Deck 5: Cystic Fibrosis

Question 1

What finding during chest auscultation would suggest a more advanced case of cystic fibrosis?

A)Expiratory wheezing
B)Loud P-2
C)Coarse crackles
D)A gallop rhythm

Answer 1

B

Question 2

What finding is common in patients with cystic fibrosis during the general examination?

A)Patient appears malnourished
B)Patient appears younger than their stated age
C)Patient appears to be in pain
D)Patient appears to be sleepy

Answer 2

A

Question 3

Which of the following symptoms is suggestive of a new respiratory infection in the patient with cystic fibrosis?

A)New onset of cough
B)Sudden decrease in FEV1
C)Lack of expected weight gain
D)All the above

Answer 3

D

Question 4

If your patient with cystic fibrosis has bilateral polyphonic wheezing,what therapy would you initially recommend?

A)Inhaled albuterol
B)Inhaled corticosteroids
C)Oral aminophylline
D)Inhaled atropine

Question 5

Why are patients with cystic fibrosis frequently malnourished?

A)Pancreatic exocrine insufficiency
B)Inadequate food intake
C)High metabolic demand from infections
D)All the above

Question 6

Which of the following statements is most true regarding the prognosis of cystic fibrosis patients today?

A)Most don't live past the age of 15 years
B)Many now live past the age of 30 years
C)The prognosis has not changed in the past several decades
D)The prognosis varies with age of diagnosis

Question 7

Your patient with cystic fibrosis appears to have a pulmonary infection.What organism is most likely involved?

A)Pseudomonas aeruginosa
B)Klebsiella
C)Staphyolococcus aureus
D)E.coli

Question 8

Which of the following pulmonary problem(s)is/are seen in cystic fibrosis patients with an advanced stage of the disease?

A)Cor pulmonale
B)Respiratory failure
C)Severe hemoptysis
D)All the above

Question 9

Common pulmonary problems associated with cystic fibrosis include all the following except:

A)Bronchiectasis
B)Pneumonia
C)Bronchial hyperactivity
D)Pleural effusion

Question 10

What is the most significant health threat to patients with cystic fibrosis?

A)Pancreatic fibrosis
B)Recurrent pulmonary infections
C)Bowel obstructions
D)Sepsis

Question 11

What finding on the chest radiograph is NOT typical for patients with cystic fibrosis?

A)Tram tracks
B)Hilar adenopathy
C)Enlarged heart
D)Atelectasis

Question 12

What are the chances that a couple will have a child with cystic fibrosis if both parents are carriers of the gene?

A)1:2
B)1:4
C)1:8
D)1:16

Question 13

What sweat chloride concentration is associated with the diagnosis of cystic fibrosis in children?

A)Greater than 40 mEq/L
B)Greater than 60 mEq/L
C)Greater than 80 mEq/L
D)Greater than 100 mEq/L

Question 14

Which of the following upper airway problems is common in patients with cystic fibrosis?

A)Nasal polyps
B)Sinusitis
C)Deviated septum
D)A and B only

Question 15

What therapy has been shown to significantly slow the progression of lung disease in cystic fibrosis patients?

A)Chest physical therapy
B)Aerosolized antibiotics
C)Ibuprofen
D)Oral corticosteroids

Question 16

Which of the following pulmonary function results is NOT typical for cystic fibrosis?

A)Reduced FEV1
B)Reduced FVC
C)Reduced RV
D)Reduced expiratory flows

Question 17

What therapy has been shown to reduce sputum viscosity in the number of exacerbations in patients with cystic fibrosis?

A)Use of mucomyst
B)Aerosolized saline
C)Use of bronchoscopy to clear retained mucus
D)Use of DNase

Question 18

What is the medical problem that most cystic fibrosis patients first seek help for?

A)Recurrent pulmonary infections
B)Frequent episodes of hyperglycemia
C)Bowel obstructions
D)Weight loss

Question 19

What is the most common abnormality found on the arterial blood gas study in the patient with mild cystic fibrosis?

A)Mild hypoxemia
B)Mild respiratory alkalosis
C)Mild respiratory acidosis
D)Metabolic acidosis

Question 20

Your patient with cystic fibrosis is having frequent pulmonary infections and her doctor asks you for a suggestion on therapy that might help reduce the frequency of these exacerbations.What do you suggest?

A)Better hydration for the patient
B)Aerosolized tobramycin
C)Home chest physical therapy
D)A home humidifier