Term
| Who is most at risk for cystic fibrosis? |
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Definition
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Term
| How is cystic fibrosis spread? |
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Definition
|
|
Term
| What is the prevalence of having one gene for cystic fibrosis? |
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Definition
|
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Term
| What is the rate of cystic fibrosis dx? |
|
Definition
| 1 in 2000-2500 live births |
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Term
| What is the number one fatal inherited dz? |
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Definition
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Term
| What is the main mechanism of cystic fibrosis? |
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Definition
| abnormal chloride transport channels in epithelial cells |
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Term
| What is the median life expectancy for cystic fibrosis pt's? |
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Definition
|
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Term
| What was the median life expectancy for cystic fibrosis in the 1940s? |
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Definition
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Term
| Is Cl- absorption or secrection impaired in cystic fibrosis? |
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Definition
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Term
| What ionic imbalance results from Cl- hyposecretion? |
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Definition
| Na+ reabsorption fails --> cystic fibrosis |
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Term
| What does Na+ reabsorption in lung tissues cause in cystic fibrosis? |
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Definition
| hypertonic sol'n inside cells --> H2O reabsorption --> thickened airway secretions --> lumen obstruction --> hyperinflammatory lung tissues |
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Term
| How does cystic fibrosis lead to increased infection rates? |
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Definition
| lumen obstruction leads to hyperinflammatory response and leaves lungs susceptible to pseudomonas and other normal bacterial flora which then siezes the opportunity |
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Term
| What is the end sx of cystic fibrosis (besides death)? |
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Definition
| the persistent inflammation and infections cause damage to airway walls and obliteration of small airways. Lots of scarring results. |
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Term
| What is the #1 way that cystic fibrosis pt's die? |
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Definition
| pulmonary failure secondary to infection |
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Term
| While cystic fibrosis mainly affects the lungs, does it caues other systemic problems? |
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Definition
|
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Term
| Besides the lungs, where else does cystic fibrosis present problems? |
|
Definition
poor digestion of fats
poor absorption of proteins and carbs
infertility
cirrhosis |
|
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Term
| What are tx for cystic fibrosis? |
|
Definition
airway clearance techniques
Oral AB's
Inhaled AB's
bronchodilators
nutrition
inhaled 7% NaCl sol'n
ibuprofen
lung transplantation |
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|
Term
| What are airway clearance techniques for cystic fibrosis? |
|
Definition
| postural drainage w/ percussion of lungs; b.i.d. |
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Term
|
Definition
| A pulmozyme that is used to ease sputum clearance in cystic fibrosis |
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Term
| How much does dornase alpha cost? |
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Definition
| Very expensive, ~25k per year. |
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Term
| What is an ocular SE of dornase alpha? |
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Definition
|
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Term
| Are oral AB's effective long term for cystic fibrosis tx? |
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Definition
|
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Term
| What oral AB's are used for short term cystic fibrosis pt's? |
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Definition
| oral azithromycin 3x /week |
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Term
| What is the mechanism of oral AB's in CF tx? |
|
Definition
| unknown, but may be anti-inflammatory or anti-bacterial |
|
|
Term
| How are inhaled AB's used in CF tx? |
|
Definition
| Cycle of 28 days of use, 28 days not used |
|
|
Term
| What is the #1 way to increase lifespan in CF pt's? |
|
Definition
|
|
Term
| What inhaled AB's are used for CF tx? |
|
Definition
high dose tobramycin 15 min tx b.i.d.
Aztreonam for 5 min t.i.d. |
|
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Term
| What bronchodilators are used in CF tx? |
|
Definition
| Beta-2 adrenergic agonists |
|
|
Term
| Why is nutrition so important for CF tx? |
|
Definition
| They have poor GI absorption so malnutrition occurs readily |
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|
Term
| How is NaCl administered to CF pt's? |
|
Definition
| 7% sol'n inhaled for 15 minutes b.i.d. |
|
|
Term
| What benefits does inhaling NaCl provide CF pts? |
|
Definition
| reduced lung exacerbations and improved lung fxn |
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Term
| What drug is more useful in younger CF pts? |
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Definition
|
|
Term
| What is a risk of high dose ibuprofen CF tx? |
|
Definition
| risks of kidney damage and GI bleeding |
|
|
Term
| What is the survival rate for CF pt's after a lung transplant? |
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Definition
|
|
Term
| What is the most common cause of death in CF lung transplant pts? |
|
Definition
|
|
Term
| What are 3 potential future tx for CF? |
|
Definition
growth hormone, may help offset frailty due to malnutrition
gene therapy
meds that increase Cl- transport in lungs |
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Term
| In restrictive lung dz, what lung capacities and volumes are decreased? |
|
Definition
|
|
Term
| What is normal in restrictive lung dz? |
|
Definition
|
|
Term
| What expansion is decreased in restrictive lung dz? |
|
Definition
|
|
Term
| What are 3 infiltrative lung dz's? |
|
Definition
pneymoconiosis
collagen vascular dz
sarcoidosis |
|
|
Term
| What are 3 chest wall abnormalities seen in restrictive lung diseaes? |
|
Definition
kyphoscoliosis
obesity
respiratory muscle fatigue |
|
|
Term
| What is the typical pathophysiology of infiltrative lung dz? |
|
Definition
| diffuse injury and inflammation leads to fibrosis of alveoli --> thickening and stiffening of septae --> decreased lung elasticity --> decreased gas exchange |
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|
Term
|
Definition
| lung dz from inhalation of inorganic dust |
|
|
Term
| Is there tx for pneumoconiosis? |
|
Definition
| nothing beyond removing the environment providing the dust |
|
|
Term
| What used to be a particularly common form of pneumoconiosis? |
|
Definition
|
|
Term
| What is the #1 way to prevent asbestosis? |
|
Definition
| limit the dose; has therefore been phased out of as many products as possible |
|
|
Term
| Are asbestos fibers cleared by the body? |
|
Definition
| No, they tend to stay long term, well past the point where exposure is stopped |
|
|
Term
| What does asbestos increase? |
|
Definition
| risk for bronchogenic carcinoma increases 59x |
|
|
Term
| What is the timeframe for the onset of asbestosis? |
|
Definition
| very long after the exposure is when cancer presents |
|
|
Term
| What is the main sx of asbestosis? |
|
Definition
| pleural effusion causes progressive pulmonary fibrosis |
|
|
Term
| What types of cancer does asbestosis cause? |
|
Definition
| mesotheliomas of pleura and peritoneum |
|
|
Term
| What causes 'black lung? What kind of dz does it fall under? |
|
Definition
| coal dust, silica, and cigarette smoke cause a form of pneumoconiosis |
|
|
Term
| What is the typical timeframe from asbestos exposure to cancer onset? |
|
Definition
|
|
Term
| What is pleural effusion? |
|
Definition
| abnormal fluid exposure in the lungs |
|
|
Term
| What is the most prevalent occupational dz in the world? |
|
Definition
|
|
Term
| What are causative agents of silicosis? |
|
Definition
|
|
Term
| What are sources of inorganic dust that can cause silicosis? |
|
Definition
sandblasting
mining
pottery
brick |
|
|
Term
|
Definition
| multisystem disorder of unkown etiology |
|
|
Term
| What are the primary systems affected in sarcoidosis? |
|
Definition
|
|
Term
| Who are most at risk to develop sarcoidosis? |
|
Definition
young people
women
african descent |
|
|
Term
| What is the primary sx of sarcoidosis? |
|
Definition
| granulomas develop throughout the body |
|
|
Term
| What is suspected to be the primary risk factor for sarcoidosis? |
|
Definition
|
|
Term
| What % of sarcoidosis pt's will have ocular SE? |
|
Definition
|
|
Term
| What is the #1 ocular SE of sarcoidosis? |
|
Definition
| anterior uveitis w/ pigment dispersion |
|
|
Term
| What are ocular SE of sarcoidosis? |
|
Definition
anterior uveitis
granulomas on lids, iris, retina
conjunctivitis
lacrimal gland inflammation
retinal vasculitis
ONH edema
cranial neuropathies |
|
|
Term
| What respiratory sx are found in 95% of sarcoidosis pt's? |
|
Definition
cough and dyspnea
paratracheal and hilar adenopathy
infiltrates and fibrosis possible
fatigue and fever
lymphadenopathy |
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