Cystic Fibrosis Flashcards
1
Q
What are the common symptoms of cystic fibrosis in infant/neonates?
A
- meconium ileus
- prolonged jaundiced
- pancreatic insufficiency
- steatorrhea (fatty diarrrhea)
- failure to thrive
2
Q
What are the common symptoms of cystic fibrosis in childhood?
A
- anemia
- hyponatremia/hypoproteinemia
- heat intolerance
- wheezing/chronic cough/hemoptysis
- intestinal obstruction
- clubbing
3
Q
What are the common symptoms of cystic fibrosis in adults?
A
- COPD
- infertility
4
Q
You suspect your patient has cystic fibrosis, what investigations should you order?
A
-sweat chloride test x 2
False pos - malnutritions, celiac, adrenal insufficiency
False neg - peripheral edema, atopic dermatitis, hypo-PTH
5
Q
How would you treat patients with cystic fibrosis?
A
NUTRITION-high cal diet, pancreatic enzyme supps
CHEST-physio, exercise, bronchodilators, aerosolized DNAase, ABx (cipro, cephalosporins for Pseudomonas coverage)
GENETIC COUNSELLING
*Lung transplant
6
Q
What are the complications of cystic fibrosis?
A
- resp failure
- pneumothorax
- cor pulmonale
- gallstones
- cirrhosis with portal HTN
- infertility
- low life expectancy (avg survival 30yr)
