Wong's Nursing Care of Infants and Children 9th Edition by Marilyn J. Hockenberry, David Wilson - Test Bank

Wong’s Nursing Care of Infants and Children 9th Edition by Marilyn J. Hockenberry, David Wilson – Test Bank

 

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Hockenberry: Wong’s Nursing Care of Infants and Children, 9^th Edition

 

Chapter 05: Hereditary Influences on Health Promotion of the Child and Family

 

Test Bank

 

MULTIPLE CHOICE

 

1. Which of the following genetic terms 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 expression 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 80

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

 

2. Which of the following genetic terms 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 81

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

 

3. Which of the following 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 81

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

 

4. The nurse is assessing a neonate who was born 1 hour ago to healthy, Caucasian parents in their early forties. Which of the following findings would 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 109

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

 

5. Which of the following abnormalities is a common sex chromosome defect?

a.	Down
b.	Turner
c.	Marfan
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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 86

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

 

6. Turner syndrome is suspected in an adolescent girl with short stature. This is caused by which of the following?

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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 107

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

 

7. Which of the following 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).

 

DIF:    Cognitive Level: Comprehension     REF:   p. 86

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

 

8. Which of the following statements 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 gene is necessary to cause the disorder. When a parent is affected, there is a 50% chance that the chromosome with the gene for 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 90

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

 

9. Which of the following 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 95

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

 

10. Which of the following 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. Since 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 98

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

 

11. A father with an X-linked recessive disorder asks the nurse what the probability is that his sons will have the disorder. The nurse answers:

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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 98

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

12. The inheritance of which of the following 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 dominant trait.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 97

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

 

13. Chromosome analysis of the fetus is usually accomplished through the testing of which of the following?

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, 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 107

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

 

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 the couple is ready to start their family
c.	Now, if one of them has a family history of congenital heart disease
d.	Now, if the couple 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 108

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

15. A woman, age 43, is 6 weeks pregnant. It is important that she be informed of the:

a.	need for a therapeutic abortion.
b.	increased risk for Down syndrome.
c.	increased risk for Turner syndrome.
d.	need for an immediate amniocentesis.

 

 

ANS:  B

Women who are over age 35 at the birth of a single child or 31 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 109

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

16. 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 1:4. Which of the following statements 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. Since 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.

 

DIF:    Cognitive Level: Comprehension     REF:   p. 109

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

17. A couple expecting their first child has a positive family history for several congenital defects and disorders. Prenatal diagnostic testing is recommended. The couple tells the nurse that they are opposed to abortion for religious reasons. Which of the following should the nurse consider when counseling this couple?

a.	Couple should be encouraged to have recommended diagnostic testing.
b.	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. Should the child have 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.

 

DIF:    Cognitive Level: Comprehension     REF:   pp. 106,107

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

MULTIPLE RESPONSE

 

1. The nurse is teaching a group of new nurses who will be working in the neonatal intensive care unit. Which of the following assessment findings for genetic disorders should the nurse include in the teaching? Select all that apply.

a.	Poor urinary output
b.	Limb abnormalities
c.	Hypertonic muscle tone
d.	Increased respiratory rate
e.	Cardiac defect

 

 

ANS:  B, C, E

A variety of assessment clues, including limb abnormalities, hypertonic muscle tone, and cardiac defects, point to the possibility of a genetic disorder. Poor urinary output and increased respiratory rate are not indications of a genetic problem.

 

DIF:    Cognitive Level: Application           REF:   p. 109

TOP:   Integrated Process: Teaching/Learning

MSC:  Client Needs: Health Promotion and Maintenance

 

2. Which of the following 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.

 

DIF:    Cognitive Level: Application           REF:   pp. 90, 95

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