Midterm - Pathology Flashcards

2
Q

at 15 weeks gestational age, the lungs are in this phase

A

pseudoglandular phase

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3
Q

by 24 weeks gestational age, the lungs are now in this phase

A

cannalicular phase

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4
Q

mature alveolar septa are composed of?

A

type 1 epithelium overlying endothelium with occasional type 2 epithelium (surfactant)

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5
Q

large ducts in the bronchial wall drain this

A

mucin from submucosal glands

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6
Q

what type of epithelium is found in the bronchi?

A

ciliated pseudocolumnar epithelium with goblet cells (that produce mucin)

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7
Q

in what direction do respiratory cilia beat?

A

upwards to remove debris

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8
Q

bronchiolar walls are lacking what two components found in the bronchi?

A

cartilage and glands

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9
Q

what separates type 1 epithelium and endothelium?

A

basement membrane, location of gas exchange

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10
Q

what is the purpose of type 2 epithelium?

A

produce surfactant which breaks water tension and prevents alveolar collapse, also pluripotent and can regenerate new type 1 cells

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11
Q

what type of cells are found in the pleura?

A

mesothelial cells

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12
Q

peribronchial and interstitial stroma supports airway and is rich is this component

A

elastin

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13
Q

where are the arteries and veins found within the secondary lobule?

A

arteries track with bronchioles, veins along interlobular septae

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14
Q

what is the purpose of pleura?

A

frictionless vaccuum system to keep lung inflated

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15
Q

name two genetic non-obstructive causes of bronchiectasis

A

cystic fibrosis, primary ciliary dyskinesia

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16
Q

pathology of bronchiectasis

A

permanent dilation of bronchi, peri-bronchial inflammation, and organization (fibroblastic proliferation)

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17
Q

radiology of bronchiectasis

A

airway dilation extending into periphery (seen on CT)

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18
Q

what is found in the dilated bronchi of a patient with CF?

A

muco-purulent debris plugging the bronchi

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19
Q

which microbial product leads to mucus hypersecretion and structural damage of airways in patients with CF?

A

elastase

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20
Q

what’s wrong with the cilia in CF?

A

nothing; just not mechanically strong enough to move abnormally thick mucus

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21
Q

which gene is mutated in CF, and what is the most common allele?

A

CFTR gene; delta F508

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22
Q

CF exhibits what inheritance pattern?

A

AR

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23
Q

what causes exceptionally thick mucus in patients with CF?

A

interrupted chloride pump leading to too much water absorption into the airway epithelium

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24
Q

this simple test is still used to diagnose CF in some patients

A

sweat test (measured chloridge levels in sweat, high in pts with CF)

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25
Q

what is the genetic abnormality in PCD?

A

loss of 1 or both dynein arms, leading to poor ciliary function

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26
what would radiology of asthma show?
alternating atelectasis and overexpansion
27
pathology of asthma
edema, smooth muscle thickening, BM thickening, mucous cell hyperplasia, increased eosinophils, thickened mucus
28
what are the primary clinical features of asthma?
acute, usually reversible bronchial narrowing due to airway hyperresponsiveness, chronic inflammation of the airways
29
what are charcot-leyden crystals?
caused by high levels of eosinophils and found in asthma
30
what are curschmann spirals?
dehydrated mucus that causes mucus casts, seen in asthma
31
clinical definition of chronic bronchitis
productive cough at least 3 mo out of the year for 2 years or more
32
pathology of chronic bronchitis
mucus cellular and glandular hyperplasia + submucosal chronic inflammation
33
two major causes of COPD
cigarette smoking or alpha1-antitrypsin deficiency
34
emphysema from cigarette smoking leads to ______lobular destruction predominantly in the _____ lobes
centrilobular; upper lobes
35
emphysema from A1AT deficiency leads to ______lobular destruction predominantly in the _____ lobes
panlobular; lower lobes
36
emphysema pathology
dilation of distal airspaces due to septal destruction; increased elastase activity
37
smoking causes elastic damage by?
recruiting elastase-sereting leukocytes and inhibiting anti-elastase
38
inheritance pattern of A1AT deficiency
AR
39
at what age does A1AT deficiency present?
middle age, under 40
40
A1AT is due to loss of function of this gene, and this allele leads to particularly severe disease
SERPINA1 gene; Z allele
41
AIAT deficiency leads to disease of the lung and this organ
liver (Z protein aggregates in rER of hepatocytes)
42
clinical presentation of BOOP/COP (organizing pneumonia)
acute onset cough, dyspnea, fever, malaise from drugs, infxn, coll-vasc dz
43
treatment of BOOP/COP
corticosteroids WORK WELL
44
pathology of BOOP/COP
patchy fibromyxoid plugs (fibroblastic prolif) in distal bronchioles and alveoli +/- endogenous lipid pneumonia
45
peribronchovascular stroma is composed of what?
fibrous connective tissue rich in lymphocytes and macrophages
46
interstitium
tissue separating alveoli
47
pathology of sarcoidosis
tight, well-formed non-caseating granulomata
48
granulomata are composed of these characteristic cell types
epithelioid histiocytes, occasional giant cells
49
radiological findings in sarcoidosis
interstitial infiltrates in broncial tree + hilar LN adenopathy
50
who is at greatest risk for sarcoidosis?
adults, black > white, females > males
51
hypersensitivity pneumonia (HP) is also known as?
extrinsic allergic alveolitis (EAA)
52
what causes hypersensitivity pneumonia?
organic dusts, animal proteins, synthetic organic chemicals, hot tubs, AC, hay, sugarcane, maple bark
53
pathology of HP
patchy peri-bronchiolar and interstitial chronic inflammation and loosely formed granulomata; visible lymphocytes
54
pneumoconioses are caused by?
inorganic dusts from occupational or environmental exposure --> lung fibrosis
55
three major causes of pneumoconiosis
silica, asbestos, coal
56
why do inorganic dusts cause fibrosis of the lung?
body cannot metabolize them; digested by macrophage, gives off cytokines that promote a fibrogenic response
57
early silicosis is rich in?
macrophages
58
late stage silicosis shows?
fibrotic nodules (pink on slide)
59
asbestosis causes this unique pathological finding
asbestos fibers; crystals less than 7mm that are brittle and undigestible; surrounded by iron released by macrophages --> ferruginous bodies
60
fibrosis caused by asbestos is usually patchy and confined to the ______ region
sub-pleural
61
coal-worker's pneumoconiosis leads to?
coal dust macules, black lung, no interference with gas exchange
62
usual interstitial pneumonia is also known as?
idiopathic pulm fibrosis
63
what is the prognosis of UIP?
poor; avg survival 3-5 yrs after dx
64
UIP can resemble what other diseases radiologically?
asbestosis, autoimmune dz (all three cause patchy subpleural infiltrates
65
important pathological finding that suggests UIP
temporal heterogeneity: alternating between fibroblasts, mature fibrosis, and normal tissue
66
late stage sub-pleural fibrosis resembles?
honeycomb change
67
diffuse alveolar damage (DAD)
shock --> fibrin rich alveoli --> after 1 week the fibrin is cleared, leading to lung remodeling --> decreased pulm compliance --> impaired mechanical ventilation
68
prognosis of DAD
50% mortality
69
pathology of DAD
interstitial +/- intra-alveolar fibroblastic proliferation in sites of fibrin accumulation (uniform fibrosis)
70
major difference between UID and DAD
both cause interstitial fibrosis, but with DAD it is temporally uniform and occurs predictably after shock
71
pathology of a hamartoma
solitary, lobulated, cartilagenous benign tumor made up of normal tissue in excess/disarray (can have popcorn calcification)
72
who presents with hamartomas?
adolescence to adulthood (never congenital)
73
what are the 4 types of malignant epithelial neoplasms in the lung (carcinomas)?
squamous cell, adenocarcinoma, large cell undiff, small cell undiff
74
what is the 5 year survival rate of lung cancer (unspecified subtype)?
15%; very high mortality
75
what % of lung carcinoma deaths occur in cigarette smokers?
more than 85%
76
cigarette smoking causes death by what three main disease outcomes?
lung cancer, ischemic heart disease, COPD
77
what % of tobacco particulates are cleared by cilia?
50%; the remainder are phagocytosed and transported through the lymphatics --> tatooing
78
describe the cause, location, pathological findings, and paraneoplastic phenomena assoc. with squamous cell carcinoma
cause: SMOKING, location: central (usually bronchi due to increased turbulence), path: desmosome bridges, keratin pearls; secretes PTH-like compound --> hypercalcemia
79
what are the three stages of adenocarcinoma
ACIS/BAC (less than 3cm, noninvasive); MIA (less than 3cm w/ small invasion), invasive (greater than 3cm OR large invasion)
80
describe the cause, location, pathological findings, and paraneoplastic phenomena assoc. with adenocarcinoma
can occur in non-smokers, peripheral, path: glands and/or mucin
81
how to detect mucin
PASd stain; mucin looks red
82
what is unique about BAC?
can be multifocal --> must distinguish from mets; not associated with smoking; butterfly-like growth pattern; VERY TREATABLE
83
how about large cell undiff?
carcinoma negative for all of the traits assoc with squamous cell and adenocarcinoma
84
prognostic variables for NSCLC
stage, performance status, weight loss (NOT age, histology)
85
describe the cause, location, pathological findings, and paraneoplastic phenomena assoc. with small cell carcinoma
20%; undifferentiated neuroendocrine carcinoma that presents late stage with rapid, necrotic growth of a primary central mass and metastasis to LN and liver; commonly causes SIADH leading to water retention and hyponatremia
86
therapy for small cell carcinoma
chemo/RT; very responsible initially but cancer always comes back and kills the patient
87
prognostic variables for small cell lung cancer
stage, performance status, women do better than men
88
mesothelioma
malignant neoplasm of the pleura caused by asbestos; deeply invasive growth pattern; immuno positive for keratin & calretinin, long microvilli; can appear epithelial, spindle cell, or biphasic
89
metastatic carcinomas that end up in the lung
breast, GI, renal, head/neck
90
metastatic melanoma is unique in that?
it can appear to be a primary cancer of the lung since it is often difficult to find to primary cancer