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17 Cards in this Set

  • Front
  • Back
5 common presenting features of cystic fibrosis
1. pulmonary sxs
2. failure to thrive/malnutrition
3. steatorrhea
4. meconium ileus
5. family hx known
1. What is the CFTR?
2. Where is it found genetically?
3. Found in what cells?
1. cystic fibrosis transmembrane regulator

2. chrom 7

3. all epithelial cells
CFTR fxn with Cl- in:
1. sweat glands
2. lungs
3. GI tract
1. reabsorption

2. reabsorption

3. secretion w/ H2O
1. Most common mutation in CF of N American Caucasians
1. Phe508 (F508)
In CF, describe the following theories of pulmonary sxs:
1. high salt model
2. low volume model
1. no Cl- reabs, more Na+ retained in apical surface, more H2O --> clogged airway

2. No Cl- reabs, upreg of Na+ ch expression & Na reabs, increase H2O reabs to match --> decreased fluid, no ciliary beating
4 Pulmonary sequelae of CF
1. infection/inflammation
2. obstruction
3. bronchiolitis
4. bronchiectasis
1. 3 triggers in CF pulmonary exacerbations
2. 4 sxs of exacerbation
3. 2 predominant bugs in respiratory infections
1. changed bacterial density, viral infections, host inflam response

2. change in cough/sputum/PFTs, SOB, fever, weight loss,

3. P. aeruginosa, S. aureus
3 sinus effects of CF
1. hypertrophy/plasia of secretory elements

2. inflam & edema

3. polyps
GI tract effects of CF in:
1. intestine
2. rectum
3. hepatobiliary system (5)
1. meconium ileus or distal intestinal obstruction syndr

2. rectal prolapse

3. focal biliary cirrhosis, hypoplastic GB, gallstones, fatty liver, stenosed comm bile duct
2 pancreatic effects of CF
1. obstructed ducts dilate forming cysts w/ fibrosis

2. exocrine insufficiency causes fat malabsorption
2 bone/joint effects of CF
1. osteopenia/perosis

2. episodic arthritis
GU effects of CF in:
1. men
2. women
1. mostly infertile due to poor transport of sperm

2. may be anovulatory, endocervicitis from distended mucous glands
CF effects on sweat glands
1. increased sensitivity to dehydration in hot weather
2. Na/Cl homeostasis abNL -- salty sweat
4 Nutritional concerns w/ CF pts
1. increased caloric demand, especially during exacerbations

2. decrease intake & malabsorption

3. can have CF related diabetes

4. good nutrition & weight assoc'd w/ better outcomes
2 types of CF dx
1. clinical evidence

2. lab evidence (sweat Cl- level, genotyping)
List 7 categories of CF tx
1. airway clearance
2. ABX
3. bronchodilators
4. nutrition
5. anti-inflam
6. mucolytics
7. lung transplant
2 pronged management of pulmonary exacerbations in CF
1. IV ABX

2. increased airway clearance frequency