CE Test 2.3 Hours: Genomic Breakthroughs in the Diagnosis and Treatment of Cystic Fibrosis

Contrada, Emily

AJN, American Journal of Nursing: June 2014 - Volume 114 - Issue 6 - pp 44,45
doi: 10.1097/01.NAJ.0000450429.20255.36
Feature Articles

Emily Contrada

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Genomic Breakthroughs in the Diagnosis and Treatment of Cystic Fibrosis

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To provide information about empirical research into cystic fibrosis (CF) and the impact of new genomic advances on CF diagnosis and treatment.

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After reading this article and taking this test, you will be able to

* recognize recent findings in the study of cystic fibrosis genetics.

* identify the implications of these findings for patient teaching and treatment.

1. How does cystic fibrosis (CF) rank as a common, potentially life-shortening genetic disorder affecting U.S. children?

a. first

b. second

c. third

d. fourth

2. CF is most common among which of the following groups?

a. white Americans

b. Asian Americans

c. Hispanic Americans

d. African Americans

3. Infants born with CF today can be expected to live beyond

a. the first or second decade.

b. the second or third decade.

c. the fourth or fifth decade.

d. the sixth or seventh decade.

4. CF is characterized by abnormally thick secretions in exocrine glands, especially in the

a. liver.

b. sweat glands.

c. salivary glands.

d. reproductive organs.

5. Men who have CF tend to be infertile owing to

a. congenital bilateral absence of the vas deferens.

b. an enlarged varicose vein in the spermatic cord.

c. long-term or intensive exposure to toxins.

d. a low sperm count or impaired sperm motility.

6. If both parents are carriers, what is their child's chance of having CF?

a. no chance

b. 1 in 2

c. 1 in 3

d. 1 in 4

7. Of the 1,965 cystic fibrosis transmembrane conductance regulator (CFTR) mutations, how many sufficiently impair CFTR function to produce CF symptoms?

a. 15

b. 127

c. 368

d. 792

8. Symptom-causing mutations interfere with the protein that transports which of the following substances across the cell membrane?

a. sodium

b. calcium

c. chloride

d. potassium

9. Patients who have which of the following classes of CF mutations tend to have milder CF symptoms?

a. I

b. II

c. III

d. IV

10. Which of these is the gene mutation responsible for most cases of CF?

a. F508del

b. R117H

c. 1717-1G>A

d. 2789+56G>A

11. The Cystic Fibrosis Foundation recommends that newborn screening panels include how many of the most common symptom-causing mutations?

a. 10

b. 23

c. 36

d. 47

12. When multimutation panels are used, about 97% of infants found to have only 1 CF mutation through newborn screening

a. have CF.

b. are CF carriers.

c. do not have CF.

d. have abnormal sweat test results.

13. Which sweat test value confirms a CF diagnosis for all age groups?

a. below 30 mEq/L

b. below 39 mEq/L

c. between 30 and 59 mEq/L

d. 60 mEq/L or higher

14. Sweat test values below which of the following rule out a CF diagnosis in children and adults?

a. 30 mEq/L

b. 39 mEq/L

c. 59 mEq/L

d. 60 mEq/L

15. Those with a clear CF diagnosis have evidence of 2 symptom-causing mutations, in some cases confirmed by the presence of

a. hematologic involvement.

b. cardiac involvement.

c. neurological involvement.

d. pulmonary involvement.

16. Disorders that can be associated with CFTR-related disease include

a. myasthenia gravis.

b. disseminated bronchiectasis.

c. amyotrophic lateral sclerosis.

d. disseminated intravascular coagulation.

17. Two studies found that the presence of a single CFTR mutation, usually a CF-causing mutation, can be associated with

a. chronic pancreatitis.

b. diabetes.

c. chronic kidney disease.

d. chronic obstructive pulmonary disease.

18. The drug ivacaftor (Kalydeco or VX-770)

a. is indicated for infants with CF.

b. is pending approval by the Food and Drug Administration for treatment of CF.

c. is indicated for CF patients with F508del, a class II mutation, when combined with VX-809.

d. increases the ability of affected CFTR proteins to transport ions across the cell membrane at the cell surface.

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