Midterm - Function Flashcards
2
Q
asthma results in ____ exhaled NO from airways
A
INCREASED
3
Q
pathogenesis of asthma
A
periodic airway hyperreactivity/bronchoconstriction & mucus chronic plugging/inflammation
4
Q
describe the death rate from asthma
A
varies WIDELY and we do not understand why
5
Q
PFT diagnosis of asthma shows these 5 results:
A
- obstructive ventilatory defect, 2. auto-PEEP (increased end-exp pressure), 3. high DLCO, 4. hyperresponsiveness to methacholine challenge, 5. reversible airflow obstruction with bronchodilator
6
Q
bronchodilator responsiveness in asthma vs COPD
A
reversibility in both, but there is much greater and more consistent reversibility in asthma
7
Q
methacholine challenge tests?
A
bronchial hyperreactivity
8
Q
repeated challenge tests in asthma are different in that?
A
they do NOT plateau with time, instead the slope goes straight up (can kill them)
9
Q
beta agonists prevent ____ hyperreactivity, whereas steroids prevent ______ response
A
initial; late stage
10
Q
allergic inflammation in asthma is caused by abnormal levels of what helper T cell?
A
Th2, leading to increased mast cells and eosinophils
11
Q
sputum of an asthmatic may show what four pathologic findings?
A
charcot-leyden crystals (from eosinophils), mucus casts (curschmann’s spirals), creola bodies (ciliated columnar cells), and eosinophils
12
Q
what is extrinsic asthma?
A
atopic version, presents at young age, family hx of allergies, specific allergic triggers
13
Q
what is intrinsic asthma?
A
usually adult-onset following severe resp illness, perennial sx refractory to tx, not well understood
14
Q
lecturer’s clear favorite tx for asthma
A
LABA + ICS
15
Q
what is samter’s triad?
A
NSAID sensitivity, late-onset asthma, rhinitis and nasal polyps
16
Q
salicylate sensitivity can cause?
A
asthma
17
Q
what is COPD?
A
progressive, not fully-reversible airflow limitation usually due to noxious particles
18
Q
COPD mortality is rising/declining, especially in men/women?
A
rising, especially in women
19
Q
what percent of COPD occurs in smokers?
A
80-90%
20
Q
what percent of smokers develop COPD?
A
15-20%
21
Q
what are three causes of chronic obstruction, and what are their corresponding diseases? Hint: 2/3 are the same disease
A
small airway remodeling (chronic bronchitis), alveolar wall destruction (emphysema), mucus hypersecretion (chronic bronchitis)
22
Q
what proteases contribute to COPD pathology?
A
elastase, matrix metalloproteases
23
Q
what counteracts the effects of these proteases, and is missing is genetic COPD?
A
alpha-1 antitrypsin
24
Q
what are two sources of increase elastolytic activity in COPD?
A
impaired mucociliary clearance –> bacterial colonization –> increased elastase from bacteria, as well as increased inflammation which produces elastlate
25
Q
what are the consequences of loss of elastic recoil in emphysema?
A
- lower driving pressure for expiration, 2. loss of radial traction leading to airway collapse, 3. chest wall expansion
26
symptoms of COPD
SOB, cough (may not be productive), wheezing, fatigue and weakness from working hard to breath, LATE = morning headaches from high CO2, edema
27
diagnosis of COPD
requires spirometry; specifically a low FEV1, increased TLC and RV, decreased IC
28
classification of COPD is based on?
degree of obstruction, as defined by FEV1
29
why should helium dilution not be used to measure lung volumes in COPD? What should be used instead?
helium underestimates degree of abnormality in COPD; whole-body plethysmograph is better
30
chest radiograph changes of COPD occur ____ and include?
late; expanded chest, retrosternal air space, low and flat diaphragm, increased CVA, decrease in peripheral vascularity
31
exercise causes ____ in pts with COPD
dynamic hyperinflation, leading to dyspnea
32
what effect does COPD have on DLCO?
DLCO is decreased because diffusion decreases as a result of alveolar destruction
33
blood gas analysis in COPD shows?
high CO2, relatively normal pH due to chronic state of high CO2 and compensation
34
when should oxygen therapy be given in lung dz?
O2 sat <55% (only drug that can decrease mortality from COPD)
35
chronic hypoxemia in COPD leads to?
RHF, cor pulmonale
36
best tx for COPD
smoking cessation
37
when should pulmonary rehabilitation therapy be included in a COPD tx regimen?
State II: Moderate Disease
38
who is a good candidate for surgery to treat COPD?
has stopped smoking for at least 4 mo, is able to undergo pulm rehab, has potentially removeable areas of lung that are poorly fnc
39
what 3 things can reduce mortality in COPD?
1. stop smoking, 2. oxygen tx, 3. lung volume reduction surgery in the right patient
40
one in how many caucasians are a carrier for the CFTR gene?
1 in 29 (so affects 1 in 3,300 births)
41
common problems in patients with CF
recurrent infection, allergic rhinitis/sinusitis (nasal polyps), pancreatic insufficiency (most on enzymes), bleeding duodenal ulcers, liver disease and cirrhosis
42
long-term side effects of CF
osteopenia, malnutrition, diabetes mellitis, respiratory failure and need for transplant
43
median survival age for CF is now?
late 30s!
44
what is locus heterogeneity?
ONE GENE with multiple different mutations that can cause a disease; see this in CF
45
what is the most common genotype of CF?
deltaF508
46
which gender has a survival disadvantage?
females
47
in addition to genetics, what factors contribute to CF phenotype/survival?
socioeconomic status, tobacco, nutrition, access to care, adherence
48
why do CF patients have chronic infection of the airways?
thick mucus + high cellular metabolism = great niche for anaerobic organisms like pseudomonas to form biofilms
49
how does chronic infection lead to lung damage?
neutrophils come to clear the infection, which doesn't work but damages the epithelium anyway (from release of elastase, etc.)
50
which organisms preferentially inhabit young vs older CF patients?
young = haemophilus influenzae; old = pseudomonas aeruginosa
51
diagnostic criteria for cystic fibrosis
1+ typical phenotypic features (sinopulm dz, GI sx, or fam hx) and evidence of CFTR malfunction (sweat test, gene test)
52
when are the majority of CF patients diagnosed?
first year of life
53
major complications of CF
hemoptysis, pneumothorax, resp failure, diabetes, arthritis/vasculitis, gallbladder/kidney stones, infertility in males especially, peri-osteal bone formation (HPOA) (risk increases with age, so does frequency of acute exacerbations)
54
"acute" therapy for CF includes?
IV antibiotics, airway clearance (hypertonic saline, special vests)
55
"maintenance" therapy for CF includes?
inhaled/oral abx, airway clearance (mucolytics, saline, rhDNase)
56
how expensive is it to treat CF?
HUGE BURDEN OF TX
57
lung transplant survival for CF
on high end of survival curve, but still only 25% survival rate 15 years post-transplant
58
key clinical feature of bronchiectasis
copious amounts of purulent sputum
59
pathologic definition of bronchiectasis
pathologic dilatation of airways with or without obstruction; same as CF but without a known cause
60
other pathologic features of bronchiectasis
floppy and inflamed with mucus impaction and complete loss of tapering; INFLAMMATION; br wall may be destroyed while arteries may be hypertrophied; squamous metaplasia; plasma cells, eosinophils; ulceration leading to hemoptysis
61
what is the most common cause of bronchiectasis?
post-infection (some recall bias), but 50% is idiopathic
62
clinical sx of bronchiectasis
cough, mucopurulent sputum, dyspnea, wheeze, chest pain, fatigue, anorexia, weight loss, hemoptysis, exacerbations, finger clubbing, cyanosis/cor pulmonale end stage
63
clinical sx of PCD
neonatal resp problems, recurrent otitis media, sinus inversus, infertility
64
gold standard investigation for bronchiectasis
sputum microbiology
65
bacterial colonization in bronchiectasis
mycobacteria, same as CF, no organisms at least 20% of the time
66
if a HRCT scan is positive for bronchiectasis, what should you do?
follow up diagnostic tests to confirm CF, CVID, A1AT, PCD (nasal NO), CTD, ABPA
67
how do you diagnose PCD?
nasal NO measures significantly low
68
treatment for bronchiectasis
abx, airway clearance, bronchodilators, anti-inflammatory agents, surgery if local, transplant if severe, oxygen tx prolongs life
69
what constitutes a sufficiently severe FEV1 to consider transplant in pt with bronchiectasis?
FEV1 <30% predicted
70
which airway clearance tx used in CF should NOT be used in pts with other forms of bronchiectasis?
rhDNase (pulmozyme)
71
full time tx with this antibiotic can be used for its anti-inflammatory abilities in patients with bronchiectasis; shown to decrease exacerbations
macrolides (only if confident no mycobacteria infection)
72
there have been several recent studies considering ______ antibiotics for improved QOL and decreased exacerbation in patients with bronchiectasis
inhaled
73
when to use IV abx?
during exacerbation, refractory to oral, hemoptysis, resistant gram neg, previous hx
74
what medicines should be given before/during a lung transplant?
tacrolimus, mycophenolate, and steroids
75
what are the 3 requirements for normal inspiration?
1. compliant chest wall, 2. normal muscle strength, 3. compliant lungs
76
obstructive lung disease causes impaired ______, while restrictive lung disease causes impaired ______
expiration; inspiration
77
major abnormalities causing decreases compliance of the chest wall
1. fibrothorax, 2. kyphoscoliosis, 3. morbid obesity
78
weakness of respiratory muscles occurs in?
1. myasthenia gravis, 2. ALS
79
abnormalities of the lung parenchyma that cause decreased lung compliance
sarcoidosis, idiopathic pulm fibrosis (UIP)
80
interstitial lung disease would better be called?
diffuse parenchymal lung disease (expansion extends beyond the interstitium)
81
DPLD/ILD mimics these two common diseases
CHF, pulmonary infection (pneumonia)
82
DPLD leads to diffusion impairment due to?
decreased lung compliance and pulm HTN?
83
lung volumes in IDL
low TLC, FRC, and RV
84
lung volumes in pts with muscle weakness
low TLC, high RV
85
lung volumes in pts with obesity
low FRC
86
most severe side effect of ILD
pulmonary HTN --> RVH --> cor pulmonale/RHF
87
the majority of DPLD is caused by?
unknown etiology
88
known etiologies of DPLD include?
inhaled organic dusts (hypersens pneumonia), pneumoconiosis, iatrogenic (radiation, drugs)
89
DPLDs with unknown etiology
sarcoidosis, idiopathic pulm fibrosis (UIP), BOOP/COP, eosinophilic pneumonia, CTD-related ILD
90
sarcoidosis is most common in?
black females 20-40 years old
91
best theory on cause of sarcoidosis
Th1 mediated immune response from something inhaled interacting with T cells and APCs; greatest genetic linkage in MHC-related genes
92
putative antigens in sarcoid
mycobacteria, propionibacteria
93
Lofgren's syndrome (seen in sarcoid)
fever, adenopathy, erythema nodosum, symmetrical ankle arthritis
94
cutaneous involvement occurs in ____% of sarcoid
25
95
stage I sarcoidosis
intrathoracic LN only (spont remission in 2/3)
96
stage II sarcoidosis
parenchymal involvement + adenopathy
97
stage III sarcoidosis
parenchymal involvement, NO adenopathy
98
stage IV sarcoidosis
upper lobe fibrosis with hilar retraction, honeycomb changes, large bullae and cysts (lungs clear on exam)
99
lupus pernio
indurated plaques and discoloration on the face, indicates prolonged course of sarcoid, can biopsy and find non-caseating granulomas
100
diagnostic test for sarcoid
bronchoscopy to exclude infection and take a biopsy
101
diagnostic criteria for sarcoid
1) granulomas, 2) no other cause of granuloma (TB, fungus, cancer-lymphoma or epithelioid)
102
treatment of sarcoid
often don?t need tx, start with corticosteroids, then chemotx, anti-TNF
103
who gets IPF/UIP?
older adults
104
why is it important to diagnose UIP?
it has a much poorer prognosis and decreased survival time relative to other IIPs
105
what is important to rule out in a case that resembles UIP?
connective tissue diseases, autoimmune diseases
106
what is the pathogenesis of IPF?
defective injury repair: injury to alveoli epithelium --> tissue damage and death --> damage to BM and release of cytokines --> stimulation of fibroblasts --> collagen deposition (impeding normal recovery)
107
risk factors for IPF
family hx, smoking, environmental dust exposure, chronic aspiration from GERD, infectious agents
108
clinical presentation of IPF
age >50, gradual onset, progressive dyspnea on exertion, nonproductive paroxysmal cough, bibasilar crackles like velcro (80%), digital clubbing (25-50%)
109
late stage IPF may present with?
sleep apnea, cyanosis, cor pulmonale, peripheral edema
110
pulmonary function in IPF
reduced FVC and FEV1, high FEV1/FVC, reduced lung volumes across the board, decreased diffusion capacity (low DLCO), decreased PaO2 and increased A-a gradient, desaturation on exercise
111
IPF on x-ray
subpleural and basilar distribution of infiltrate; usually bilateral but not necessarily symmetrical
112
High res CT can be diagnostic for IPF. What 4 things should you find?
1. subpleural honeycomb, 2. basilar distribution, 3. traction bronchiectasis, 4. minimal ground glass opacity
113
when clinical and radiological assessment is inconclusive for ruling out UIP/IPF, you should do a?
video-assisted thoracoscopic (VATS) biopsy
114
histopathology of IPF
temporal heterogeneity!!
115
treatment of IPF
oxygen tx (to prevent/delay RHF), lung transplant
116
cancer deaths from lung cancer are greater than the next ____ cancers combined
5!
117
lung cancer is most common in and kills more ___ than any other demographic
african american men
118
risk factors for lung cancer (6)
smoking, second hand smoke, family hx BIG, previous tobacco-related cancer, occupational exposure, COPD
119
women are more likely to get this type of lung cancer
adenocarcinomas, non-smoking related lung cancer
120
evidence suggests that the biological difference seen in women with lung cancer is, in part, due to?
estrogen signaling
121
areas of previous scarring (such as fibrotic lung dz) may be a precurser for this type of cancer
adenocarcinomas
122
this type of non-small cell lung cancer is most likely to metastasize
adenocarcinomas
123
hypertrophic periosteoarthritis and clubbing are signs of?
adenocarcinoma
124
these lungs cancers are usually central
squamous cell, small cell
125
this lung cancer is usually peripheral
adenocarcinoma
126
BAC is now known as?
non-mucinous adenocarcinoma with lepidic features (adenocarcinoma in situ)
127
is ACIS more common in men or women? Smokers or non-smokers?
women, non-smokers
128
this is the only primary lung cancer that can present with multiple nodules
BAC/ACIS
129
what does ACIS look like on a HRCT?
ground glass, infiltrate/consolidation with air bronchograms
130
what lung cancer frequently causes frothy sputum?
BAC/ACIS
131
this lung cancer occurs in smokers, causes obstruction of the airway with distal atelectasis, may cavitate, and presents with hypercalcemia and clubbing
squamous cell
132
this substance is the cause of hypercalcemia in lung cancer
PTH-like substance produced by tumor
133
hypercalcemia is never seen in this type of lung cancer
small cell lung cancer
134
bulky central tumor with extensive mediastinal lymph node involvement, grows rapidly and presents with SIADH
small cell lung cancer
135
what are the symptoms of SIADH
low sodium assoc with mental status changes
136
specific symptoms assoc with lung cancer
cough, hemoptysis, dyspnea, hoarseness, SVC syndrome, headache, mental status changes, sx of pneumonia, chest wall or bone pain
137
what are paraneoplastic syndromes?
clinical disorders not related to size or metastasis of cancer; may even precede diagnosis
138
paraneoplastic syndromes seen in non-small cell lung cancer
hypercalcemia, HPO, clubbing
139
paraneoplastic syndromes seen in small cell lung cancer
SIADH, Cushing's, Lambert-Eaton (muscle weakness), cerebellar degen, periph neuropathy, weight loss
140
best diagnostic test for primary lung cancer
endobronchial biopsy
141
what are the downsides to sputum cytology?
need more than 3 specimens, negative result does not exclude cancer
142
what should be done to assess whether a pleural effusion is malignant or paramalignant?
thoracentesis
143
when and how should transthoracic needle biopsies be performed?
to diagnose a peripheral lesion; perform under CT guidance
144
non-small cell lung cancer is usually diagnosed at what stage?
stage IV
145
treatment for early stage non-small cell lung cancer
surgical resection (plus chemo after sometimes for hilar node, mediastinal node, large tumor)
146
treatment of locally advanced NSCLC
chemo + surgery, chemo + radiation (combination tx)
147
treatment of stage IV NSCLC
considered incurable, palliative care only; chemotx improves survival, sx control, and QoL; standard of care to differentiate subtype
148
specific mutations seen in NSCLC
EGFR, KRAS
149
predominant risk factor for SCLC
smoking
150
median survival for SCLC without tx
2-4 months
151
what are the stages of SCLC?
limited vs extensive
152
limited SCLC
confined to hemithorax
153
extensive SCLC
tumor beyond radiation port, including malignant pleural effusion
154
treatment of limited SCLC
chemo + XRT, prophylactic cranial radiation (1.5 year survival)
155
treatment of extensive SCLC
same as limited, but still only 5-10% survival at 2 years (less than 1 year survival)
156
what happens if you screen 55-79 year old smokers with low dose spiral CT?
decreased mortality, but LOTS OF FALSE POSITIVES
157
smoking cessation decreases pathology of these diseases
COPD, CVD, lung cancer
