Reading...
Play button
Play button
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;
18 Cards in this Set
- Front
- Back
|
Genetics of CF
|
AR affecting "cystic fibrosis transmembrane conductance regulator" or CFTR
>1800 individual CFTR mutations (locus heterogeneity) |
|
|
Most common CF gene mutation
|
delta F508
|
|
|
Common first lobe of CF application
|
right upper lobe
|
|
|
Medical tx impacts on CF
|
adherence to meds
access to care clinic variations |
|
|
Environmental exposures impacting CF phenotype
|
microorganisms
tobacco smoke nutrition gender stress |
|
|
Major bacteria in CF patients (adult)
|
mucoid pseudomonas aeruginosa
|
|
|
Some clinical manifestations of CF
|
liver disease
pancreatic insufficiency clubbing nasal polyps stunted height and weight |
|
|
When are a majority of pts diagnosed for CF? What can they present with at birth?
|
71% in 1st year
Often with meconium obstruction at birth |
|
|
Diagnostic criteria of CF
|
sinopulmonary disease
GI symptoms: pancreas, DIOS, liver Family history: obstructive azoospermia in males |
|
|
Lab evidence of CF
|
elevated sweat chloride (>60mmol/L)
mutations in CFTR Bioelectric abnormalities |
|
|
What are some symptoms of CF?
|
cough, sputum, dyspnea, can become chronic w/ periods of exacerbation (feel flu-like)
|
|
|
Main bacterias for kids w/ CF
|
Staph, Staph aureus
H. influenza |
|
|
hemoptysis thoughts in CF?
|
can be major complication when massive
|
|
|
3 common co-diseases w/ CF
|
bone disease, diabetes, depression
|
|
|
What can you have in the extremities in CF?
|
purpuric rash or new periosteal bone formation
|
|
|
Data to analyze for pts with CF
|
lung function trends
last IV therapy nutrition trends |
|
|
Typical tx for pseudomonas
|
(2 classes at least)
anti-pseudomonas penicillin aminoglycosides |
|
|
Wonder drug for CF recently released
|
ivacaftor (for specific gene mutation)
|
