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AJN, American Journal of Nursing:
doi: 10.1097/01.NAJ.0000450429.20255.36
Feature Articles

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

Contrada, Emily

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Emily Contrada

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TEST INSTRUCTIONS

* To take the test online, go to our secure Web site at www.nursingcenter.com/ce/ajn.

* To use the form provided in this issue, record your answers in the test answer section of the CE enrollment form below. Each question has only one correct answer. You may make copies of the form.

* Complete the registration information and course evaluation. Mail the completed enrollment form and registration fee to: Lippincott Williams and Wilkins, CE Group, 74 Brick Blvd., Bldg. 4, Suite 206, Brick, NJ 08723. You will receive your certificate in four to six weeks. For faster service, include a fax number and we will fax your certificate within two business days of receiving your enrollment form. You will receive your CE certificate of earned contact hours and an answer key to review your results. There is no minimum passing grade.

* Registration deadline is June 30, 2016.

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DISCOUNTS AND CUSTOMER SERVICE

* Send in together two or more tests from any nursing journal published by Lippincott, Williams and Wilkins (LWW), and deduct $0.95 from the price of each test.

* We also offer CE accounts for hospitals and other health care facilities online at www.nursingcenter.com. Call 1-800-787-8985 for details.

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PROVIDER ACCREDITATION

LWW, publisher of AJN, will award the number of contact hours indicated for each continuing nursing education activity. LWW is accredited as a provider of continuing nursing education by the Commission on Accreditation of the American Nurses Credentialing Center (ANCC).

These activities are also provider approved by the California Board of Registered Nursing, Provider Number CEP 11749 for the number of contact hours indicated. LWW is also an approved provider of continuing nursing education by the District of Columbia and Florida #50-1223. Your certificate is valid in all states.

The ANCC's accreditation status of the LWW Department of Continuing Education refers to its continuing nursing educational activities only and does not imply Commission on Accreditation approval or endorsement of any commercial product.

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CE TEST QUESTIONS

Genomic Breakthroughs in the Diagnosis and Treatment of Cystic Fibrosis

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GENERAL PURPOSE:

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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LEARNING OBJECTIVES:

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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