Airway Disorders

Question 1

What is the most common cause of severe chronic lung disease in young adults?

Answer 1

Cystic Fibrosis (CF)

Question 2

What is the hallmark lab finding in all CF patients?

Answer 2

Sweat Cl >60 on TWO occasions

Question 3

What is the typical CF pt look like (general appearance)?

Answer 3

Young patient who looks malnourished or who has a low BMI

Question 4

What is pathophysiology behind CF?

Answer 4

Its a genetic disorder that causes mucous buildup and tissue damage in the lungs due to abnormalities in a membrane chloride channel.

Question 5

Where do you hear the crackles in a CF patient? apical or base?

Answer 5

apical crackles

Question 6

Is there a cure for CF?

Answer 6

NO, you just basically treat symptoms and REFER to CF center!

Question 7

What are some symptoms that CF patients present with?

Answer 7

Chronic PRODUCTIVE cough
Dyspnea
Wheezing
low exercise tolerance 
Hemoptysis 
Chronic rhinosinusitis sx 
steatorrhea, diarrhea, abdominal pain

Question 8

What disease is caused by abnormal dilation and destruction of the bronchial walls?

Answer 8

Bronchiectasis

Question 9

Bronchiectasis favors M or W?

Answer 9

Women!

Question 10

What are some causes of Bronchiectasis?

Answer 10

CF!!, Obstruction, Infection

Question 11

Where do you hear the crackles in a patient with Bronchiectasis?

Answer 11

Bronchiectasis = BASE

Question 12

Patients with bronchiectasis have similar symptoms to those with CF, what is a unique sx of bronchiectasis?

Answer 12

FOUL smelling sputum

Question 13

What imaging modality best diagnoses Bronchiectasis?

Answer 13

HIGH RES CT!

Question 14

When you see imaging descriptions such as “tram tracks”, “ring shadow” or “gloved finger” you should think of what disease as part of your differential?

Answer 14

Bronchiectasis

Question 15

How do you treat Bronchiectasis?

Answer 15

EMPIRIC ABX- Amoxicillin
Inhaled bronchodilators - to help them breath due to mucous accumulation
HYDRATE
VACCINE

Question 16

Stridor is best heard on expiration or inspiration?

Answer 16

Inspiration, best over upper chest and back

Question 17

in severe bronchiectasis, what PE finding is always present?

Answer 17

Clubbing

Question 18

What is is the most common organism recovered from non-cystic fibrosis patients with bronchiectasis?

Answer 18

H. Flu –> this is why you need the vaccine in the management

Question 19

T/F. Cystic fibrosis patients have an increased risk of malignancies of the GI tract, osteopenia, and arthropathies?

Answer 19

true

Question 20

What are non-pulmonary disorders that are commonly seen in patients presenting with undiagnosed CF?

Answer 20

Pancreatitis and infertility

Question 21

Nearly all men with CF have congenital bilateral absence of what?

Answer 21

vas deferens with azospermia (remember infertility!)

Question 22

What kind of pattern is seen on PFTs with CF?

Answer 22

mixed obstructive and restrictive pattern

Question 23

What is the only definitive treatment for advanced CF?

Answer 23

lung transplant

Question 24

Beside the seasonal flu shot which other vaccine is recommended in CF patients?

Answer 24

pneumococcal.

Question 25

Death from cystic fibrosis is a result of:

Answer 25

pulmonary complications (pneumonia, pneumothorax, hemoptysis), or terminal respiratory failure and cor pulmonale.

Question 26

T/F. CF have a smaller incidence of atypical mycobacterial colonization.

Answer 26

False. Yearly screening with sputum acid-fast bacilli cultures is advised

Question 27

2nd highest death rate of CF, behind caucasians?

Answer 27

American Indians