Professional Documents
Culture Documents
1
Formulas to Memorize
PAO2 Alveolar O2 tension
𝑷𝒂 𝑪𝑶𝟐
Alveolar gas equation: 𝑷𝑨 𝑶𝟐 = 𝑭𝑰 𝑶𝟐 × 𝑷𝑩 − 𝟒𝟕 − ( 𝟎.𝟖
) FIO2 Fraction inspired O2
PB Barometric pressure
If on room air: simplify it! 𝑷𝑨 𝑶𝟐 = 𝟏𝟓𝟎 − (
𝑷𝒂 𝑪𝑶𝟐
𝟎.𝟖
) (corrected for water
pressure, - 47)
Use to calculate A-a difference (alveolar – arterial) PaCO2 Arterial CO2
Normal values: < 20 on room air (20% O2) < 100 on 100% O2
Increase: suggests venous admixture (poorly oxygenated blood reaching circulation)
2
Lung Mechanics Review
Note: there’s probably way more to review than this lecture covered
Transmural pressure = pressure inside – pressure outside
Transpulmonary pressure (PL)= (alveolar pressure) – (pleural pressure) = elastic recoil pressure
Transdiaphragmatic pressure (Pdi) = abdominal pressure = pleural pressure
PBS = body surface pressure, Ppl = pleural pressure, PCW = chest wall pressure
So the pressure across the respiratory system is PL – PCW = (Palv – Ppl) – (Ppl – Pbs) =
PRS = Palv - Pbs
Lung Compliance
3
Chest Wall Compliance
Note: chest wall; lung compliances are similar over range of breathing pressures
How does this relate to FRC, RV, TLC, and all that stuff?
See graph – just adding the pressures of CW & lung to get RS
FRC: inward recoil of lungs = outward recoil of relaxed thorax
o Graph: chest wall pressure is same distance from zero as lung pressure
RV: all airways are closed
TLC: inward recoil of RS = outward recoil of maximally contracting
inspiratory muscles
Flow patterns
concentric layers of air flow slipping past Resistance:
Laminar each other @ different velocities (faster in independent of gas density
middle)
Resistant: strongly
molecules tumbling around (still with a net
Turbulent vector in flow direction)
dependent on gas density
(↑ with ↑ density)
4
Reading a CXR
Not on the exam; just a few pictures & concepts that seemed helpful
Assessing quality
Not too much lung field above clavicle (bending over?)
Should be able to just make out outlines of vertebrae through mediastinum (exposure good?)
Note: left hemidiaphragm “stops” (mediastinum & abdominal contents are same opacity)
Distribution
Masses (>4cm)?
Upper or lower? Infiltrates? Effusions?
Unilateral or bilateral?
Nodules (<4cm)?
Alveolar? Mixed?
Interstitial?
Water
Reticular (lines)
Blood
Nodular
Cells
Combined
Pus
Honeycomb
Protein
Ground glass
Calcium
5
Pulmonary Function Testing
Spirometry:
Inhale to TLC
Exhale as rapidly/completely as you can
Measure exhaled volume vs. time
Results:
FEV1 : Volume exhaled in 1st second
FVC: total volume exhaled
FEV1/FVC: fraction of total volume exhaled in 1st second (FEV1%)
o Normally ~0.8
Interpretation of Spirometry
Ventilatory defect: FVC FEV1/FVC
Restrictive ↓ Normal or ↑
Obstructive Normal or ↓ ↓
Flow-volume curves
Spirograms show expired volume and time; you can also plot
flow per time. Either way lets you calculate FEV1 and FVC
6
Can also have patient inhale as fast as possible
at end of spirometry to generate flow-volume
loop (see right)
Lesion Affects…
Upper airway inspiratory flow >
obstruction expiratory
expiratory flow >
COPD
inspiratory
Fixed obstructions (e.g.
could affect both
tumor around trachea)
Alveolar gas equation: memorize this: PAO2 = [FIO2 x (PB-47)] – (PaCO2/0.8) PAO2 Alveolar O2 tension
FIO2 Fraction inspired O2
If on room air: simplify it! PAO2 = 150 – PaCO2/0.8
PB Barometric pressure
o Room air close to 20% oxygen (corrected for water
Inspired [O2] changed by water vapor & CO2 pressure, - 47)
PaCO2 Arterial CO2
A-a gradient (i.e. who cares about the alveolar gas equation?)
Calculate PAO2 (alveolar PO2) and compare it to arterial PO2 – are you getting oxygen from alveoli to arteries?
Normal values: < 20 on room air (20% O2) < 100 on 100% O2
Increase: suggests venous admixture (poorly oxygenated blood reaching circulation)
7
Causes of Hypoxemia
Cause Description A-a Response to
O2
Maldistribution of V relative to Q (ventilation / flow)
V/Q Mismatch Some areas are overventilated (↑ PaO2) ↑ Corrects
but doesn’t correct for underventilated areas (↓ PaO2)
Extreme V/Q mismatch
8
Can’t measure DLO2, although we’d like to – backpressure (dissociates from Hb)
Use DLCO instead! Doesn’t have backpressure (binds really tightly to Hb)
o Pt inhales 0.3% CO in 10% He (both diluted equally, He is marker), holds breath 10 seconds
o Exhaled mixed alveolar gas sampled, exhaled [CO], [He}]measured,
o Calculate: how much CO was able to diffuse?
Interpreting DCO
Sensitive, non-specific (something’s wrong, but huge DDx); wide normal range
↓ DCO: ↓ alveolar-capillary SA for gas exchange
↑ DCO: ↑ pulmonary capillary blood volume
Lung Volumes
How to measure residual volume?
The rest we can get from spirometry
Need to use GAS DILUTION (He, nitrogen, Ar, methane)
Gas dilution
Measures only ventilated lung units
Breathe in & out to equilibrate
Calculate measure diluted [He]
Use known initial volumes & initial / final [He] to figure out how
much lung capacity was around (TLC), then calculate RV by TLC-VC
Key Points
Patients can be pathophysiologically categorized with use of:
Spirometry and Flow-volume curves
ABGs
DCO
Lung volumes
9
Interstitial Lung Disease
Pathogenesis of restrictive diseases:
Stiff lungs (don’t expand) ILD: A restrictive disorder
Stiff chest wall (or too small) ↓ TLC (by def’n)
Respiratory muscle weakness (diaphragm rises small lungs) ↓ FVC (almost always)
Normal FEV1/FVC
Interstitial Lung Disease: STIFF LUNGS (no flow restriction)
150+ clinical entities can cause ILD ↓ DLCO (specific to stiff
“Diffuse parenchymal lung diseases” would be better lung restrictive dz)
o Lung is simple
tubes (airways: asthma & COPD are Dz)
blood vessels (pulmonary HTN)
parenchyma (alveoli) – the stuff that isn’t tubes or vasculature
10
CXR:
↑ interstitial markings
Fibrosis
Predominantly LOWER LOBE involvement
& SUBPLEURAL
CT:
Architectural distortion
o (traction bronchiectasis: bronchi
pulled apart by stiffness)
Honeycombing is diagnostic (radiographic hallmark of IPF)
o Don’t need biopsy anymore!
Pneumoconiosis
Accumulation of dust in the lungs; results in tissue reaction
Types of Pneumoconiosis
Reaction can be collagenous or non-collagenous
Excludes dust exposure that results in: Silicosis
o malignancy, asthma, bronchitis, or emphysema Asbestosis
Coal workers’ pneumoconiosis
Talcosis
Silicosis Berylliosis
Most prevalent chronic occupational lung dz in the world! Hard-metal pneumoconiosis
Inhalation of silica, usually in quartz form o Tungsten carbide
Settings (esp. if not using appropriate respiratory protection) o Cobalt dust
o Mining (hard rock/ anthracite coal), foundries, brickyards, glass/ceramic
manufacturing, industrial sandblasting
Clinical Presentation:
Dyspnea & cough (>20yrs low-moderate exposure, 5-10yrs high-level exposure)
CXR:
Small rounded opacities in upper lung zones
Conglomerate (>10mm) opacities
o Called progressive massive fibrosis (PMF)
o Looks like tumor
o Small opacities can progress to PMF
PFTs : identical to IPF
May see airflow obstruction, ↓ FEV1/FVC occasionally
Pathogenesis:
1. Silica particles deposit in alveoli
2. Mϕ gobble them up
3. Mϕ injured / cell death happens
4. Release of intracellular proteolytic enzymes lung injury / fibrosis
5. Silicotic nodules form!
11
Diagnosis of Interstitial Lung Disease
Patient history is crucial!
o Check for exposures, how long have Sx been going on, any Hx autoimmune dz, other sx?
Chest radiograph useful (& chest CT)
Fiberoptic bronchoscopy
o Broncheoalveolar lavage: rule out infection, look for eosinophils
o Transbronchial biopsy: e.g. for sarcoidosis
Thorascopic / open lung biopsy too
12
COPD
COPD: chronic disease characterized by REDUCED EXPIRATORY AIRFLOW Risk factors for COPD
CIGARETTE SMOKING
Diseases included in COPD: both COPD: Clinical Course Older age
caused by cigarette smoking Progressive ↓ in pulmonary function Male gender (?)
Emphysema Punctuated by acute exacerbations Airway hyperreactivity
Chronic bronchitis Low socioeconomic status
Eventually: disability & premature death
Others: asthma / Alpha-1 anti-trypsin deficiency
asthmatic bronchitis / bronchiectasis / CF technically COPD too, but not in
common parlance
If you quit:
Rate of FEV1 drop goes back to normal! Delay onset of Sx
13
Pan-Acinar vs Centrilobular Emphysema
Remember: acini / lobules are the functional unit surrounding one respiratory bronchiole
Alpha-1
Entire Base >
Pan-Acinar antitrypsin
acinus apex
deficiency
Evidence:
Alpha-1 antitrypsin (normally inactivates proteases) deficiency leads to
premature emphysema
Proteases such as elastase causes severe emphysema in lab animals
Cigarette smoking causes inflammatory cells to secrete proteases (which
accumulate in the terminal airspaces)
Cigarette smoke inactivates anti-proteases
Pink Puffers & Blue Bloaters (typical COPD pt has elements of both)
Findings Picture
Bronchitic
High PaCO2
Less hyperinflated
Better O2sat w/ exercise
Blue Bloater Obese physique
Cyanotic
Not dyspneic
14
Pathophysiologic Abnormalities in COPD
Probably good to memorize these lists of 3 things
Major pathophysiologic abnormalities in COPD
Airflow Obstruction: causes 1. Airflow obstruction (Early)
1. ↓ elastic recoil (emphysema) 2. Hypoxemia (Mid-course)
2. ↑ airway resistance (chronic bronchitis) 3. Pulmonary HTN (Late)
3. ↑ airway smooth muscle tone (asthmatic bronchitis)
Hypoxemia: causes
1. V/Q mismatch (because of non-linear Hb dissociation curve)
2. Hypoventilation (late in course; more common in chronic bronchitic)
3. Diffusion impairment (exercise or high altitude;
more common in emphysema)
Flow-volume loop:
More curvilinear (some areas emptying very slowly – bullae, etc)
Smaller in general (less volume)
CT findings with
density masking:
better for Dx
Too much air
(↓ density)
15
Gas exchange: problems progress over course of disease
Treatment of COPD
Chronic oxygen improves survival in pts with hypoxemia
Concentrator or liquid O2
deliver with nasal cannula, Venturi mask (control concentration), or transtracheal catheter (high flow) as needed
Advanced treatment:
Lung transplant
Alpha-1 antitrypsin replacement therapy ($30K/yr, not known if benefit, only for pan-acinar)
Lung volume reduction surgery
Long-term mechanical ventilation
Exacerbations in COPD
Increase in cough, phlegm, dyspnea
Occur on average 2-3/year
50-75% caused by bacterial infection
Treated with antibiotics, steroids Treatment for Acute Respiratory Failure
Impair quality of life Non-invasive positive pressure ventilation
May result in acute respiratory failure Intubation & mechanical vent if needed
Can be prevented by inhaled bronchodilators, inhaled steroids
16
Pathophysiology of Asthma
Definition: Chronic lung disease characterized by Epidemiology
1. Chronic airway inflammation 20M (7%) in US
2. Airway hyperresponsiveness M>F in kids; F>M in adults
Most common childhood chronic dz
3. Variability in outflow obstruction
Prevalence, mortality rate ↑
Cause: UNKNOWN ↑ mortality in AA pts (big gap!)
Genetic factors (clusters in families, ↑ in atopic pts - ↑ ability to generate IgE after allergen exposure)
Environmental exposures (tobacco smoke, occupational agents, air pollutants)
Respiratory infections? Controversial: exacerbates but no good data for causation
Leads to:
Bronchoconstriction
Airway edema
Goblet cell hyperplasia (↑ mucus)
Eventually results in ↑ AIRWAY RESISTANCE and AIRFLOW OBSTRUCTION (wheezing, shortness of breath)
Airway Hyperresponsiveness
Exaggerated bronchoconstriction after environmental exposures or response to stimuli
We all do it; just more in asthma pts
Allergens (e.g. dust mite), irritants (e.g. tobacco smoke), methacholine (diagnostic)
Mechanism unclear: airway inflammation? Abnormal neural control of airways? ↓ ability to relax smooth mm?
17
Variability in Airflow Obstruction
Obstructive ventilatory defect present
LOW FEV1/FVC RATIO (<0.7)
Asthma Exacerbations
Acute ↑ in airway resistance from: bronchoconstriction, airway edema, mucus / cell debris
Usually over days-weeks but can be sudden
60% asthmatics: 1+ severe exacerbation/yr
Triggers
Clinical Presentation
VIRAL INFECTIONS (most common cause; rhinovirus, influenza)
Hx: ↑ SOB, chest tightness, wheezing, cough
Inhaled irritants (cig smoke, ozone, particulates)
PE: tachypnea, wheezing, prolonged expiration
Inhaled allergens (dust mites, animal dander – cats, mice, etc)
Radiology: hyperinflated, flattened diaphragms
Exercise or cold air (irritates!)
Occupational exposures
Physiological alterations in exacerbation:
Hyperinflation: Specific to patient (need to test & find out).
Can have delayed symptoms too – exposure, get Sx hours later!
Diaphragms flattened mechanical
disadvantage (hard to flatten more to breathe!)
↑ work breathing
o abdominal paradox: use less efficient intercostals to breathe, so abdomen goes in instead of out on inspiration
o Can lead to respiratory failure
Pulmonary HTN: alveolar capillaries compressed (↑ alveolar pressure)
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3. Hypercarbic Respiratory Failure (↑ PaCO2)
o Diaphragm fatigued (↑ work of breathing) alveolar hypoventilation with ↑ PaCO2
o Normally asthma pts hyperventilate during exacerbation so PaCO2 should be low (<40mmHg)
o A NORMAL PaCO2 is therefore a bad sign in asthma exacerbation
Treatment of Asthma
Patient education: what it is, how to manage, what drugs are, etc.
Avoid triggers: needs to be comprehensive Goals of treatment
Smoke avoidance, Pet avoidance, Roach control, Mouse control
Dust mite modifications: 1. Control symptoms
o Allergy proof covers, Wash linens in hot water weekly 2. Prevent exacerbation
o Vacuum/sweep weekly, Carpet/curtain removal, Humidity control 3. Maintain lung function as close to
Asthma management plan normal as possible
Can have pt measure peak expiratory flow rates at home • Use objective measures: pt
may not realize how bad it is!
Plan with pt: what actions to take based on peak flow
Helps with appropriate medication use (not overuse), can have pt call &
4. Avoid adverse effects from
schedule appt or prescription refill if drastic decline, etc. medications
5. Prevent irreversible airway
Pharmacologic treatment: obstruction
• Is asthma predisposition for
1. Bronchodilators: relax bronchial smooth muscle COPD?
a. Short & long-acting β2 agonists, anticholinergic meds 6. Prevent asthma mortality
b. Inhaled
c. Short-acting meds for RESCUE ONLY
2. Inhaled corticosteroids: anti-inflammatory 3. Oral corticosteroids
a. Cornerstone of daily control therapy a. If can’t control with inhaled meds
b. Add if pt needs their bronchodilator b. ↑ side effects
>2x/wk, for instance
4. Others: if refractory to tx
Drug Description
Cromolyn sodium / nedocromil Anti-inflammatory, Mast cell stabilizer (↓ degranulation)
Anti-inflammatory, less effective than corticosteroids
Leukotrine modifiers
(can use if mild asthma or corticosteroids contraindicated)
Theophylline Methylxanthine bronchodilator
Anti-IgE Really expensive; IV; only in very severe cases
Acute Exacerbations:
↑ frequency of short-acting bronchodilators with ↑ Sx
Often needs oral corticosteroids, may need subQ or parenteral β-agonists if severe
Monitor for respiratory failure
19
How to use MDIs & Spacers
Step Care
Sx, severity depend on:
frequency of exacerbations
frequency of nocturnal symptoms
variability in lung function (FEV1 / PEFR)
20
Expiratory Flow Limitation
Expiratory Flow Limitation (defn): PATHOPHYSIOLOGY OF RESPIRATORY FUNCTION: BASIC CATEGORIES
Restrictive ventilatory defects Difficulty getting air into the lungs
↑effort to exhale does NOT cause Obstructive ventilatory defects Difficulty getting air out of the lungs
↑ expiratory flow rate Difficulty with efficient movement of
Gas exchange defects
gases between alveoli & blood
21
Expiratory muscles contract
Isovolumic so Pel has to be the same (8)
↑Ppl (contraction of expiratory muscles), so ↑Palv too (keep Pel same)
↑Palv now creates a gradient, and ↑airflow out
Flow limitation:
airflow stops increasing at some point (60% max effort in normal)
resistance from ↑ Ppl cancels out ↑ airflow from ↑ Palv
Airway opens up again now the pressure is greater outside (forces closed)
Resolved by either:
Bronchus fluttering open & closed
Bronchus doesn’t really close; self-adjusts diameter to maintain it just
open enough to balance opening & closing
22
Why is this a problem in chronic obstructive lung disease?
23
Pulmonary Vascular Disease
Review from last year: pulmonary circulation
Key points about the pulmonary circulation:
Entire cardiac output goes through it
Low pressure drop (25-5)
Low resistance (low pressure drop!)
P −P
PVR = PA𝑸 LA
𝒕
o Resistance = pressure gradient over flow.
o Pressure gradient (pulmonary artery to LA) divided by the flow
(cardiac output = venous return)
So ↑ PPA with
1. ↑ left atrial pressure
2. ↑ downstream resistance
3. ↑ cardiac output
24
1. ↑ LA pressure
LV or mitral valve disease (cardiomyopathy, mitral regurgitation or stenosis, etc)
↑ LA pressure ↑ backpressure so ↑ pulmonary arterial pressure
Most common cause of pulmonary hypertension (because LH dz very common)
Treatment: unload left heart (treat underlying condition to ↓ LA pressure)
2. ↑ downstream resistance
Very uncommon to raise resistance in veins – usually arteriolar or arterial
3. ↑ cardiac output
↑ pulmonary blood flow ↑ PPA
o Anemia, hyperthyroidism (↑ CO); generally not severe; reversible
Chronic ↑ flow vascular remodeling ↑ resistance (more fixed form)
o L-to-R shunt (ASD/VSD), Sickle cell anemia too
25
Consequences of pulmonary HTN
ACUTE (e.g. Acute PE) CHRONIC (e.g. chronic lung dz, PAH)
RV not hypertrophied; fails quickly RH has time to hypertrophy
(pushing against ↑ resistance) o See BIG RV and RA
RA pressure ↑ as RV fails RA pressure ↑ over time (can’t eject everything)
↑ catecholamines to ↑ mean systemic pressure, ↑ HR pulmonary hypertension
↓ Venous return / CO shock, sudden death Venous return, CO maintained (↑ sympathetics, ↑
Acute PEs have 10-15% mortality mean systemic pressure to augment return)
Prognosis:
Correlates with mean pulmonary arterial pressure!
o See graph: ↑ PPA = ↑ mortality
Similarly:
o ↑ RA pressure (RH failing!) = 3 mo median survival
o ↓ CO (failing) = bad prognosis
o Hyponatremia (compensatory mechanisms failing) = bad prognosis
VASCULAR MODIFIERS
Prostaglandin I2 (prostacyclin) Endothelin receptor antagonists PDE5 inhibition
(e.g. Bosentan) (e.g. sildenafil / Viagra®)
Endothelin usually leads to
↑ cGMP vasodilation, inhibition of
Vasodilation & inhibition of remodeling vasoconstriction, proliferation,
remodeling
migration of smooth mm (reverse!)
Requires chronic perfusion but
Can give orally, again good functional
improvement in survival (50% at 5yrs Can give orally; good functional data
data but not good data for survival
vs 20% w/o Tx) (six minute walk) but not good data
for survival (should correlate?)
Lung transplant: cures PAH but has significant problems in its own right
5yr survival is ~50% (worst of transplants)
Summary:
Pulmonary hypertension can arise via various physiologic or pathophysiologic mechanisms (most still unclear)
Harder to diagnose than systemic HTN / often presents late
Major clinical importance of pulmonary HTN: effect on the RV (acute vs. chronic)
Strategies to decrease PVR and unload the RV have beneficial effects on patients with pulmonary hypertension
26
Obesity & Breathing Disorders
Obesity: BMI kg/m2; >25 overwt, >30 obese. Growing problem (ha!).
More obesity in Missisippi. Obstructive
(mentally recreate obesity epidemic maps) ↓ Sleep Apnea↓
Sleep apnea
Clinical Features
Upper Airway Obstruction Alterations during sleep
Snoring Excessive movements
Choking, gasping Insomnia
Cardiopulmonary dysfunction
Alterations in daytime function
Hypertension
Excessive hypersomnolence
Glucose Intolerance
Intellectual deterioration
MI / Heart Failure / Arrhythmias
Fatigue
Cor Pulmonale
27
Neuromuscular activity:
Normally, when upright, genioglossus contracts
(to pull tongue forward on inspiration to open airway)
Normally, when supine, have more genioglossus activity
(tonic activity too to keep tongue out of the way)
Apneic patients have ↓ / absent genioglossal nerve firing
In adults: combination of
structural problems and
this neuromuscular dysfunction
Obesity:
More common in upper body obesity (“apples” – mostly males)
o Fat encroaching on neck / pharyngeal tissues ↑ collapsibility
o Fat ↑ load on resp system / impedes gas exchange
o Fat cytokines / humoral factors that ↓ CNS reflexes to keep things open
28
Daytime Respiratory Complications of Severe Obesity
Hypoxemia & hypercapnia cor pulmonale
Hypercapnia:
↑ metabolic demand (more CO2 produced) if obese
Should ↑ alveolar ventilation (VA) to compensate
o Blow off extra CO2
If ventilatory drive depressed, ↑↑PaCO2
(not getting rid of the extra you’re producing)
↓ PaCO2
o ↑ VA (alveolar ventilation): blow off more CO2
Stimulant (progesterone) ↑ VA (the same way ↑ metabolic demands dealt with in pregnancy)
Mechanical ventilation / CPAP to ↑ VA if needed
o ↓ VCO2 (produce less carbon dioxide!)
WEIGHT LOSS (even moderate loss ~ 5% helps!)
Maintain oxygenation
o ↑ PIO2 (supplemental oxygen)
o Treat sleep apnea (repeated ↓ in oxygen sat)
29
Ventilatory Failure
Lung failure Pump failure
Primary feature Hypoxia Hypercarbia
Pneumonia, Asthma, COPD,
Clinical syndromes interstitial lung disease, myopathies, neuropathies,
PE, ARDS spinal cord lesions
Note: hypercarbia is LATE in pump failure: don’t miss stuff that comes before!
Accessory muscles:
Inactive in relaxed breathing; active during exercise or disease
Sternocleidomastoid is big one
Pectoralis major / minor, trapezius, serratus anterior too
Expiratory muscles
Remember that TIDAL EXPIRATION is PASSIVE
o Use active expiration during exercise, obstruction, dyspnea, pulmonary function testing
Abdominal muscles & internal intercostals
Essential for STRONG COUGH (clear mucus!)
30
Mechanisms of Respiratory Muscle Failure
IMBALANCE between DEMAND and CAPACITY
↑ demand, ↓ capacity, or both
RM demand: how much are you breathing and how hard is it to breathe?
↑ demand with … Because…
↑ CO2 production Need to get rid of CO2 (e.g. obesity)
Alveolar ventilation is needed to get rid of CO2:
Minute ventilation ↑ Dead space
↑ total ventilation if ↑ dead space to preserve VA
↑ respiratory drive By definition
↓ lung compliance
Harder to breathe if stiffer
Respiratory system mechanics ↓ chest wall compliance
↑ airway resistance Pushing against more
RM capacity ↓↓
Hyperinflation (worse with exercise)
Malnutrition (esp. protein) ↓ diaphragm mass END RESULT IN COPD
Steroids (COPD Tx) chronic myopathy ↑ demand + ↓ capacity = failure
Myopathy ↓ oxidative capacity
31
When things go wrong: Critical Illness
Many conditions cause ↓ capacity (stroke, spinal cord injury, ALS, phrenic nerve injury, Guillan-barre, myasthenia gravis, MD)
Diaphragmatic Paralysis
Causes Features
Cardiac surgery, trauma, Relatively Asx (DOE)
Unilateral
tumor, stroke, herpes zoster ↓ max voluntary vent (25%)
Severe dyspnea & ORTHOPNEA (really bad)
Neuropathies, herpes zoster,
Bilateral ↓ VC and max voluntary vent (50%)
vasculitis, Lyme dz
Diagnose by Pdi (transdiaphragmatic pressure – balloon in esophagus vs stomach)
32
Assessment of RM Function
Simple clinical observation: “TAP”
Tachypnea
Accessory muscle use
Paradoxical breathing
o Put one hand on abdomen, other on chest: should rise & fall together. If diaphragm can’t contract, not raising
abdominal pressure. Accessory muscles contract, suck abdominal contents in abdomen falls
o Respiratory alternans: periods of alternating paradoxical & normal breathing
Diaphragm works for a while, takes a break & lets accessory muscles work, etc.
Blood gases: hypercarbia (but a LATE sign! Very ominous if rising – some pts can have chronic hypercarbia)
Muscle training (exercise) – works for skeletal muscle but NOT for resp muscle Treatment strategies
Works in normals (↑ strength / endurance) but not patients Exercise
Problem: already exercising resp MM all the time (e.g. COPD) Rest
Medications
Rest (mechanical ventilation) Pacing
Allow respiratory muscles to recover from constant load Surgery
Mechanical ventilation
o Non-invasive (nasal / facial mask with positive pressure), invasive (if intubated)
o Temporary (endotracheal tube) or permanent (tracheostomy)
Medications: don’t really have any good ones! Respiratory muscle surgery : Lung volume reduction
Bronchodilators (dilate airways, ↓ Used infrequently these days
hyperinflation) Severe emphysema:
Theophylline (↑ strength, ↑ resistance to ↑RM strength by ↓ hyperinflation
fatigue) ↑ lung fxn, sx, survival in some pts, but
Androgenic steroids (mixed results) unpredictable outcomes
Routes of Injury
Inhalation Blood-borne Direct injury to lung
Sepsis
Aspiration of gastric contents Trauma
Pneumonia (diffuse bilateral) Drug overdose (narcotics, ASA) Lung contusion
Smoke inhalation Multiple transfusions Radiation
Near-drowning Pancreatitis
Venous air embolism
Predisposing factors
Other risk factors for ARDS
SEPSIS IS #1
SEPSIS / trauma /gastric acid
40% pts with sepsis develop ARDS; 40% ARDS pts had sepsis as factor aspiration
Sepsis / trauma / gastric acid aspiration = 75% of cases Older age
Severity of associated illness
Multiple factors multiply risk of ARDS development Cig smoking / chronic lung dz
Chronic alcohol abuse
34
Pathphysiology of ARDS
1. Injury to capillary endothelium (activated PMNs, Mϕ) cytokines, oxygen radicals, etc.)
2. Pulmonary Edema
o Protein rich (both water & protein leaking)
o Sensitive to small increases in capillary pressure
o Insensitive to changes in blood oncotic pressure
Normally:
fluid drains out (depends on Kf = conductance constant & driving pressure,
combination of hydrostatic pushing out minus oncotic sucking into capillary)
Lymph channels drain lung, keep the alveolus dry
35
Management of ARDS: Oxygen Therapy
Oxygen Therapy: main strategy for ARDS treatment
GOALS OF O2 THERAPY FOR ARDS
PEEP: essentially raising FRC Arterial O2sat = 90%
Keep positive airway pressure at end expiration o (too high is toxic!)
Recruits more lung: stents open compressed airways & alveoli w/ +pressure Keep FIO2 ≤ 0.60
o ↑ FRC, ↓ shunt fraction, ↑ compliance Maintain cardiac output
Mechanical ventilation
Study: reducing tidal volume in mechanical vent lead to ↓ mortality, ↑ vent-free days, ↓ organ failure
Can cause more damage with overstretching!
Avoid complications:
o Oxygen toxicity (keep FIO2 ≤ 0.60)
o Ventilator-induced injury (keep tidal vol ≤ 6 ml/kg)
o Nosocomial infections pneumonia, catheters
36
Pneumonia
Significance
th
7 leading cause of death in US Generally, patients with pneumonia do well
Leading cause among nosocomial infections (only 25% CAP hospitalized; 12% of those die)
M. tb is most deadly bacteria in world Need to recognize, assess risk, Dx, Tx
Pandemic influenza major geopolitical death
Pathogenesis
Pneumonia: infection of the lung parenchyma
Not one disease, but many common ones that share a common anatomic location
Some non-infectious processes also are called “pneumonia” – e.g. eosinophilic pneumonia
Route of Infection
Many people think it’s inhaled respiratory transmission – but not most common way!
Most: colonization (oropharynx / endotrach tube) establish there aspirated into lung!
Clinical Presentation
Symptoms: Signs:
Fevers, chills, rigors (shaking) – cytokines, etc. Infiltration crackles
Cough, sputum production, dyspnea, pleuritic Consolidation dull to percussion,
pain (sharp with inspiration / coughing) – when tubular breath sounds
more established Pleurisy (friction rub – scratchy sound on insp.)
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Pneumonia vs Acute bronchitis: important for treatment
Pneumonia Bronchitis
Sx Cough & Fever Cough + Fever
Normal VS
VS: P >100,RR >24, BP
PE Rhonchi, wheezes
Crackles, consolidation
(except flu)
X-ray Infiltrate Negative
Etiology BACTERIAL VIRAL
Antibiotics? YES NO Infiltrate: hallmark of pneumonia
Inflammation in interstitium
Interstitial
(fluid, not pus)
pneumonia
No air bronchograms
limited Ddx:
GNR, S. aureus, M. TB, fungi
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Diagnosis: Sputum & Culture
Quality of Squamous
Polys Grossly From
sputum: epithelial cells
Good: Lots Few Thick mucus Lower resp tract
Bad: Few Lots Saliva Upper resp tract
Can be mixed too: believe type III, consider type II, throw out type I
Graded by lab
Picture: left is good (thick sputum with polys); right is bad (saliva with epithelial)
Clasisfying Pneumonia
Acute vs Chronic
Acute evolves over hours / days (S. pneumo, H. flu, Legionella – fast growers)
Sub-acute / Chronic evolves over weeks-months (M. TB, fungi, anaerobic abscesses, PCP – slow growers)
Community-acquired pneumonia
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Classification of CAP: typical vs. atypical
“Typical” pneumonia “Atypical” pneumonia
Onset Acute Subacute
Nonspecific, systemic, more viral:
Symptoms Fever / chills / Rigors
Headache, pharyngitis, myalgias
Cough Productive of purulent sputum Non-productive cough
Lung exam findings Consolidation Few findings
CXR Dense infiltrate Patchy / interstitial infiltrate
Leukocytosis YES (WBC > 15k) Modest (WBC < 15k)
Etiology Strep pneumoniae, H. flu M. pneumoniae, Legionella sp., Chlamydia sp, viruses
Legionellosis
Epidemiology: 2-5% of CAP, sporadic in general pop, epidemics (hotels, hospitals)
At-risk: age > 40, COPD, immunosuppressed (old, smoking / drinking too much – like a Legionnaire)
Dx: urinary antigen detects 80% (doesn’t grow well)
Treatment: macrolide or fluoroquinolone
MORTALITY: 10-20% !
Influenza
Epidemiology: common (seasonal), also pandemic (drift/shift)
Mortality: 36k/yr, esp. elderly elderly
Sx: high fever, myalgia, headache, cough
Dx: clinical Dx > Ag test > culture
Rx: amantadine / neuraminidase inhibitors (give w/in 36hrs)
Prevention: vaccine (70-90% efficacy, ↓ severity), antiviral px, respiratory isolation, good resp precautions
Aspiration Pneumonia
Frequency: ≈ 10% CAP, common cause of HAP
At-risk: Macro-aspiration (alcoholism, drug abuse, seizure disorder, neuromuscular disorder)
Sx / signs: Cough, fever, infiltrate in dependent segment (GRAVITY)
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Where’s it coming from? Gingival crevice!
Anaerobes, etc.
See polymicrobial flora on gram stain but nothing grows on Cx (anaerobes!)
Aspiration pneumonia:
if bacterial agents get involved, ends up in dependent locations (GRAVITY)
Lower lobe if standing up
Right middle lobe if laying down / on back
Often lead to abscesses!
Abscess vs Cavity
Nosocomial Pneumonia
Hospital pathogens: Gram (-) bacilli, S. aureus
Treatment
Empiric: BROADER spectrum than CAP (more possible causative agents)
Pathogen directed when you figure out what it is
Prevention
Proper infection control (↓ transmission) Avoid unnecessary antacid therapy
Identify aspiration-prone patients, ↑ HOB (↑ bacterial contents in stomach ↑ infection if aspirate)
Limit ventilator time
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Immunocompromised pts
Type of defects depend on what kind of immune compromise you have (what usually clears the infection?)
Table for reference; probably wouldn’t memorize
Asplenia ↑ susceptibility to encapsulated organisms
HIV: CD4 count what kind of infection you’re susceptible to (table for future reference too)
CD4+ Pathogens
>500 S. pneumoniae, H. flu
200-500 S. pneumoniae, H. flu, M. TB
S. pneumoniae, Pneumocystis jiroveci, H. flu , M. TB,
50-200 Histoplasma, Cryptococcus
S. pneumoniae, P. jiroveci, M. TB, Other mycobacteria,
<50 H. capsulatum, C. neoformans, Aspergillus sp.,
Nocardia sp., Rhodococcus equi, CMV, Kaposi’s sarcoma
CXR in Immunocompromised Patients (another one not to memorize but future reference)
CXR Finding Associated pathogens
Diffuse interstitial infiltrate Pneumocystis, CMV, Kaposi sarcoma, respiratory viruses
Diffuse nodular infiltrate (miliary) Mycobacteria, Histoplasmosis, other fungi, Pneumocystis
Localized infiltrate Typical bacteria, Nocardia, Fungi, Mycobacteria
Large nodular/cavitary infiltrate Staph aureus, Mycobacteria, Nocardia, GNR, Aspergillus, other fungi, anaerobes (aspiration)
Hilar adenopathy Mycobacteria, fungi, Kaposi sarcoma, lymphoma
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Etiology by Age
Newborn Children Young adults Middle age** Elderly**
(0-6 wks) (6 wk–18 yrs) (18-40 yrs) (40-65 yrs) (> 65 yrs)
Group B strep H. flu Mycoplasma S. pneumoniae S. pneumoniae
GNR Mycoplasma C. pneumoniae Anaerobes Anaerobes
Chlamydia trachomatis Viral*** S. pneumoniae H. influenzae H. influenzae
(4-6 wks) Pneumocystis GNR
carinii (AIDS) Viral***
Agents are listed in rank order
** Major causes of nosocomial pneumonia: *** Major viral pathogens
GNR (Klebsiella sp., P. aerug, Enterobacter sp., and E. coli), RSV, parainfluenza, and adenovirus
S. aureus, anaerobes middle-aged adults: only common viral cause is influenza.
Treatment of Pneumonia
Who should I be worried about? (Predictors of BAD OUTCOME)
Older age Marked derangements in vital signs
Co-morbidities (malignancy, cardiopulmonary dz) Multiple lobe involvement
Alterations in host defense (don’t use bacteriostatic abx!) Bacteremia
Treatment
Often empiric, usually successful
Narrow spectrum when pathogen-directed
↑ need to identify specific pathogens if:
o Severe disease
o Host immunosuppressed
o Unusual features (e.g. cavity)
o Failure to improve
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The Pleural Space
Anatomy Review
Lined by parietal & visceral pleural (merge @ hilum)
Pleural cavities separated by mediastinum
2000 cm2 of surface area, 10-20 μ in diameter (thin)
Normally: slightly negative pleural pressure (lungscollapse, chest expand) – suck fluid in
Starling Equation: what determines movement of liquid into pleural space from capillaries??
Pleural effusions
Causes of Pleural Effusions
Increased Production Decreased Clearance
↑ intravascular pressure / interstitial fluid
lymphatic obstruction is #1
o LV / RV failure, PE, pneumonia, SVC syndrome, pericardial effusions
o 28 fold capacity for drainage vs normal
↓ pleural pressure
production
o Atelectasis, ↑ elastic recoil pres.
↑ systemic vascular pressures
↑ pleural fluid protein
o SVC syndrome, RV failure
↑ permeability
? disruption of aquaporins
o pleural inflammation, VGEF
o 4 types found in the lung;
↑ peritoneal fluid
o AQP1 important in peritoneal fluid transport
Disruption of thoracic duct / intrathoracic vessels
Iatrogenic
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Epidemiology:
CHF (500k), Parapneumonic (300k), Malignant (200k), PE (150k), Viral (100k) are big ones
o Parapneumonic = related to pneumonia!
Also: Cirrhosis/ascites, post-CABG, GI dz, TB, mesothelioma, asbestos exposure
Thoracentesis
(taking fluid out of pleural effusion)
Indications Contraindications
Unless you know why it’s there, take it out! Absolute & relative
Especially if: Bleeding, infection
Unilateral effusions, particularly L-sided* pneumothorax (1.3-20%, 2% need chest tube placed)
Bilateral effusions of unequal size* Also:
Normal cardiac silhouette on CXR* o vasovagal episodes / arrhythmia,
Febrile, evidence of pleurisy o tumor seeding of needle tract,
(* = indicates that effusion’s less likely to be transudate) o puncture of other organs,
o re-expansion pulmonary edema
For relief of dyspnea too
o death (rare)
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Is it an exudate? Get both peritoneal fluid & serum LDH + protein compare.
Need one of the following (looking for ↑ leakage into pleural fluid)
o Fluid : Serum protein >0.5
o Fluid : Serum LDH >0.6
o Fluid LDH >200 IU/L or > 0.45 upper limit nl for lab
If no serum labs: can use pleural fluid protein / LDH levels alone (not as good)
o (protein > 2.9, LDH > 60% upper limit nl, chol > 45 mg/dL)
Don’t wait to treat a pleural effusion! These can move quickly (e.g. PPE air pockets rupture)
Hours later: air pockets develop with After days / weeks: can rupture,
PPE, can treat by draining
gas production, would need surgery requiring thoracotomy (major surgery)
Malignant Effusions
#2 cause of exudative effusions (200k/yr in US), #1 for exudative effusions that need thoracentesis
Lung cancer, breast cancer, lymphoma responsible for 75% 1° tumor Survival
1° tumor not identified in 6% GI CA 2.3 mo
BAD SIGN: Die in average of 4 months – PALLIATE by treating effusion Lung CA 3 mo
o Primary tumor is most important predictor; performance status too Breast CA / unknown 5 mo
Mesothelioma 6 mo
Pathogenesis:
tumor emboli visceral pleura, 2° seeding of pleural space / parietal pleura (or via diaphragm)
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Work-up of malignant effusions: Paramalignant Effusions
Thoracentesis (cytology beter than closed pleural Bx) (not all effusions in cancer are malignant effusions)
Thorascopy (95% sensitivity)
NOT Bronchoscopy (little value!) related to primary tumor but
not direct neoplastic involvement of pleura
Treatment: for palliation
Don’t use much sedation – no nerves in visceral pleura Etiologies: Post-obstructive pneumonia PPE, obstruction of thoracic duct
Go in and remove malignant nodules chylothorax, PE, SVC syndrome, post-obstructive atelectasis ↓ PPL,
Talc blown in (acts as glue to hold lung to chest wall; low Ponc from cachexia, pneumonitis/trapped lung, cancer Rx, more
also prevents re-accumulation of fluid)
Pneumothorax
Moving from fluid (effusion) to air (pneumothorax) in pleural space
Tension pneumothorax:
Tachycardic: shock death
Need needle decompression HR returns to normal immediately!
Relatively new disease (1967), pulmonary disease after resp therapy of IRDS (↑ O2 conc, mechanical vent)
airway inflammation, fibrosis, smooth muscle hypertrophy
high mortality rate Common features of BPD
Abnormal CXR
Premature births in US Respiratory symptoms
11% all US births < 37 wks (premature) Hx of supplemental O2 and/or
308k with low BW (<2500g), 58k with very low BW (<1500g) mech vent in neonatal period
In US, bronchopulmonary dysplasia is the leading cause of chronic lung disease in infants
BPD can also occur in up to 20% of mechanically ventilated FULL TERM infants
Pathological Findings
ATELECTASIS, OVER-INFLATION, CYSTIC CHANGES
Hyperinflation, interstitial changes, “Mosaic changes” on CT: Histology: areas of atelectasis, other areas
cystic development dense areas, hyperinflation with alveolar enlargement; fibrosis rxn
Treatment
Has really ↑ survival for very early gestation infants
Use of bovine surfactant
Tertiary care centers take care of infants
Better ventilator techniques (less damage)
Prudent supplemental O2 use
o (high concentrations free radicalsimpairs alveolar growth)
o Remember lung keeps growing & developing (until 2 yo)
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Surfactant review
Surfactant:
Surface tension: directly proportional to pressure needed to open & keep open alveoli
2×tension
LaPlace’s Law: Pressure =
radius
If ↑ surface tension, need ↑ pressure to keep open (newborn with RDS: collapse!)
2. Infection
a. Immune systems not developed
b. pre/post-natal infections
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Diagnostic Criteria (severity of BPD)
If born ≤32 wks gestation & got >21% O2 for 28+ days: assess at 36 wks PMA or at time of discharge
Mild BPD Breathing room air
Moderate BPD Need < 30% O2
Severe BPD Need ≥ 30% O2 and/or positive pressure (nasal CPAP / PPV)
↑ need for pulm meds, hospitalization in follow up studies if more severe BPD
Medical treatment
Meds
consider if need supplemental O2
Diuretics
Preterm > 3wks – acute / chronic distal diuretics may improve pulmonary mechanics
Inhaled steroids BPD infants have ↑ airway resistance & inflammation
β-adrenergic bronchodilators use PRN (can develop tolerance)
Anticholinergics can help in respiratory aspiration
(nebulized ipratroprium, etc) IRDS – kind of like COPD lungs in a mechanistic sense
Supplemental Oxygen
Hypoxia in BPD infants (formerly thought was ↑ SIDS)
o ↑ number of central apneas
o ↑ central apneas, hypoxia bradycardias, severe hypoxemia, inability to auto-resuscitate (death)
Maintain O2 sat: better growth / development, prevent central apneas / oxygen
o ↓ risk of sudden death from acute hypoxia
Want O2Sat 92% or greater during sleep, with feeds, during activities
Nutrition: need 120-150 kcal/day for adequate growth, supplemental tube feeding if needed
Recurrent insults to lungs can worsen underlying BPD & prevent compensatory lung growth
Growth continues through 2 years – window for catching up!
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Pulmonary Outcomes in BPD infants
Respiratory Sx in 25% young adults / adolescents who had BPD:
Wheezing (↓ small airway flows) Radiographic abnormalities
Recurrent pneumonia ↑ risk of airway obstruction & reactivity
Chronic need for resp meds (↓ FEV1)
↓ exercise tolerance
Course:
Hospitalizations for resp problems ↓ by 4-5 yo
↑ frequency of chronic resp problems (esp. obstruction)
Wheezing ↑ smaller kids (in BW < 1500g
↓ resp reserve, ↑ O2 desaturation with exercise
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Cystic Fibrosis
Genetics of CFTR
1:2750 Caucasians, carrier rate 1:25
↓ in African Americans (1:17k), ↓↓ in Asians (1:70k)
CFTR : Single gene mutation
o (chromosome 7, most common mutation is ΔF508)
o cAMP-regulated chloride channel
o Autosomal recessive
Manifestations of CFTR
Systemic!
Lungs Chronic obstructive pulmonary disease Diagnosis by CLINICAL TRIAD:
Pancreas Pancreatic exocrine insufficiency (↓ enzymes to digest fat) ↑ SWEAT CHLORIDE
GI CFTR channel helps in stool transit
PANCREATIC INSUFFICIENCY
Repro glands Can’t develop (e.g. vas deferens)
Skin Sweat electrolytes ↑ (sweat test, messed up salt balance) CHRONIC PULMONARY DISEASE
Respiratory Manifestations
Chronic cough and bronchitis at first Chronic sinusitis, nasal polyps
Bronchiectasis (no matter how well you treat) Hemoptysis, pneumothorax (↑ pressure cysts can pop!)
Recurrent pneumonia (staph aureus, Chronic airways obstruction, irreversible
pseudomonas aeruginosa)
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Complications of CF lung disease
Pneumothorax: dilated peripheral airways + mucus plugging / air trapping, rupture of pleura
Chest tubes, pleural sclerosis involved too
50% recurrence rate
See these more frequently in adults now: about 1/3 CF pts are adults these days
Nasal polyposis (shouldn’t see nasal polyps in child) Pan-sinusitis: maldevelopment opacifiction, erosion
Nasal polyps + asthma in child: 99% of time it’s CF! All CF pts; Tx with abx, surgery
3% CF pts; tx with topical steroids, surgical excision
CF GI disease
GI Sx start early: pancreas blocked, no good in utero production of fat metabolizing enzymes
Meconium ileus (newborn) ≈ Distal intestinal obstruction syndrome (postnatal) – GI blockage
o Can lead to intussusception (telescoping of bowel into itself, can cause death)
Meconium peritonitis too
Pancreatic insufficiency is lifelong (malabsorption)
Hepatic cirrhosis, portal HTN, neonatal direct hyperbilirubenemia, gall bladder obstruction
Nutritional aspects (edema, hypoalbuminemia, hypovitaminosis A/K/E) – more rare these days
Pancreatic Disease
↓ volumes & bicarb content of pancreatic fluid
15% have milder mutation pancreatic sufficiency (longer life span but ↑ risk pancreatitis!)
Can lead to CF-related diabetes (3% kids, 14% adults)
o Block islets ↓ insulin and ↓ glucagon (so ketoacidosis rare)
o Stresses can trigger hyperglycemia: pregnancy, corticosteroids, pulmonary exacerbation
Hepatobiliary Dz
Eosinophilic concretions in bile ducts ↑ gall bladder dz, gall stones, microgallbladder
Cirrhosis in 3%: portal HTN, splenomegaly, esophageal varices
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Diagnosis of CF
Sweat Test
Classic test; still used as primary test
When should I get a sweat test? (these things mostly make sense)
Meconium ileus, meconium peritonitis Panopacification of sinuses/pansinusitis in CBAVD/Azoospermia at any age (but
Jaundice in infancy childhood becomes more obvious in adults)
Hypochloremic alkalosis in infancy Pancreatitis in late childhood/early Recurrent or persistent pneumonia any age
Heat prostration Infancy/adulthood (males) adulthood Staphylococcal pneumonia at any age
Failure to thrive Infancy/childhood Unexplained cirrhosis in (especially infants)
Rectal prolapse in childhood childhood/adolescence Mucoid Pseudomonas in lung at any age
Nasal polyposis in Childhood/adulthood Gallstones in late childhood/early adulthood Bronchiectasis at anyage
Family history (sibling, first cousin)
Immunoreactive Trypsin
Most CF patients develop ↑ immunoreactive trypsin, but 80% false positive rate
Dx by Genotyping
1000x mutations in CFTR; internet databases; don’t know significance of all
Commercial genotyping available
IV-V: milder mutations (If one serious, one milder: mild dominantes - milder phenotype)
IV. Altered conductance
V. Reduced synthesis
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Organ-Specific Vulnerabilities
Organs that make lots of protein & secrete slowly through long tortuous passages
Genotype: predictive of pancreatic sufficiency but not other diseases (meconium ileus, liver dz, diabetes)
Pulmonary Status
Variable rate of decline (even with identical genotype)
Complex structure / function, main cause of morbidity / mortality
CF Treatments
Aspect of CF Treatment
High Sweat Chloride Dietary Salt (a disease you ↑ salt for!)
Chest Physiotherapy/DNase
Thick Airway Mucus
Hypertonic Saline
Chronic Lung Infections Antibiotics
Inflammation Anti-Inflammatories
BiPAP
Respiratory Failure
Lung Transplant
Pancreatic Insufficiency Pancreatic Enzymes
Meconium Ileus PEG, stool softeners
Islet Cell Loss Insulin, Pancreatic Transplants
Male Infertility, CBAVD In Vitro Fertilization
Biliary tract insufficiency Bile acid salts
Lung transplant: not a good solution; limited organs available, tons of side effects, risk of death
Trading one disease for another
Median predicted survival now 38 years (↑ but still – only 50% live to be 38!)
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Disorders of the Lower Airways
“When noisy breathing is not asthma”
Clinical Approach
History
Physical Exam of the Chest Onset
Inspection: Vital signs (resp rate, SaO2), retractions, contour Alleviating / exacerbating factors
Percussion (dullness vs hyperinflation) o Position
o Diaphragmatic domes normally w/in 1-2 finger breadths of scapular tips
o Occurrence: sleep or activity
Palpation o Response to therapy
Auscultation is the big one Other associated symptoms:
o Inspiratory or Expiratory COUGH (never normal in babies!)
o Airway disease = narrowing
(laminar vs turbulent air flow depending on radius)
Something pushing in from outside!
Position Sound Ins/Exp More detail
Above thoracic output Stridor Inspiratory
High pitched Peripheral (e.g. asthma)
Below thoracic output Wheezing Expiratory
Coarse sound Central (larger airways)
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o Small incidence of malignancies
Tx: often lobectomy (no recurrence with complete excision)
Incidence: 1:20k-30k
Etiology:
Mechanical obstruction in utero(25%) – mucosal flap, lobar twisting on pedicle
Airway collapse (25%) – bronchial atresia, deficient bronchial cartilage
No clear etiology (50%)
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Tracheoesophageal Fistula
Incomplete mesodermal separation of primitive foregut
Esophagus connected to trachea
o See barium swallow to right
More common: 1st and twin pregnancies; ↑ with ↑ maternal age
2/3 have other associated abnormalities
Presents around birth (feeding / breathing abnormalities)
Vascular Rings
Can show up early but often later in life
Extrinsic obstruction of trachea & esophagus
Most common: double aortic arch; can see others too
o Wraps around trachea, compresses
o Just sever one of arches (smaller one) everything OK
CXR: look for right sided arch (sensitive but not specific – common variant)
Pulmonary swing:
Left PA originates from Right PA, courses posterior to trachea (see CT)
Results in tracheomalacia
Tracheomalacia / Bronchomalacia
Parents often aware of noisy breathing early but often don’t present until 6-12 mo
Fremitus is uniform, normal lung volumes (obstructing), lack of retractions, poor bronchodilator response
ALWAYS present if you have a TEF
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Bronchoscopy:
Left mainstem bronchus has keyhole appearance
Right mainstem bronchus has a lip (airway flopping into lumen)
Tracheal Bronchus
If airway not built right (e.g. aberrant RUL bronchus coming off of trachea instead of bronchus)
Picture: ignore arrow, actually the top bronchus branching off early
Chronic Congestion
CHRONIC WET COUGH IS A RED FLAG – something else is going on! (wheezing + cough)
o Beyond just narrowing of airways
o CF / primary/acquired dyskinesia, passive smoking, humoral immunodeficiency, retained foreign body
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Bronchiolitis
INFLAMMATION of BRONCHIOLES usu. occurring in children UNDER 2 YRS OLD resulting from VIRAL INFECTION
Disease of WINTER, infectious in nature
Pathophysiology
Airway obstruction Ventilation / perfusion mismatching
o Airway wall edema o Hypoxemia
o Mucous plugging
o Bronchospasm Paradoxical breathing
Increased airway resistance o Decreased tidal volume
o Air trapping o Decreased minute ventilation
o Decreased compliance
o Increased work of breathing
Therapy:SUPPORTIVE CARE
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Upper Airway Disorders
Anatomy Review
Nasal Cavity Turbinates and sinus ostia (ethmoid, maxillary, frontal)
Nasopharynx Airway posterior to the nasal passages, adjacent to soft palate
Oropharynx Posterior to the tongue
Hypopharynx Superior to and including the vocal cords
Larynx Airway inferior to the vocal cords
Laryngeal Position
Infants: have high larynx (C3)
o more efficient breathing with nursing
o ↓ aspiration risk
Nasopharyngeal Obstruction
Choanal atresia
Nasal cavities extend poteriorly during development, directed by palatal process’ fusion
st
Membrane separates nasal cavitiy from oral cavity thins & ruptures (mid 1 trimester)
Rupture failure = choanal atresia
o Remember infants are nasal breathers: 1° route of breathing obstructed
Dx: try to pass a #8 French catheter through each nostril to see if it’s patent!
Complications:
Aspiration from dyscoordination (2° to ↑ nasal airway resistance)
Severe hypoxemia with sleep (trying to breathe through nose obstructive apnea)
Waldeyer’s Ring
“adenoids & tonsils”
Adenoid Hypertrophy
Long face
Open mouth breathing (blocked nasal passage)
“Nasal” voice
↓ development of maxilla over time
Treatment: adenoidectomy
Oropharyngeal Obstruction
Tonsilar hypertrophy
“kissing tonsils” – see pictures
Can be graded but airway obstruction doesn’t correlate directly
o Also depends on airway tone is with sleep!
Cause airway obstruction
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Presentation (Tonsilar hypertrophy)
Snoring is most common
Muffled voice, drooling, trouble swallowing, choking on solids (more rare)
Obstructive sleep apnea: no airflow movement during breathing (dx with sleep study)
o mostly between 2-6 years old (tonsils growing, airway smaller)
o or adolescents (heavier more soft tissue)
Treatment: Tonsillectomy (80-90% successful)
Micrognathia
often associated with underlying genetic disorders
Therapy:
Mild micrognathia: may improve by school age
Severe micrognathia: can require intubation / tracheostomy
Macroglossia
Big tongue
Associated with: angioedema, congenital syndromes (e.g. Beckwith-Wiedemann), lymphangioma
Pathophysiology: Tongue displaced into hypopharynx obstructive apnea
Therapy: prone positioning, tongue debulking (rarely done)
Laryngopharyngeal obstruction
Laryngomalacia
Most common cause of stridor in infants
o Inspiratory noise, implies extrathoracic obstruction
o (Wheezing = expiratory, intrathoracic – e.g. asthma)
Pathophysiology:
Dynamic anomaly, cartilage collapses into airway
Etiology unclear (no tissue anomalies, no differences in muscle bulk – maybe muscle dyscoordination, structural variation)
Presentation:
Stridor onset since birth, minimal respiratory distress
Worse in supine position and when agitated / active
↓ noise when at rest (↑ flow ↑ turbulence – kind of like cardiac murmurs)
Normal voice quality & pitch
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Vocal Cord Paralysis
#2 common congenital laryngeal abnormality
Bilateral VCP Unilateral VCP
Etiology usually idiopathic usually recurrent laryngeal nerve damage
can be from CNS lesions (anything pressing on birth trauma or with cardiac surgery too (e.g. PDA
brainstem ligation)
Presentation Diagnosed late Normal phonation & stridor
Mild stridor, hoarse phonation, occasional aspiration Occasionally as airway emergency
(if on top of cough, cold, etc)
Treatment Correct CNS lesions, may need tracheostomy Improves (↓ inflammation, other VC compensates)
Signs of birth trauma: think VCP possibly
VCP: can be early sign of brain stem / spinal cord compression
Acquired too: local neck trauma, head trauma, viral compression (more rare in kids)
Subglottic Stenosis
Congenital Acquired
rd
Epidemiology 3 most common laryngeal anomaly Related to airway inflammation
Incomplete recanalization of larynx Inflammatory factors: prolonged intubation, traumatic
Pathophysiology
during gestation intubation, oversized endotracheal tube used, GE reflux
Presentation Recurrent / persistent croup Hx of prior intubation, airway instrumentation
If Severe stenosis: biphasic stridor, dyspnea, labored breathing
Gets much worse if they have a cough or cold already obstructed
If you’re having trouble with expected ET tube size, be careful!
Treatment: frequently requires tracheostomy or airway surgery (more than other two)
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Laryngopharyngeal Obstructions: Complication
Inability to coordinate feeding
Growth failure, aspiration
Treatment: occupational therapy or feeding tube (worst-case scenario)
Infections
Viral Croup (Laryngotracheobronchitis)
Most common infectious cause of upper airway obstruction in pediatrics
o Peak 18-24 mo
o Often post-URTI (coryzal prodrome)
Pathophysiology:
Edema narrowing; stridor from turbulence
Smaller airway, poor cell-mediated immunity predisposition of airway obstruction
Cricoid cartilage: complete ring (not C-shaped like lower down in trachea, bronchi)
o Bigger reduction in lumen (so more predisposition to obstruction) (resistance ↑ with r4)
Presentation:
Barking cough, hoarse voice, inspiratory stridor (exertion / agitation), restlessness
o Drooling / resp distress in severe cases
Symptoms worse at night
Hypoxemia / hypercarbia: severe upper airway obstruction
Hx of recurrent croup suggests underlying abnormality
(more than 3-4x in same kid)
Therapy:
Nebulized epinephrine (α-adrenergic effects vasoconstriction, ↓ edema)
o beta-agonists don’t help
o Doesn’t affect duration of croup
o REBOUND can occur – keep watching the kid for a while!
Heliox mixtures ↓ turbulence but no large studies
Most studies: no benefit with humidified air
Corticosteriods: supported by evidence but type, route, dosing regimen debated
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Epiglottitis
Pathophysiology of Epiglottitis
HiB 99% of cases historically
o Other bacteria & some viruses since vaccine
o HiB still in non-immunized, vaccine failure
(trisomy 21, prematurity, malignancy, immunodeficiency)
Presentation
Rapid for HiB, more gradual for strep
Sore throat / dyspnea muffled voice, drooling, tripod position, toxic appearance
o Picture: kid tripoding (leaning forward, on hands; retractions too)
Stridor isn’t prominent but can occur with worsening obstruction
Diptheria
Incidence ↓↓↓ with vaccination
Exudative material clogs / blocks airway (gray films)
Antitoxin is mainstay of therapy
Important Points
Upper airway obstruction can present as a medical emergency
Secure the airway first, then worry about diagnoses
Nasal obstruction can pose significant problems for obligate nasal breathers (infants)
Obtain polysomnography (sleep study) to assess severity of obstructive apnea
Pulse-oximetry alone is not adequate
Asymptomatic examination while awake can be misleading
Why we treat:
Obstructive apnea can lead to growth failure, developmental delays, and right ventricular failure
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