Cystic Fibrosis

Question 1

CF

Answer 1

• autosomal recessive
• abnl sweat and thick mucus from exocrine glands
• sweat rich in Na, K, Cl (>60 meq/l)
• pulm fibrosis, restrictive lung dz

Question 2

pulm probs of CF

Answer 2

• persistent bronchial infections, irreversible fibrotic changes
• airway obstruction + respiratory insufficiency
• atelectasis, empyema, abscess, formation, PNA, PTX, cor pulmonale, COPD
• wheezing, decreased pulmonary compliance

Question 3

GI probs of CF

Answer 3

-pancreatic obstruction
(Vit A deficiency=inc ICP, Vit E deficiency=hemolysis and short RBC life)
-biliary tract obstruction (poor absorption Vit K, abnl hemostasis)
-intestinal obstruction in infancy (mucus in gut)

Question 4

Preop surgery

Answer 4

• PFTs, ABGs
• PT/PTT-severity of biliary obstruction, clotting status
• EKG-p waves-cor pulmonale

Question 5

premed

Answer 5

-atropine decreases secretions, anti-vagal, bronchodilator

Question 6

Intraop

Answer 6

• volatile anesthetics=bronchodilation

- Nitrous oxide-avoid if corpulmonale or large pulmonic blebs