Question 1

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

Accepted Answer

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

Question 2

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

Accepted Answer

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

Question 3

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

Accepted Answer

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

Question 4

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

Accepted Answer

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

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?

Accepted Answer

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

Question 6

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

Accepted Answer

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

Question 7

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?

Accepted Answer

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. 
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.

Question 8

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

Accepted Answer

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

Question 9

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

Accepted Answer

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

Question 10

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

Accepted Answer

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

Question 11

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

Accepted Answer

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

Question 12

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

Accepted Answer

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

Question 13

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

Accepted Answer

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

Question 14

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?

Accepted Answer

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. 
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 15

Which of the following radiographic features is consistent with CF?

Accepted Answer

A) Air bronchograms 
B) Dome-shaped diaphragms 
C) Bronchiectatic changes 
D) Meniscus sign 
A) Air bronchograms 
B) Dome-shaped diaphragms 
C) Bronchiectatic changes 
D) Meniscus sign

Question 16

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

Accepted Answer

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

Question 17

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

Accepted Answer

A) More than 5% 
B) More than 20% 
C) More than 50% 
D) More than 70% 
A) More than 5% 
B) More than 20% 
C) More than 50% 
D) More than 70%

Question 18

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

Accepted Answer

A) Airway clearance techniques 
B) Bronchodilators 
C) Antihistamines 
D) PEP therapy 
A) Airway clearance techniques 
B) Bronchodilators 
C) Antihistamines 
D) PEP therapy

Exam 28: Cystic Fibrosis

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

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

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

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

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?

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

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?

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

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

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

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

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

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

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?

Which of the following radiographic features is consistent with CF?

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

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

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

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