Deck 15: Cystic Fibrosis

Question 1

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.

Answer 1

an elevated potassium level in the sweat.

Question 2

Which of the following is NOT recommended in children with cystic fibrosis?

A) Systemic corticosteroids
B) High-dose ibuprofen
C) Inhaled antibiotics
D) Inhaled DNase (Dornase alpha) (Pulmozyme)

Answer 2

Systemic corticosteroids

Question 3

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

Answer 3

Spontaneous pneumothorax

Question 4

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%

Question 5

All of the following chest assessment findings may be seen in a patient with cystic fibrosis EXCEPT:

A) decreased tactile and vocal fremitus.
B) dull percussion note.
C) diminished breath sounds.
D) decreased heart sounds.

Question 6

Which of the following may be recommended to reduce bronchial inflammation in a 12-year-old child with cystic fibrosis?

A) High-dose ibuprofen
B) High-dose acetaminophen
C) High-dose naproxen
D) High-dose corticosteroids

Question 7

Which of the following are commonly used in the management 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 8

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 fatal childhood inherited disorder.
3) The mutation is a dominant trait.
4) The mutation stops ciliary function.

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

Question 9

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

Question 10

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

A) African-Americans
B) Asians
C) Hispanics
D) Caucasians

Question 11

A cystic fibrosis patient in generally good condition may be a candidate for which of the following?

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

Question 12

Men with cystic fibrosis have difficulty reproducing because the:

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

Question 13

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 14

A 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 15

A common nonrespiratory clinical manifestation of cystic fibrosis is:

A) sinusitis.
B) hyperpigmentation.
C) rheumatoid arthritis.
D) glaucoma.

Question 16

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.

Question 17

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 18

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 FEF50%.

Question 19

The major pathologic or structural changes associated with cystic fibrosis include:
1) partial airway obstruction leading to hyperinflation.
2) bronchospasm.
3) thick, tenacious mucus.
4) total airway obstruction leading to atelectasis.

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