Test CF

Question 2

CF What are complications of Cystic Fibrosis from head to toe?

Answer 2

1. Nasal polyps 2. Chronic sinusitis 3. Chronic cough 4. Bronchiectasis 5. Chronic colonization with staph, hemophilus, pseudomonas, burkholderia seppacia 6. GERD secondary to obstructive lung disease, hyperinflation and diaphragms pushing on stomach 7. Meconium ileus 8. Failure to thrive 9. Pancreatic insufficiency (with delta F508 mutation) –> Fat soluble vitamin deficiency, steatorrhea, eventual diabetes 10. Liver cirrhosis - from thickened bile 11. Congenital absence of vas deferens in males 12. Infertility in both males and females - in females, thick cervical secretions or secondary amenorrhea from malnutrition 13. Rectal prolapse - from chronic diarrhea, malnutrition and weak musculature 14. Clubbing 15. Cor pulmonary from pulmonary hypertension

Question 3

CF What is the pattern of colonization in CF patients?

• by age group-

Babies

Toddlers

School age children

Teenagers

Answer 3

Babies - staph aureus

Toddlers - hemophilus b influenza

School age children - pseudomonas

Teenagers

• Burkholderia seppacia
• stenotrophomonas maltophilia

Question 4

How do you interpret a PFT?

Answer 4

1. Look at flow-volume curve: is it reproducible (aka straight exp line vs squiggly)? 2. Look at effort curve: kids < 12 should exhale for at least 6 seconds, kids > 12 should exhale for at least 10 seconds 3. Look at FEV1: mild (70-79), moderate (60-69), severe (50-59), very severe (<49) airflow limitation 4. Look at FVC (normal in obstructive, decreased in restrictive) 5. Look at FEF25-75 (N = 70-120): evidence of small airway flow limitation 6. Look at lung volumes if present 7. Look at post-bronchodilator response if present (>12% change is significant, CHEO uses > 10%) 8. Compare FEV1 to last PFT: has it changed?

Question 5

What is the differential diagnosis of wheeze? (10)

Answer 5

1. Asthma 2. Aspiration penumonitis: ask about coughing/choking with feeds, reflux symptoms, rule out TEF 3. BPD 4. Bronchiolitis obliterans: chronic wheezing after adenovirus infection 5. Congenital cardiac lesion: left to right shunts causing increased pulmonary blood flow 6. Cystic fibrosis: ask about recurrent diarrhea, failure to thrive 7. Foreign body aspiration 8. Vascular rings/slings 9. Airway malformations: tracheomalacia, lung cysts, mediastinal mass 10. Primary ciliary dyskinesia: ask about recurrent sinusitis, ear infections