Chapter 36: Hemolytic Disorders and Congenital Anomalies
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.
PTS: 1 DIF: Cognitive Level: Apply
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-negativNeUmR heIr N
otS anGdTaBfa.thCeOMho is Rh positive and heterozygous
rw
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.
PTS: 1 DIF: Cognitive Level: Understand
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 child’s 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.
PTS: 1 DIF: Cognitive Level: Applying
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. Potential for impaired parent-infant attachment
b. Inadequate nutrition
c. Potential for infection
d. Reduced 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 inadequate
nutrition 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 diaphragmN atU
icRhS
erI
niN
a.GTT
hB
e n.uC
tritiM
onal needs of this infant may be a clearly
identified need; however, at this time the nurse should be most concerned about reduced gas
exchange. This infant has the potential for infection, especially after 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.
PTS: 1 DIF: Cognitive Level: Apply
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.
PTS: 1 DIF: Cognitive Level: Understand
, 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.
PTS: 1 DIF: Cognitive Level: Understand
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 tN
heUnR
eeSdIfoNrGseTriB
al.cC
astiM
ng.
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.
PTS: 1 DIF: Cognitive Level: Apply
TOP: Nursing Process: Planning
MSC: Client Needs: Health Promotion and Maintenance
8. Most congenital anomalies of the central nervous system (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
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.
PTS: 1 DIF: Cognitive Level: Apply
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-negativNeUmR heIr N
otS anGdTaBfa.thCeOMho is Rh positive and heterozygous
rw
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.
PTS: 1 DIF: Cognitive Level: Understand
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 child’s 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.
PTS: 1 DIF: Cognitive Level: Applying
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. Potential for impaired parent-infant attachment
b. Inadequate nutrition
c. Potential for infection
d. Reduced 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 inadequate
nutrition 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 diaphragmN atU
icRhS
erI
niN
a.GTT
hB
e n.uC
tritiM
onal needs of this infant may be a clearly
identified need; however, at this time the nurse should be most concerned about reduced gas
exchange. This infant has the potential for infection, especially after 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.
PTS: 1 DIF: Cognitive Level: Apply
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.
PTS: 1 DIF: Cognitive Level: Understand
, 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.
PTS: 1 DIF: Cognitive Level: Understand
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 tN
heUnR
eeSdIfoNrGseTriB
al.cC
astiM
ng.
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.
PTS: 1 DIF: Cognitive Level: Apply
TOP: Nursing Process: Planning
MSC: Client Needs: Health Promotion and Maintenance
8. Most congenital anomalies of the central nervous system (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
