Cystic Fibrosis DSA Flashcards
cystic fibrosis
autosomal recessive
mutation in CFTR
affects lung, pancreas, intestines, liver, sweat glands, sinuses, vas deferens
life - to 37yo
CF lungs
decreased Cl secretion
-impaired mucociliary clearing
infections, bronchiectasis, fibrosis, mucous plugging
CF sinuses
nasal and sinus polyps
CF pancreas
acinar dilation, cystic and fibrotic pancreas, exocrine insufficiency
CF intestine
meconium ileus secondary to severe obstruction
CF sweat gland
failure of Cl absorption
elevation of Cl and Na in sweat
CF vas deferens
95% men infertile
vitamin absorption in CF
can’t absorb fat soluble
-ADEK
CF MSK
decreased bone density secondary to decreased vit D absorption
CF kidney
nephrolithiasis
CF diagnosis
immunoreactive trypsinogen marker for pancreatic injury
genetic mutation analysis
confirmation - sweat test**
sweat test
confirm Dx of CF
pilocarpine iontophoresis - stimulates sweat
CXR CF
hyperinflation
CXR PFT
obstruction
progressive irreversible airflow obstruction
COPD
20-30yr after exposure
leading cause of COPD
smoking
40mL per year decrease in FEV1
also work dusts, mining, cotton mills, grain-handling
alpha1 antitrypsin
deficiency - risk fx for COPD
neutrophil elastase
emphysema
enlarged air spaces distal to terminal bronchiole with destruction of alveolar wall
centriacinar
emphysema
-distal to terminal bronchiole
smoking
panacinar
emphysema
-alveolar ducts and adjacent alveoli
alpha1 antitrypsin
enlargement of bronchial mucous glands and increased epithelial goblet cell production leads to cough and mucous production
chronic bronchitis and bronchiolitis
hypoxemia leads to vasoconstriction and increased pulmonary vascular resistance in small pulmonary arteries
pulmonary HTN
vascular remodeling including medial smooth m enlargement and intimal fibrosis
lung mechanics in COPD
elastic recoil decreased in COPD
and airway resistance increased in COPD
distal airways less than 2mm diamter**
hypoxemia
mild in early COPD
hypercapnea - severe COPD
blue bloaters
chronic bronchitis COPD
pink puffers
emphysema COPD
mild COPD
FEV1/FVC < 70
FEV1 > 80%
moderate COPD
FEV1/FVC < 70
FEV1 50-80%
severe COPD
FEV1/FVC < 70
FEV1 30-50%
very severe COP
FEV1/FVC < 70
< 50%
CXR COPD
hyperinflation, flat diaphragms, bullae
SABAs
albuterol
levalbuterol
LABAs
salmeterol
formoterol
anticholinergics short acting
ipatroprium bromide
anticholinergic long acting
tiotroprium
methylxanthines
theophylline
antagonizes adenosine recepors
-increased cAMP, weak bronchodilator, requires close monitoring
roflumilast
PDE4 inhibitor
-increase FEV1 by 50 mL and reduce exacerbations in moderate to severe exacerbations of COPD
prednisone
systemic corticosteroid
corticosteroid in COPD
inhaled - reduced exacerbations
COPD immunizations
influenza and pneumococcus
lung transplant COPD
survival 5 years
for severe incapacitation
COPD exacerbation infections
h flu, streptococcus, moraxella, pseudomonas (hospitalized), rhinovirus, influenza, parainfluenza, RSV
50% patients FEV1 at 40%
will survive 5 years
