Deck 28: Cystic Fibrosis

Question 1

Which of the following respiratory signs and symptoms should prompt the therapist for the evaluation of cystic fibrosis in a child?
I) Seasonal wheezing
II) Frequent thick sputum production
III) Chronic cough
IV) Nasal polyps

A) I and II only
B) III and IV only
C) I, II, and III only
D) II, III, and IV only

Answer 1

D
Box 28-1 Signs and Symptoms That May Prompt Evaluation for Cystic Fibrosis
Recurrent wheezing
Chronic cough
Frequent thick sputum production
Severe, prolonged, or recurrent sinopulmonary infections
Respiratory infections with pathogens associated with cystic fibrosis
Persistently abnormal chest radiograph
Nasal polyps
Clubbing of the nail beds

Question 2

A sweat chloride test is performed in a 14-year-old child who has adrenal insufficiency. What effect can this condition have on the results of this diagnostic test?

A) It can produce a false-positive sweat test.
B) It can generate a false-negative sweat test.
C) Adrenal insufficiency can cause either a false-positive or a false-negative result.
D) Adrenal insufficiency has no known effect on the result of a sweat test.

Answer 2

A
The sweat is obtained by stimulating the skin on the forearm with pilocarpine iontophoresis (see Figure 28-1 in the textbook). Technical error can result in false-negative and false-positive results. In addition to inadequate sweat collection, malnutrition, edema, and hypoalbuminemia can also give false-negative results. Therefore, patients with clinical features suggestive of CF but normal or borderline sweat test results should have the test repeated. Conditions that can produce false-positive results include malnutrition, eczema, adrenal insufficiency, pseudohypoaldosteronism, and hypothyroidism.

Question 3

What are the primary characteristics of cystic fibrosis?
I) Chronic obstruction and inflammation of the airways
II) Exocrine pancreatic insufficiency
III) Malabsorption and small bowel obstruction
IV) Decreased sweat chloride concentration

A) I and IV only
B) I, II, and III only
C) I, II, and IV only
D) II, III, and IV only

Answer 3

B
The signs and symptoms of classic CF are related to the overproduction of thick, viscous secretions in multiple organ systems:
Chronic obstruction, infection, and inflammation of the airways;
Exocrine pancreatic insufficiency with malabsorption and small bowel obstruction;
Infertility in males;
Elevated sweat chloride levels.

Question 4

Which of the following medications should the therapist routinely administer during the management of hospitalized patients with cystic fibrosis?
I) Albuterol
II) Hypertonic saline
III) DNAse
IV) Salmeterol

A) I and II only
B) II and III only
C) I and IV only
D) I, II, and III only

Question 5

A sweat chloride test and CFTR mutation analysis performed in a 10-year-old child with signs and symptoms consistent with CF are inconclusive. Which of the following tests should the therapist suggest at this time?

A) Sodium in urine
B) Nasal potential difference
C) CT scan of the chest with contrast
D) Immunoreactive trypsinogen

Question 6

On pulmonary function testing, which of the following lung abnormalities are common in patients with severe cystic fibrosis?
I) Obstructive pattern
II) Restrictive pattern
III) Airway hyperreactivity
IV) Normal pattern when asymptomatic

A) I and IV only
B) II and IV only
C) II and III only
D) I, II, and III only

Question 7

Approximately what percentage of patients with cystic fibrosis present with pulmonary symptoms?

A) 100%
B) 80%
C) 50%
D) 30%

Question 8

What are the chances of two CFTR gene carriers having a child with two normal alleles?

A) 100%
B) 75%
C) 50%
D) 25%

Question 9

Which of the following radiographic features is consistent with CF?

A) Air bronchograms
B) Dome-shaped diaphragms
C) Hyperinflation and flattened diaphram
D) Meniscus sign

Question 10

Which of the following aerosolized antibiotics is nebulized to treat infections caused by Pseudomonas aeruginosa in patients with CF?

A) Gentamycin
B) Tobramycin
C) Amiloride
D) Ibuprofen

Question 11

Which of the following is the earliest pathologic change that causes airway dysfunction?

A) Infection
B) Inflammation
C) Bronchospasm
D) Plugging of the submucosal gland ducts

Question 12

What percentage of patients with adult cystic fibrosis are chronically infected with Pseudomonas?

A) More than 3%
B) More than 23%
C) More than 53%
D) More than 73%

Question 13

Which of the following therapeutic interventions should be the focus of the treatment of patients with cystic fibrosis?

A) Hypertonic saline
B) Bronchodilators
C) Antihistamines
D) Removing thickened mucus from the airway

Question 14

Which of the following microorganisms commonly colonize the airways of patients with cystic fibrosis?
I) Actinomyces israelii
II) Haemophilus influenzae
III) Pseudomonas aeruginosa
IV) Staphylococcus aureus

A) III only
B) I and II only
C) II and IV only
D) II, III, and IV only

Question 15

Which of the following methods is the most commonly recommended for newborn screening?

A) Sodium in urine
B) Nasal potential difference
C) CT scan of the chest with contrast
D) Immunoreactive trypsinogen

Question 16

A 10-year-old child has had two sweat tests, each indicating a sweat chloride concentration of 30 mEq/L. How should the therapist interpret these data?

A) The child is a carrier of CF.
B) The child has CF.
C) The child is likely to develop CF.
D) The child does not have CF.

Question 17

Which of the following is a common nonpulmonary effect of CF?

A) Myocardial infarction
B) Hypertension
C) Gout
D) Pancreatic insufficiency

Question 18

Which of the following drugs should the therapist give before administering nebulized 7% saline to a patient with CF?

A) rhDNase
B) N-Acetylcysteine
C) Albuterol
D) Amiloride

Question 19

Which of the following anti-inflammatory agents should be considered in patients with CF to slow the progression of the lung disease?

A) Prednisone
B) Any inhaled corticosteroid
C) Aspirin
D) Ibuprofen