Question 1

What finding during chest auscultation would suggest a more advanced case of cystic fibrosis?&#10;A)Expiratory wheezing&#10;B)Loud P-2&#10;C)Coarse crackles&#10;D)A gallop rhythm

Accepted Answer

Loud P-2 is a sign of advanced cystic fibrosis, as it indicates the presence of thick, tenacious secretions in the airways.

Question 2

What finding is common in patients with cystic fibrosis during the general examination?&#10;A)Patient appears malnourished&#10;B)Patient appears younger than their stated age&#10;C)Patient appears to be in pain&#10;D)Patient appears to be sleepy

Accepted Answer

Patients with cystic fibrosis often have malnutrition due to poor absorption of nutrients. This may present as weight loss, muscle wasting, and overall physical weakness.

Question 3

Which of the following symptoms is suggestive of a new respiratory infection in the patient with cystic fibrosis?&#10;A)New onset of cough&#10;B)Sudden decrease in FEV1&#10;C)Lack of expected weight gain&#10;D)All the above

Accepted Answer

A new onset of cough, sudden decrease in FEV1, and lack of expected weight gain are all suggestive of a new respiratory infection in a patient with cystic fibrosis. It is important to promptly identify and treat any new infections to prevent further lung damage and deterioration.

Question 4

If your patient with cystic fibrosis has bilateral polyphonic wheezing,what therapy would you initially recommend?&#10;A)Inhaled albuterol&#10;B)Inhaled corticosteroids&#10;C)Oral aminophylline&#10;D)Inhaled atropine

Accepted Answer

Polyphonic wheezing indicates airway narrowing which can be improved by inhaled bronchodilators such as albuterol. Inhaled corticosteroids may be used if there is evidence of chronic airway inflammation, but they are not the first-line therapy for acute wheezing. Oral aminophylline has a narrow therapeutic window and potential for toxicity, and is not recommended as first-line therapy. Inhaled atropine is not commonly used for respiratory conditions and can worsen airway narrowing.

Question 5

Why are patients with cystic fibrosis frequently malnourished?&#10;A)Pancreatic exocrine insufficiency&#10;B)Inadequate food intake&#10;C)High metabolic demand from infections&#10;D)All the above

Accepted Answer

Patients with cystic fibrosis can have pancreatic exocrine insufficiency, which means their pancreas cannot produce enough enzymes to break down food and absorb nutrients properly. Additionally, cystic fibrosis can increase metabolic demand from frequent infections, leading to increased calorie needs. These factors, along with other variables such as difficulty digesting fat, can result in inadequate food intake and ultimately malnutrition. Therefore, all of the options (A, B, and C) contribute to the frequent malnourishment seen in cystic fibrosis patients.

Question 6

Which of the following statements is most true regarding the prognosis of cystic fibrosis patients today?&#10;A)Most don't live past the age of 15 years&#10;B)Many now live past the age of 30 years&#10;C)The prognosis has not changed in the past several decades&#10;D)The prognosis varies with age of diagnosis

Accepted Answer

With advancements in treatment options, many cystic fibrosis patients are now able to live past the age of 30 years. While the prognosis still varies based on the age of diagnosis and severity of the disease, overall, the outlook for patients has improved greatly in recent years. Option A is not correct because cystic fibrosis patients are now living longer than 15 years. Option C is not correct because the prognosis has improved over the years. Option D is partially correct, but B is the best choice because it reflects the overall positive trend in cystic fibrosis prognosis.

Question 7

Your patient with cystic fibrosis appears to have a pulmonary infection.What organism is most likely involved?&#10;A)Pseudomonas aeruginosa&#10;B)Klebsiella&#10;C)Staphyolococcus aureus&#10;D)E.coli

Accepted Answer

Pseudomonas aeruginosa is a common organism found in the respiratory tract of patients with cystic fibrosis and is associated with chronic pulmonary infections. It is often resistant to many antibiotics, making treatment challenging. Klebsiella and E.coli are not commonly associated with pulmonary infections in patients with cystic fibrosis. Staphylococcus aureus can also be seen in these patients but is less common compared to Pseudomonas aeruginosa.

Question 8

Which of the following pulmonary problem(s)is/are seen in cystic fibrosis patients with an advanced stage of the disease?&#10;A)Cor pulmonale&#10;B)Respiratory failure&#10;C)Severe hemoptysis&#10;D)All the above

Accepted Answer

In advanced stages of cystic fibrosis, patients are at an increased risk of developing cor pulmonale, respiratory failure, and severe hemoptysis due to the chronic pulmonary infections and inflammation. Therefore, all the options are correct.

Question 9

Common pulmonary problems associated with cystic fibrosis include all the following except:&#10;A)Bronchiectasis&#10;B)Pneumonia&#10;C)Bronchial hyperactivity&#10;D)Pleural effusion

Accepted Answer

Pleural effusion is not commonly associated with cystic fibrosis. The other three options, bronchiectasis, pneumonia, and bronchial hyperactivity, are frequently seen in patients with cystic fibrosis.

Question 10

What is the most significant health threat to patients with cystic fibrosis?&#10;A)Pancreatic fibrosis&#10;B)Recurrent pulmonary infections&#10;C)Bowel obstructions&#10;D)Sepsis

Accepted Answer

Recurrent pulmonary infections are the most significant health threat to patients with cystic fibrosis, as they can lead to chronic lung damage and respiratory failure. While pancreatic fibrosis, bowel obstructions, and sepsis can also occur in patients with cystic fibrosis, they are not typically the primary cause of mortality or morbidity in this population.

Question 11

What finding on the chest radiograph is NOT typical for patients with cystic fibrosis?&#10;A)Tram tracks&#10;B)Hilar adenopathy&#10;C)Enlarged heart&#10;D)Atelectasis

Accepted Answer

The answer of What finding on the chest radiograph is...

Question 12

What are the chances that a couple will have a child with cystic fibrosis if both parents are carriers of the gene?&#10;A)1:2&#10;B)1:4&#10;C)1:8&#10;D)1:16

Accepted Answer

The answer of What are the chances that a couple...

Question 13

What sweat chloride concentration is associated with the diagnosis of cystic fibrosis in children?&#10;A)Greater than 40 mEq/L&#10;B)Greater than 60 mEq/L&#10;C)Greater than 80 mEq/L&#10;D)Greater than 100 mEq/L

Accepted Answer

The answer of What sweat chloride concentration is associated with...

Question 14

Which of the following upper airway problems is common in patients with cystic fibrosis?&#10;A)Nasal polyps&#10;B)Sinusitis&#10;C)Deviated septum&#10;D)A and B only

Accepted Answer

The answer of Which of the following upper airway problems...

Question 15

What therapy has been shown to significantly slow the progression of lung disease in cystic fibrosis patients?&#10;A)Chest physical therapy&#10;B)Aerosolized antibiotics&#10;C)Ibuprofen&#10;D)Oral corticosteroids

Accepted Answer

The answer of What therapy has been shown to significantly...

Question 16

Which of the following pulmonary function results is NOT typical for cystic fibrosis?&#10;A)Reduced FEV1&#10;B)Reduced FVC&#10;C)Reduced RV&#10;D)Reduced expiratory flows

Accepted Answer

The answer of Which of the following pulmonary function results...

Question 17

What therapy has been shown to reduce sputum viscosity in the number of exacerbations in patients with cystic fibrosis?&#10;A)Use of mucomyst&#10;B)Aerosolized saline&#10;C)Use of bronchoscopy to clear retained mucus&#10;D)Use of DNase

Accepted Answer

The answer of What therapy has been shown to reduce...

Question 18

What is the medical problem that most cystic fibrosis patients first seek help for?&#10;A)Recurrent pulmonary infections&#10;B)Frequent episodes of hyperglycemia&#10;C)Bowel obstructions&#10;D)Weight loss

Accepted Answer

The answer of What is the medical problem that most...

Question 19

What is the most common abnormality found on the arterial blood gas study in the patient with mild cystic fibrosis?&#10;A)Mild hypoxemia&#10;B)Mild respiratory alkalosis&#10;C)Mild respiratory acidosis&#10;D)Metabolic acidosis

Accepted Answer

The answer of What is the most common abnormality found...

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

Accepted Answer

The answer of Your patient with cystic fibrosis is having...

Deck 5: Cystic Fibrosis