Chapter 36: Hemolytic Disorders and Congenital Anomalies My Nursing Test Banks

Chapter 36: Hemolytic Disorders and Congenital Anomalies

Lowdermilk: Maternity & Womens Health Care, 11th Edition

MULTIPLE CHOICE

1. To explain hemolytic disorders in the newborn to new parents, the nurse who cares for the newborn population must be aware of the physiologic characteristics related to these conditions. What is the most common cause of pathologic hyperbilirubinemia?

a.

Hepatic disease

b.

Hemolytic disorders

c.

Postmaturity

d.

Congenital heart defect

ANS: B

Hemolytic disorders in the newborn are the most common cause of pathologic hyperbilirubinemia (jaundice). Although hepatic damage, prematurity, and congenital heart defects may cause pathologic hyperbilirubinemia, they are not the most common causes.

DIF: Cognitive Level: Apply REF: p. 882 TOP: Nursing Process: Diagnosis

MSC: Client Needs: Physiologic Integrity

2. Which infant is most likely to express Rh incompatibility?

a.

Infant of an Rh-negative mother and a father who is Rh positive and homozygous for the Rh factor

b.

Infant who is Rh negative and a mother who is Rh negative

c.

Infant of an Rh-negative mother and a father who is Rh positive and heterozygous for the Rh factor

d.

Infant who is Rh positive and a mother who is Rh positive

ANS: A

If the mother is Rh negative and the father is Rh positive and homozygous for the Rh factor, then all the offspring of this union will be Rh positive. Only Rh-positive offspring of an Rh-negative mother are at risk for Rh incompatibility. Only the Rh-positive offspring of an Rh-negative mother are at risk. If the mother is Rh negative and the father is Rh positive and heterozygous for the factor, a 50% chance exists that each infant born of this union will be Rh positive, and a 50% chance exists that each will be born Rh negative. No risk for incompatibility exists if both the mother and the infant are Rh positive.

DIF: Cognitive Level: Understand REF: p. 883 TOP: Nursing Process: Planning

MSC: Client Needs: Health Promotion and Maintenance

3. What is the highest priority nursing intervention for an infant born with myelomeningocele?

a.

Protect the sac from injury.

b.

Prepare the parents for the childs paralysis from the waist down.

c.

Prepare the parents for closure of the sac when the child is approximately 2 years of age.

d.

Assess for cyanosis.

ANS: A

A major preoperative nursing intervention for a neonate with a myelomeningocele is the protection of the protruding sac from injury to prevent its rupture and the resultant risk of central nervous system (CNS) infection. The long-term prognosis in an affected infant can be determined to a large extent at birth, with the degree of neurologic dysfunction related to the level of the lesion, which determines the nerves involved. A myelomeningocele should be surgically closed within 24 hours. Although the nurse should assess for multiple potential problems in this infant, the major nursing intervention is to protect the sac from injury.

DIF: Cognitive Level: Understand REF: p. 892

TOP: Nursing Process: Planning | Nursing Process: Implementation

MSC: Client Needs: Physiologic Integrity

4. Which nursing diagnosis is most appropriate for a newborn diagnosed with a diaphragmatic hernia?

a.

Risk for impaired parent-infant attachment

b.

Imbalanced nutrition, related to less than body requirements

c.

Risk for infection

d.

Impaired gas exchange

ANS: D

Herniation of the abdominal viscera into the thoracic cavity may cause severe respiratory distress and represent a neonatal emergency. Oxygen therapy, mechanical ventilation, and the correction of acidosis are necessary in infants with large defects. Although imbalanced nutrition, related to less than body requirements, may be a factor in providing care to a newborn with a diaphragmatic hernia, the priority nursing diagnosis relates to the oxygenation issues arising from the lung hypoplasia that occurs with diaphragmatic hernia. The nutritional needs of this infant may be a clearly identified need; however, at this time the nurse should be most concerned about impaired gas exchange. This infant is at risk for infection, especially once the surgical repair has been performed. The extent of the herniation may have hindered normal development of the lungs in utero, resulting in respiratory distress.

DIF: Cognitive Level: Apply REF: p. 894 TOP: Nursing Process: Diagnosis

MSC: Client Needs: Physiologic Integrity

5. What is the clinical finding most likely to be exhibited in an infant diagnosed with erythroblastosis fetalis?

a.

Edema

b.

Immature red blood cells

c.

Enlargement of the heart

d.

Ascites

ANS: B

Erythroblastosis fetalis occurs when the fetus compensates for the anemia associated with Rh incompatibility by producing large numbers of immature erythrocytes to replace those hemolyzed. Edema occurs with hydrops fetalis, a more severe form of erythroblastosis fetalis. The fetus with hydrops fetalis may exhibit effusions into the peritoneal, pericardial, and pleural spaces, as well as demonstrate signs of ascites.

DIF: Cognitive Level: Understand REF: p. 883

TOP: Nursing Process: Assessment MSC: Client Needs: Physiologic Integrity

6. Which statement regarding congenital anomalies of the cardiovascular and respiratory systems is correct?

a.

Cardiac disease may demonstrate signs and symptoms of respiratory illness.

b.

Screening for congenital anomalies of the respiratory system need only be performed for infants experiencing respiratory distress.

c.

Choanal atresia can be corrected with the use of a suction catheter to remove the blockage.

d.

Congenital diaphragmatic hernias are diagnosed and treated after birth.

ANS: A

The cardiac and respiratory systems function together; therefore, initial findings will be related to respiratory illness. Screening for congenital respiratory system anomalies is necessary, even for infants who appear normal at birth. All newborns should have critical congenital heart disease (CCHD) screening performed before discharge. Choanal atresia requires emergency surgery. Congenital diaphragmatic hernias are prenatally discovered on ultrasound.

DIF: Cognitive Level: Understand REF: p. 889

TOP: Nursing Process: Assessment MSC: Client Needs: Physiologic Integrity

7. When attempting to screen and educate parents regarding the treatment of developmental dysplasia of the hip (DDH), which intervention should the nurse perform?

a.

Be able to perform the Ortolani and Barlow tests.

b.

Teach double or triple diapering for added support.

c.

Explain to the parents the need for serial casting.

d.

Carefully monitor infants for DDH at follow-up visits.

ANS: D

Because DDH often is not detected at birth, infants should be carefully monitored at follow-up visits. The Ortolani and Barlow tests must be performed by experienced clinicians to prevent fracture or other damage to the hip. Double or triple diapering is not recommended because it promotes hip extension, thus worsening the problem. Serial casting is recommended for clubfoot, not DDH.

DIF: Cognitive Level: Apply REF: p. 899 TOP: Nursing Process: Planning

MSC: Client Needs: Health Promotion and Maintenance

8. The nurse is assigned a home care visit of a 5-day-old infant for the treatment of jaundice. A thorough assessment is completed, and a health history is obtained. Which sign or symptom indicates that the infant may be displaying the initial phase of encephalopathy?

a.

High-pitched cry

b.

Severe muscle spasms (opisthotonos)

c.

Fever and seizures

d.

Hypotonia, lethargy, and poor suck

ANS: D

The early and most subtle symptoms of bilirubin encephalopathy include hypotonia, lethargy, poor suck, and a depressed or absent Moro reflex. Should the infant display symptoms such as a high-pitched cry, severe muscle spasms, hyperreflexia, or an arching of the back, the nurse should be aware that the baby has progressed beyond the more subtle signs of the first phase of encephalopathy. Medical attention is immediately necessary. Symptoms may progress from the subtle indications of the first phase to fever and seizures in as few as 24 hours. Only approximately one half of these infants survive, and those that do will have permanent sequelae, including auditory deficiencies, intellectual deficits, and movement abnormalities.

DIF: Cognitive Level: Analyze REF: p. 884

TOP: Nursing Process: Assessment MSC: Client Needs: Health Promotion and Maintenance

9. Most congenital anomalies of the CNS result from defects in the closure of the neural tube during fetal development. Which factor has the greatest impact on this process?

a.

Maternal diabetes

b.

Maternal folic acid deficiency

c.

Socioeconomic status

d.

Maternal use of anticonvulsant

ANS: B

All of these environmental influences may affect the development of the CNS. Maternal folic acid deficiency has a direct bearing on the failure of neural tube closure. As a preventative measure, folic acid supplementation (0.4 mg/day) is recommended for all women of childbearing age.

DIF: Cognitive Level: Analyze REF: p. 891 TOP: Nursing Process: Planning

MSC: Client Needs: Physiologic Integrity

10. The condition, hypospadias, encompasses a wide range of penile abnormalities. Which information should the nurse provide to the anxious parents of an affected newborn?

a.

Mild cases involve a single surgical procedure.

b.

Infant should be circumcised.

c.

Repair is performed as soon as possible after birth.

d.

No correlation exists between hypospadia and testicular cancer.

ANS: A

Mild cases of hypospadias are often repaired for cosmetic reasons, and repair involves a single surgical procedure, enabling the male child to urinate in a standing position and to have an adequate sexual organ. These infants are not circumcised; the foreskin will be needed during the surgical repair. Repair is usually performed between 1 and 2 years of age. A correlation between hypospadias and testicular cancer exists; therefore, these children will require long-term follow-up observation.

DIF: Cognitive Level: Apply REF: p. 902

TOP: Nursing Process: Implementation MSC: Client Needs: Physiologic Integrity

11. The nurse is instructing a family how to care for their infant in a Pavlik harness to treat DDH. What information should be included in the teaching?

a.

Apply lotion or powder to minimize skin irritation.

b.

Remove the harness several times a day to prevent contractures.

c.

Return to the clinic every 1 to 2 weeks.

d.

Place a diaper over the harness, preferably using an absorbent disposable diaper.

ANS: C

Infants have a rapid growth pattern. Therefore, the child needs to be assessed by the practitioner every 1 to 2 weeks for possible adjustments. Lotions and powders should not be used with the harness, and the harness should not be removed, except as directed by the practitioner. A thin disposable diaper can be placed under the harness.

DIF: Cognitive Level: Understand REF: p. 900

TOP: Nursing Process: Implementation MSC: Client Needs: Physiologic Integrity

12. A neonate is born with mild clubfeet. When the parents ask the nurse how this will be corrected, how should the nurse respond?

a.

Traction is tried first.

b.

Surgical intervention is needed.

c.

Frequent, serial casting is tried first.

d.

Children outgrow this condition when they learn to walk.

ANS: C

Serial casting, the preferred treatment, is begun shortly after birth and before discharge from the nursery. Successive casts allows for gradual stretching of skin and tight structures on the medial side of the foot. Manipulation and casting of the leg are frequently repeated (every week) to accommodate the rapid growth of early infancy. Surgical intervention is performed only if serial casting is not successful. Children do not improve without intervention.

DIF: Cognitive Level: Understand REF: p. 901 TOP: Nursing Process: Planning

MSC: Client Needs: Physiologic Integrity

13. Which statement regarding hemolytic diseases of the newborn is most accurate?

a.

Rh incompatibility matters only when an Rh-negative child is born to an Rh-positive mother.

b.

ABO incompatibility is more likely than Rh incompatibility to precipitate significant anemia.

c.

Exchange transfusions are frequently required in the treatment of hemolytic disorders.

d.

The indirect Coombs test is performed on the mother before birth; the direct Coombs test is performed on the cord blood after birth.

ANS: D

An indirect Coombs test may be performed on the mother a few times during pregnancy. Only the Rh-positive child of an Rh-negative mother is at risk. ABO incompatibility is more common than Rh incompatibility but causes less severe problems; significant anemia, for instance, is rare with ABO. Exchange transfers infrequently are needed because of the decrease in the incidence of severe hemolytic disease in newborns from Rh incompatibility.

DIF: Cognitive Level: Understand REF: pp. 884, 885 TOP: Nursing Process: Planning

MSC: Client Needs: Health Promotion and Maintenance

MULTIPLE RESPONSE

1. Cleft lip or palate is a common congenital midline fissure, or opening, in the lip or palate resulting from the failure of the primary palate to fuse. Multiple genetic and, to a lesser extent, environmental factors may lead to the development of a cleft lip or palate. Which factors are included? (Select all that apply.)

a.

Alcohol consumption

b.

Female gender

c.

Use of some anticonvulsant medications

d.

Maternal cigarette smoking

e.

Antibiotic use in pregnancy

ANS: A, C, D

Factors associated with the potential development of cleft lip or palate are maternal infections, alcohol consumption, radiation exposure, corticosteroid use, use of some anticonvulsant medications, male gender, Native-American or Asian descent, and maternal smoking during pregnancy. Cleft lip is more common in male infants. Antibiotic use in pregnancy is not associated with the development of cleft lip or palate.

DIF: Cognitive Level: Understand REF: p. 895 TOP: Nursing Process: Planning

MSC: Client Needs: Physiologic Integrity

2. The most widespread use of postnatal testing for genetic disease is the routine screening of newborns for inborn errors of metabolism (IEM). Which conditions are considered metabolic disorders? (Select all that apply.)

a.

Phenylketonuria (PKU)

b.

Galactosemia

c.

Hemoglobinopathy

d.

Cytomegalovirus (CMV)

e.

Rubella

ANS: A, B, C

PKU is an IEM that can be diagnosed with newborn screening. Galactosemia is a metabolic defect that falls under the category of an IEM. Sickle cell disease and thalassemia are hemoglobinopathies that can be detected by newborn screening. CMV and rubella cannot be detected by newborn screening and are not metabolic disorders; rather, they are viruses contracted by the fetus.

DIF: Cognitive Level: Understand REF: p. 904 TOP: Nursing Process: Planning

MSC: Client Needs: Health Promotion and Maintenance

3. The nurse is caring for an infant with DDH. Which clinical manifestations should the nurse expect to observe? (Select all that apply.)

a.

Positive Ortolani click

b.

Unequal gluteal folds

c.

Negative Babinski sign

d.

Trendelenburg sign

e.

Telescoping of the affected limb

ANS: A, B

A positive Ortolani test and unequal gluteal folds are clinical manifestations of DDH observed from birth to 2 to 3 months of age. A negative Babinski sign, Trendelenburg sign, and telescoping of the affected limb are not clinical manifestations of DDH.

DIF: Cognitive Level: Apply REF: p. 900 TOP: Nursing Process: Planning

MSC: Client Needs: Health Promotion and Maintenance

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