1 Permanent DILATION of the bronchi and bronchioles
causing repeated episodes of airway infection and
inflammation << Due to destruction of cartilage and
elastic tissue by chronic necrotizing infections
2.1 Cystic fibrosis
2.1.1 Autosomal recessive disease
126.96.36.199 (1) Most common mutation is a three-nucleotide
deletion on chromosome 7 that normally codes
for phenylalanine (70% of cases).
188.8.131.52 (2) Mutation causes defective protein folding in the
cystic fibrosis transmembrane conductance
184.108.40.206 (3) Defective CFTR is degraded in the Golgi apparatus.
220.127.116.11 (4) Loss of CFTR causes decreased Cl– reabsorption in
the sweat glands
18.104.22.168 Effect of loss of CFTR in
22.214.171.124.1 (a) Increased Na+ and water reabsorption from
126.96.36.199.2 (b) Decreased Cl– secretion out of epithelial cells
into luminal secretions
188.8.131.52.3 (c) Net effect of these electrolyte alterations is
dehydration of body secretions due to lack of NaCl.
2.2.1 (a) TB is the most common cause worldwide.
2.2.2 (b) Mycobacterium avium-intracellulare (typically involves the
right middle lobe and lingula), adenovirus, Staphylococcus
aureus, Haemophilus influenzae
2.3 Bronchial Obstruction
2.4 Primary ciliary dyskinesia
2.4.1 (a) Dynein arm in cilia is absent.
2.4.2 (b) Dynein arm contains ATPase (adenosine
triphosphatase) for movement of the cilia.
2.5 Allergic bronchopulmonary aspergillosis
3 Gross Findings
3.1 a. Most commonly occurs in the lower lobes
3.2 b. Dilated bronchi and bronchioles are filled
3.2.1 (1) Dilated airways extend to the lung
3.2.2 (2) Dilations are tube-like
4.1 a. Cough productive of copious sputum (often cupfuls)
4.2 b. Hemoptysis that is sometimes massive
4.3 c. Digital clubbing
4.4 d. Cor pulmonale
4.5 Chest cardiograph & CT scan Findings
4.5.1 Crowded bronchial markings extend to the lung periphery