Test 3 Flashcards
Cystic firbrosis leads the excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree secondary to ____________________________.
Inadequate hydration of the periciliary fluid layer (sol layer)
Partial obstruction leads to what?
Over distention of the alveoli
Complete obstruction leads to ____________________.
Patchy areas of atelectasis and in some cases bronchiectasis
What are the predominant features of CF in the advanced stages?
- Excessive bronchial secretions
- Bronchial obstruction
- Hyperinflation of the lungs
What are the major respiratory pathologic or structural changes associated with CF? (6)
- Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree secondary to inadequate hydration of the periciliary layer.
- Partial bronchial obstruction (mucus plugging)
- Hyperinflation of the alveoli
- Total bronchial obstruction (mucus plugging)
- Atelectasis
- Bronchiectasis
What is the most common fatal inherited disorder in childhood?
Cystic Fibrosis
What type of gene disorder is CF?
Autosomal recessive gene disorder caused by mutations in a pair of genes located in Chromosome 7
Under normal conditions, every cell in the body has _______ chromosomes.
46 chromosomes - 23 pairs
More than _________ different mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator have been described.
1700
The most common genetic defect linked to CF involves the absence of three base pairs in ___________ that codes for phenylalanine on chromosome 7 (band q31.2)
Codon 508 (ΔF508)
The absence of three base pairs in codon 508 (ΔF508) accounts for ___________ of patients with CF tested.
70-75%
The abnormal expression of the CFTR results in what?
Abnormal transport of sodium and chloride ions across many types of epithelial surfaces, including those lining the bronchial airways, intestines, pancreas, liver ducts, and sweat glands.
The CFTR mutations that is classified a gating defect means ________________.
The channel does not open
The CFTR mutations that is classified as conductance defect means
________________.
The channel is open, but chloride does not move effectively.
Because CF is a recessive gene disorder, the child must inherit _______ copies of the defective gene disorder.
2 copies, one from each parent to have the disease
If both parents carry the CF gene, the possibility of their children having CF follows the ________________.
Standard Mendelian Pattern
When both parents carry a CF gene mutation, there is a _____ in four chance that the chid will have CF.
One.
1/4
It is estimated that how many Americans are unknowing, symptomless carriers of the mutant CF gene?
10 million
According to the CF Foundation, CF affects about how many children and adults in the US and how many worldwide?
US - 30,000
Worldwide - 70,000
About ______ new cases of CF are diagnosed each year in the US.
1000
More than ___% of patients are diagnosed with CF by newborn screening.
90
More than ____% of the patient population with CF are 18 years or older.
50
What is the median age of survival for individuals with CF?
In the late 30s, but many patients with CF live into their 40s and beyond.
CF occurs most often in what race?
Caucasians (1:3000)
The diagnosis of CF is based on what three things?
- Clinical manifestations associated with CF
- Family history of CF
- Lab findings
What two criteria must be met to diagnose CF?
- Clinical symptoms consistent with CF in at least one organ
- Clinical evidence of CTFR dysfunction
What is classified as clinical evidence of CTFR dysfunction?
- Elevated sweat chloride greater than 60 mEq/L (on two occasions)
- Molecular diagnosis (genetic testing). Presence of two disease-causing mutations in CFTR.
- Abnormal nasal potential difference
Newborn screening for CF has been part of the newborn genetic testing protocol in all 50 states since _____.
2011
Most infants with CF have an elevated blood level of immunoreactive trypsin, which can measured by ________________.
- Radioimmunoassay
- An enzyme-linked immunoassay
Immunoreactive trypsin is also called what?
Trypsin-like immunoreactivity or serum trypsin
The immunoreactive trypsin level (IRT) is measured from the ___________ collected on all newborn infants on the Guthrie cards.
blood dots
The CF screening protocol varies among states and will identify more than ____% of infants wit CF.
90
What is the most common CF protocol?
Perform DNA screening for 32 to 85 of the most common CF mutations on 2-5% of the samples with the highest IRT levels
The diagnosis of CF is established by what?
A positive sweat test and/or genetic analysis for CF mutations
What identifies a newborn as a CF carrier?
Negative or normal sweat test
_________________ is the gold standard diagnostic test for CF.
Sweat test or sweat chloride test
The sweat test is a reliable test for the identification of about ____% of patients with CF.
98
CF Pancreas Mnemonic
Chronic Respiratory Disease
Failure to Thrive
Polyps
Alkalosis, Metabolic
Neonatal intestinal obstruction
Clubbing of fingers
Rectal prolapse
Electrolyte increase in sweat
Aspermia/absent vas deferens
Sputum: Staphylococcus aureus, Pseudomonas aeruginosis
What does the sweat test measure?
Amount of sodium and chloride in the patient’s sweat
During the sweat test procedure, a small amount of a colorless, odorless sweat-producing chemical is applied to the patient’s arm or leg. What is this chemical called?
Pilocarpine
How many times is the sweat test usually done?
Twice
How is sweat produced during a sweat test?
An electrode is attached to the chemically prepared area, and a mild electric current is applied to stimulate sweat production.
Although the sweat glands of patients with CF are microscopically normal, the glands secrete up to ________ the normal amount of sodium and chloride.
FOUR TIMES
In both infants and adults, a sweat chloride concentration greater than _______ is considered to be a diagnostic sign of CF.
60 mEq/L
With a sample of the patient’s _______________, a genetic test can be performed to analyze DNA for the presence of CFTR gene mutations.
Blood or cheek cells
Most of the diagnostic laboratories in the US are able to screen for at least ________ of the most common mutations.
30-100
What is the sweat test interpretations?
Infants 6 mo. or YOUNGER
Normal: Less than or equal to 29 mmol/L
Intermediate: 30-59 mmol/L
Abnormal: Greater or equal to 60 mmol/L
Infants Older than 6 mo, Children & Adults
Normal: Less than or equal to 39 mmol/L
Intermediate: 40-59 mmol/L
Abnormal: Greater or equal to 60 mmol/L
It is estimated that genetic testing can confirm CF in about how many of the patients tested?
90-96%
As the sodium and chloride ions move across the epithelial cell membrane, they generate what is called an __________________.
Electrical Potential Difference
Define Electrical Potential Difference.
The amount of energy required to move an electrical charge from one point to another.
Where can the nasal potential difference be measured?
With a surface electrode over the nasal epithelial cells lining the inferior turbinate
What does an increased nasal potential difference suggest?
CF
The nasal potential difference is recommended for what type of patients?
Patient with clinical features of CF who have borderline or normal sweat test values and nondiagnostic CF genotyping.
If desired, a fetus can be tested for CF by amniocentesis during what trimester?
First
Fetal cells are tested for the presence of CF mutations and identified as what?
- CF affected
- CF carrier
- Normal
___________________ is very important in all cases of prenatal testing for CF to explain the uncertainty or residual risk to prospective parents.
Genetic counseling
Explain fecal fat test.
It measures the amount of fat in the infant’s stool and the percentage of dietary fat that is not absorbed by the body.
Infants with CF and pancreatic insufficiency will have a fecal elastance of ____________.
Less than 50 µg/g
What is the normal fecal elastance value?
Greater than 300 µg/g
What is the stool fecal fat test used to evaluate?
How the liver, gallbladder, pancreas and intestines are functioning.
What are the major anatomic alterations of the lungs associated with CF? (3)
- Bronchospasm
- Excessive bronchial secretions
- Atelectasis
Define spontaneous pneumothorax.
No obvious injury could have caused the pneumothorax.
Side note: Once you’ve have one, there’s about a 50% chance that it will recur.
Describe the physical examination of a CF patient.
- Increased RR (Tachypnea)
- Increased HR and BP
- Use of accessory muscles of inspiration/expiration
- Pursed-lip breathing
- Cyanosis
- Digital clubbing
- Peripheral edema and venous distention
- Diminished heart and breath sounds
- Decreased or increased tactile fremitus
- Hyperresonant percussion note
- Bronchial breath sounds
- Crackles
- Wheezing
-Spontaneous pnuemothorax
What would an ABG look like for a patient MILD TO MODERATE CF?
Acute Alveolar Hyperventilation with Hypoxemia (Acute Respiratory Alkalosis)
What would an ABG look like for a patient SEVERE STAGE CF?
Chronic Ventilatory Failure with Hypoxemia (Compensated Respiratory Acidosis)
What are the abnormal lab test associated with CF?
Increased hematocrit and hemoglobin
Increased WBC
Hypoxemia makes the blood produce more blood cells
What are some radiologic findings of CF? (8)
- Translucent (dark) lung fields
- Depressed or flattened diaphragm
- Right ventricular enlargement (Cor pulmonale)
- Areas of atelectasis or fibrosis
- Tram tracks
- Bronchiectasis (often a secondary complication)
- Pnuemothorax (spontaneous)
- Abscess formation
About _____% of men with CF are infertile.
99
Nasal polyps are seen in between _________ of patients with CF.
10% and 30%
What is meconium ileus?
Obstruction of the small intestine of the newborn that is caused by the impaction of thick, dry, tenacious meconium, usually at or near the ileocecal valve.
What are the primary goals for patients with CF?
- Prevent pulmonary infections
- Reduce the amount of thick bronchial secretions
- Improve airflow
- Provide adequate nutrition
Hypoxemia may NOT respond well to oxygen therapy when ____________ is present.
True or capillary pulmonary shunting
What are keys to reversing consolidation caused by mucous plugging?
Deep breathing and effective cough
When do you want to administer bronchodilators to CF patients?
- Before patient receives chest physiotherapy or exercise
- Before patient receives inhaled nebulized hypertonic saline, antibiotics and/or dNASE to offset bronchial constriction
What are recommended bronchodilators for CF?
SABA - Albuterol
LABA - Salmeterol and Formoterol
What is Ivacaftor’s brand name?
Kalydeco
What may be used to hydrate thick mucus in the airways of patients with CF who are 6 years of age or older, have a chronic cough or have a reduced FEV1?
Inhaled hypertonic saline
Inhaled hypertonic saline is usually given with a bronchodilator how often?
Twice a day.
What medication has not been proven to be effective in treating patients with CF?
Inhaled N-acetylcysteine
Drugs that help mutated CFTR reach the epithelial cell surface where the CFTR protein normally functions as a transmembrane regulator of chloride movement out of the cell and sodium transport into the cell.
Correctors
Drugs that help mutated CTFR function more effectively at the epithelial cell surface transporting chloride out of the cell and inhibiting the movement of sodium into the cell.
Potentiators
An oral potentiator molecule that has been proved effective to improve cell function and clinical status in patients with CF with the G551D mutation.
Ivacaftor (Kalydeco)
Which of the following organisms are commonly found in the tracheobronchial tree secretions of patients with. CF?
- Staphylococcus
- Haemophilus influenzae
- Pseudomonas aeruginosa
About 80% of all patients with CF demonstrate a deficiency in which of the following vitamins?
D, A, K, E
Bronchiectasis is an acquired disorder of the major bronchi and bronchioles characterized by what?
Chronic dilation and distortion of one or more bronchi
Bronchiectasis is commonly limited to a lobe or segment and is commonly found in which part of the lungs?
Lower lobes
Reid classification subdivides bronchiectasis into what following three patterns?
- Varicose (fusiform)
- Cylindrical (tubular)
- Cystic (saccular)
The bronchi are dilated and constricted in an irregular fashion, ultimately resulting in a distorted bulbous shape.
Varicose (fusiform) bronchiectasis
The bronchi progressively increase in diameter until they end in large, cystlike sacs in the lung parenchyma.
Cystic (saccular) bronchiectasis
The bronchi are dilated and rigid and have regular outlines similar to a tube.
Cylindrical (tubular) bronchiectasis
With cylindrical (tubular) bronchiectasis, X-ray shows that the dilated bronchi fail to taper for _______ generations and then appear to end abruptly because of mucous obstruction.
6-10
Which form of bronchiectasis causes the greatest damage to the tracheobronchial tree?
Cystic (saccular) bronchiectasis
With cylindrical (tubular) bronchiectasis, the bronchial walls becomes composed of _______ alone.
Fibrous tissue,
Cartilage, elastic tissue and smooth muscle are all absent
What are the major pathologic or structural changes associated with bronchiectasis? (7)
- Chronic dilation and distortion of bronchial airways
- Excessive production of often foul-smelling sputum
- Bronchospasm
- Alveoli hyperinflation
- Atelectasis
- Parenchymal consolidation and fibrosis
- Hemoptysis secondary to bronchial arterial erosion
Most causes of bronchiectasis include some combination of ____________ and ______________.
Bronchial obstruction and infection
In developed countries, ______________ is the most common causes of bronchiectasis in children.
CF
In the US, the incidence of NCFB is about _______________.
4.2 per 100,000 young adults
The low incidence of NCFB in developed countries is most often attributed to what?
Early medical management.
What is the most common causes of NCFB?
Pulmonary infection
The causes of bronchiectasis are commonly classified in what following categories?
- Acquired bronchial obstruction
- Congenital anatomic defects
- Immunodeficiency states
- Abnormal secretion clearance
- Miscellaneous disorders
A routine chest radiograph of bronchiectasis may reveal what? (11)
- Overinflated lungs
- Marked volume loss
- Increased opacities
- Dilated fluid-filled airways
- Crowding of the bronchi
- Translucent (dark) lung fields
- Depressed and flattened diaphragms
- Long and narrow heart pulled down by diaphragm
- Enlarged hearted when heart failure present
- Tram tracks
- Areas of consolidation and/or atelectasis may or may not be seen
What has replaced bronchograms as the gold standard for the diagnosis of NCFB?
HR-CT (High-Resolution Computed Tomogram)
The diagnosis of bronchiectasis is made on the basis of what three things?
- Internal diameter of the bronchus that is wider than its adjacent pulmonary artery
- Failure of the bronchi to taper
- Visualization of bronchi in the outer 1-2 cm of lung fields
The HR-CT is used to better clarify the findings of the chest radiograph and standard CT scans, and allows ____________ of airway abnormalities that cannot be identified on routine films of the chest.
Lung Mapping
What can be used to determine if the bronchiectasis demonstrates primarily an obstructive or restrictive lung pathophysiology?
Spirometry
What can be used to determine if the patient has mild, moderate or severe gas exchange compromise?
ABG
You will see pursed-lip breathing in bronchiectasis patients when their pathology is primarily ___________________.
Obstructive
State the clinical manifestations of bronchiectasis. (4)
- Excessive bronchial secretions
- Bronchospasm
- Consolidation
- Increased alveolar-capillary membrane thickness
What is the hallmark of bronchiectasis?
Chronic cough with large production of foul-smelling sputum
Which anaerobic organisms are commonly cultured from the sputum of bronchiectasis patients?
- Haemophilus influenzae
- Streptococcus
- Psuedomonas aeruginosa
The productive cough seen in patients with bronchiectasis is triggered by __________________.
The large amount of secretions that fill the trachebronchial tree
The subepithelial mechanoreceptors are found in the trachea, bronchi and bronchiole but are predominantly located ____________.
In the upper airways
What are chest assessment findings in bronchiectasis that is primarily obstructive?
- Decreased tactile and vocal fremitus
- Hyperresonant percussion note
- Diminished breath sounds
- Wheezing
- Crackles
What are chest assessment findings in bronchiectasis that is primarily restrictive? (5)
- Increased tactile and vocal fremitus
- Bronchial breath sounds
- Crackles
- Whispered pectoriloquy
- Dull percussion note
How does a PFT look for a moderate or severe bronchiectasis patient?
Everything normal or DECREASED.
RV/TLC ratio normal
Bronchiectasis: Hematology
- Increased hematocrit and hemoglobin
- Elevated WBC if patient is acutely infected
Parallel or curved opacity lines of varying length caused by bronchial wall thickening.
Tram tracks
Which type of bronchiectasis would tram-track opacitites most likely be seen in?
Cylindrical bronchiectasis
What is the general treatment plan for bronchiectasis?
Control pulmonary infections, airway secretions and airway obstruction and preventing complications
USE OF CORTICOSTEROIDS IS DISCOURAGED WITH BRONCHIECTASIS.
JUST KNOW
What are some airway clearance techniques used for bronchiectasis?
- Directed cough
- CPT (Postural drainage)
- Suctioning
- PEP
- High-frequency chest wall oscillation
What are the major pathologic or structural changes associated with pnuemonia? (3)
- Inflammation of the alveoli
- Alveolar consolidation
- Atelectasis
Atelectasis is often associated with patients who have ___________________.
Aspiration pneumonia
What are some causes of pneumonia? (9)
- Bacteria
- Viruses
- Fungi
- Protozoa
- Parasites
- TB
- Anaerobic organisms
- Aspiration
- Inhalation of irritating chemicals such as chlorine
Polymorphonuclear leukocytes move into the infected area and engulf and kill invading bacteria on the alveolar walls. What is this called?
Surface phagocytosis
Taken together, pneumonia and influenza combined are the _________ leading cause of death among Americans.
eigth
Taken together, pneumonia and influenza combined are the _________ leading cause of death among Americans over the age of 65.
sixth
It is estimated that ____________ Americans die of pneumonia each year.
50,000
What is the leading cause of morbidity and mortality in children beyond the neonatal period?
Pneumonia
Pneumonia often mimics a __________________.
Cold or flu
What is characterized by a patchy pattern of infection that is limited to the segmental bronchi and surrounding lung parenchyma?
Bronchopneumonia
Bronchopneumonia usually involves both lungs and is often seen in which part of the lung?
Lower lobes
_____________ is a widespread of diffuse alveolar inflammation and consolidation confined to one or more lobes of the lungs.
Lobar pneumonia
Interstitial pneumonia is usually a diffuse and often bilateral inflammation that primarily involves ___________________.
The alveolar septa and interstitial spaces
Which type of pneumonia is typically the end result of a severe or long term bronchopneumonia?
Lobar pneumonia
When both lungs are involved, the condition is sometimes called _______________.
“double pneumonia” by laypersons.
What term is often used to describe a mild case of pneumonia?
Walking pneumonia
What are some risk factors for pneumonia?
- Age over 65 years
- AIDS
- Rib fractures
- Cancer
- Viral respiratory infections
- Prolonged bed rest
- Chronic respiratory disease
__________________ refers to a pneumonia acquired from normal social contact.
CAP - Community-acquired pneumonia
What type of pneumonia accounts for more than 80% of all bacterial pneumonias?
Streptococcus pneumoniae pneumonia
Is streptococcus gram-positive or negative?
Positive
What type of S. pneumoniae organism is the most virulent?
Serotype 3
There are more than ___ types of S. pneumoniae.
80
How is streptcocci generally transmitted?
By aerosol from a cough or sneeze of an infected person.
S. pneumoniae and H. influenzae is commonly cultured from the sputum of patients having an acute exacerbation of ___________.
Chronic bronchitis
What are two major groups of staphylococcus?
- staphylococcus aureus, most responsible for “staph” infections in humans
- staphylococcus albus and staphylococcus epidermidis, part of the normal skin flora
______________ are a common cause of hospital-acquired pneumonia or nosocomial pnueumonia.
Staphylococci, becoming increasingly antibiotic resistant
_____________ is a common inhabitant of human pharyngeal flora.
Haemophilus influenzae
Most strains of S. pneumoniae are sensitive to __________________.
Penicillin and its derivatives
A gram-positive, nonmotile coccus that is found singly, in pairs and in irregular clusters
Staphylococcus
Rod-shaped organisms that are the major gram-negative organisms responsible for pneumonia.
Bacilli
A gram-positive, nonmotile bacterium that occurs singly, in pairs, and in short chains.
Streptococcus
How is staphylococcus aureus transmitted?
By air from a cough/sneeze of an infected person and indirectly via contact with contaminated floors, bedding, floors and clothes.
There are 6 types of H. influenzae, designated A to F, but only type ____ is commonly pathogenic.
B
How does H. influenzae appear on gram stain?
Coccobacilli
Is H. influenzae gram-negative or positive?
Negative
Pneumonia caused by H. influenzae is most often seen where?
Children 1 month to 6 years old.
H. influenzae type B is almost always the cause of _________________.
Acute epiglottitis