inactive. This inactivity decreases the effect of the gene." c. "The incidence of X-linked recessive disorders is higher in males because they do not have a second X chromosome to balance expression of the gene." d. "Males have only one X chromosome, which allows the X-linked recessive disorder to be transmitted from father to son."

ANS: C Because the number of X chromosomes in males and females is not the same (1:2), the number of X-linked chromosome genes in the two genders is also unequal. Males have only one X chromosome, a condition called hemizygosity, for any gene on the X chromosome. As a result, X-linked recessive genes have a dominant expressive pattern of inheritance in males and a recessive expressive pattern of inheritance in females. This difference in expression occurs because males do not have a second X chromosome to balance the expression of any recessive gene on the first X chromosome. It is incorrect to say that one X chromosome of a pair is always inactive in females, or that females have a decreased penetrance rate for this gene mutation. X-linked recessive disorders cannot be transmitted from father to son, but the trait is transmitted from father to all daughters who will be carriers.

In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxia-ischemia cycle. What information should the nurse share with parents in a teaching plan? a. Encourage drinking. b. Keep accurate records of output. c. Check for moist mucous membranes. d. Monitor the concentration of the child's urine.

ANS: C Children with SCA have impaired kidney function and cannot concentrate urine. Parents are taught signs of dehydration and ways to minimize loss of fluid to the environment. Encouraging drinking is not specific enough for parents. The nurse should give the parents and child a target fluid amount for each 24-hour period. Accurate monitoring of output may not reflect the child's fluid needs. Without the ability to concentrate urine, the child needs additional intake to compensate. Dilute urine and specific gravity are not valid signs of hydration status in children with SCA.

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 is an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets? a. Aplastic anemia b. Thalassemia major c. Idiopathic thrombocytopenic purpura d. Disseminated intravascular coagulation

ANS: C Idiopathic thrombocytopenic purpura is an acquired hemorrhagic disorder characterized by an excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and normal bone marrow. Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasma.

Parents ask the nurse about the characteristics of autosomal dominant inheritance. Which statement is characteristic of autosomal dominant inheritance? a. Females are affected with greater frequency than males. b. Unaffected children of affected individuals will have affected children. c. Each child of a heterozygous affected parent has a 50% chance of being affected. d. Any child of two unaffected heterozygous parents has a 25% chance of being affected.

ANS: C In autosomal dominant inheritance, only one copy of the mutant gene is necessary to cause the disorder. When a parent is affected, there is a 50% chance that the chromosome with the gene for the disorder will be contributed to each pregnancy. Males and females are equally affected. The disorder does not "skip" a generation. If the child is not affected, then most likely he or she is not a carrier of the gene for the disorder. In autosomal recessive inheritance, any child of two unaffected heterozygous parents has a 25% chance of being affected.

What statement best describes b-thalassemia major (Cooley anemia)? a. It is an acquired hemolytic anemia. b. Inadequate numbers of red blood cells (RBCs) are present. c. Increased incidence occurs in families of Mediterranean extraction. d. It commonly occurs in individuals from West Africa.

ANS: C Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia. Thalassemia is inherited as an autosomal recessive disorder. An overproduction of RBCs occurs. Although numerous, the red blood cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.

Which is a sex chromosome abnormality that is caused by the presence of one or more additional X chromosomes in a male? a. Turner b. Triple X c. Klinefelter d. Trisomy 13

ANS: C Klinefelter syndrome is characterized by one or more additional X chromosomes. These individuals are tall with male secondary sexual characteristics that may be deficient, and they may be learning disabled. An absence of an X chromosome results in Turner syndrome. Triple X and trisomy 13 are not abnormalities that involve one or more additional X chromosomes in a male (Klinefelter syndrome).

The nurse is teaching the family of a child, age 8 years, with moderate hemophilia about home care. What should the nurse tell the family to do to minimize joint injury? a. Administer nonsteroidal anti-inflammatory drugs (NSAIDs). b. Administer DDAVP (synthetic vasopressin). c. Provide intravenous (IV) infusion of factor VIII concentrates. d. Encourage elevation and application of ice to the involved joint.

ANS: C Parents are taught home infusion of factor VIII concentrate. For moderate and severe hemophilia, prompt IV administration is essential to prevent joint injury. NSAIDs are effective for pain relief. They must be given with caution because they inhibit platelet aggregation. A factor VIII level of 30% is necessary to stop bleeding. DDAVP can raise the factor VIII level fourfold. Moderate hemophilia is defined by a factor VIII activity of 4.9. A fourfold increase would not meet the 30% level. Ice and elevation are important adjunctive therapy, but factor VIII is necessary.

Phenylketonuria is a genetic disease that results in the body's inability to correctly metabolize which? a. Glucose b. Thyroxine c. Phenylalanine d. Phenylketones

ANS: C Phenylketonuria is an inborn error of metabolism caused by a deficiency or absence of the enzyme needed to metabolize the essential amino acid phenylalanine. Individuals with this disorder can metabolize glucose. Thyroxine is one of the principal hormones secreted by the thyroid gland. Phenylketones are metabolites of phenylalanine excreted in the urine.

The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA? a. SCA is not inherited. b. All siblings will have SCA. c. Each sibling has a 25% chance of having SCA. d. There is a 50% chance of siblings having SCA.

ANS: C SCA is inherited as an autosomal recessive disorder. In this inheritance pattern, each child born to these parents has a 25% chance of having the disorder, a 25% chance of having neither SCA nor the trait, and a 50% chance of being heterozygous for SCA (sickle cell trait). SCA is an inherited hemoglobinopathy.

Which ethnic group is at risk for Tay-Sachs disease? a. Black African b. Mediterranean c. Ashkenazi Jewish d. Southern and Southeast Asian

ANS: C The Ashkenazi Jewish ethnic group is at higher risk for Tay-Sachs disease. The black African, Mediterranean, and Southern and Southeast Asian ethnicities are at higher risk for sickle cell anemia disease.

A mother states that she brought her child to the clinic because the 3-year-old girl was not keeping up with her siblings. During physical assessment, the nurse notes that the child has pale skin and conjunctiva and has muscle weakness. The hemoglobin on admission is 6.4 g/dl. After notifying the practitioner of the results, what nursing priority intervention should occur next? a.Reduce environmental stimulation to prevent seizures. b.Have the laboratory repeat the analysis with a new specimen. c.Minimize energy expenditure to decrease cardiac workload. d.Administer intravenous fluids to correct the dehydration.

ANS: C The child has a critically low hemoglobin value. The expected range is 11.5 to 15.5 g/dl. When the oxygen-carrying capacity of the blood decreases slowly, the child is able to compensate by increasing cardiac output. With the increasing workload of the heart, additional stress can lead to cardiac failure. Reduction of environmental stimulation can help minimize energy expenditure, but seizures are not a risk. A repeat hemoglobin analysis is not necessary. The child does not have evidence of dehydration. If intravenous fluids are given, they can further dilute the circulating blood volume and increase the strain on the heart.

The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration? a. Decreased blood viscosity b. Deficiency in coagulation c. Increased red blood cell (RBC) destruction d. Greater affinity for oxygen

ANS: C The clinical features of SCA are primarily the result of increased RBC destruction and obstruction caused by the sickle-shaped RBCs. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. SCA does not have a coagulation deficit. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension.

A child with severe anemia requires a unit of red blood cells (RBCs). The nurse explains to the child that the transfusion is necessary for which reason? a.Allow her parents to come visit her. b.Fight the infection that she now has. c.Increase her energy so she will not be so tired. d.Help her body stop bleeding by forming a clot (scab).

ANS: C The indication for RBC transfusion is risk of cardiac decompensation. When the number of circulating RBCs is increased, tissue hypoxia decreases, cardiac function is improved, and the child will have more energy. Parental visiting is not dependent on transfusion. The decrease in tissue hypoxia will minimize the risk of infection. There is no evidence that the child is currently infected. Forming a clot is the function of platelets.

48. 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.

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.

46. 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.

Which genetic term refers to the transfer of all or part of a chromosome to a different chromosome after chromosome breakage? a. Trisomy b. Monosomy c. Translocation d. Nondisjunction

ANS: C Translocation is the transfer of all or part of a chromosome to a different chromosome after chromosome breakage. It can be balanced, producing no phenotypic effects, or unbalanced, producing severe or lethal effects. Trisomy is an abnormal number of chromosomes caused by the presence of an extra chromosome, which is added to a given chromosome pair and results in a total of 47 chromosomes per cell. Monosomy is an abnormal number of chromosomes whereby the chromosome is represented by a single copy in a somatic cell. Nondisjunction is the failure of homologous chromosomes or chromatids to separate during mitosis or meiosis.

The nurse is teaching parents of a child with cri du chat syndrome about this disorder. The nurse understands parents understand the teaching if they make which statement? a. "This disorder is very common." b. "This is an autosomal recessive disorder." c. "The crying pattern is abnormal and catlike." d. "The child will always have a moon-shaped face."

ANS: C Typical of this disease is a crying pattern that is abnormal and catlike. Cri du chat, or cat's cry, syndrome is a rare (one in 50,000 live births) chromosome deletion syndrome, not autosomal recessive, resulting from loss of the small arm of chromosome 5. In early infancy this syndrome manifests with a typical but nondistinctive facial appearance, often a "moon-shaped" face with wide-spaced eyes (hypertelorism). As the child grows, this feature is progressively diluted, and by age 2 years, the child is indistinguishable from age-matched control participants.

Early diagnosis of congenital hypothyroidism (CH) and phenylketonuria (PKU) is essential to prevent which? a. Obesity b. Diabetes c. Cognitive impairment d. Respiratory distress

ANS: C Untreated, both PKU and CH cause cognitive impairment. With newborn screening and early intervention, cognitive impairment from these two disorders can be prevented. Obesity, diabetes, and respiratory distress do not result from both CH and PKU.

A father with an X-linked recessive disorder asks the nurse what the probability is that his sons will have the disorder. Which response should the nurse make? a. "Male children will be carriers." b. "All male children will be affected." c. "None of the sons will have the disorder." d. "It cannot be determined without more data."

ANS: C When a male has an X-linked recessive disorder, he has one copy of the allele on his X chromosome. The father passes only his Y chromosome (not the X chromosome) to his sons. Therefore, none of his sons will have the X-linked recessive gene. They will not be carriers or be affected by the disorder. No additional data are needed to answer this question.

A client is typed and crossmatched for a unit of blood. Which statement by the nurse indicates a need for further genetic education? a. "Blood type is formed from three gene alleles: A, B, and O." b. "Each blood type allele is inherited from the mother or the father." c. "If the client's blood type is AB, then the client is homozygous for that trait." d. "If the client has a dominant and a recessive blood type allele, only the dominant will be expressed."

ANS: C There are three possible gene alleles: A, B, and O. Blood type is determined by only two of the three specific gene alleles. The blood type OO is homozygous in contrast to the blood type AB, which is heterozygous. In the blood type AO, the gene allele A is dominant and will be expressed as blood type A. It is true that each blood type allele is inherited from the mother or the father.

The nurse is teaching parents of a child being discharged from the hospital after a splenectomy about the risk of infection. What should the nurse include in the teaching session? (Select all that apply.) a. Avoid obtaining the pneumococcal vaccination for the child. b. Avoid obtaining the meningococcal vaccination for the child. c. The child should receive prophylactic penicillin for certain procedures. d. Have the child immunized with the Haemophilus influenzae type b vaccination. e. Notify the health care provider if your child develops a fever of 38.5° C (101.3° F).

ANS: C, D, E Because of the risk of life-threatening bacterial infection after splenectomy, these children are immunized with the pneumococcal, meningococcal, and H. influenzae type b vaccines before surgery and receive prophylactic penicillin for several years after splenectomy. The parents should be instructed in the importance of seeking immediate medical attention if their child develops a fever of 38.5° C (101.3° F) or higher as a common sign of infection or postsplenectomy sepsis.

A client who tests positive for a mutation in the BRCA1 gene allele asks a nurse to be present when she discloses this information to her adult daughter. How should the nurse respond? a. "I will request a genetic counselor who is more qualified to be present for this conversation." b. "The test results can be confusing; I will help you interpret them for your daughter." c. "Are you sure you want to share this information with your daughter, who may not test positive for this gene mutation?" d. "This conversation may be difficult for both of you; I will be there to provide support."

ANS: D A nurse should provide emotional support while the client tells her daughter the information she has learned about the test results. The nurse should not interpret the results or counsel the client or her daughter. The nurse should refer the client for counseling or support, if necessary.

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.

Parents ask the nurse if there was something that should have been done during the pregnancy to prevent their child's cleft lip. Which statement should the nurse give as a response? a. "This is a type of deformation and can sometimes be prevented." b. "Studies show that taking folic acid during pregnancy can prevent this defect." c. "This is a genetic disorder and has a 25% chance of happening with each pregnancy." d. "The malformation occurs at approximately 5 weeks of gestation; there is no known way to prevent this."

ANS: D Cleft lip, an example of a malformation, occurs at approximately 5 weeks of gestation when the developing embryo naturally has two clefts in the area. There is no known way to prevent this defect. Deformations are often caused by extrinsic mechanical forces on normally developing tissue. Club foot is an example of a deformation often caused by uterine constraint. Cleft lip is not a genetic disorder; the reasons for this occurring are still unknown. Taking folic acid during pregnancy can help to prevent neural tube disorders but not cleft lip defects.

Parents of a child with hemophilia A ask the nurse, "What is the deficiency with this disorder?" Which correct response should the nurse make? a. "Hemophilia A has a deficiency in red blood cells." b. "Hemophilia A has a deficiency in platelets." c. "Hemophilia A has a deficiency in factor IX." d. "Hemophilia A has a deficiency in factor VIII."

ANS: D Hemophilia A is deficient in factor VIII. Glucose-6-phosphate dehydrogenase (G6PD) deficiency shows low red blood cells (hemolytic anemia). Immunosuppression may be the cause of a deficient number of platelets. Hemophilia B is deficient in factor IX.

What statement best describes iron deficiency anemia in infants? a. It is caused by depression of the hematopoietic system. b. Diagnosis is easily made because of the infant's emaciated appearance. c. It results from a decreased intake of milk and the premature addition of solid foods. d. Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.

ANS: D In iron-deficiency anemia, the child's clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed. The bone marrow produces red blood cells that are smaller and contain less hemoglobin than normal red blood cells. Children who have iron deficiency from drinking excessive quantities of milk are usually pale and overweight. They are receiving sufficient calories but are deficient in essential nutrients. The clinical manifestations result from decreased intake of iron-fortified solid foods and an excessive intake of milk.

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.

The majority of children in the United States with human immunodeficiency virus (HIV) infection acquired the disease by which means? a. Through sexual contact b. From a blood transfusion c. By using intravenous (IV) drugs d. Perinatally from their mothers

ANS: D More than 90% of the children with HIV under 13 years who were reported to the Centers for Disease Control and Prevention acquired the infection during the perinatal period. With intervention, the number of children infected can be decreased. Sexual contact and IV drug use are the leading causes of infection in the 14- to 19-year age group. This number is less than the number of cases in the under 13-year age group. Transfusion has accounted for 3% to 6% of all pediatric acquired immunodeficiency syndrome cases to date. Before 1985 and routine screening of donated blood products, children with hemophilia were at great risk from pooled plasma products.

A nurse cares for a client of Asian descent who is prescribed warfarin (Coumadin). What action should the nurse perform first? a. Schedule an international normalized ratio (INR) test to be completed each day. b. Initiate fall precautions and strict activity limitations. c. Teach the client about bleeding precautions, including frequent checks for any bruising. d. Confirm the prescription starts warfarin at a lower-than-normal dose.

ANS: D Most individuals of Asian heritage have a single nucleotide polymorphism in the CYP2C19 gene that results in low activity of the enzyme produced. This mutation greatly reduces the metabolism of warfarin, leading to increased bleeding risks and other serious side effects. Any person of Asian heritage who needs anticoagulation therapy should be started on very low dosages of warfarin and should have his or her INR monitored more frequently. The nurse can always teach about the risk of bleeding and can monitor for any bruising. The priority action is for the nurse to check the prescription and confirm the dose prior to administering the medication. It is not necessary to initiate fall precautions and to limit activity based on the administration of warfarin.

The clinic nurse is evaluating lab results for a child. What recorded hemoglobin (Hgb) result is considered within the normal range? a. 9 g/dl b. 10 g/dl c. 11 g/dl d. 12 g/dl

ANS: D Normal hemoglobin (Hgb) determination is 11.5 to 15.5 g/dl.

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.

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.

14. A couple asks the nurse about the optimal time for genetic counseling. They do not plan to have children for several years. When should the nurse recommend they begin genetic counseling? a. As soon as the woman suspects that she may be pregnant b. Whenever they are ready to start their family c. Now, if one of them has a family history of congenital heart disease d. Now, if they are members of a population at risk for certain diseases

ANS: D Persons who seek genetic evaluation and counseling must first be aware if there is a genetic or potential problem in their families. Genetic testing should be done now if the couple is part of a population at risk. It is not feasible at this time to test for all genetic diseases. The optimal time for genetic counseling is before pregnancy occurs. During the pregnancy, genetic counseling may be indicated if a genetic disorder is suspected. Congenital heart disease is not a single-gene disorder.

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 condition is an inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity? a. Fanconi syndrome b. Wiskott-Aldrich syndrome c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency syndrome (SCIDS)

ANS: D SCIDS is a genetic disorder that results in deficits of both humoral and cellular immunity. Fanconi syndrome is a hereditary disorder of red blood cell production. Wiskott-Aldrich syndrome is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. AIDS is not inherited.

The nurse is reviewing a client's prenatal history. Which prescribed medication does the nurse understand is not considered a teratogen and prescribed during pregnancy? a. Phenytoin (Dilantin) b. Warfarin (Coumadin) c. Isotretinoin (Accutane) d. Heparin sodium (Heparin)

ANS: D Teratogens, agents that cause birth defects when present in the prenatal environment, account for the majority of adverse intrauterine effects not attributable to genetic factors. Types of teratogens include drugs (phenytoin [Dilantin], warfarin [Coumadin], isotretinoin [Accutane]). Heparin is the anticoagulant used during pregnancy and is not a teratogen. It does not cross the placenta.

35. A child with hemophilia A will have which abnormal laboratory result? a. PT (ProTime) b. Platelet count c. Fibrinogen level d. PTT (partial thromboplastin time)

ANS: D The basic defect of hemophilia A is a deficiency of factor VIII. The partial thromboplastin time measures abnormalities in the intrinsic pathway (abnormalities in factors I, II, V, VIII, IX, X, XII, HMK, and KAL). The prothrombin time measures abnormalities of the extrinsic pathway (abnormalities in factors I, II, V, VII, and X). Fibrinogen level is not dependent on the intrinsic pathway. Platelets are not affected with hemophilia A.

What therapeutic intervention is most appropriate for a child with b-thalassemia major? a. Oxygen therapy b. Supplemental iron c. Adequate hydration d. Frequent blood transfusions

ANS: D The goal of medical management is to maintain sufficient hemoglobin (>9.5 g/dl) to prevent bone marrow expansion. This is achieved through a long-term transfusion program. Oxygen therapy and adequate hydration are not beneficial in the overall management of thalassemia. The child does not require supplemental iron. Iron overload is a problem because of frequent blood transfusions, decreased production of hemoglobin, and increased absorption from the gastrointestinal tract.

The nurse is preparing to administer a unit of packed red blood cells to a hospitalized child. What is an appropriate action that applies to administering blood? a. Take the vital signs every 15 minutes while blood is infusing. b. Use blood within 1 hour of its arrival from the blood bank. c. Administer the blood with 5% glucose in a piggyback setup. d. Administer the first 50 ml of blood slowly and stay with the child.

ANS: D The nurse should administer the first 50 ml of blood or initial 20% of volume (whichever is smaller) slowly and stay with the child. Vitals signs should be taken 15 minutes after initiation and then every hour, not every 15 minutes. Blood should be used within 30 minutes, not 1 hour. Normal saline, not 5% glucose, should be the IV solution.

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.