CF

Question 1

What is cystic fibrosis (CF)?

Answer 1

An autosomal recessive defect that primarily affects the lungs, leading to progressive pulmonary involvement and increased morbidity and mortality.

Question 2

What is the primary cause of cystic fibrosis?

Answer 2

Defects in the CF transmembrane conductance regulator (CFTR).

Question 3

What is the function of the CFTR protein?

Answer 3

It is a cyclic adenosine monophosphate (cAMP)-regulated chloride channel expressed on airway epithelial cells.

Question 4

How does defective CFTR affect airway function?

Answer 4

Leads to reduced chloride conductance, upregulation of ENaC activity, and impaired mucociliary clearance.

Question 5

What are the initial bacterial infections commonly found in CF patients?

Answer 5

Staphylococcus aureus and Haemophilus influenzae.

Question 6

What is the significance of mucoid Pseudomonas aeruginosa in CF?

Answer 6

It is associated with chronic infection and poorer prognosis.

Question 7

True or False: Invasive fungal infections are common in cystic fibrosis patients.

Answer 7

False. Invasive fungal infections are rare.

Question 8

What role do neutrophils play in CF lung disease?

Answer 8

They contribute to airway inflammation and release proteases that can damage airway structures.

Question 9

What is bronchiectasis?

Answer 9

A predominant pathological feature of CF characterized by abnormal dilation of the bronchi.

Question 10

List some common symptoms of CF lung disease.

Answer 10

• Cough
• Exercise intolerance
• Dyspnea
• Digital clubbing

Question 11

What is the treatment for pulmonary exacerbation in CF?

Answer 11

Aggressive airway clearance techniques and antibiotic therapy.

Question 12

What is the typical treatment for nasal polyposis in CF?

Answer 12

Systemic antibiotic therapy and possibly surgical intervention.

Question 13

What is allergic bronchopulmonary aspergillosis (ABPA)?

Answer 13

An inflammatory complication associated with CF, characterized by an intense immunologic response to Aspergillus fumigatus.

Question 14

Fill in the blank: The primary symptom of CF lung disease is _______.

Answer 14

Cough

Question 15

What does a decline in FEV1 indicate in CF patients?

Answer 15

It is a key marker for disease progression.

Question 16

What is cor pulmonale?

Answer 16

A complication of hypoxemia commonly seen in older patients with advanced cystic fibrosis.

Question 17

What are the common imaging studies used to monitor lung disease in CF?

Answer 17

Chest imaging studies, including high-resolution computed tomography (HRCT).

Question 18

What is the average annual reduction in FEV1 for CF patients in the United States?

Answer 18

Approximately 2% per year.

Question 19

True or False: Blood gas measurements are typically abnormal early in CF disease.

Answer 19

False. Blood gas measurements are normal until late in the disease.

Question 20

What is the significance of high levels of proinflammatory cytokines in CF patients?

Answer 20

They are found even in the absence of detectable infection and contribute to airway inflammation.

Question 21

What are the potential outcomes of chronic inflammation in the CF lung?

Answer 21

Bronchiectasis, airway obstruction, and increased susceptibility to infections.

Question 22

What is the role of airway clearance techniques in CF management?

Answer 22

They help mobilize purulent secretions and reduce bacterial burden.

Question 23

What is the relationship between CF and exercise tolerance?

Answer 23

Exercise intolerance and dyspnea are seen as lung disease progresses.

Question 24

List some complications associated with advanced CF lung disease.

Answer 24

• Hemoptysis
• Pneumothorax
• Cor pulmonale

Question 25

What is the characteristic finding in HRCT for CF patients?

Answer 25

Progressive bronchiectasis and other abnormalities before detection on plain radiographs.

Question 26

What is a useful index of disease severity in cystic fibrosis?

Answer 26

Rate of FEV1 decline ## Footnote Deteriorating FEV1 is a key marker for disease progression.

Question 27

What is the average annual reduction in FEV1 in the United States?

Answer 27

Approximately 2% per year

Question 28

What is the 2-year mortality rate for CF patients with a baseline FEV1 of 30% of predicted for age?

Answer 28

50%

Question 29

When do blood gas measurements typically show abnormalities in cystic fibrosis patients?

Answer 29

Late in the course of disease

Question 30

What is related to worsening ventilation-perfusion inequalities in advanced lung disease?

Answer 30

Gas exchange abnormalities

Question 31

What type of assessments are regularly conducted for CF patients?

Answer 31

Comprehensive microbiological assessments of specimens from the lower respiratory tract

Question 32

True or False: Negative oropharyngeal cultures can exclude lower airway infections.

Answer 32

True

Question 33

What predicts the development of bronchiectasis in CF patients?

Answer 33

Presence of detectable neutrophil elastase in lavage fluid early in life

Question 34

Are inflammatory markers in the blood useful for assessing lung disease in CF?

Answer 34

No

Question 35

What are the components of current management for cystic fibrosis?

Answer 35

* Antibiotics * Inhaled mucolytics * Vigorous airway clearance * Nutritional support * Antiinflammatory agents * Small molecule potentiators and correctors

Question 36

What is the purpose of airway clearance in CF management?

Answer 36

To relieve airway obstruction, reduce infection, and decrease airway inflammation

Question 37

Name one active airway clearance therapy (ACT).

Answer 37

* Positive expiratory pressure * Active-cycle-of-breathing technique * Autogenic drainage

Question 38

What adjunct to airway clearance techniques can mobilize secretions?

Answer 38

Aerobic exercise

Question 39

Fill in the blank: Hypertonic saline acts directly as an _______.

Answer 39

osmotic agent

Question 40

What is the effect of inhaled mannitol in CF treatment?

Answer 40

Improves lung function

Question 41

What does inhaled recombinant DNAse do?

Answer 41

Reduces sputum viscosity and increases mucus clearance

Question 42

What is N-acetyl-L-cysteine's role in CF treatment?

Answer 42

It hydrolyzes disulfide bonds in mucins

Question 43

In what settings is antibiotic therapy utilized for CF patients?

Answer 43

* Acute infections * Pulmonary exacerbations * Eradication of organisms in asymptomatic young children

Question 44

What is the most common combination of intravenous therapy for P. aeruginosa infections?

Answer 44

Aminoglycoside with a β-lactam antibiotic

Question 45

What must be monitored in CF patients receiving systemic aminoglycosides?

Answer 45

Drug concentrations

Question 46

What is the recommended approach for infections caused by nontuberculous mycobacteria in CF patients?

Answer 46

First treat other organisms found on culture

Question 47

What is Ivacaftor?

Answer 47

First FDA-approved therapy to address the basic defect in CF

Question 48

What is the effect of high-dose ibuprofen in CF treatment?

Answer 48

Slows the annual rate of decline in lung function

Question 49

What vaccination is recommended annually for CF patients?

Answer 49

Influenza vaccine

Question 50

What are the contraindications to lung transplantation in CF patients?

Answer 50

* Presence of B. cenocepacia * Multiorgan failure

Question 51

What is an emerging therapy for cystic fibrosis?

Answer 51

Somatic gene therapy

Question 52

What factors negatively affect prognosis in cystic fibrosis?

Answer 52

* Malnutrition * Diabetes mellitus * Infection with P. aeruginosa * B. cepacia complex * Frequency of pulmonary exacerbations

Question 53

True or False: Patients with atypical forms of CF have a slower decline in pulmonary function.

Answer 53

True