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Bronchiectasis Description
- -abnormal dilation of bronchi with failure to taper
- -visualize on CT or bronchography
- -Characteristics: signet ring sign, tram tracks etc
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Signet Ring Sign
- -hollow airways with small opaque "stone" sitting on them
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3 Patterns of Bronchiectasis
- 1. Cylindrical
- 2. Varicose
- 3. Saccular
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Bronchiectasis Pathology
- 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
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Etiology
- Almost always due to predisposing infection
- -Rubeola (measles), Bordetella (whooping cough)
- -Mycobacterial disease
- -Lady Windermere Syndrome
- -Typical Pneumonias
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Clinical Presentation/Physical Exam
- 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
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Diagnosis
1. Hypoxemia or mild hypercarbia
- 2. Obstructive pattern on PFT
- -almost always reduced FEV1/FVC
- 3. Imaging
- -CXR
- -CT
- -Bronchography
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Imaging
- -diffuse reticular pattern of scarring
-airway pus, cysts
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Special Situations
- 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
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Cystic Fibrosis Epidemiology
- -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
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Cystic Fibrosis Diagnosis
- 1. Clinical suspicion
- 2. Genetic analysis (homozygous for known mutation)
- 3. Positive sweat Chloride (> 60 mEq Cl-)
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Cystic Fibrosis Sweat Test
- 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
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Bronchiectasis Treatment
1. Antibiotics
- 2. Airway Clearance Therapies
- -mechanical devices
- -aerobic exercise
- -postural drainage
- -sputum liquefaction
- 3. Anti-Inflammatories
- -Macrolide abx
- -Corticosteroids
- -NSAIDs
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Antibiotics
- 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)
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Airway Clearance Therapies
- 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
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Morbidity and Mortality
- 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
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Role of Surgery
- 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)
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