Week 2: Bronchiectasis and Cystic Fibrosis

Question 1

Definition of bronchiectasis

Answer 1

-abnormal dilatation of the bronchi

Question 2

Manifestation of bronchiectasis

Answer 2

• inflamed and collapsible airways
• airway obstruction of airflow demonstrated by spirometry
• chronic daily cough
• viscous sputum production
• radiological: dilated, tortured airways, tramtrack appearance

Question 3

COPD vs bronchietasis: gross appearance

Answer 3

• COPD: outpouchings of dilated and thickened airways

- bronchiectasis: parenchyma appear normal, dilated and torturous airways

Question 4

Kartagener’s syndrome

Answer 4

• primary ciliary dyskinesia (immotile cilia syndrome)

- bronchiectasis, sinusitis, male infertility or female decreased fertility, and situs inversus

Question 5

Radiological findings in bronchiectasis

Answer 5

• parallel line shadows “tramlines”
• regional distribution of increased, thickened linear densities to cylindrical or saccular cavities
• Axial CT scan: signet ring sign due to dilated bronchus

Question 6

Pathophysiology of bronchiectasis

Answer 6

1. Infectious insult
   - childhood infections: pertussis, measles
   - bacterial, viral, fungal, infections
2. impairment of drainage
   - bronchial obstruction: foreign body, neoplasm
   - hilar adenopathy
   - COPD
   - mucoid impaction: Lady windermere syndrome-MAI infection
3. host defense: immunodeficiency states
   - IgG, IgA, deficiency
   - Leukocyte dysfunciton
4. hereditary
   - Tracheobronchial: e.g. bronchomalacia
   - vascular: pulm sequestration
   - ciliary defects: kartageners
   - a1 antitrypsin
   - CF

Question 7

Bronchiectasis symptoms and signs

Answer 7

SYMPTOMS
Cough
daily sputum
dyspnea
hemoptysis
recurrent pleurisy
SIGNS
crackles
rhonchi
wheezing
digital clubbing

Question 8

MAIC

Answer 8

mycobacterium avium intracellalure complex
-causes a diffuse bronchiectasis in older patients without a prior history of lung idsease or known risk factors for developing lung disease

Question 9

Presentation of CF

Answer 9

CF PANCREAS
chronic respiratory disease
failure to thrive
polyps
alkalosis, metabolic
neonatal intestinal obstruction
clubbing of fingers
rectal prolapse
electrolyte increased in sweat
aspermia/absent vas deferens
sputum-s. aureus/P.aeruginosa