Bronchiectasis

Question 1

Bronchiectasis definition

Answer 1

• Chronic dilation of bronchi, characterized by obstruction and inflammation
• Can be localized or diffuse, if diffuse think of multi organ disease

Question 2

Localized vs diffuse bronchiectasis

Answer 2

• Localized is often an acquired problem, due to environmental/occupational exposures
• Diffuse is usually multi organ disease caused by congenital abnormality
• One ex of diffuse: primary ciliary dyskinesia (immotile cilia, AKA Kartagener’s syndrome)

Question 3

Pathophysiology of bronchiectasis

Answer 3

• Can be due to one of the following
• Infectious insult: bacteria, viral, fungal
• Impairment of drainage (obstruction): aspiration, neoplasm, COPD, hilar adenopathy
• Host defense impaired: IgG, IgA deficiency
• Or can be hereditary: Ciliary defects, A1AT, CF

Question 4

Signs and Sx of bronchiectasis

Answer 4

• Cough, sputum daily, dyspnea, hemoptysis, recurrent pleurisy
• Crackles, rhonchi, wheezing, clubbing

Question 5

CF and bronchiectasis

Answer 5

• CF causes a diffuse bronchiectasis
• It is often associated w/ mucoid pseudomonas aeruginosa infections (recurrent)
• CF Dx (one of these): sweat chloride ≥60 w/ clinical Sx, high respiratory epithelial transmembrane potential w/ clinical Sx, abnormal CF gene genotyping
• Delta F508 mutation most common form of CF

Question 6

Presentation of CF

Answer 6

• CFPANCREAS
• Chronic respiratory disease
• Failure to thrive
• Polyps
• Alkalosis (met)
• Neonatal intestinal obstruction
• Clubbing
• Rectal prolapse
• Electrolytes elevated in sweat
• Aspermia (absent vas deferens)
• Sputum discolored (S aureus/P aeruginosa)

Question 7

Pathophysiology in CF

Answer 7

• Since there are no or few functioning Cl channels at the apical membrane, there is decreased Cl in bronchial secretions
• This leads to low water content and thus thickened mucus in airways (susceptible to infection b/c cilia don’t work properly)
• Once infection happens there is a cycle of infection causing inflammation/damage which promotes more infection
• Infective agents: H influenza, S aureus, P aeruginosa
• The process of inflammation causes release of elastases and proteases, which cause destruction of bronchi and lead to bronchiectasis

Question 8

Rx of CF

Answer 8

• Bronchodilators to open airway

- Loosen the mucous mechanically (chest vests) and medically (DNAse, N acetyl cystine)

Question 9

Signs and Sx of pulm exacerbation in CF

Answer 9

• Increased cough, sputum, weight, dyspnea, school/workf loss
• New chest findings (rales, wheezing), decreased exercise tolerance, decreased FEV (down 10%)
• New radiographic findings

Question 10

Malabsorption in CD

Answer 10

• Second most frequently involved organ is pancreas

- Need to watch diet and put on oral digestive nzs replacement Rx