CH 15 Cystic Fibrosis

Which of the following are considered classifications of CFTR mutations? 1. Gating defect 2. Conductance defect 3. Sol layer defect 4. Periciliary defect a. 1, 2 b. 1, 3, 4 c. 2, 3, 4 d. 1, 2, 3

ANS: A The CFTR mutations are also classified as either a gating defect where the channel does not open or a conductance defect where the channel is open, but chloride does not move efficiently.

A common nonrespiratory clinical manifestation of cystic fibrosis is: a. sinusitis. b. hyperpigmentation. c. rheumatoid arthritis. d. glaucoma.

ANS: A Nasal polyps are seen in between 10% and 30% of patients with CF. The other listed medical conditions are not associated with cystic fibrosis.

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

ANS: A 1,2 There are many variations in the genetic mutation that causes CF, the most common childhood inherited disorder. The defective CF gene is recessive, not dominant. It causes the dehydration of secretions. The cilia are normal.

Which of the following chest assessment findings may be seen in a patient with cystic fibrosis? 1. Decreased tactile and vocal fremitus 2. Dull percussion note 3. Diminished breath sounds 4. Decreased heart sounds a. 1, 2 b. 1, 3, 4 c. 2, 3, 4 d. 1, 2, 3

ANS: B Due to the airway obstruction and retained secretions, the following chest assessment findings would be expected: decreased or increased tactile and vocal fremitus, hyperresonant percussion note, diminished breath sounds, diminished heart sounds, and bronchial breath sounds (over atelectasis). A hyperresonant percussion note would be expected with the hyperinflation of alveoli, not a dull percussion note.

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.

ANS: B Men with cystic fibrosis are usually infertile because the vas deferens is missing or underdeveloped.

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.

ANS: B Patients with advanced cystic fibrosis primarily have obstructive lung disease changes. Restrictive changes play a minor role.

Which of the following are commonly used in the management of cystic fibrosis? 1. Pancreatic enzymes and vitamins 2. Antibiotics 3. Postural drainage 4. Antitussives a. 1, 4 b. 2, 3 c. 1, 2, 3 d. 2, 3, 4

ANS: C Antitussives are not used in the treatment of cystic fibrosis.

. Which of the following pulmonary function findings would be expected in a patient with moderate to severe cystic fibrosis? 1. Increased FVC 2. Increased RV 3. Decreased PEFR 4. Decreased FEF50% a. 1 b. 2, 3 c. 2, 3, 4 d. 1, 2, 3

ANS: C Because of the obstructive lung problems associated with cystic fibrosis, the FVC will be decreased, not increased. Obstruction will lead to all expiratory flows being low. Air trapping will cause the RV to be increased.

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. 2, 3, 4

ANS: C Patients with cystic fibrosis have thick, tenacious mucus. This can lead to either a partial airway obstruction (and hyperinflation) or total airway obstruction (and atelectasis). Bronchospasm is only occasionally found.

Which of the following can be used in the diagnosis of cystic fibrosis? 1. An elevated potassium level in the sweat 2. An elevated chloride level in the sweat 3. Genetic testing of the parents 4. An elevated blood level of immunoreactive trypsin a. 1 b. 2, 3 c. 2, 3, 4 d. 1, 2, 3

ANS: C There is no change in the potassium level in the sweat. All of the other listed options can be used to help in the diagnosis of a person with cystic fibrosis.

Cystic fibrosis patients can have: 1. malnutrition. 2. a meconium ileus (bowel obstruction). 3. excessive, viscous pulmonary secretions. 4. a tendency for status asthmaticus. a. b. c. d. 1 2, 3 2, 3, 4 1, 2, 3

ANS: D Asthma is a separate condition that is not associated with cystic fibrosis. All of the other listed options can be found in patients with cystic fibrosis.

Which of the following are recommended in children with cystic fibrosis? 1. Systemic corticosteroids 2. High-dose ibuprofen 3. Inhaled antibiotics 4. Inhaled DNase (Dornase alpha) (Pulmozyme) a. 1, 3 b. 1, 2, 4 c. 1, 2, 3 d. 2, 3, 4

ANS: D Systemic glucocorticoids are not recommended in children and adolescents with CF. The benefits of systemic glucocorticoids are outweighed by the adverse effects on growth retardation, glucose metabolism, development of CF-related diabetes, and cataract risks. High-dose ibuprofen is recommended in children and young adolescents with mild CF. Ibuprofen has been shown to reduce bronchial inflammation without hindering bacterial clearance. Antibiotics are commonly administered to prevent or combat chronic respiratory tract infections. Inhaled DNase (Dornase alpha) (Pulmozyme) has been shown to be especially helpful in the management of patients with moderate to severe CF. This aerosolized agent is an enzyme that breaks down the DNA of the thick bronchial mucus associated with chronic bacterial infections with CF.

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

ANS: D The bacteria P. aeruginosa, H. influenza, and S. aureus are commonly found in the mucus of patients with cystic fibrosis. Although other bacteria are also found, Klebsiella is not among them.

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

B. 2 and 4

Cystic fibrosis is a gene disorder caused by mutations in a pair of genes located on chromosome ______. a. 3 b. 7 c. 21 d. 23

B. 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

C. Spontaneous pneumothorax

Which ethnic group has the greatest number of people with cystic fibrosis? a. African-Americans b. Asians c. Hispanics d. Caucasians

D) Caucasians

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

D) Lung transplant

Which of the following may be recommended to reduce bronchial inflammation in a 10-year-old child with cystic fibrosis? a. High-dose ibuprofen b. High-dose acetaminophen c. High-dose naproxen d. High-dose corticosteroids

High-dose ibuprofen

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)

Tobramycin (TOBI)

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%

c. 50%