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Gen Med Unit 6 > Bronchiectasis, Cystic Fibrosis > Flashcards

Bronchiectasis, Cystic Fibrosis Flashcards

1
Q

Define Bronchiesctasis

A

Abnormal and permanent dilation of bronchi and destruction of bronchial walls

Usually due to infectious process

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2
Q

What does bronchiectasis affect?

A

Affects proximal and medium sized bronchi

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3
Q

What is the pathophys of bronchiectasis?

A
  1. Airways become inflamed
    A. Then become edematous with development of scarring, ulceration and neovascularization
    B. Can lead to airway collapse
  2. Airflow is obstructed
  3. Clearance of secretions impaired
  4. Colonization/infection develops
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4
Q

What is the etiology of bronchiectasis?

A
  1. Congenital
    A. Secondary to cystic fibrosis
    -50% of cases
  2. Recurrent infections
  3. Obstruction
    A. Tumor
    B. Foreign body
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5
Q

What etiology makes up 50% of bronchiectasis cases?

A

Congenital

Secondary to cystic fibrosis

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6
Q

What is the most common pathogen asst. with bronchiectasis?

A

Hemophilus influenzae

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7
Q

What other pathogens are involved in bronchiectasis?

A
  1. Hemophilus influenzae
  2. 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)

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8
Q

What are the symptomsof bronchiectasis?

A 
Chronic daily cough
Mucopurulent sputum lasting months/years
Thick, tenacious
Hemoptysis
Dyspnea
Pleuritic chest pain
Weight loss 
Weakness
Little or no tobacco history
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9
Q

What are the signs of bronchiectasis?

A 
Rales
Rhonchi
Wheezing
Clubbing
Cyanosis
May develop right sided heart failure (Cor pulmonale)
Peripheral edema, JVD, hepatomegaly 
Wt loss/wasting
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10
Q

What si the dx study of choice for bronchiectasis?

A

Chest CT

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11
Q

What are the Chest CT results in bronchiectasis?

A 
Dilated and thickened bronchi 
“Tram Tracks”
Thickening of bronchial walls
Increased pulmonary markings
Atelectasis
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12
Q

What are the Chest XR results in bronchiectasis?

A

Honeycombing & atelectasis

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13
Q

What are the bronchoscopy results in bronchiectasis?

A

Evaluate hemoptysis

Remove secretions

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14
Q

What is the treatment for bronchiectasis guided by?

A

Sputum culture results

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15
Q

What is the abx treatment for bronchiectasis?

A
  1. Amoxicillin (beta lactam neg), otherwise:
  2. Amoxicillin clavulanate (Augmentin)
  3. Macrolides
    A. Azithromycin (Zithromax)
    B. Clarithromycin (Biaxin)
  4. Doxycycline
  5. Fluoroquinolone (if no sputum cx)
    A. Levofloxacin (Levaquin), moxifloxacin (Avelox)
    B. Ciprofloxacin (Cipro) if Hx of Pseudomonas
  6. Aerosolized gentamicin or aerosolized tobramycin
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16
Q

When is Aerosolized gentamicin or aerosolized tobramycin used in bronchiectasis?

A

pts wth cystic fibrosis

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17
Q

How is the mucus mobilized and loosened in bronchiectasis?

A

Chest physiotherapy and postural drainage/Vest

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18
Q

What other treatments can be used in bronchiectasis?

A
  1. Inhaled bronchodilators
  2. Mucolytics
    A. Guafenisin (Mucinex)
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19
Q

When and what surgery is indicated in braonchiectasis?

A

In advanced disease, surgery sometimes indicated:
Lung resection
Surgery has little long term benefit

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20
Q

What does a bronchiectasis pt need to be educated about?

A
  1. Avoid tobacco
  2. Avoid second hand smoke exposure
  3. Nutritional support
  4. Immunizations
    A. Influenza
    B. Pneumovax/Prevnar
    C. Pertussis
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21
Q

What is the drug of choice if a bronchiectasis pt has a history of pseudomonas infection?

A

Ciprofloxacin

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22
Q

Define Cystic fibrosis

A

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

23
Q

What are the reproductive effects of cystic fibrosis?

A
  1. ↑ infertility in females due to thick cervical mucus

2. 98% males infertile due to vas deferens development failure

24
Q

What is the pathophys of cystic fibrosis?

A
  1. 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
25
Q

What are the clinical sxs of CF?

A 
Chronic or recurrent cough 
Excessive sputum production
Dyspnea
Wheezing
Decreased exercise tolerance
Clubbing
Inc AP diameter
Steatorrhea  
Diarrhea
Failure to thrive infants
26
Q

What are the common pathogens that cause pneumonia or bronchiectasis in CF pts?

A 
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
27
Q

What are the ABG results in CF?

A

Compensated respiratory acidosis

Hypoxemia

28
Q

What are the PFT results in CF?

A

Mixed obstructive & restrictive pattern

29
Q

What are the CXR results in CF?

A

Hyperinflation, increased interstitial markings

30
Q

What is the CT Chest result in CF?

A

Bronchiectasis

31
Q

What test confirms diagnosis in CF?

A
  1. 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
32
Q

What CF treatment restores the function of mutant CF protein? Who is it approved for?

A
  1. Ivacaftor (Kalydeco) 150 mg po q 12 h

2. > 6 yrs

33
Q

How are resp infections treated outpt in CF? What are the causative agents?

A
  1. Pseudomonas aeruginosa & Staph aureus

A. Azithromycin is only oral med used

34
Q

What is the prophylactic treatment for CF?

A
  1. 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
35
Q

How are lower airways cleared in CF pts?

A
  1. Chest physiotherapy and postural drainage/Vest
  2. Inhaled dornase alpha (Pulmozyme) qd
    A. Hydrolyzes extracellular DNA-thins mucus
36
Q

How are pancreatic enzymes replaced in CF?

A

Pancrelipase (Creon) w/ meals & snacks

37
Q

How is bronchoconstriction treated in CF?

A

Inhaled bronchodilators prn only

38
Q

What is the only definitive treatment for CF?

A

Lung transplantation

Double lung or heart lung transplant is required

39
Q

What is the 3 yr survival rate post transplant?

A

55%

40
Q

What is the prognosis for CF?

A

Longevity of pts with CF is increasing

Median survival is 35 years after diagnosis

41
Q

What is CF mortality asst with?

A 
1. Pulmonary complications
A. Pneumonia
B. Pneumothorax 
C. Hemoptysis
2. Result of chronic resp failure and cor pulmonale
42
Q

What is BOOP?

A
  1. Bronchiolitis Obliterans Organizing Pneumonia (also called Cryptogenic Organizing Pneumonia (COP))
  2. Noninfectious pneumonia
  3. Rare
  4. inflammation of bronchioles and alveoli
43
Q

What is the etiology of BOOP?

A
  1. Idiopathic
  2. Complication of chronic inflammatory diseases
    A. RA
    B. Scleroderma
    C. SLE
  3. Complication of Amiodarone
44
Q

What is the clinical presentation of BOOP?

A
  1. Starts w/flu-like symptoms
  2. Cough persists
  3. DOE
  4. Weight loss
  5. Does not respond to antibiotics
45
Q

What are the sputum culture results in BOOP?

A

Negative

46
Q

What are the CXR results in BOOP?

A
  1. Appears like unilat. or bilat. patchy infiltrates that does not clear in 1 month. Infiltrates may migrate/change
47
Q

What are the CT scan results in BOOP?

A

Bronchograms
Ground glass appearance
Hazy opacities

48
Q

What are the inpt treatment options for infection in CF?

A
  1. Amoxicillin (beta lactam neg), otherwise:
  2. Amoxicillin clavulanate (Augmentin)
  3. Macrolides
    A. Azithromycin (Zithromax)
    B. Clarithromycin (Biaxin)
  4. Doxycycline
  5. Fluoroquinolone (if no sputum cx)
    A. Levofloxacin (Levaquin), moxifloxacin (Avelox)
    B. Ciprofloxacin (Cipro) if Hx of Pseudomonas
  6. Aerosolized gentamicin or aerosolized tobramycin
49
Q

What are the bronchoscopy results in BOOP?

A
  1. Lavage-40% lymphocytes
  2. Bx- “Masson Bodies”
    - Polypoid plugs of loose connective tissue
50
Q

What immunizations are indicated in BOOP?

A

Flu vaccine

Pneumovax/Prevnar

51
Q

What other treatments indicated in BOOP?

A
  1. Long term steroids to treat the inflammation
    A. Wean over 6-12 months, taper very slowly
  2. Immunosuppressant Tx
    A. Cyclophosphamide
52
Q

What is the dx study order in BOOP?

A
  1. CXR
  2. CT scan
  3. Bronchoscopy
53
Q

What is dx in BOOP?

A

Bx- “Masson Bodies”

Polypoid plugs of loose connective tissue

Gen Med Unit 6 (14 decks)

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