Alterations of Pulmonary Function

Terminology:
o Measurements of lung volume
Tidal Volume(TV)- 500 ml, volume of exhaled air after normal inspiration
Inspiratory reserve volume (IRV)-3000 -3300 ml
Expiratory reserve volume (ERV) 1000-1200 ml
Residual Volume (RV)-1200 ml the air that remains trapped in the alveoli
Total lung capacity (TLC)- 5700-6200 ml
Vital capacity (VC)- 4500-5000 ml, is the amount of air that can be forcibly expired
after a maximal inspiration and indicates the largest amount of air that can enter and
leave the lungs during respiration
Functional residual capacity (FRC)-
Inspiratory capacity (IC)
o Hypercapnia state of increased carbon dioxide in the blood (PaCO2)
o Hypoxemia decreased oxygen in the arterial blood leading to a decrease in the partial
pressure of oxygen (PaO2)
o Hypoxia cellular deprivation of O2
o Ventilation-perfusion mismatch- areas of the lungs are ventilated but not perfused or that
the lung is perfused but not ventilated.
Ventilation Perfusion
o Effective gas exchange or diffusion of O2 from the alveoli to the blood is dependent on an
even distribution of gas, such as in ventilation, and blood, such as profusion, into all portions
of the lungs
o Under normal conditions profusion is a little bit greater in the lung bases than ventilation so
the normal V/Q ratio is .8 to 1
If this number goes higher, its not good, it means that O2 is available but its not
being profused
o Diffusion of O2 from alveoli to blood
o Depends on balance:
Air getting into the alveoli (V)
Amount of blood perfusion in the capillaries around the alveoli (Q)
Ratio expressed a V/Q
Normally around .8 because ventilation is slightly less than profusion
o An abnormal V/Q is most common cause of hypoxemia because of shunting( when you have
deoxygenated blood, blood goes through but doesnt pick up any oxygen)
V/Q Mismatch
o Abnormal distribution of ventilation and perfusion
o Most common cause of hypoxemia
o So if you have an abnormal distribution of ventilation and profusion
One cause is: inadequate ventilation of the well perfused areas= LOW V/Q
The gases are in the alveoli but they cannot profuse through all of the
creations in the alveoli
Ex: shunting due to atelectasis, asthma, pulmonary edema
Another cause: poor perfusion of well-ventilated portions of lung=HIGH V/Q
Alveolar dead space alveoli ventilated but there is nothing to profuse it
Examples: Pulmonary embolus blocking flow to area
Have plenty of O2 gases in your alveoli but no blood to carry them
Pulmonary artery vasoconstriction
o In order for the lungs to operate efficiently an d gas exchange it is essential that the
pulmonary blood flow be directed to well ventilated areas of the lungs
o Hypoxic Pulmonary Vasoconstriction (HPV)
Mechanism for control of blood distribution within the lungs
The precise mechanism that under lines HPV is unresolved, we dont know why
Pulmonary arteries constrict with alveolar hypoxia (PAO2) partial pressure of arterial
O2
Protective mechanism
Pulmonary vascular resistance is increases so that meant that your pulmonary
arterials constrict and blood is shunted, moved around, from an area of poor
oxygenation to an area that is richer in O2 content
So you can see that if you have some atelectasis in one area how this might come
into place, it might shunt the blood around that and send it to a place that is better
oxygenated
The pulmonary circulatory system works a little differently from the systemic
pulmonary system
In pulmonary arteries when there is a moderate hypoxia these arteries
constrict but in the systemic circulation (kidneys, toes, fingers) the arteries
dilate
Its just that little bit of opposite in the pulmonary circulation and thats why
this HPV mechanism works in the lungs
This phenomenon, HPV is responsible for maintaining the V/Q ratio during localized
alveolar hypoxia
If the area that is poorly oxygenated is resolved then this vasoconstriction of the
pulmonary arterioles will be corrected. kind of a compensatory mechanism for the
respiratory system
If you have chronic alveolar hypoxia this can result in permanent pulmonary
hypertension because of the increase pressure and that eventually leads to right
heart failure (cor pulmonale)
Mechanism that tries to save your lungs in an episode of acute hypoxia that shunts
the blood from an area that is poorly oxygenated to an area that is better
oxygenated, once that has resolved itself the constriction goes away.
Pulmonary Disease
o Acute-of short duration but usually severe
o Chronic- persist for a long time or is a commonly reoccurring phenomenon
o Infectious
o Non-infectious
o Obstructive
o Restrictive (not covered in this lecture)
Respiratory Tract Infection
o Disease of upper airway
Flu
Virus incubation is about 2 days( it sits inside your body gaining strength)
by the 2
nd
day the disease will last from 7-10 days and usually your body is
able to fight it off without any complications
Upper respiratory disease
Once you are infected you get necrosis of the cilia and serous membrane in
nose and trachea, these cells die and sloth off all down in your lungs and you
cough them up. This gunk that falls down into your lungs as an upper airway
disease can develop into a lower airway disease. Because your lungs are
warm moist and dark (bacteria loving)
So you can get secondary bacteria pneumonia as a complication from a viral
flu
Clinical Manifestations
o Cough
o Chills/Fever
o Malaise- dont feel good
Can cause a secondary disease process- pneumonia
o Disease of lower airway
Involves the bronchioles, the alveoli, and the parenchyma(lung tissue)
Pneumonia
TB
Pneumonia
o Acute infection of the upper airways
o Bacterial (typical infections) streptococcus pneumoniae or staphylococcus aureus
o With infection the alveoli fill with mucus preventing them from exchanging gases
o The more surface area of the lung that is infected the sicker the patient- walking pneumonia
o The accumulation of exudate leads to dyspnea, V/Q mismatching (Low V/Q), and hypoxemia
o Viral (atypical infections)-flu, fungus, mycoplasma, herpes simplex, and chicken pox
o Non-Infectious-from inhaled gases or irritants, aspiration pneumonia such as vomit ( the HCl
which is a strong irritant initiates the inflammatory process, produces exudate or mucus
causing the gas exchange to decrease)
o Usually defined by the organism that is causing it but it is also defined by the location so you
can have a lobar pneumonia(involves a lobe of the lung)
o It is bronchial pneumonia if it involves more than one lobe
o Clinical Manifestations
Generally preceded by an URI (frequently viral)
Fever, chills
Productive or dry cough
Dyspnea
Malaise
Pleural pain
Hemoptysis (coughing up blood)
Elevated WBC- because of the infection
Chest x-ray infiltrates
o Chest x-ray with right lobe consolidation
o Can be community acquired or nosocomial infection (got it in the hospital)
If you are admitted to the hospital and develop pneumonia before 48 hours that is
community acquired
Treated with antibiotics and you do just fine
If you are admitted to the hospital and develop pneumonia after 48 hours it is
classified as nosocomial because of the incubation period
More difficult to treat because it usually a gram-negative bacteria(that is
more difficult to treat because they have already been exposed to the
antibiotics and developed a resistance)
o Risk factors
Severe illness- because the body is using its reserves to fight the initial illness
Mental impairment- can be associated with poor hygiene
Antibiotic treatment
Respiratory equipment- equipment in the hospital that has not been cleaned
properly or the technician is not using proper hygiene
Intubation- with trachea tube goes through the mouth or nose
In systemic infections where the body can transmit it: ex- septicemia(can be
transported through the blood by gas exchange)
Chronic cardio-pulmonary disease- have decreased profusion
Immunocompromised decreased immune response are at risk, patients with HIV or
an active TB
Age- very old or very young
Aerosolized inhaled bacteria that is spread through droplets, or patients who have
inhalers that can become contaminated
Normal pathogens- become virulent
Tuberculosis (TB)
o Agent: Mycobacterium Tuberculosis
o Transmitted by droplets
o Bacilli multiply and initiate the immune response
o Tubercle: neutrophils and macrophages seal off colonies of bacilli
o The tubercle is then incased in a collagenous scar tissue and within about 10 days the
bacteria is encapsulated, it dies forming a cheese like substance called caseation necrosis,
immunity develops and the TB may remain dormant for life or it may be reactivated in the
presence of an immune system that is impaired
Ex: patients with HIV, poor nutrition, on antirejection drugs, or other debilitating
diseases
o If the live bacilli escapes into the bronchi active disease occurs and may spread to other
organs
o Many individuals who are infected with TB are asymptomatic
o Clinical manifestations
Fatigue
Weight loss
Lethargy
Anorexia
Low-grade fevers- generally in the afternoon
Cough with purulent sputum
Night sweats
Dyspnea
Hemoptysis (blood streaked sputum, purulent discharge is puffy)
o Evaluation and Treatment
Diagnosed by:
Positive TB skin test (PPD)
Sputum culture
Chest x-ray
Treatment
Combination antibiotic therapy
Disease or injury
o Pulmonary aspiration
o Pulmonary atelectasis
o Pulmonary edema
o Pneumothorax
o Pleural effusion
Conditions caused by Pulmonary Disease or Injury
o Aspiration
Impairment of normal swallowing or cough reflex
Right lung more susceptible
Preventive measures are more effective than treatment
Rate of deaths from aspiration pneumonitis is >50%
o Atelectasis(anytime the alveoli collapse)
Collapse of lung tissue
Compression atelectasis- caused by external pressure
Absorption atelectasis-inhalation of concentrated oxygen or anesthetic
agents (not discussing)
Tends to occur after surgery
Clinical manifestations- dyspnea, cough, fever, and leukocytosis
Pulmonary edema(accumulation of fluid in the air sacs(alveoli) in the lungs)
Occurs when there is water in the lungs
Most frequently caused my cardiac disease
o Predisposing factors include
Heart disease (most common)
ARDS (acute respiratory distress syndrome)
Inhalation of toxic substances- causes the water to move out of the capillaries and
into the lungs
o Pathogenesis
Increased left atrial pressure
The left ventricle fails, results in an increase in the pulmonary capillary
hydrostatic pressure, meaning that fluid moves out of the capillaries into the
interstitial space and as the flow of fluid in the capillaries exceeds the ability
of your lymphatic system to remove it pulmonary edema develops)
Injury to capillary endothelium can be caused by ARDS
Blockage of lymphatic vessels- obstruction of the lymphatic with things like edema or
tumors
o Clinical manifestations
Dyspnea
Orthopnea (difficulty breathing while you are lying down)
Hypoxemia
Pink, frothy sputum
o Treatment is geared toward cause
Pneumothorax
o Presence of air or gas in the pleural space caused by a rupture in the visceral pleura or
parietal pleura
o Disrupts the equilibrium between elastic recoil of lung and chest wall
o Lung tends to collapse
o A spontaneous pneumothorax occurs unexpectedly in healthy individuals
For some unknown reason blister like formations that are called blebs form on the
visceral plural, and when they rupture the elastic recoil of the lungs is disrupted and
the lung collapses toward the hilum
o Secondary or traumatic pneumothorax is caused by trauma, could be a rib fracture, gun
shot, stab wound, can occur with patients with COPD (Chronic obstructive pulmonary
disease), or patients who are on ventilators
o Clinical manifestations of spontaneous and secondary pneumothorax
Pain
Tachypnea
Mild dyspnea
o Can either be an open or tension pneumothorax
Open pneumothorax (such as a stab wound) some air exchange between the open
wound and the chest
Tension pneumothorax there is no air exchange, inspired air gets trapped, causes a
compression which leads to the displacement of the heart and the great vessels. Is
life threatening
Pleural effusion
o Presence of fluid in the pleural space
Hemothorax: blood
Empyema: pus
Patients with have sinusitis, fever, tachycardia, cough, pleural pain
Causes include complications of pneumonia, surgery, trauma, or bronchial
obstruction from a tumor.
Treatment includes antibiotics
All pneumothorax and pleural effusions have to be drained with a chest tube
o Clinical Manifestations
Dyspnea
Pleural pain with inflamed pleura
Obstructive lung disease
o COPD (chronic obstructive pulmonary disease )
Emphysema
Chronic bronchitis
Even though their pathophysiology is very different their clinical manifestations and
treatment modality are the same
o Asthma
o Emphysema
Abnormal permanent enlargement of gas exchange airways
Destruction of alveolar walls
Obstruction results from changes in lung tissue, rather than mucus production and
inflammation
Is irreversible
Major mechanism in decreased air flow because of the loss of elastic recoil
Loss of elastic recoil results in difficulty with expiration and hyperinflation of the
alveoli
Primary emphysema inherited deficiency of the enzyme
1
antitrypsin(suggested
in individuals who develop emphysema before the age of 40 or in non-smokers
Secondary emphysema excessive accumulation of neutrophils within the lung
tissue; cells release proteolytic enzymes that damage respiratory
Most common cause is smoking- smoke triggers the immune response, the
neutrophils and macrophages are activated but they are retained in the lung
tissue, the release proteolytic enzymes that destroy the extracellular matrix
such as the elastin in the lungs
o The reduces elasticity in the lungs reduces the ability of the alveoli to
recoil and release CO2, the air spaces become large and ineffective,
the alveolar walls collapse and air is trapped in the alveoli and that
means the effective O2 intake is compromised as well as the release
of CO2
Clinical Manifestations
Related to chronic hypoxemia and hypercapnia (high CO2)
Dyspnea on exertion dyspnea even at rest
No cough (early)
Minimal sputum production (early)
Use of accessory muscle with ventilation
Barrel chest
Posture leading forward to help with breathing
Treatment
Maintain optimal lung function
Best treatment is prevention
o Stop smoking
o Chronic Bronchitis
COPD
The presence of a persistent productive cough with excessive mucus that last for 3
months or long for 2 or more consecutive years
Diagnosis is made only after all other diagnosis are excluded
Irritation of the bronchi caused by:
Irritants (cigarette smoke or air pollutants )
Infection
Cigarette smoke or the exposure to second-hand smoke is the primary cause
of chronic bronchitis symptoms
Pathogenesis
o Bronchial edema- caused by continual bronchial inflammation
o Hypersecretion of mucus- inflammation results in the increase of the
size and number of the secretion of the goblet cells that produce a
thick hypersecretion of mucus
o Chronic productive cough
o Impaired ciliary function
o Bronchospasm as the lungs defense mechanisms are compromised
there is an increase in the acceptability in pulmonary infections and
injury which are further complicated by bronchospasm
o Initially effects only the larger bronchi but over time all airways are
involved
The airways collapse early in expiration and gas is trapped in the distal
portion of the lungs
Obstruction eventually leads to:
o Ventilation perfusion mismatch
o Hypoventilation muscles are compromised leased to
hypoventilation and hypercapnia
o Hypoxemia
Clinical manifestations:
o Wheezing and shortness of breath
o Chronic productive cough
o Increased sputum production
o Abnormal spirometry
o Hypoxemia
o Polycythemia- compensatory mechanism in the body, the increase
production of red blood cells in the blood, helps to increase the
amount of O2 that can be transported
o Pulmonary hypertension
Treatment modalities: !!!!(Dont need to know)!!!!
o The best treatment is prevention
Asthma
Inflammatory disease resulting in bronchospasm
Unlike COPD it is more acute and is intermediate
A familial disorder with over 20 genes identified that may contribute to the
accessibility and pathogenesis of asthma
The major pathologic feature with asthma is inflammation resulting in hyper-
responsiveness of the airways
Involves many factors:
o Biochemical
o Autonomic
o Immunologic
o Infectious
o Endocrine
o Psychologic
Inflammatory process produces
o Bronchial smooth muscle spasm
o Vascular congestion
o Increased vascular permeability
o Edema
o Production of thick tenacious mucus
o Impaired mucociliary function- cannot be cleared
o Thickening of airway walls
The expiratory air flow is decreased and this causes air trapping and
hyperinflation distal to the obstruction which increases the work of
breathing, as the alveolar gas pressure rises perfusion to the alveoli is
decreased which leads to a V/Q mismatch causing hypoxemia. Initially there
is hyperventilation which causes an increase in the pH with a respiratory
alkalosis.
But as the obstruction becomes more severe the number of alveoli being
ventilated and profused decreases, this leads to hypoventilation with
retention of CO2 in respiratory acidosis. This signals respiratory failure
If the bronchospasm is not reversed the patient is considered to have status
asthmaticus which is life threatening
Airway obstruction
o Increased resistance
o Decreased flow rates
Decreased air flow decreased ventilation
o Hypoxemia
o Respiratory alkalosis
o Continued inadequate ventilation
o CO2 retention
o Respiratory acidosis
o Respiratory failure
Clinical Manifestations:
o Dyspnea
o Marked respiratory effort
o Prolonged expiration
o Decreased breath sounds
o Wheezing
o Alterations in arterial blood gases
o Alterations in flow rates and lung capacities
Treatment: (NOT ON TEST)
Pulmonary Vascular Disease
o Pulmonary embolism
Occlusion of a portion of the pulmonary vascular bed by an embolus
Thrombus (blood clot)
Tissue fragment
Lipids (fats)
Foreign body or air bubble
Most common are thrombi dislodged from deep veins in the calf
Impact depends on
Extent of pulmonary blood flow obstruction
Size of affected vessels
Nature of embolus
Secondary effects
Massive occlusion:
Shock
Hypotension
Tachypnea
Tachycardia
Sever pulmonary hypertension
Chest pain
Manifestations associate with infarction (tissue death)
Pleural pain
Dyspnea
Pleural friction rub
Pleural effusion
Hemoptysis
Fever
Leukocytosis
Embolus without infarction (no tissue death)
Tachypnea
Tachycardia
Dyspnea
Unexplained anxiety
Occasional fainting
Anticoagulant therapy is the primary treatment
If the embolus does not cause infarction, the clot is dissolved by the fibrinolytic
system
If infarction occurs, shrinking and scarring develop in the affected area
Risk factors:
Disorders that promote blood clotting
o Venous stasis (seen in patient who stays in one place for a long time-
particularly postoperatively
o Hypercoagulability
o Injuries that cause bleeding
Frequent complication of hospitalization
Important cause of death, especially in elderly and hospitalized individuals
Clinical manifestations
Nonspecific
More than 90% of pulmonary emboli are a result of clots formed in the legs
or pelvis
o Leg swelling
o Duskiness
o Positive Homan sign (bend toes back and get pain in the back of leg)
o Calf asymmetry
Prevention
Leg elevation
Bed exercises
Position changes
Early post-op ambulation
Pneumatic calf compression
o Pulmonary Hypertension
Abnormally high blood pressure in the arteries of the lungs
It is either primary or secondary, since primary is very rare we are only talking about
secondary pulmonary hypertension
Results from diseases of the respiratory system that cause hypoxemia, with
pulmonary arteriolar vasoconstriction and arterial remodeling
It makes the right side of the heart need to work harder than normal
Often not detected until sever
Clinical manifestations
Fatigue
Chest discomfort
Tachypnea
Dyspnea, mainly with exertion
o Cor Pulmonale
Creates a chronic pressure overload in the right ventricle, increases the work load of
the heart
Normally the thin walls of the heart muscle enlarge (hypertrophy) which eventually
leads to dilation and failure of the right ventricle
Normally it is the left side of the heart that produces the high blood pressure in order
to pump the blood to the body, the right side of the heart only needs to pump blood
through the lungs and is under a much lower pressure but when the right ventricle is
unable to pump against these high pressures from pulmonary hypertension you get
right sided heart failure called Cor Pulmonale
Failure of the right side of the heart brought on by long-term high blood pressure in
the pulmonary arteries and right ventricle of the heart.
An enlargement of the right ventricle due to high blood pressure in the arteries of
the lungs usually caused by chronic lung disease.
Difficult to detect and may be obscured by primary respiratory disease
Clinical manifestations
Jugular venous distension
Hepatosplenomegaly
Peripheral edema
Alterations of Pulmonary Function in Children
o Upper Airway Disorders
Croup
Airway obstruction below the vocal cords (in subglottic region of trachea)
o Slightly narrower than trachea
o More loosely attached
o More vascular
o Leads to airway compromise in children
As the child struggles to move air a negative pressure is generated and this
may cause airway structures higher up to collapse with inspiration
Symptoms
o Rhinorrhea- snotty nose
o Sore throat
o Low grade fever for a few days
o Followed by a barking cough that is very distinctive
Usually self-limiting
o If you have a child that has an inspiratory stridor or any sort of
respiratory distress this suggest that it is a more severe situation and
may require hospitalization with treatment of nebulized epinephrine
Manifestations are produces primarily by the inflammatory edema of the
upper trachea
A child with sever croup may have deep retraction, stridor, agitation,
tachycardia, pallor, or cyanosis
Breathes harder to move air
o Excessive negative pressure can cause airway structures (above
obstruction) to collapse upon inspiration
Turbulent flow across obstruction can cause stridor on inspiration
o Sometimes on expiration
Spasmodic croup
o Usually seen in older children
o Sudden onset, usually at night
o Usually no prodomal symptoms(rhinorrhea, fever, sore throat and
cough)
o Similar hoarseness, barking cough and stridor as in croup
But occurs in older children
o Manifestations due to inflammatory edema of upper trachea
Acute epiglottis
Caused by the H. influenza type B, has been decreased because of
vaccination
Caused by group A streptococcus
Sever, rapidly progressive, life-threatening
o Infection of the epiglottis and surrounding area
o Child suddenly develops fever, inspiratory stridor and severe
respiratory distress
Clinical Manifestations:
o Appears anxious
o Voice sounds muffled
o Drooling and dysphagia are common
o Death can occur in a few hours
Aspiration of foreign body
Clinical manifestations of airway occlusion
o Cough
o Stridor
o Hoarseness, inability to speak
o Respiratory distress
o Agitation or panic
Clinical manifestations of bronchial foreign bodies
o Cough or wheezing
o Atelectasis
o Pneumonia
o Lung abcess
o Blood-streaked sputum
o Lower Airway
Respiratory Distress (RDS)
Surfactant deficiency
AKA hyaline membrane disease
Primarily seen in premature infants who have a structural immaturity and
normally do not secret surfactant until after about 30 weeks gestation
Structural immaturity
o Underdeveloped and small alveoli
o Weak chest wall/ highly compliant
o Surfactant secretion around 30 weeks gestation
So if you have a baby that is born 26 or 27 weeks they
havent began the secretion with the type II cells
These babies have wide spread atelectasis, respiratory distress, and
pulmonary hypertension
Results of RDS
o Atelectasis (collapsed alveoli)
o Hypoxemia and hypercapnia
Pulmonary vasoconstriction and increased pulmonary
vascular resistance
o Shunting (or return to fetal circulation ) and hypoperfusion of lungs
o Increase ulmonary capillary permeability
o Damage to alveolar epithelium
Plasma proteins leak into alveoli and interfere with function
of surfactant
o Fibrin deposits (hyaline membranes)
Breathing is difficult and leads to atelectasis, and require a significant
amount of negative inspiratory pressure to overcome the atelectasis and to
open up the alveoli
More O2 is used to sustain the work of breathing resulting in the hypoxemia
and hypercapnia
Clinical manifestations are seen immediately at birth
o Have pronounced inspiratory and expiatory retraction
o Nasal flaring
o Poor coloring
Require mechanical ventilation and the administration of exogenous
surfactant
The prevention of preterm birth is the primary treatment
Bronchopulmonary dysplasia (BPD)
AKA Chronic lung disease of infancy
Persistent lung disease following neonatal lung injury
Although the instances are decreasing with advanced treatment and cases of
prematurity its a significant predictor of BPD because of ventilation on the
day of birth
Pathophysiology and clinical manifestations mirror RDS
These children have long term effects and are at an increased risk for asthma
Disrupted lung development
o Few alveoli (large in size) = decreased surface area for gas exchange
o Alterations in the growth of pulmonary capillaries
Results:
o Impaired gas exchange, ventilation-perfusion mismatch, poor
capacity to exercise
Bronchiolitis
Viral infection of the lower pulmonary tract usually caused by respiratory
syncytial virus
In most cases the infant makes a full recover
Respiratory Syncytial Virus (RSV)
o 50 to 70 % of cases
Necrosis of bronchial epithelium
Destruction of ciliated epithelial cells
Inflammation
Narrowing of airways from edema, debris
Bronchospasms, atelectasis, air trapping (as seen with RSD, and BPD)
Increased in work of breathing, hypoxemia and hypercapnia in severe cases
Clinical manifestations
o Significant rhinorrhea, cough, lethargy, fever, wheezing heard with
auscultation
Treatment based on the severity of the disease, age of the infant, and the
underlined lung disease
Asthma : Allergen- Induced
Inflammatory obstructive airway disease thats reversible and has bronchial
hyper-reactivity
Early asthmatic response
o Immediately after exposure lasting up to 2 hours
o Allergen binds to IgE of mucosal mast cells
Triggers a degranulation of the mast cell, release of the
mediators (histamine, prostaglands and certain cytokines)
o Production of mediators
Smooth muscle constriction (bronchospasm)
Increased vascular permeability (mucosal edema)
Mucus secretion
o Late Asthmatic Response
4-8 hours post exposure lasting up to 24 hours
See page 758 for illustration
Airway hyper-responsiveness
Obstructions
o Obstruction of airway -> air trapping!
o Increases work of breathing
o Decreased perfusion of alveoli
o Hyperventilation
o Decreased PaCO2 and increased pH
Respiratory Alkalosis
o Eventually- without intervention
o Hypoventilation
o CO2 retention
Respiratory Acidosis
o Precursor to an acute respiratory failure
Cystic fibrosis (CF)
Autosomal recessive inherited disease
o Defective epithelia chloride ion transport
o CF gene on chromosome 7
Mutation results in abnormal expression of the protein Cystic fibrosis
transmembrane conductance regulator (CFTR)
o CFTR is a chloride channel that is normally present on the surface of
many types of epithelial cells
o We see these chloride channels in our airways, bile ducts, pancreas,
or sweat glands, and vas deferens
Features
o The exocrine or mucus producing glands secrete abnormally thick
mucus and because of the affective epithelial ion transport , because
of this mutation of the CFTR, the bronchiole are obstructed in the
lungs, predisposing the lungs to chronic infection
o Mucus plugging
Increased production & altered properties of mucus (viscous)
Impaired ciliary mobility
o Chronic inflammation
The abnormal cytokine profile promotes a pro-inflammatory
state
Neutrophils release oxidants and proteases- cant be
removed and buildup causing damage to the lung structures
such as elastin
Contributes to the long term lung damage
This cycle continues to cause problems
o Chronic infection
Environment favor bacterial colonization
o Although it is a multisymptom disease the most important effects are
its effects on the lungs
o Patients have respiratory arrest or respiratory failure, its the
common cause of their death
o Treatment is focused on pulmonary and nutritional need
o Clinical Manifestations
Respiratory
Persistent cough or wheeze
Recurrent or sever pneumonia
Over time : barrel chest and digital clubbing
Gastointestinal
Meconium ileus at birth (unable to pass meconium
at birth)
Failure to thrive
Malabsorptive symptoms (frequent loose oily stools)