CF and Bronchiectasis

Question 1

abnormally functioning chloride channels that leads to altered chloride transport and water movement, with increased NaCl in sweat and decreased NaCl everywhere else

Answer 1

cystic fibrosis

Question 2

in which unscreened patients should CF be suspected? (3)

Answer 2

Hx of chronic lung disease (bronchiectasis)
pancreatitis
infertility

Question 3

a patient presents with cough + sputum, decreased exercise tolerance, recurrent hemoptysis, chronic rhinosinusitis, steatorrhea, diarrhea, chronic abdominal pain, malnourished, digital clubbing, increased anteroposterior chest diameter, hyperresonance to percussion, and apical crackles. what are they likely experiencing?

Answer 3

cystic fibrosis

Question 4

what are 5 diagnostics that I can order for a patient with CF?

Answer 4

blood gas
spirometry
chest CT
sweat test
DNA genotyping

Question 5

what would a blood gas in a CF patient present? (2)

Answer 5

hypoxemia
compensated respiratory acidosis

Question 6

what would a spirometry show in a patient with CF?

Answer 6

mixed obstructive/restrictive

Question 7

a patient with CF shows what in their chest CT? (5)

Answer 7

hyperinflation
peribronchial thickening
mucus plugging
focal atelectasis
peripheral opacities (mucus)

Question 8

what would be required if a suspected CF patient tests negative on the sweat test?

Answer 8

DNA genotyping

Question 9

at a CF center, what is the treatment for a patient 6 years or older? (4)

Answer 9

chest physiotherapy
inhaled hypertonic solution
dornase alfa (mucolytic)
bronchodilators

Question 10

what is the treatment for a patient with CF who has bacteria / respiratory tract infections?

Answer 10

inhaled tobramycin

Question 11

what is the only treatment in CF patients with end-stage pulmonary disease?

Answer 11

lung transplantation

Question 12

irreversible airway dilation (chronic inflammation) involving the lung in focal or diffuse manner, and can be infectious or noninfectious

Answer 12

bronchiectasis

Question 13

what causes 50% of bronchiectasis cases?

Answer 13

cystic fibrosis

Question 14

a patient presents with chronic cough, copious purulent sputum, hemoptysis, pleuritic chest pain, weight loss, foul-smelling breath, crackles/rales, anemia, hypoxemia, and nail clubbing. what are they likely experiencing?

Answer 14

bronchiectasis

Question 15

what 4 diagnostics should be order for a patient with suspected bronchiectasis?

Answer 15

CBC
CXR
Chest CT
Flu test

Question 16

what would a CBC show in a patient with bronchiectasis?

Answer 16

decreased Hb and Hct due to hemoptysis

Question 17

what would a chest xray show in a patient with bronchiectasis? (3)

Answer 17

ring-like markings / tram tracks
cysts
increased interstitial markings

Question 18

what would a Chest CT of a patient with bronchiectasis show?

Answer 18

dilated bronchi

Question 19

what is the most common organism recovered in microbio testing of bronchiectasis?

Answer 19

H. flu

Question 20

what are the 4 basic treatments for a patient with bronchiectasis?

Answer 20

antibiotics
chest physiotherapy
chest bronchoscopy
surgical resection

Question 21

what antibiotics can be given to a patient with bronchiectasis? (3)

Answer 21

amoxicillin
augmentin
ciprofloxacin

Question 22

what medication can be used prophylactically for patients with bronchiectasis to prevent future infections?

Answer 22

macrolide

Question 23

when can surgical resection be used in a patient with bronchiectasis?

Answer 23

when bronchiectasis is localized

Question 24

what are 3 complications of bronchiectasis?

Answer 24

anemia
cor pulmonale
visceral abscess