Deck 14: Cystic Fibrosis

Question 1

Cystic fibrosis patients can have all of the following EXCEPT:

A) malnutrition.
B)meconium ileus (bowel obstruction).
C)excessive, viscous pulmonary secretions.
D)tendency for status asthmaticus.

Answer 1

tendency for status asthmaticus.

Question 2

Which of the following are commonly cultured from the mucus in the tracheobronchial tree of a patient with cystic fibrosis?
1) Klebsiella
2) Pseudomonas aeruginosa
3) Haemophilus influenzae
4) Staphylococcus aureus

A)1, 2
B)2, 4
C)1, 2, 3
D)2, 3, 4

Answer 2

2, 3, 4

Question 3

If both the mother and the father are carriers for the cystic fibrosis gene, what are the chances that their child will be a cystic fibrosis carrier?

A) 0%
B)25%
C)50%
D)75%

Answer 3

50%

Question 4

If a cystic fibrosis patient is in generally good condition, what might be done to permanently fix the lung problem?

A) Cryosurgery
B)Total DNA replacement
C)Diaphragmatic pacemaker
D)Lung transplant

Question 5

total airway obstruction leading to atelectasis.

A) 1, 2
B)3, 4
C)1, 3, 4
D)1, 2, 3, 4

Question 6

Which ethnic group has the greatest number of people with cystic fibrosis?

A) African Americans
B)Asians
C)Hispanics
D)whites

Question 7

What complicating pulmonary problem is likely to happen to a patient with cystic fibrosis?

A) Pulmonary edema
B)Pleural effusion
C)Spontaneous pneumothorax
D)Cardiac tamponade

Question 8

All of the following pulmonary function findings would be expected in a patient with moderate to severe cystic fibrosis EXCEPT:

A) increased FVC.
B)increased RV.
C)decreased PEFR.
D)decreased FEF 50%.

Question 9

During the advanced stages of cystic fibrosis, the anatomic alterations cause the patient to have:

A) a primarily restrictive lung disease.
B)a primarily obstructive lung disease.
C)equal parts restrictive and obstructive lung disease.
D)normal airways with emphysema.

Question 10

Men with cystic fibrosis have difficulty reproducing because:

A) the fallopian tubes are blocked.
B)the vas deferens is missing or underdeveloped.
C)the men are impotent.
D)the women do not ovulate.

Question 11

Which of the following is/are commonly used in the treatment of cystic fibrosis?
1) Pancreatic enzymes and vitamins
2) Antibiotics
3) Postural drainage
4) Expectorants

A)1, 4
B)2, 3
C)1, 2, 3
D)1, 2, 3, 4

Question 12

Your patient has a lung infection with Pseudomonas aeruginosa. What antibiotic should be used against it?

A) Tobramycin (TOBI)
B)Dornase alpha (Pulmozyme)
C)Levalbuterol (Xopenex)
D)Pentamidine (NebuPent)

Question 13

How can the genetic mutation found with cystic fibrosis (CF) be characterized?
1) There are many variations in the mutation.
2) CF is the most common childhood inherited disorder.
3) The mutation is a dominant trait.
4) The mutation stops cilia function.

A)1, 2
B)3, 4
C)1, 3
D)2, 3, 4

Question 14

Common chest assessment findings in a patient with cystic fibrosis include:
1) tracheal deviation.
2) breath sounds reveal crackles and rhonchi.
3) dull percussion note.
4) hyperresonant percussion note.

A)1, 3
B)2, 4
C)1, 2
D)2, 3, 4

Question 15

All of the following can be used in the diagnosis of cystic fibrosis EXCEPT:

A) an elevated potassium level in the sweat.
B)an elevated chloride level in the sweat.
C)genetic testing of the patient and/or parents.
D)chronic lung infections from an early age.