cystic fibrosis Flashcards
list the 4 major systems involved
- GI
- sweat glands
- reproductive
- respiratory
GI sx of CF (6)
- meconium ileus– surgical emergency!!!
- prolonged jaundice
- rectal prolapse
- steatorrhea (cant digest fat)
- belly pain
- distal intestinal obstruction syndrome
2 sequlae of salt crystal formation
irritation
nonspecific rash
what is CF
when mutated CFTR protein causes really thick sticky secretions that builds up in many organ sys
what is the quantittive pilocarpine iontrophoresis detecting??
high levels of chloride in the sweat
its the diagnostic test of choice to confirm
if there is no mutation in DNA test, does this mean that the person does not have CF?
nope. there is a rare mutation that standard testing cannot detect
Negative sweat test but CF is still suspected. what do you do?
repeat sweat test & do expanded analysis
when can you dx pancreatic insufficiency
elastase below 200 micrograms/gram
two common bacteria seen in the thick respiratory mucus plug in CF
staph aureus
pseudomonas aeruginosa
consequence of bacterial infection and inflammation in CF
bronchiectasis– permanent dilation of bronchi filled with mucus
5 resp sx of CF
- Chronic cough; Wheezing
- Recurrent pneumonia
- Sinusitis
- Nasal polyps
- bronchiectasis
what would PFT show in CF
obstructive pattern– decreased FEV1 and FEV1/FVC
what causes the bulging fontanel in CF
Vit A malabsorption
6 other sx of CF
- Low Na, Cl- metabolic alkalosis
- Male sterility
- Edema, hypoproteinemia and anemia
- Portal hypertension
- Clubbing; Failure to thrive
- Pseudotumor cerebri
most commonly missed PE in CF
clubbing
