Bronchiectasis and CF Flashcards
Most common cause of Bronchiectasis? 2nd most common?
1st= Cystic fibrosis- ~50%
2nd= Haemophilus influenza
(others= copd, primary infection, congenital defects, autoimmune)
What is bronchiectasis?
Abnormal dilation of proximal and medium-large sized bronchi (>2mm in diameter)
Is bronchiectasis mostly congenital or acquired?
can be congenital but most often acquired
Describe what happens in the development of bronchiectasis
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
Sx of bronchiectasis
- 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
On PE, what would you find in a pt with bronchiectasis?
-crackles/rhonchi w/ infection (~75% pts)
- may have scattered wheezing
- clubbing (~2-3%)
- rarely: cyanosis, wasting
On sputum anaysis, what finding is specific to Bronchiectasis?
Dittrich plugs (small white/yellow concentrations)
What organisms on gram stain would be suggestive of bronchiectasis? (aka what organisms are involved?)
Pseudomonas
E. coli
What is ordered for all pts with possible bronchiectasis? What might it show?
Plain CXR (PA/lateral)
increased pulmonary markings, ring-like structures, atelectasis, dilated and thickened airways (tram lines) and mucus plugging
(CXR might be nl)
What is gold standard imaging for dx of bronchiectasis?
High resolution CT (HRCT)
What genetic condition might result in bronchiectasis? (deficiency)
Alpha1-antitrypsin
What kind of Spirometry pattern would a pt with bronchiectasis show?
Obstructive!
- reduced or nl forced FVC
- low FEV1
- low FEV1/FVC
What is the mainstay of tx of bronchiectasis?
Antibiotics
Outpatient abx options for bronchiectasis?
Severe illness requiring hospitalization?
Outpatient:
- amoxicillin
- tertacycline
- trimethoprim
- sulfamethoxazole
- macrolide
- 2nd gen cephalosporin
- fluoroquinolone
Severe:
-IV abx
What is very important to treatment of bronchiectasis? How is it done?
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
In pts with severe bronchiectasis, what are management options?
- lung resection
- transplant
What is Cystic fibrosis?
- autosomal recessive genetic disorder
- dz of EXOCRINE gland fx
CF is the most fatal hereditary disorder in what population?
Caucasians in the U.S.
What are the 2 ways that CF can present (vary with age of pt)?
- pulmonary infections (90%)
- pancreatic enzyme insufficiency
What is the defect in CF? What does it result in?
defect in the CFTR gene which results in abnormal chloride transport channels
Explain what happens in CF
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
What is the primary cause of death in CF patients?
end-stage lung dz
What are problems see in pts with CF? (think organs it affects)
- 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
BOTH criteria must be met to dx CF, what are they?
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)
What is the primary test for the diagnosis of CF?
Sweat chloride test
What do you need to vaccinate pts with CF for?
influenza and PNA
What are some ways to manage CF? (sorry long list)
- 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
