Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
17 Cards in this Set
- Front
- Back
|
what is chronic therapy for CF like |
ADEK creon pancreas enzyme pulmozyme break up mucus azithromycin inflam abx albuterol- dilate prilosec- Cl in stomach |
|
|
what is the classic CF mutation |
deltaF508 |
|
|
what are the five classes of CF mutation |
I: stop signal in CF gene so no CFTR II: CFTR misfolded*** III: CFTR made and delivered but does not function IV: CFTR opening broken V: not enough CFTR made VI: CFTR degrades too fast |
|
|
what all organs involved in CF? |
upper airways: chronic sinusitis
lungs: recurrent stahp, pseudomonas, bronchiectasis.
pancreas: insuffiency, DM
liver: cirrhosis intestines: meconium ileus, distal interstinal obstruction reproductive tract -> infertility for men |
|
|
how do you clear mucous? |
cilia beat together in a layer of fluid made by the Cl channels |
|
|
how do you dx CF? |
one of each
screening test, sibling with CF, one phenotypic feature
elevated Cl in sweat, 2 CFTR mutations, abnormal nasal potential difference |
|
|
why do CF pts have crackles? |
atelectasis bronchiectasis** |
|
|
why do CF pts get bronchiectasis? |
defective CFTR situation means less Cl secreted, increased Na absorption -> bronchial obstruction -> infection -> inflam -> bronchiectasis
get a lot of infections because mucous, airway obstruction, defect in host defense |
|
|
how does bronchiectasis present? |
sx cough, sputum, hemoptysis
signs: crackles, wheezing, clubbing |
|
|
what is aspergillus like?
how does it relate to CF pt? |
spore bearing fungus all over environment
hypersensitivity is predominantly in pts with asthma and cystic fibrosis. This chronic situation further pushes for bronchiectasis, fibrosis, and respiratory compromise |
|
|
how do u tx the ABPA |
steriods
|
|
|
what is most important pathogen in CF lung dz why?
how does this happen |
pseudomonas aeruginosa
most rapid decline inducer. Build up sensitivity
infection is non-mucoid, pan sensitive then evolves with virulence factor alginate to become mucoid and then you're screwed. Have to tx before that happens |
|
|
how do we treat PA in america |
tobramycin and ciprofloxacin |
|
|
what is mutation where channel opening is broken?
what is drug for that? |
G551D
ivacaftor- potentiates the channel |
|
|
how do you tx class II mutation |
use lumacaftor which helps get to surface |
|
|
what is inhertance
what gene and what is its purpose |
AR in the CFTR gene
channel for ion transport across and w/in epithelial cells in multiple organs |
|
|
what is the principal path process for loss of lung function in CF? |
bronchiectasis |
