Chap 14 and 15- bronchiectasis/ CF Flashcards
Bronchiectasis is characterized by
chronic dilation and distortion of one or more bronchi- usually as a result of extensive inflammation and destruction of the bronchial wall cartilage, blood vessels, elastic tissue, and smooth muscle components
One or more lungs may be involved, but commonly
limited to a lobe or segment and is frequently found in the lower lobes. the smaller bronchi, with less supporting cartilage, are predominantly affected
Because of bronchial wall destruction,
normal mucocillary clearance is impaired. This result in the accumulation of copious amounts of bronchial secretions and blood that often become foul smelling because of secondary colonization with anaerobic organisms.
Condition leads to one or both of the following anatomic alterations
- hyperinflation of the distal alveoli as a result of expiratory check valve obstruction or 2. atelectasis, consolidation, and fibrosis as a result of complete bronchial obstruction
3 types of bronchiectasis
varicose (fusiform)
Cylindrical (tubular)
Cystic (saccular)
Varicose (fusiform) bronchiectasis
bronchi are dilated and constricted in an irregular fashion similar to varicose veins, ultimately resulting in a disorted, bulbous shape
Cylindrical (tubular) bronchiectasis
bronchi are dilated and rigid and have regular outlines similar to a tube. Dilated bronchi fail to taper for 6 to 10 generations and then appear to end abruptly because of mucous obstruction
Cystic (saccular) bronchiectasis
bronchi progressively increase in diameter until they end in large, cystlike sacs in the lung parenchyma. This type causes the greatest damage to the tracheobronchial tree.
-Bronchial walls become composed of fibrous tissue alone-cartilage, elastic tissue, and smooth muscle are all absent
Major pathologic or structural changes associated with bronchiectasis
- Chronic dilation and disortion of bronchial airways
- excessive production of often foul-smelling sputum
- bronchospasm
- hyperinflation of alveoli (air trapping)
- Atelecctasis
- Consolidation and parenchyma fibrosis
- Hemoptysis secondary to bronchial arterial erosion
Most causes of bronchiectasis include some combination of
Bronchial obstruction and infection. In dev. countries, CF is the most common cause of bronchiectasis
NCFB
Noncystic fibrosis bronchiectasis
-most common cause is pulmonary infection
significant lung infection during early childhood
causes anatomic alterations of the developing lung that allows persistent bacterial infections as a result, the continuous bacterial infections lead to bronchiectasis
-Individuals with NCFB have mucociliary disorder
Mucociliary disorder- Promary ciliary dyskinesia
Immunodeficiency disorder involving low levels of immunoglobulin F, IgM, and IgA
Causes of bronchiectasis are commonly classified into the following categories
- Acquired bronchial obstruction
- Congenital anatomic defects
- Immunodeficiency states
- Abnormal secretion clearance
- Miscellaneous disorders
Diagnosis
- Routine chest radiograph
- Bronchograms
- CT scan- computed tomography scan!!
- Spirometry testing (obstructive or restrictive)
- ABG (mild, moderate, or severe)
Abnormal secretion clearance diseases with bronchiectasis
- Ciliary defects of airway mucosa- Kartageners syndrom
- CF (mucoviscidosis)
- Youngs syndrome
Major anatomic alterations of the lungs associated with bronchiectasis
Bronchospasm, consolidation, atelectasis, Increased alveolar-capillary membrane thickness
If the majority of the bronchial airways are only partially obstructed
obstructive lung disorder
the majority of the bronchial airways are completely obstructive
The distal alveoli collapse, atelectasis results, and the bronchiectasis manifests primarily as a restrictive disorder.
Vitals of bronchiectasis
- Tachypnea
- hypoxemia
- decreased lung compliance and increased ventilatory rate
- anxiety
- increased HR and BP
- Use of accessory muscles during insp/exp
- Pursed lip breathing
- Barrel chest (obstructive)
- Cyanosis
- Digital clubbing
Hallmark of bronchietasis
large quantities of foul smelling sputum
The stagnant secretions in bronchiectasis stimulate the
Subepithelial mechanoreceptors which in turn produce a vagal reflex that triggrs the cough. Found in the trachea, bronchi, and bronchioles, but they are predominantly located in the upper airways
Chest assessment in bronchiectasis in primarily obstructive
- Decreased tactile and vocal fremitus
- hyperresonant percussion note
- diminished breath sounds
- wheezing
- Crackles
Chest assesment in bronchieactasis in primarily restrictive
- Increased tactile and vocal fremitus
- bronchial breath sounds
- crackles
- whispered pectoriloquy
- dull percussion note
Sputum culture results and sensitivity
Streptococcus pneumoniae
Haemophilus influenzae
Pseudomonas aeruginosa
Anaerobic organisms
Chest radiograph in obstructive nature
- Translucent lung fields
- depressed or flattened diaphragms
- long and narrow heart
- enlarged heart
- tram tracks
- areas of consolidation and or atelectasis may or may not be seen
Tram-track opacities
may be seen in cylindrical bronchiectasis. They are parallel, or curved opacity lines of varying length caused by bronchial wall thickening
Chest radiograph in restrictive nature
- Atelectasis and consolidation
- Infiltrates (suggesting pneumonia)
- Increased opacity
Bronchography
- the injection of an opaque contrast material into the tracheobronchial tree
- Was routinely performed on pts with bronchiectasis
Computed tomography (CT scan)
with this technique increased bronchial wall opacity is often seen. The bronchial walls may appear
- thick
- dilated
- charac. by ring lines or clusters
- signet ring shaped
- flame shaped
- Specific type of bronchiectasis can be confirmed with the CT scan, as well as other things
Lung Mapping
CT scan has the ability to map out and determine precisely where chest physical therapy would be delivered, or exactly where surgical resection of lung should be performed
General treatment plan for bronchiectasis
controlling pulmonary infections, airway secretions, and airway obstruction and preventing complications.
- Daily chest percussion, postural drianage, and effective coughing exercises to remove secretions.
- Antiobiotics, bronchodilators, and expectorants
Oxygen therapy is used to
treat hypoxemia, decrease work of breathing, and decrease myocardial work.
- Hypoxemia that develops in bronchiectasis is usually caused by pulmonary shunting associated with the disorder.
- May not respond well to true shunting
Initially in CF
pt has bronchial gland hypertrophy and metaplasia of goblet cells. Leads to excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree secondary to inadequate hydration of the perciliary fluid layer (sol layer)
Partial obstruction in CF leads to
overdistention of the alveoli, and complete obstruction leads to patchy areas of atelectasis and , in some cases, bronchiectasis.
CF may develop signs and symptoms of
recurrent pneumonia, chronic bronchitis, bronchiectasis, and lung abscesses
Pathologic or structural changes associated with CF are
- Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree secondary to inadequate hydration of the periciliary fluid layer
- Mucous plugging (total or partial)
- Hyperinflation of the alveoli
- Atelectasis
- Bronchiectasis
CF is the most common fatal inherited isorder in childhood. CF is an
autosomal recessive gene disorder caused by mutation in a pair of genes located on chromosome 7
CFTR
Cystic fibrosis transmembrane conductance regulator
The abnormal expression of the CFTR results in
abnormal transport of sodium and chloride ions across many of epithelial surfaces, including those lining the bronchial airways, intestines, pancreas, liver ducts, and sweat glands.
-Result thick, viscous mucus accumulates in the lungs, and mucus blocks the passageways of the pancreas, preventing enzymes from the pancreas from reaching the intestines
CFTR inhibits
the digestion of protein and fat, which in turn leads to deficiencies of fat-soluble vitamins A,D,E and K
Meconium ileus
infants with CF sometimes are born with a blockage of the intestine
Most men with CF are
infertile (azospermic) as a result of a missing or an underdeveloped vas deferens. Fertility is decreased in women with CF secondary to thick cervical mucus
Because CF is a recessive gene disorder,
the child must inherit two copis of the defective CF gene-one from each parent (carriers) to have the disease
Both parents carrying the CF gene- Standard Mendelian pattern
-there is a 25% chance that each child will have a CF, a 25% chance that each child will be completely normal (and not carry the gene), and a 50% chance that each child will be a carrier
There is a what chance that the child will have CF if both patients carry a CF gene mutation
1/4
Diagnosis of CF is based on
newborn screening, clinical manifestations associated with CF, family history of CF, and laboratory findings
Two following criteria must be met to diagnose CF
- Clinical symptoms consistent with CF in at least one organ system
- Clinical evidence of CCF transmembrane conductance regulator (CFTR) dysfunction
CFTR dysfuntion any of the following
- Elevated sweat chloride >60mEq/L
- Molecular diagnosis (genetic testing)
- abnormal nasal potential difference
Most infants with CF have an
elevated blood level of immunoreactive trypsin, which can be measured by radioimmunoassay or by an enzyme linked immunoassay
Immunoreactive trypsin level (IRT)
is measured from the blood dots collected on all newborn infants on the buthrie cards
-Detection of at least one CF mutation is considered a positive screen and is referred to a CF center in most states for further testing
The diagnosis of CF is established by a
positive sweat test and or genetic analysis for CF mutations
Sweat test/ sweat chloride test
Gold standard diagnostic test for CF
- test measures the amount of sodium and choride in the pts sweat.
- Small colorless, odorless, sweat-producing chemical called pilocarpine is applied to pts arm/leg and an electrode is attached to the chemically prepared area.
- test is usually done 2
CF glands secrete of to
4 times the normal amount of sodium and chloride. The actual volume however is no greater than that produced by normal indiv.
Genetic test-genotype test, gene mutation test, or mutation analyze
analyze deoxyribonucleic acid (DNA) for the presence of CFTR gene mutation
Nasal Potential difference
As the Na and Cl ions move across the epithelial cell membrane they generate what is called an electrical potential difference- the amount of NRG required to move an electrical charge from one point to another
-an increase NPD strongly suggests CF
Amniocentesis
Fetus test after first trimester
Stool fecal fat tests
measures the amount of fat in the infants stool and the percentage of dietary fat that is absorbed by the body.
-An elevated stool fecal fat value is associated with CF (more than 7g of fat per 24 hours)
Fecal elastance
is an easier test of pancreatic function as it only requires a small stool sample for analysis (less than 50ug/g of stool)
Major anatomic alterations of the lungs associated with CF
Atelectaiss, bronchospasm, excessive bronchial secretions
Sputum Examination in CF
Increased WBC Gram positive bacteria -Staphylococcus aureus -Haemophilus influenzae Gram negative bacteria -Pseudomonas Aeruginosa -Stenotrophomonas Maltophilia -Burkholderia Cepacia complex
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.
- Fail to pass meconium within 12 hours after birth.
- Equivalent to intestinal obstruction
Pacreatic ducts in CF
become plugged with mucus, which leads to fibrosis of the pacrease, leads to deficiency of vitamins A, D, E and K.
Vitamin K deficiency may be cause of
easy bruising and bleeding
-80% CF have vitamin deficiencies
Nasal Polyps
may cause nasal obstruction. and may cause disortion of normal facial features
CF treatment protocols
- Bronchopulmonary hygiiene therapy
- Lung expansion therapy (deep breathing)
- Bronchodilators and mucolytic agents
- Mechanical ventilation protocol
- Antibiotics
- Ibuprofen
E.anti-leukotriene
Accolate
Singular
Zyflo
F.Mucolytic/ Expectorant
Pulmozyme
Muconex
Mucomyst
Water
BLong Acting Bronchodilator
Spiriva(anticholinergic, 1st drug, lasts 24hrs)
Serevent (lasts 12 hours)
Advair (corticosteroid, maintenance drug lasts lunger than albuterol)
C.Corticosteroid
Flovent (Steroid component of advair) Advair (long acting, maintenance drug lasts long albut) Prednisone (p.o) Azmacort Solumedrol (IV route
D.Mast cell stabilizer
Intal (cromolyn) -aerosol neb
A. short acting bronchodilator
Atrovent (anticholinergic-atropine, dry mouth)
Duoneb (albuterol/atrovent)
Xopenex (albuterol with less cardiac effect)
Albuterol (beta alergenic 2.5mg .83%)
When two carriers of cystic fibrosis produce children there is a
25% chance that the boby will be completely normal
50% chance carrier
25% affected
When one parent is carrier/ one CF
50% affected
50% unaffected carrier
When one parent is not a carrier/ one CF
100% carrier
when one parent is not a carrier/ one is a carrier
50% unaffected
50% carrier
In cystic fibrosis the patient commonly demonstrates which of the following
- Increased FEVT
- Decreased MVV
- Increased RV
- Decreased FEV1/FVC ratio
2,3,4
During the advance stages of cystic fibrosis , the pt generally demonstrates which of the following
Bronchial breath sounds, diminished breath sounds, hyperresonant percussion notes
Clinical manifestations are associated with severe cystic fibrosis
Increased central venous pressure
Decreased breath sounds
Increased pulmonary vascular resistance
First drug of choice used for maintenance of asthma is
Inhaled steroids
oral form of corticosteroids
Prednisone
what biomarker would indicate the presence of airway inflammation in a patient with asthma
FeNO concentration
pt exercise induced asthma and nonsymptomatic, PFT?
Volumes and flows are in their predicted normal range
maintenance therapy drug
ICS inhaled corticosteroid
Learning about how to gain control of pt astha step. initiate therapy?
Step 2
Kirschman spirals are casts of
terminal and smallest airways
Which of the following forms of bronchiectasis are the bronchi dilated and constricted in an irregular fashion
Fusiform and Cylindrical
Common causes of acquired bronchiectasis
Kartageners syndrom and CF
Primarily obstructive form of bronch the pt commonly demonstrates
Decreased PEFR
