L35 CF and Non-CF Bronchiectasis

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Author:
jknell
ID:
202833
Filename:
L35 CF and Non-CF Bronchiectasis
Updated:
2013-02-23 16:29:56
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Pulmonary II
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Pulmonary II
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  1. Bronchiectasis Description
    • -abnormal dilation of bronchi with failure to taper
    • -visualize on CT or bronchography
    • -Characteristics: signet ring sign, tram tracks etc
  2. Signet Ring Sign
    • -hollow airways with small opaque "stone" sitting on them
  3. 3 Patterns of Bronchiectasis
    • 1. Cylindrical
    • 2. Varicose
    • 3. Saccular

  4. 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
  5. Etiology
    • Almost always due to predisposing infection
    • -Rubeola (measles), Bordetella (whooping cough)
    • -Mycobacterial disease
    • -Lady Windermere Syndrome
    • -Typical Pneumonias
  6. 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
  7. Diagnosis
    1. Hypoxemia or mild hypercarbia

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

    • 3. Imaging
    • -CXR
    • -CT
    • -Bronchography
  8. Imaging
    • -diffuse reticular pattern of scarring

    -airway pus, cysts

  9. 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
  10. 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
  11. Cystic Fibrosis Symptoms
  12. Cystic Fibrosis Diagnosis
    • 1. Clinical suspicion
    • 2. Genetic analysis (homozygous for known mutation)
    • 3. Positive sweat Chloride (> 60 mEq Cl-)
  13. 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
  14. Bronchiectasis Treatment
    1. Antibiotics

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

    • 3. Anti-Inflammatories
    • -Macrolide abx
    • -Corticosteroids
    • -NSAIDs
  15. 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)
  16. 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
  17. 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
  18. 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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