Bronchiectasis & CF

Question 1

Bronchiectasis

Answer 1

Bronchiectasis is an abnormal,
permanent, irreversible dilatation of
the bronchi with destruction of the
bronchial walls

Question 2

It is a chronic lung condition that
results from recurrent infection or
inflammation of the airways

Answer 2

Bronchiectasis

Question 3

Bronchiectasis etiology

Answer 3

○ About half of all cases are due to Cystic Fibrosis
○ Chronic or recurrent significant lung infection is a major cause
■ Tuberculosis
○ Immunodeficiencies with associated lung insult
○ Alpha-1-antitrypsin deficiency
○ Rheumatic disease (Rheumatoid Arthritis, Sjogren syndrome)
○ Localized airway obstruction (tumor, mucoid impaction, etc.)

Question 4

Pathophysiology of Bronchiectasis

Answer 4

○ Chronic or recurrent significant infection leads to copious amounts of mucus production in the larger airways.
○ Thick mucus causes chronic large airway obstruction and aggressive inflammatory
response in bronchial walls.
○ Eventually, bronchial dilatation and destruction occur with cysts and mucus

Question 5

Bronchiectasis clinical presentation

Answer 5

■ Chronic, copious purulent sputum (nearly 100% of cases)
● Often foul-smelling
■ Chronic cough
■ Dyspnea (75%)
■ Wheezing (75%)
■ Hemoptysis
■ Pleuritic chest pain
■ Recurrent infections (like pneumonia)

Question 6

PE for Bronchiectasis

Answer 6

■ Crackles at the lung base
■ Clubbing (infrequent until severe disease)

Question 7

Bronchiectasis diagnosis

Answer 7

○ High-resolution CT scan is the diagnostic test of choice, although classic findings are often initially seen on CXR.
○ Imaging will reveal dilated, tortuous airways.
■ Crowded bronchial markings
■ Cystic spaces
■ Tram-Track markings

Question 8

Bronchiectasis Diagnosis- Microbial Cultures:

Answer 8

○ Recurrent bacterial infections are very
common as the copious amounts of mucus
create a warm, moist growth environment.
○ Pseudomonas aeruginosa is commonly
cultured from those with Cystic Fibrosis
○ Haemophilus influenzae is the most common
microbe recovered from non-CF patients

Question 9

Bronchiectasis Treatment

Answer 9

○ Bronchoscopy is sometimes necessary to evaluate hemoptysis
and stop acute bleeding, as well as to remove retained secretions
○ Acute exacerbations are treated with a combo of the following:
■ Supplemental O2 PRN for hypoxemia
■ Antibiotics (preferably based on sputum culture, empiric if not)
● Augmentin, Ceftriaxone, Doxycycline, or a Fluoroquinolone
■ Daily chest physiotherapy
■ Inhaled bronchodilators
■ Mucolytics
○ Preventive or suppressive medications are sometimes given

Question 10

Some significant complications of Bronchiectasis include:

Answer 10

■ Hemoptysis
■ Pulmonary hypertension
■ Cor pulmonale
■ Secondary visceral abscesses in distant sites
○ Lung transplant may be an option for some patients in severe,
late disease

Question 11

Cystic Fibrosis

Answer 11

an autosomal recessive genetic disorder that results in
abnormal production of mucus.
PULM-CF-1
○ This occurs in almost all exocrine glands, causing
obstruction of those glands and ducts

Question 12

the most common chronic lung disease in young adults

Answer 12

CF

Question 13

Cystic Fibrosis inheritance

Answer 13

○ CF is inherited in an autosomal recessive,
mendelian-type fashion.
○ Because it is recessive, both mom and dad have to be carriers of the mutation.
○ With each pregnancy, there is a 50% chance the child will be a carrier too, and a 25%
chance they will have CF

Question 14

Pathophysiology of CF

Answer 14

○ There are over 2000 known genetic mutations for the gene that encodes for CFTR protein
○ The most common mutation (Delta F508)
accounts for a huge portion of CF cases.
○ The CFTR protein is part of a membrane
chloride channel.
○ Because of the abnormality in chloride transport, nearly all exocrine glands produce an abnormally high amount of mucus
○ The thick mucus blocks glands and ducts, which eventually leads to tissue damage.
○ In the respiratory tract, inadequate hydration of the tracheobronchial epithelium leads to impaired ciliary function and high sputum viscosity.
○ This leads to chronic airway inflammation and recurrent infections, ultimately resulting in Bronchiectasis

Question 15

Cystic Fibrosis clinical presentation (children)

Answer 15

■ Intestinal obstruction at birth (Meconium Ileus)
■ Failure to thrive
■ Respiratory compromise
■ Recurrent respiratory infections
■ Chronic cough with rales or rhonchi
■ Nasal polyps
■ Sinus tenderness or purulent nasal discharge

Question 16

Cystic Fibrosis diagnosis

Answer 16

○ The diagnostic test of choice is a Sweat Chloride test.
■ Quantitative Pilocarpine Iontophoresis Sweat Test
○ The test will reveal elevated Na and Cl
levels in the sweat (> 60 mEq/L).
○ Two tests performed on different
days are required for accuracy.
○ A negative test does not
completely exclude the Dx.
■ Would move on to genetic
testing if high suspicion

Question 17

In advanced disease of Cystic fibrosis, ABG may reveal ____

Answer 17

compensated
respiratory acidosis

Question 18

The most common mutation in CF

Answer 18

This is that Delta F508 (AKA “F508del”)

Question 19

Cystic Fibrosis treatment:

Answer 19

○ The majority of treatment is focused
on lung preservation, respiratory infection prevention, and pancreatic enzyme supplementation.
○ Every patient diagnosed with CF should be referred to the Regional CF Center (ours is in Salt Lake City).
○ Several daily medications and daily
chest physiotherapy is the normal.

Question 20

Cystic fibrosis Mgt (pharmacology)

Answer 20

○ Mucolytics are generally given twice daily as a nebulized breathing treatment during chest physiotherapy.
○ Short-term Abx are used to treat active airway infection based on culture and sensitivity testing.
○ Inhaled bronchodilators (such as albuterol) should be considered in patients who demonstrate an increase of at least
12% in FEV1 after an inhaled bronchodilator
○ An inhaled corticosteroid should be added to the treatment regimen for patients who have cystic fibrosis with persistent
asthma or allergic bronchopulmonary mycosis
○ Long-term antibiotic therapy is sometimes utilized in patients
who are frequently positive for Pseudomonas

Question 21

Vaccinations and mortality in CF patients

Answer 21

○ Vaccination against pneumococcal,
COVID, and influenza are very important.
○ Death in CF occurs from pneumonia,
pneumothorax, hemoptysis, or as a result
of chronic respiratory failure and cor
pulmonale

Question 22

Lung transplant survival in pts with cystic fibrosis

Answer 22

○ Lung Transplantation is the only
definitive treatment for advanced CF lung
disease.
■ The 3-year survival rate after the
procedure is about 55%

Question 23

When a Sweat Chloride Test is found to be positive for a pt with cystic fibrosis, the patient should be sent for _____

Answer 23

CFTR genetic testing