CF and Bronchiectasis

Question 1

Cause of Cystic Fibrosis

Answer 1

autosomal recessive disorder of the exocrine gland

Question 2

Pathophysiology of CF

Answer 2

Affects plasma membrane chloride/sodium channels of epithelia cells

Question 3

Common bacteria in CF patients (resides in biofilm)

Answer 3

Pseudomonas aeruginosa

Question 4

Presentation of CF

Answer 4

Persistant, purulent cough

Dyspnea w/ hypoxia

Chronic sinusitis

Persistant wheezing

Recurrent PNA

Diabetes and weight loss

Question 5

CF spirometry readings:

Answer 5

Initially obstructive and then restrictive once dz progresses

Inc progression/age –> dec FEV1

Inc. RV/TLC d/t hyperinflation and bronchectasis

Question 6

What is the primary diagnostic test for CF?

Answer 6

Skin sweat test

> 60 mEq/L

Question 7

Tx mild CF exaccerbation

Answer 7

Oral abx

Question 8

Tx severe exacerbation

Answer 8

IV abx

Question 9

Preventions for CF

Answer 9

Inhaled tobramycin

Exercise

Chest physiotherapy

PEP mask

Inhaled DNAse

Hypertonic saline inhalation

Vaccines

Question 10

What is bronchiectasis

Answer 10

Chronic dilation of the bronchi or bronchioles as a consequence of inflammatory/mucous obstruction of the airways