L35 CF and Non-CF Bronchiectasis

• -abnormal dilation of bronchi with failure to taper
• 
• -visualize on CT or bronchography
• -Characteristics: signet ring sign, tram tracks etc

• 
• -hollow airways with small opaque "stone" sitting on them

• 1. Cylindrical
• 2. Varicose
• 3. Saccular

• 
• 

• 1. Thickened walls with mural fibrosis
• 2. Bronchial Inflammation
• 3. Neutrophilic infiltrates (chronic acute infection)
• 4. Loss of cuboidal epithelial layer
• 5. Loss of cilia
• 6. Mucus impactions

• Vicious cycle:
• -infection triggers inflammation
• -inflammation causes obstruction
• -obstruction leads to more infection

• Almost always due to predisposing infection
• -Rubeola (measles), Bordetella (whooping cough)
• -Mycobacterial disease
• -Lady Windermere Syndrome
• -Typical Pneumonias

• Symptoms:
• -chronic cough (80% are productive)
• -SOB
• -frequent pneumonias or chest colds
• -hemoptysis
• -weight loss

• Physical Exam:
• -crackles, wheezes, coarse breath sounds
• -chest hyperinflation (barrel-chested)
• -clubbing
• -cachexia

1. Hypoxemia or mild hypercarbia

• 2. Obstructive pattern on PFT
• -almost always reduced FEV1/FVC

• 3. Imaging
• -CXR
• -CT
• -Bronchography

• 
• -diffuse reticular pattern of scarring

-airway pus, cysts

• 1. Immunodeficiency
• -hypogammaglobulinemia
• -post stem cell transplant
• -CGD
• -other

• 2. Ciliary Dyskinesias
• -Kartagener syndrome

• 3. Foreign Body Obstruction
• -ie: broncholith (calcified LN that erodes from outside airway into inside airway)
• 

• Allergic Bronchopulmonary Aspergillus
• -presents as tough to manage asthma
• -caused by inflammatory reaction to aspergillus
• -elevated IgE
• -Tx: Prednisone, Antifungal

• -recessive mutation in CFTR
• -over 1300 mutations (ΔF508 most common in US)
• -prevalence of mutations vary by region

• -1/2500 live births (most common fatal genetic disease)
• -1:30 are heterozygotes
• -heterozygotes are asymptomatic

• 1. Clinical suspicion
• 2. Genetic analysis (homozygous for known mutation)
• 3. Positive sweat Chloride (> 60 mEq Cl-)

• Normal Sweat:
• -isotonic
• -Na and Cl are reabsorbed as they travel down the duct --> mostly water is excreted

• Cystic Fibrosis Sweat:
• -defect in reabsorption
• -sweat tends to have Cl- levels similar to serum

1. Antibiotics

• 2. Airway Clearance Therapies
• -mechanical devices
• -aerobic exercise
• -postural drainage
• -sputum liquefaction

• 3. Anti-Inflammatories
• -Macrolide abx
• -Corticosteroids
• -NSAIDs

• Primarily GN infections:
• -pseudomonas
• -H flu
• -E coli

• Seen Early:
• -S aureus
• -S pneumo

Always treat broad spectrum even if they don't grown multiple species on culture

• Acute Therapies:
• -IV β Lactams (piperacillin, ceftazidime, cefepime)
• -IV aminoglycosides (tobramycin, gentamicin, amikacin)
• -IV polymyxin E (colistin)
• -Oral/IV fluoroquinolones

• Chronic Antibiotics:
• -inhaled tobramycin or aztreonam
• -chronic oral abx (oto and nephrotoxicity)

• 1. Mechanical devices
• -the vest
• -acapella or flutter valve
• -intrapulmonary percussive ventilator
• -percussor

2. Postural Drainage

3. Aerobic exercise

4. Hypertonic Saline

5. Dornase alfa (DNAse)

6. Mannitol

• All Bronchiectasis
• -chronic daily impairement
• -frequent pulmonary exacerbations
• -hemoptysis
• -shorter life expectancy
• -die WITH not from respiratory complications

• Cystic Fibrosis
• -continued progress in life expectancy
• -most recent: median death ~37 years old
• -typically die FROM respiratory complications

• 1. Focal, symptomatic disease
• -fear of contaminating good lung
• -inadequate sx control with other tx

• 2. Life threatening hemoptysis
• -lobectomy
• -high morbidity

• 3. Lung transplant
• -good success with CF patients (median survival 7 years, up to 11 if live through first)
• -do better than other patients (younger, used to complicated tx, fewer comorbidities)