Bronchiectasis and CF Flashcards

1
Q

Most common cause of Bronchiectasis? 2nd most common?

A

1st= Cystic fibrosis- ~50%

2nd= Haemophilus influenza

(others= copd, primary infection, congenital defects, autoimmune)

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2
Q

What is bronchiectasis?

A

Abnormal dilation of proximal and medium-large sized bronchi (>2mm in diameter)

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3
Q

Is bronchiectasis mostly congenital or acquired?

A

can be congenital but most often acquired

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4
Q

Describe what happens in the development of bronchiectasis

A

infection-> impaired drainage-> airway obstruction

  • muscular and elastic components of the bronchial wall and often peribronchial alveolar tissue damaged from the inflammatory process
  • overall result= retained secretions colonized w/ pathogenic organisms resulting in copious amounts of purulent sputum
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5
Q

Sx of bronchiectasis

A
  • cough and daily mucopurulent sputum lasting months to years
  • blood-streaked or hemoptysis due to airway damage
  • pleuritic chest pain
  • dyspnea
  • wheezing
  • weight loss
  • fever
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6
Q

On PE, what would you find in a pt with bronchiectasis?

A

-crackles/rhonchi w/ infection (~75% pts)

  • may have scattered wheezing
  • clubbing (~2-3%)
  • rarely: cyanosis, wasting
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7
Q

On sputum anaysis, what finding is specific to Bronchiectasis?

A

Dittrich plugs (small white/yellow concentrations)

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8
Q

What organisms on gram stain would be suggestive of bronchiectasis? (aka what organisms are involved?)

A

Pseudomonas

E. coli

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9
Q

What is ordered for all pts with possible bronchiectasis? What might it show?

A

Plain CXR (PA/lateral)

increased pulmonary markings, ring-like structures, atelectasis, dilated and thickened airways (tram lines) and mucus plugging
(CXR might be nl)

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10
Q

What is gold standard imaging for dx of bronchiectasis?

A

High resolution CT (HRCT)

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11
Q

What genetic condition might result in bronchiectasis? (deficiency)

A

Alpha1-antitrypsin

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12
Q

What kind of Spirometry pattern would a pt with bronchiectasis show?

A

Obstructive!

  • reduced or nl forced FVC
  • low FEV1
  • low FEV1/FVC
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13
Q

What is the mainstay of tx of bronchiectasis?

A

Antibiotics

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14
Q

Outpatient abx options for bronchiectasis?

Severe illness requiring hospitalization?

A

Outpatient:

  • amoxicillin
  • tertacycline
  • trimethoprim
  • sulfamethoxazole
  • macrolide
  • 2nd gen cephalosporin
  • fluoroquinolone

Severe:
-IV abx

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15
Q

What is very important to treatment of bronchiectasis? How is it done?

A

Bronchial hygiene
-postural drainage w/ percussion and vibration loosens and mobilizes secretions

  • devices: flutter device, intrapulmonic percussive ventilation device
  • “vest” system
  • nebulizers w/ concentrated (7%) sodium chloride solution
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16
Q

In pts with severe bronchiectasis, what are management options?

A
  • lung resection

- transplant

17
Q

What is Cystic fibrosis?

A
  • autosomal recessive genetic disorder

- dz of EXOCRINE gland fx

18
Q

CF is the most fatal hereditary disorder in what population?

A

Caucasians in the U.S.

19
Q

What are the 2 ways that CF can present (vary with age of pt)?

A
  • pulmonary infections (90%)

- pancreatic enzyme insufficiency

20
Q

What is the defect in CF? What does it result in?

A

defect in the CFTR gene which results in abnormal chloride transport channels

21
Q

Explain what happens in CF

A

Reduced Cl- transport across the epithelial cells of mucus membranes

  • Less H2O in respiratory and pancreatic secretions
  • results in thick, viscous mucus secretions in the respiratory tract, pancreas, GI tract -> plugging

Chronic lung infection occur due to bacterial colonization

22
Q

What is the primary cause of death in CF patients?

A

end-stage lung dz

23
Q

What are problems see in pts with CF? (think organs it affects)

A
  • Chronic hacking cough (recurrent bronchiolitis in infants, possible post-tussive emesis)
  • GI manifestations (meconium ileus, obstruction, malabsorption of fats and proteins)
  • Male reproductive tract (infertility, undescended testes)
  • Sinus infection
24
Q

BOTH criteria must be met to dx CF, what are they?

A

Clinical sx c/w CF in at least 1 organ system

AND

Evidence of (CFTR) dysfunction (any of the following):

  • elevated sweat chloride
  • genotyping
  • abnormal nasal potential different-this one is rare)
25
Q

What is the primary test for the diagnosis of CF?

A

Sweat chloride test

26
Q

What do you need to vaccinate pts with CF for?

A

influenza and PNA

27
Q

What are some ways to manage CF? (sorry long list)

A
  • Bronchodilators (B-agonists and anticholinergics)
  • agents to promote clearance of secretions
  • chest physiotherapy
  • antiinflammtory therapy (macrocodes, ibuprofen, corticosteroids)
  • Negative partial pressure ventilation
  • nutritional support
  • transplant