Bronchiectasis, Cystic Fibrosis Flashcards
Define Bronchiesctasis
Abnormal and permanent dilation of bronchi and destruction of bronchial walls
Usually due to infectious process
What does bronchiectasis affect?
Affects proximal and medium sized bronchi
What is the pathophys of bronchiectasis?
- Airways become inflamed
A. Then become edematous with development of scarring, ulceration and neovascularization
B. Can lead to airway collapse - Airflow is obstructed
- Clearance of secretions impaired
- Colonization/infection develops
What is the etiology of bronchiectasis?
- Congenital
A. Secondary to cystic fibrosis
-50% of cases - Recurrent infections
- Obstruction
A. Tumor
B. Foreign body
What etiology makes up 50% of bronchiectasis cases?
Congenital
Secondary to cystic fibrosis
What is the most common pathogen asst. with bronchiectasis?
Hemophilus influenzae
What other pathogens are involved in bronchiectasis?
- Hemophilus influenzae
- Pseudomonas aeruginosa
A. Accelerated course
B. Associated with more rapid decline in pulmonary function and more frequent exacerbations
Moraxella catarrhalis
Staphylococcus aureus
Streptococcus pneumoniae (uncommon)
What are the symptomsof bronchiectasis?
Chronic daily cough Mucopurulent sputum lasting months/years Thick, tenacious Hemoptysis Dyspnea Pleuritic chest pain Weight loss Weakness Little or no tobacco history
What are the signs of bronchiectasis?
Rales Rhonchi Wheezing Clubbing Cyanosis May develop right sided heart failure (Cor pulmonale) Peripheral edema, JVD, hepatomegaly Wt loss/wasting
What si the dx study of choice for bronchiectasis?
Chest CT
What are the Chest CT results in bronchiectasis?
Dilated and thickened bronchi “Tram Tracks” Thickening of bronchial walls Increased pulmonary markings Atelectasis
What are the Chest XR results in bronchiectasis?
Honeycombing & atelectasis
What are the bronchoscopy results in bronchiectasis?
Evaluate hemoptysis
Remove secretions
What is the treatment for bronchiectasis guided by?
Sputum culture results
What is the abx treatment for bronchiectasis?
- Amoxicillin (beta lactam neg), otherwise:
- Amoxicillin clavulanate (Augmentin)
- Macrolides
A. Azithromycin (Zithromax)
B. Clarithromycin (Biaxin) - Doxycycline
- Fluoroquinolone (if no sputum cx)
A. Levofloxacin (Levaquin), moxifloxacin (Avelox)
B. Ciprofloxacin (Cipro) if Hx of Pseudomonas - Aerosolized gentamicin or aerosolized tobramycin
When is Aerosolized gentamicin or aerosolized tobramycin used in bronchiectasis?
pts wth cystic fibrosis
How is the mucus mobilized and loosened in bronchiectasis?
Chest physiotherapy and postural drainage/Vest
What other treatments can be used in bronchiectasis?
- Inhaled bronchodilators
- Mucolytics
A. Guafenisin (Mucinex)
When and what surgery is indicated in braonchiectasis?
In advanced disease, surgery sometimes indicated:
Lung resection
Surgery has little long term benefit
What does a bronchiectasis pt need to be educated about?
- Avoid tobacco
- Avoid second hand smoke exposure
- Nutritional support
- Immunizations
A. Influenza
B. Pneumovax/Prevnar
C. Pertussis
What is the drug of choice if a bronchiectasis pt has a history of pseudomonas infection?
Ciprofloxacin
Define Cystic fibrosis
Autosomal recessive disorder that results in abnormal production of mucus by almost all exocrine glands, causing obstruction of those glands/ducts
median survival rate 35 years
What are the reproductive effects of cystic fibrosis?
- ↑ infertility in females due to thick cervical mucus
2. 98% males infertile due to vas deferens development failure
What is the pathophys of cystic fibrosis?
- Defect in cystic fibrosis trans-membrane regulator gene
A. Regulates apical/luminal chloride channels
B. Profound effect on ion and fluid transport
-Lungs
-Pancreas
C. Critical for chloride to be transported into lumen of conducting airways & pancreatic ducts, drawing Na & H2O
D. ↓ chloride results in thickened secretions
What are the clinical sxs of CF?
Chronic or recurrent cough Excessive sputum production Dyspnea Wheezing Decreased exercise tolerance Clubbing Inc AP diameter Steatorrhea Diarrhea Failure to thrive infants
What are the common pathogens that cause pneumonia or bronchiectasis in CF pts?
1. Pseudomonas aeruginosa A. Most common in adults 2. Staph aureus A. Most common in children B. Increasing in adults C. ↑ MRSA 3. Hemophilus influenzae A. Children > adults
What are the ABG results in CF?
Compensated respiratory acidosis
Hypoxemia
What are the PFT results in CF?
Mixed obstructive & restrictive pattern
What are the CXR results in CF?
Hyperinflation, increased interstitial markings
What is the CT Chest result in CF?
Bronchiectasis
What test confirms diagnosis in CF?
- Sweat Chloride Test: a mild electrical current pushes medicine into skin to cause sweating, sweat is collected and salt content is measured
A. Positive test on 2 occasions confirms diagnosis
B. ≥ 60 mmol/L = CF
What CF treatment restores the function of mutant CF protein? Who is it approved for?
- Ivacaftor (Kalydeco) 150 mg po q 12 h
2. > 6 yrs
How are resp infections treated outpt in CF? What are the causative agents?
- Pseudomonas aeruginosa & Staph aureus
A. Azithromycin is only oral med used
What is the prophylactic treatment for CF?
- Prophylaxis via nebulizer
A. Inhaled tobramycin (Tobi) 300 mg bid x 28 d, then off x 28 d
B. Inhaled aztreonam (Cayston) 75 mg tid x 28 d, then off x 28 d
How are lower airways cleared in CF pts?
- Chest physiotherapy and postural drainage/Vest
- Inhaled dornase alpha (Pulmozyme) qd
A. Hydrolyzes extracellular DNA-thins mucus
How are pancreatic enzymes replaced in CF?
Pancrelipase (Creon) w/ meals & snacks
How is bronchoconstriction treated in CF?
Inhaled bronchodilators prn only
What is the only definitive treatment for CF?
Lung transplantation
Double lung or heart lung transplant is required
What is the 3 yr survival rate post transplant?
55%
What is the prognosis for CF?
Longevity of pts with CF is increasing
Median survival is 35 years after diagnosis
What is CF mortality asst with?
1. Pulmonary complications A. Pneumonia B. Pneumothorax C. Hemoptysis 2. Result of chronic resp failure and cor pulmonale
What is BOOP?
- Bronchiolitis Obliterans Organizing Pneumonia (also called Cryptogenic Organizing Pneumonia (COP))
- Noninfectious pneumonia
- Rare
- inflammation of bronchioles and alveoli
What is the etiology of BOOP?
- Idiopathic
- Complication of chronic inflammatory diseases
A. RA
B. Scleroderma
C. SLE - Complication of Amiodarone
What is the clinical presentation of BOOP?
- Starts w/flu-like symptoms
- Cough persists
- DOE
- Weight loss
- Does not respond to antibiotics
What are the sputum culture results in BOOP?
Negative
What are the CXR results in BOOP?
- Appears like unilat. or bilat. patchy infiltrates that does not clear in 1 month. Infiltrates may migrate/change
What are the CT scan results in BOOP?
Bronchograms
Ground glass appearance
Hazy opacities
What are the inpt treatment options for infection in CF?
- Amoxicillin (beta lactam neg), otherwise:
- Amoxicillin clavulanate (Augmentin)
- Macrolides
A. Azithromycin (Zithromax)
B. Clarithromycin (Biaxin) - Doxycycline
- Fluoroquinolone (if no sputum cx)
A. Levofloxacin (Levaquin), moxifloxacin (Avelox)
B. Ciprofloxacin (Cipro) if Hx of Pseudomonas - Aerosolized gentamicin or aerosolized tobramycin
What are the bronchoscopy results in BOOP?
- Lavage-40% lymphocytes
- Bx- “Masson Bodies”
- Polypoid plugs of loose connective tissue
What immunizations are indicated in BOOP?
Flu vaccine
Pneumovax/Prevnar
What other treatments indicated in BOOP?
- Long term steroids to treat the inflammation
A. Wean over 6-12 months, taper very slowly - Immunosuppressant Tx
A. Cyclophosphamide
What is the dx study order in BOOP?
- CXR
- CT scan
- Bronchoscopy
What is dx in BOOP?
Bx- “Masson Bodies”
Polypoid plugs of loose connective tissue
