Term
|
Definition
| a congenital malformation where a web forms at the vocal cords |
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Term
| what is a tracheoesophageal fistula? |
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Definition
| a congenital fistula between the esophagus and tracha due to an incomplete division of the foregut, respiratory, and digestive tracts. this can lead to respiratory problems and failure to thrive |
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Term
| what is tracheal stenosis/atresia? |
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Definition
| a congenital malformation that may be incompatible w/life and may need to be surgically removed |
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Term
| what is pulmonary sequestration? |
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Definition
| a partial or complete separation of a lobe from the surrounding lung including no connection to the main bronchial tree. in this congenital malformation, blood supply comes from the aorta and not the pulmonary arteries. this can lead to infection or respiratory problems or simply be asymptomatic |
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Term
| what are bronchogenic cysts? |
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Definition
| cysts are structures lined by epithelium and filled with various materials (fluid, solid materials, etc). bronchogenic cysts are *typically single*, filled with mucinous secretions/air, if poor blood supply - infection is possible which may rupture into the pleural cavity. ruptures of bronchogenic cysts in the pleural cavity may lead to pneumothorax, compression atelectesis and if in the bronchi - bronchitis or pneumona |
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Term
| what are cystic adematoid malformations? |
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Definition
| these are cyst-like structures w/glandular components. cystic adematoid malformations are usually solitary (*one lesion can have multiple cysts of varying size*), in the lower lobe, and lined with adenomatous epithelium and smooth muscle proliferation (glandular lining). they are typically seen in neonates who present with various degrees of respiratory distress |
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Term
| what is the most common lethal genetic disease affecting white populations? |
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Definition
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Term
| what is the genetic defect present in CF? |
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Definition
| a gene present on chromosome 7 which encodes the CFTR (cystic fibrosis transmembrane conductance regulator) and causes defective transportation of Cl- across the epithelia |
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Term
| what pathophysiology does the defect in chr 7 in CF pts lead to? |
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Definition
| the defect in the CFTR gene on chr 7 causes defective transportation of Cl- across the epithelia into the lumen of the lungs which gives rise to a viscous mucus that is devoid of water which plugs airways and leads to infection (staph and pseudomonas). sweat gland are also unable to take up Cl- from secretions, leading to high levels of Na+ in secretions. |
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Term
| what characterizes the damage CF can incur upon the lungs? |
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Definition
| beyond infection, CF can cause parenchymal damage to the lungs, empyema, and *bronchiectesis* - a necrosis of the surrounding pulmonary parenchyma |
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Term
| how can CF affect the pancreas? |
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Definition
| mucin accumulates w/dilitation of the ducts and the glands atrophy, which can develop in to fibrocystic disease with formation of cyst-like structures which eventually can lead to various degrees of malnutrition (w/CF pts: make sure dietary intake and nutrition are good) |
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|
Term
| what may be the first sign of CF in newborns? |
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Definition
| small bowel obstruction (ileus, biliary cirrhosis may develop) |
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Term
|
Definition
| males will likely have sterility problems due to duct/gland problems |
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|
Term
| can percussion therapy help pts w/CF? |
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Definition
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|
Term
| what is atelectasis? what generally causes it? |
|
Definition
| a collapse of the lung or inability of the lung to completely expand which can lead to shunting of blood around these areas and hypoxia. |
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Term
| what is resorption atelectesis? |
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Definition
| resorption atelectasises are due to obstructions of the airway and are mostly unilateral. these obstructions cause the portion of the lung distal to the obstruction to absorb the air and collapse. aspirating an object can rapidly cause this while tumors can cause this more progressively |
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Term
| what is compression atelectesis? |
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Definition
| mechanical collapse of the lung due to compression keeping it from expanding. this can happen bilaterally and involve large portions of the lung. pneumothorax (air between the lung and pleura), hemothorax (blood between the lung and pleura), and emypema (pus between the lung and pleura) can all cause this |
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Term
| what is microatelectasis? |
|
Definition
| loss of surfactant, "shock lung", where the surfactant produced by the pneumocytes which generally breaks up surface tension is lacking due to premature babies or hypervolemic shock. this tends to be bilateral and associated with the lung being unable to expand |
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|
Term
| what is contraction atelectesis? |
|
Definition
| fibrotic changes such as a build up of scar tissue in the pleura prevent the airway from expanding |
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|
Term
| what defines obstructive lung disease? what are some examples? |
|
Definition
| decreased outflow of air, problems with exhalation, increased resistance. asthma, emphysema, chronic bronchitis and bronchiectasis are all examples of OLD and may overlap (of which cigarette smoking is a common cause) and may or may not be reversible |
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Term
| where does chronic bronchitis affect? what are the major pathologic changes? what is the etiology? signs/symptoms? |
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Definition
| chronic bronchitis affects the bronchus and causes *mucus gland hyperplasia/hypersecretion*. it is caused by tobacco smoke/pollutants and presents as a cough w/sputum production. (this is only one of these in the list that is a clinical dx) |
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Term
| where does bronchiectasis affect? what are the major pathologic changes? what is the etiology? signs/symptoms? |
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Definition
| bronchiectasis affects the bronchus, causing airway dilation and scarring. it is caused by persistent or severe infections (s aureus, pseudomonas) and presents as cough, purulent sputum and fever |
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Term
| where does asthma affect? what are the major pathologic changes? what is the etiology? signs/symptoms? |
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Definition
| asthma affects the bronchous, causing smooth muscle hyperplasia, excess mucus and inflammation. it is caused by immunologic or undefined causes and presents as episodic wheezing, cough, and dyspnea. asthma is reversible. |
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Term
| where does emphysema affect? what are the major pathologic changes? what is the etiology? signs/symptoms? |
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Definition
| emphysema affects the acinus, causing airspace enlargement and wall destruction. it is caused by tobacco smoke and presents as dyspnea |
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Term
| where does small airway disease/bronchiolitis affect? what are the major pathologic changes? what is the etiology? signs/symptoms? |
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Definition
| bronchiolitis/small airway disease affects the bronchiole, causing inflammatory scarring/obliteration. it is caused by tobacco smoke, pollutants, misc and presents as cough and dyspnea |
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Term
| where is asthma a particularly strong public heath concern? |
|
Definition
| in cities due to living condictions, antigen exposure, and pollutants. there has been an increase in asthma pts and related deaths recently |
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Term
|
Definition
| a chronic inflammatory disease with acute exacerbations dependent on many factors. it is reversible, meaning that the lungs will appear normal when exacerbations are not occuring, but during attacks, cough/dyspnea/wheezing/secretions occur |
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|
Term
| what is the etiology of asthma? |
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Definition
|
|
Term
| what is the pathogenesis of asthma? |
|
Definition
| *bilateral exaggerated bronchoconstriction makes up a major component, with varying degrees of *bronchial inflammation causing exhalation to be difficult. often a *hypersensistivity to histamine may lead to bronchoconstriction and mucus secretion - therefore antihistamines can play a role in controlling the disease |
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Term
| what is extrinsic asthma? |
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Definition
| asthma initiated by a type 1 hypersensitivity reaction induced by exposure to a variety of extrinsic antigen including atopic asthma, occupational asthma and pulmonary aspergillosis (allergic rxn to fungus) |
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Term
| what is intrinsic asthma? |
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Definition
| asthma due to nonimmune capabilities, ASA hypersensitivity rxn, pulmonary infection, and exercise |
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Term
| what is the most common type of asthma? |
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Definition
| atopic asthma, which usually begins in childhood and is triggered by environmental antigens such as dusts, pollens, animal dander and foods. it may initially present subtly. |
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Term
| what is the pathogenesis of atopic asthma? |
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Definition
| antigen presentation to CD4 T cells which release IL4/IL5, leading to synthesis and release of *IgE for that antigen from B cells. this promotes mast cell and particularly eosinophil proliferation |
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|
Term
| what are mast cell activators? |
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Definition
| LT C4, D4, E4. PGD2. LTB4. PAF. all of these eventually lead to bronchoconstriction and mucus secretion |
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Term
| how does a bronchus affected by asthma appear? |
|
Definition
| bronchoconstriction, increased mucus production, hyperplasia of muscle cells, increased number of glandular cells, increased thickening of basement membrane cells, and increased vascular permeability = swelling |
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|
Term
| what is the morphology of lungs affected by asthma? |
|
Definition
| hyperinflation (can breathe in ok, but not out), occlusion of the bronchi w/mucus (can lead to resorption ateletcesis), curschman's spirals (microscopic whirls of shed epithelium), and charcot-leydin crystals (broken up eosinophils seen microscopically), inflammation of the bronchial walls (some neutrophils/lymphocytes), thickened BM, an increase in mucus glands, and hypertrophy/hyperplasia of smooth muscle (due to glands working harder) |
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|
Term
|
Definition
|
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Term
| what are symptoms of asthma? |
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Definition
| dyspnea, wheezing that may last 1-3 hrs. asthma attacks may develop into status asthmaticus: difficulty oxygenating, rapid onset, and potentially death |
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Term
| why is COPD difficult to define? |
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Definition
| there is often overlap between the 2 components: chronic bronchitis and emphysema |
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|
Term
|
Definition
| *permanent enlargement of the airspaces, destruction of the alveolar walls and a strong association with cigarette smoking (can also be linked to alpha1 anti trypsin deficiency and linked to cirrhosis) |
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|
Term
| what is centrilobular emphysema? |
|
Definition
| the morphology most commonly seen in smokters, where the middle of the acinus is involved |
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|
Term
| what is panacinar emphysema? |
|
Definition
| the morphology of emphysema most associated with alpha 1 anti trypsin deficiency which involves the entire acinus, alveolar duct and alveoli |
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|
Term
| what is the general pathogenesis of emphysema? |
|
Definition
| the lungs lose their elastic ability, they may expand, but don't necessarily return to their natural state. this can result in barrel chest from chronic overexpansion |
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|
Term
| how does alpha1 antitrypsin deficiency lead to emphysema? |
|
Definition
| alpha1 antitrypsin is an antielastase which dampens the effect of elastase that normally breaks down elastic tissue. if there is a deficiency in alpha1 antitrypsin, the elastase will over-break down the elastic features of the lung = emphysema, or a loss of elasticity |
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|
Term
| how does smoking lead to emphysema? |
|
Definition
| smoking can decreased the antielastic function of alpha1 antitrypsin as well as increase the infiltration of PMNs and macrophages which relase elastase, leading to elastic damage, resulting in hyperexpanded airways and emphysema. |
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Term
| where in the lungs is centriacinar emphysema seen in the lung? |
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Definition
| centriacinar emphysema has a preponderance for the *upper lobes and is associated w/smoking and sparing of the distal portions of the acini. |
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|
Term
| where is panacinar emphysema seen in the lung? |
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Definition
| in panacinar emphysema, which is associated with alpha1 antitrypsin deficiency and features acini uniformly enlarged is mostly found in the *lower lobes |
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|
Term
| where in the lung is distal acinar emphysema seen? |
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Definition
| distal acini in the lung, such as those adjacent to the pleura are involved and therefore distal acinar emphysema is associated with spontaneous pneumothorax (as the parenchyma begin to be destroyed, this may result in a rupture into the pleural space and give rise to a pneumothorax - causing a compression atelectesis) |
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|
Term
| does large expansion of the airway generally indicate disease? |
|
Definition
|
|
Term
|
Definition
| little balloon-like extrusions from the lungs created by progressive alveoloar breakdown which can result in pneumothorax - pts with this will have symptoms such as O2 dependence, dyspnea, etc |
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|
Term
| can emphysema be initially asymptomatic? |
|
Definition
| yes though with continued insult, such as cigarette smoking, it can be come disabling and may require home O2 therapy |
|
|
Term
| what is the pathogensis of emphysema? |
|
Definition
| excess elastase activity and or lack thereof leading to elastic damage and destruction of alveoli. nicotine from smoking will increase neutrophils/macrophages levels which then release their own elastases etc which exacerbate the problem and cause further inflammation. oxidants in cigarette smoking also inhibit alpha1 antitrypsin and its antielastase activity. |
|
|
Term
| how do emphysema pts present? |
|
Definition
| dyspnea, enlarged chest (costal flaring/hyperresonant lungs), pink puffers (pts who are pink b/c they are oxygenated but using more energy to breathe = thinner), blue bloaters (not oxygenated well, cyanotic, may have CHF/edema), pulmonary failure and right sided heart failure |
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|
Term
| is chronic bronchitis a clinical or pathologic dx? who does it affect? |
|
Definition
| clinical: persistent productive cough for at least 3 months in at least 2 consecutive years (white/yellow sputum, smokers hack). it more commonly affects cigarette smokers and urban dwellers |
|
|
Term
| what are the 4 subtypes of chronic bronchitis? |
|
Definition
| simple chronic bronchitis, chronic mucopurulent bronchitis, chronic obstructive bronchitis |
|
|
Term
| what is simple chronic bronchitis? |
|
Definition
| mucoid sputum with no obstruction of airflow (nagging cough) |
|
|
Term
| what is chronic mucopurulent bronchitis? |
|
Definition
| sputum that has pus from secondary infections |
|
|
Term
| what is chronic asthmatic bronchitis? |
|
Definition
| chronic bronchtis with occasional episodes of asthma |
|
|
Term
| what is chronic obstructive bronchitis? |
|
Definition
| chronic outflow obstruction, more emphysematous |
|
|
Term
| what are the different ways that chronic bronchitis may cause outflow obstruction? can it co-oexist with emphysema? |
|
Definition
| chronic bronchitis can cause airflow obstruction via inflammation,fibrosis and narrowing of the bronchioles. chronic bronchitis can be coexistent with emphysema |
|
|
Term
| what is the morphology of chronic bronchitis? |
|
Definition
| hyperemic edematous mucosal lining (from coughing/irritation to the airway), enlargement of the mucous secreting glands, squamous metaplasia, (ciliated columnar cells are replaced w/squamous, lessens ability to trap antigens and leads to higher infection risk -> can lead to SCC), inflammatory cells (eosinophils/neutrophils in exacerbation, lymphocytes in chronic) and chronic bronchiolitis |
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|
Term
| what is the clinical course of chronic bronchitis? |
|
Definition
| cough and sputum production, outflow obtruction, and overlap emphysema |
|
|
Term
|
Definition
|
|
Term
|
Definition
| a permanent dilation of the bronchi and bronchioles due to destruction of muscle and elastic tissue associated with necrotizing pneumonia, CF, etc (often the end product of severe infections). |
|
|
Term
| can bronchiectatis be seen on x ray? |
|
Definition
|
|
Term
| what is the gross appearance of lung affected by bronchiectasis? |
|
Definition
|
|
Term
| what can cause bronchiectasis? |
|
Definition
| bronchial obstruction, hereditary conditions (CF, immunodeficiency, kartagener's syndrome), necrotizing pneumonia (staph), and pts with underlying problems (intubated, on ventilation, series of mucus plugging events) -> not a primary disease, but is secondary to a lot of diseases |
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|
Term
| what is kartagener's syndrome? |
|
Definition
| kartanger's syndrome, which is associated with bronchiectasis, is also associated with cystis inversis (organs on opposite side of body) and is a defect in the microtubules of cilia. this inhibits their use in removal of pollutants, allergens, and also results in abnormal sperm |
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|
Term
| how does bronchiectasis present clinically? |
|
Definition
| pts will have a severe cough, foul smelling sputum (necrosis), and hemoptysis |
|
|
Term
| what generally characterizes restrictive lung diseases? |
|
Definition
| reduced compliance/increased stiffness of the lungs of which there are acute and chronic forms likely featuring dyspnea/hypoxia |
|
|
Term
| what is an important restrictive lung disease? |
|
Definition
| ARDS - adult respiratory distress syndrome |
|
|
Term
| what characterizes ARDS (shock lung/DAD - diffuse alveolar damage)? |
|
Definition
| decreased arterial O2 pressure, decreased lung compliance, pulmonary infiltrates, and diffuse alveolar damage (DAD - describes morphology). it is also referred to as "shock lung" - first seen in vietnam war, pts had hypovolemic shock that even when corrected incurred permanent lung damage to the lungs ability to oxygenate |
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|
Term
| what defines the DAD, the morphologic component of ARDS? |
|
Definition
| DAD/diffuse alveolar damage consists of injury to the epi- and endothelium, leading to the formation of hyaline membranes -> protein rich membrane structures that fill up the alveoli and edema is often present. the cumulative effect of DAD is difficulty in oxygenation - which makes tx difficult b/c you want to oxygenate - but over oxygenating can increase the DAD. |
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|
Term
| is ARDS associated with a high morbidity/mortality rate? |
|
Definition
|
|
Term
| can other things besides hypovolemic shock cause ARDS? |
|
Definition
| yes, viral infections, pneumonias, and toxic substance inhalation can also cause it |
|
|
Term
| what is the injury causing DAD in ARDS mediated by? |
|
Definition
| neutrophils and macrophages damage the alveolar epithelium and endothelium via toxic mediators - causing necrosis and release of protein to the interstitium between the alveoli. the resultant edema and proteinaceous buildup can eventually can become fibrotic. grossly the lungs will look red/firm/airless initially followed by a more wooden appearance. |
|
|
Term
| what are the phases of ARDS in order of occurence? |
|
Definition
| exudative, proliferative, and fibrotic |
|
|
Term
| what is the exudative phase of ADRS? |
|
Definition
| the exudative phase of ARDS is marked by congestion, edema, and hemorrhage. there is necrosis of the alveolar epithelial cell and *formation of hyaline membranes. *many people die at this phase* |
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|
Term
| what is the proliferative phase of ADRS? |
|
Definition
| the proliferative phase of ADRS consists of proliferation of type II pneumocytes and fibroblasts |
|
|
Term
| what is the fibrotic phase of ADRS? |
|
Definition
| the fibrotic phase of ARDS consists of permanent diffuse interstitial fibrosis resulting in life-long oxygenation problems |
|
|
Term
| what is the clinical course of ARDS? |
|
Definition
| most cases have a generally poor outcome w/mortality around 50%. O2 therapy may complicate the clinical course |
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