Chapter 15 Robbins Flashcards

1
Q

endemic area for coccidiomycosis

A

sw and W us and mexico

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2
Q

autoimmune pap

A

autoimmune
-abs for GM-CSF, 90% of cases, lacks familial predisp, loss of GM-CSF blocks terminal diff of alveolar macrophages impairing ability to catabolize surfactanct

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3
Q

legionella pneumonia common in what individuals

A

with predisposing conditions like cardiac, renal, imune, hematologic

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4
Q

focal areas of cell consoidation of lung substance chiefly in periphery of lung fields

  • heavey aggregates of lymphocytes and eosinophil lesions in septal walls and alveolar spaces
  • pts have cough, fever, night sweats, dyspnea, and weight loss
  • response to corticosteroid therapy
A

idiopathic chronic eosinophilic pneumonia

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5
Q

adenocarcinma and genetics

A

GOF mutation in GF receptor signaling path

genes encoding RTKs like
-EGFR,ALK,ROS,MET,RET MR EAR

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6
Q

prognosis of goodpasture impvoed by

A

intensive plasmapheresis, also give simultaneous immunosuppressive therapy

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7
Q

____ pneumonia is localized lesion in ICP with or without regional LN involvement

A

chronic

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8
Q

associated with URI and LRI
most common infection in young children, elderly, and ICP
can cause bronchioloitis and pneumonia
first infection in childhood, reinfections common throughout life
clinically indistinguihsable from those caused by RSV and often mistaken for influenza

A

human petapneumovirus

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9
Q

symptom in viral laryngotracheobronchitis and bronchiolitis

A

vocal cord swelling and abundant mucus production

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10
Q

complications of lung abscess

A

extension into pleural cavity, hemorrhage, and development of brain abscesses or meningitis from septic emboli

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11
Q

what infection: associated with lung abscess and emyema

importnant cause of hospital acquired pneumonia

important cause of secondary bacterial pneumonia in children and healthy adults following viral resp illness

A

staphy aureus

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12
Q

evidence that hypersensitivity pneumonitis is immuno mediated

A

specimens have increased MIP1a and IL-8

  • increased CD4 and CD8
  • abs against causitve agent in serum
  • noncaseating granulomas = t cell mediated IV hypersens
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13
Q

infrequent complications in lung transplant

A

accelerated pulmonary arteriosclerosis in the graft and EBV associated B cell lymphoma

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14
Q

mode of transmission legionella

A

inhalation of aerosolized organisms or aspiration of contaminated drinking water

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15
Q

PHTN clinical course

A

most common in women ages 20-40 and occasionally in young children

  • dyspnea, fatigue, some pts have chest pain
  • overtime respiratory distress, cyanosis, rvh, death from cor pulmunale within 2-5 yrs of 80% of pts
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16
Q

radiolograph of PAP

A

bilateral patchy asymmetric pulmonary opacifications

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17
Q

morphology of lung rejection

A

lymphocytes, plasma cells, few neutrophils and esoinophils around small vessels, in submucosa of airways or both

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18
Q

adenocarcinoma that spreads forming satellite tumors

-solitary or multiple nodule

A

mucinous adenocarcinomas

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19
Q

1 year survival rate of lung transplant

A

79%

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20
Q

during cardiopulmonary resusciation from PE pt frequently has ____

A

electromechanical dissociation

-ECG has rhythm but no pulses palpated bc no blood entering pulmonary arterial circulation

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21
Q

location of adenocarcinoma

A

periphery

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22
Q

most common bacterial cause of actute exacerbation of COPD

A

haem infl

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23
Q

surfactant protein B clinical presentation

A

infant is full term and rapidly develpos progressive respiratory distress shortly after birth
death btwn 3-6 months

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24
Q

variable amt of intra alveolar pink granular material, type II pneumocyte hyperplasia, interstitial fibrosis and alveolar simplification

abnormalities in lamerllar bodies in type II pneumocytes

A

surfactant dysfunction disorder morphology

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25
Q

treatment for human MPV

A

ribavirin

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26
Q

imaging in pulmonary langerhans cell histiocytosis

A

imaging shows characterisitc cystic and nodular abnormalities

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27
Q

cardinal histologic change in all abscesses is

A

suppurative destruction of lung parenchyma within the central area cavitation

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28
Q

etiology and pathogenesis of lung abscess

A

common organism are aerobic and anerobic streptococci, staph aureus, and gram negative organisms

anerobic organsism normally in oral cavity include bacteriodes, fusobacterium, and peptococcus

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29
Q

hereditary PAP

A

extremely rare

occurs in neonates caused by mutations that disrupt genes involved in GM-CSF signaling

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30
Q

morphology of histoplasmosis infection

A

granulomas with caseating necrosis and consolidation

-differentiate from TB, sarcoidosis and coccidio by identification of 3-5 micromietere yeast forms in tissues

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31
Q

epidemiology of goodpatrures

A

most cases occur in teens or 20s in male smokers

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32
Q

clinical features of hypersensitivity pneumonitis

A

acute attacks, of recurring episodes of fever dyspnea, cough, leukocytosis

  • micronodular interstitial infiltrates on radiograph
  • symptoms may last for 12 hours to several days
  • if chronic exposure can lead to progressive resp failure
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33
Q

CD4+ count under 50 HIV infections

A

CMV, fungal, mycobacterium avium

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34
Q

neutrophils containing lancet shaped diplococci

A

streptococcus pneumoniae

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35
Q

squamous cell carcinoma genetics

A

chrom deletion involving 3p, 9p (site of CDKN2A gene) and 17p (site of TP53) gene are early events in tumor evolution

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36
Q

lymphangioleiomyomatosis population

A

primarily affects young women of childbearing age

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37
Q

3 types of blastomycosis

A

pulmonary, disseminated, and rare primary cutaneous form

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38
Q

bronchioloitis obliterans

A

major morphologic corelate of chronic rejection

occluion of small airways by fibrosis
patchy and therefore difficult to diagnose

-bronchiectasis and pulmonary fibrosis may develop in long standing cases

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39
Q

lobar pneumonia 4 stages of inflammatory response

A

CRGR

congestion
-few neutrophils, numerous bact, heavy, boggy, red lung

Red hepatization
-confluent exudation, neutrophils, red cells, fibrin fill alveolar spaces, lobe is red, firm, airless, liver consist

gray hepatization
-disintegration of red cells and persistence of fibroinosuppurative exudate = grayish brown color

resolution

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40
Q

what mutation is associated with pneumococcal pneumonias

A

mutation in MyD88 (adapter for TLRs for activation of NFkB)

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41
Q

chronic rejection of lung transplant occurs when and in how many pts

A

1/2 of pts within 3-5 yrs

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42
Q

DIP presentation

A

40s and 50s, sex equal
all pts smoke cigs
insidious onset of dyspnea and dry cough over weeks or month, with associated clubbing of digits

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43
Q

most common organism with health care associated pneumonia

A

MESR and P aeruginosa

these pts have higher mortality than those with community acquired pneumonia

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44
Q

pulmonary htn causes

A

chronic obstructive or interstitial lung diseases

antecedent congenital or acquired heart disease (MS)

recurerent thromboemboli

autoimmune disease (systemic sclerosis w/ pulmonary vasculature involvement)

obstructive sleep apnea

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45
Q

SARS is a ___virus that infects

A

coronavirus that infects LRT and spread throughout body

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46
Q

diagnosis of PE and dvt

A

pe- spiral computed tomographic angiography

DVT diagnoses with ultrasound

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47
Q

haem influenze with nonencapsulated forms

A

= nontypeable forms
spread along surface of URT and produce otitis media, sinusitis, and bronchopneumonia

neonates with prematuritiy, malignancy, and immunoddef more at risk

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48
Q

DIP treatment

A

excellent response to steroid therapy and smoking cessation

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49
Q

Desquamative interstitial pneumonia morphology

A

accumulation of large number of macrophages with dusty brown pigment in airspaces (smokers macrophages)

alveolar septa are thickened and lined by plump cuboidal pneumocytes

emphysema often present

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50
Q

tumors with FGFR1 amplification

A

squamous cell carcinoma

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51
Q

most common cause of death in goodpasture

A

uremia

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52
Q

lymphangioleiomyomatosis proliferation and markers

A

of perivascular epitheloid cells that express markers of melanocytes and SM cells

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53
Q

hypersensititvity pneumonitis caused by thermophilic bacteria in heated water reservoirs

A

humidifier or AC lung

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54
Q

langerhans cells positive for

A

S100, CD1a, CD207

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55
Q

pulmonary htn

A

defined as mean pulmonary artery pressure greater than or equal to 25mm Hg at rest

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56
Q

morphology of fulminant disseminated histoplasmosis

A

in immunosuppressed, granulomas do not form

focal accumulation of mononuclear phagocytes filled wwith fungal yeasts

57
Q

CD4+ count under 200 in HIV infection

A

pneumocystis pneumonia

58
Q

most commnly causes hostpital acauired infections, pts who are neutropenic and it has propensity to invade blood vessels with extrapulmnary spread

associated with CF and immunocomp pts

A

pseudomonas aeruginosa

59
Q

foci of bronchopneumonia

A

consolidated areas of acute suppurative inflammation

  • confined to one lobe but more often multilobar and bilateral and basal
  • well developed lesions, granular ,gray, red, yellow
  • neutrophil rich exudate
60
Q

what hypersensitivity pneumonitis develops from exposure to dusts from hay that permits rapid proliferation of spores of thermophilic actinomycetes

A

farmers lung

61
Q

5 year survival rate lung transplant

A

53%

62
Q

where is histoplasma capsulatum endemic

A

mississippi rivers and caribbean

63
Q

intermittent diffuse alveolar hemorrhage
young children, some adults
insidious onset of productive cough, hemoptysis and anemia
favorable response to long term immunosuppresion with prednisone and or azathioprine

A

idiopathic pulmonary hemosiderosis

64
Q
inflammatory myofibroblastic tumor
who gets it
imaging
microscoping
mutation
A

children, equal M:F

imaging: single round well-defined usually peripheral mass with calcium deposits
- grayish white 3-10 cm in diameter

microscop: prolif of fibroblasts

mutation in ALK gene on chrom 2

65
Q

what can show a coin lesion on an xray

A

histoplasmosis

66
Q

control of influenza infection depends on

A

interfreon a and b produced by infected cells
-these upregulate expression of MX1 gene which is a GTPase that disrupts influenza gene txn

  • cell mediated response, NKC
  • Abs against H and N proteins
67
Q

where does histoplasmosis capsulatum lung infection spread to

A

mediastinum
adrenals
liver
meninges

68
Q

morphology of c immitis

A

c immitis is within macrophages or giant cells

  • thick walled, nonbudding speherules 20-60 micrometers in diameter with small endospores
  • pyogenic reaction superimposed when spherules rupture and release endospores
69
Q

hypersensitivity pneumonitis provoked by proteins from serum, excreta, or feathers of birds

A

pigeon breeder’s lung

70
Q

PE morphology

A

3/4 of in lower lobes
lines of zahn in the thrombus
hemorrhagic, raised, red blue in early stages
pleural surface covered by fibrinous exudate
-w/in 48 hrs becomes paler, then red brown as hemosiderin produced
-can have septic infarcts

71
Q

CDKN2A is inactivated and its protein product P16 is lost in 65% of these tumors

A

squamous cell carcinoma

72
Q

symptoms of respiratory bronchioloitis associated interstiial lung disease

A

mild with gradual onset of dyspnea and cough of smokers in 40s and 50s

-cessation results in improvement
pack yr history usually over 30

73
Q

treatment for PAP

A

whole lung lavage and GM-CSF therapy for pts with autoimmune pap

74
Q

atp binding cassete protein member 3 clinical course

A

pt presents first few months of life with rapid progressive respiratory failure followed by death

75
Q

human metapneumovirus is a

A

paramyxovirus

76
Q

predomiant reaction in viral infectiont

A

interstitial inflammatory reaction involving the walls of the alveoli
-alveoli septa are widened and edematous and usually have mononuclear inflammatory infiltrate

77
Q

large collections of macrophages in airspaces in current or former smoker

A

desquamative intersititial pneumonia

DIP

78
Q

less than 3 cm and composed entirely of dysplastic cells growing along preexisting alveolar septae

A

adenocarcinoma in situ

79
Q

fungal infections during lung transplant mainly by

A

aspergillus and candida

80
Q

bacterial pneumonia has 2 patterns of anatomic distribution

A

lobular and lobar
patchy consolidation is bronchopneumonia
consolidation of a large portion of a lobe is lobar pneumonia

81
Q

chronic pneumonia is/is not granulomatous and caused by

A

is
M tuberculosis
histoplasma capsulatum

82
Q

rare disease caused by defects related to GM-CSF or pulmonary macrophage dysfunction that results in accumulation of surfactant in intra-alveolar and bronchilar spaces

A

pulmonary alveolar proteinosis

83
Q

HLA involved in goodpasture

A

HLADRB1*1501 and 1502

84
Q

emboli have 2 deleterious pathophysiologic consequences

A

respiratory compromise due to nonperfused, although ventilated segment

hemodynamic compromise due to increased resistance to pulmonary blood flow through the lungs

85
Q

complications of pneumonia

A

tissue destruction and necrosis = abscess formation (particualllry w/ klebsiella infection)

spread of infection to pleural cavity = empyema

bacteremic dissemination, = metastatic abscesses, endocarditis, meningitis, suppurative arthritis

86
Q

abca3 mutation in surfactant disorder differentiation

A

small lamerllar bodies with electron dense cores

87
Q

pulmonary infiltrates with noninfectious diseases in HIV

A

kaposi sarcoma
non hodgkin lymphoma
lung cancer

88
Q

soild inhabiting dimorphic fungus

causes disease in central and SE US

A

blastomycosis dermatitidis

89
Q

PAP presentation

A

adult pts present with cough and abundant sputum that often contains funks of gelatinous material

-progressive respiratory failure may occur and pts are at risk for infetion

90
Q

what reactant producnts are more raised in bacterial infections than viral

A

CRP, procalcitonin

91
Q

bacterial lower respiratory tract infections are among the most serious pulmonary disorders in HIV infections, culprits are

A

S pneumoniae
S aureus
H influenza
gram neg rods

PAIG pulm HIV

92
Q

pulmonary eosinophilia responds to

A

corticosteroids

93
Q

what environment does legionaella thrive in

A

artificial aquatic environments like water cooling towers and tubing systems of domestic water supplies

94
Q

most frequent cause of gram negative bacterial pneumonia

afflicts debilitated and malnourished people particularly chronic alcoholics

A

klebsiella pneumoniae

95
Q

makers for squamous cell carcinoma

A

p63 and p40

96
Q

high mortality rate laryngotraheobronchitis results in airway obstruction as bronchi plugged by desne fibrin rich exudates containing neutrophils

pulmonary consolidation usually lobular and patch but can involve all of lobe

acute conjunctivitis in children

this is an infection with what

A

haem infl

97
Q

10 year survival of lung transplant

A

30%

98
Q

haemophilus influenzae structure

A

pleomorphic, gram neg, encapsulated and noncencapsulated forms

99
Q

secondary PAP

A

uncommon
associated with hematopoietic disorders, malignancies and more

diseases impair macrophage maturation

100
Q

pulmonary langerhans cell histiocytosis

cell level

mutation

A

accumulation of langerhans cells to form nodules along with fibroblasts, macrophages, and numerous eosinophils

some lesions behave as reactive process, others show BRAF mutations

101
Q

pulmonary blastomycosis symptoms, chest xray and treatment

A

symptoms: fever, ab pain, night sweats, chills, anorexia, weight loss

chest x ray: lobar consolidation, perihilar infiltrates,
-upper lobes most freq involved

treat: most resolve spontaneously

102
Q

diffuse infiltrates and bronchoalveolar lavage fluid contains more than 25% eosinophils

A

acute pulmonary eosinophilia with respiratory failure

103
Q

moraxella catarrhalis

A

cause of bacterial pneum especially in elderly
second most common bacterial cause of acute exacerbation of COPD
-1 of 3 most common causes of otitis media in chidlren

104
Q

chest radiograph and PE

A

12-36 hours after occurance may find a wedge shaped infiltrate

105
Q

CD4+ count over 200 in HIV and infection

A

TB and bacterial infections

106
Q

microinvasive adenomcarcinoma

-outcome?

A

tumors that are 3 cm or less with small invasive component associated with scarring and peripheral lepididc growth pattern
-better outcome than invasive carcinoma of same size

107
Q

surfactant protein C

A

second most common of 3 surfactant disorders

108
Q

hospital acquired pneumonia

A

pts on mechanial ventilation are at particularly high risk

gram positive S aureus and S pneumonia and gram negative rods (enterobacteriaceae and pseudomonas)

109
Q

surfactant dysfunction disorders

A

ATP binding cassete protein member 3 (AR)

surfactant protein C (AD)

surfactant protein B (AR)

110
Q

PHTN morphology

A

medial hypertrophy of pulmonary muscular and elastic arteries, pulmonary arterial atherosclerosis and RVH

arterioles and small arteries are most prominently affected by striking medial hypertrophy and intimal fibrosis

extreme is plexiform lesion, spans lumens of dilated thin walled small arteries and may extend outside vessel

  • prominent in idiopathic and familial phtn group 1
  • also unrepaired congenital heart disease with L-R shunts (group 2)
  • pulmonary htn associated with HIV infection
111
Q

causes legionaires disease and pontiac fevere

A

legionaella pneumophila

112
Q

pts in pulmonary langerhans cell histiocytosis

A

usually young smokers

113
Q

polyangiitis with granulomatosis diagnosis

A

transbronchial lung biopsy

capillartiis, scattered, poorly formed granulomas

114
Q

3 types of pap

A

autoimmune
secondary
hereditary

115
Q

respiratory bronchiolitis associated interstitial lung disease

A

chronic inflammation and peribronchiolar fibrosis
common in cig smokers
pigmented intraluminal macrophages in respiratory bronchioles

116
Q

symptoms of coccidio infection

A

most asymptomatic
10% get lung lesions, fever, cough, pleuritic pains with erythema nodusum or erythema multiforme

less than 1% get C immitis infection which involves skin and meninges

117
Q

frequent cause of death in aspiration pneumonia and organisms recovered

A

necrotizing pneumonia
more than one organism, most often aerobes
-abscesses

118
Q

treatment for PHTN

A

vasodilators for group 1 or refractory disease belonging to other groups

119
Q

idiotpathic pulmonary arterial HTN

A

encountered in pts whom all known causes are excluded

  • 80% have genetic basis inherited in AD trait
  • BMPR2 gene (TGF-B family)
  • haploinsufficiecny leads to dysfunction and proiferation of endothelial cells and vascular SM cells
  • 2 hit model like RB
120
Q

encapsulated forms of haemophilus influenzae

A

a-f

b most virulant

121
Q

3 most common causes of otitis media in children

A

s pneumoniae, h influenza, m catarrhalis

122
Q

location of localized lung disease with histoplasmoisis

A

lung apices

123
Q

spectrum of immune mediated predominantly interstitial lung disorders caused by intense often prolonged exposure to inhaled organic antigens

A

hypersensitivity pneumonitis

124
Q

morphology box for pulmonary alveolar proteinosis

A

homogenous, granular precipitate containing surfactant proteins within alveoli
-causes focal to confluent consolidation of large area of lung with minimal inflamation

alveoli filled with granular pink precipitate

-marked increase in size and weight of lung

periodic acid schiff positive, contains cholesterol clefts and surfactant proteins

125
Q

______ infectivity is bc infective arthroconidia when ingested by alveolar macrophages block fusion of phagosome and lysosome so resist intracellular killling

A

coccidiodomycosis immitis

126
Q

lymphangioleiomyomatosis presentation and treatment

A

dyspnea and spontaneous pneumothorax

need lung transplant

127
Q

what pts are most susceptible to histoplasmosis infection

A

immunocompromised

128
Q

highest frequency of TP53 mutations of all lung carcinomas is

A

squamous cell carcinoma

129
Q

diagnosing legionella

A

culture is gold standard

130
Q

cell mediated immune defects increases pulmonary infections with

A

mycobacteria, herpres, and pneumocystis jiroveci

131
Q

morphology of hypersensitivity pneumonitits

A

histologic changes centered on bronchioles
-interstitial pneumonitis, consisting primarily of lymphocytes, plasma cells, and macrophages (eosinophils rare)

  • noncaseating granulomas in 2/3 of pts
  • interstitial fibrosis with fibroblastic foci, honeycombing, and obliterative bronchiolitis
  • intraalveolar infiltrate in over half pts
132
Q

what chromosomes are associated with hamartoma

A

6 and 12

133
Q

lymphangioleiomyomatosis mutation

A

TSC2, linked to tuberus scleoris (tuberin)

-mutation increases mTOR activity

134
Q

which bacterial superinfection on top of influenza virus can produce ARDS and be life threatening

A

staph aureus

135
Q

organisms are introduced in lung abscesses the following ways

A

MAANS in (LA)

miscellanesous
direct traumatic penetrations of lungs, spread from neighbor organ, hematogen seeding of lung-

aspiration

antecedent primary lung infection: S aureus, S pneumoniae

neoplasm

Septic emboli

136
Q

small cell carcinoma genetics

A

loss of TP53 in 75-90% and loss of RB in almost 100%

amplification of MYC family

137
Q

respiratory bronchioloitis associated interstitial lung disease

A

changes are patchy with bronchiolocentric distribution

dusty brown smoker macrophages similar to those seen in DIP

138
Q

what is being described: small lesion less than or equal to 5mm characterized by dysplastic pneumocytes lining alveolar walls that are mildly fibrotic

A

atypical adenomatous hyperplasia