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Key Features

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Essentials of Diagnosis
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  • Chronic cough with sputum production

  • Rhonchi or wheezes (or both) on chest auscultation

  • Diagnosis is confirmed by high-resolution CT scan

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General Considerations
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  • Defined as the permanent dilation of bronchi resulting from airway obstruction by retained mucus secretions or inflammation in response to chronic or repeated infection

  • Occurs either as a consequence of

    • Preceding illness (severe pneumonia or foreign body aspiration) or

    • Manifestation of an underlying systemic disorder (cystic fibrosis, primary ciliary dyskinesia, chronic aspiration, or immunodeficiency)

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Clinical Findings

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  • Chronic cough, purulent sputum

  • Fever

  • Weight loss

  • Recurrent respiratory infections and dyspnea on exertion

  • Finger clubbing

  • Rales, rhonchi, and decreased air entry often noted over the bronchiectatic areas

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Diagnosis

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Laboratory Findings
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  • Most common bacteria detected in cultures from the lower respiratory tract include

    • Streptococcus pneumoniae

    • Staphylococcus aureus

    • Nontypeable Haemophilus influenzae

    • Pseudomonas aeruginosa

  • Nontuberculous mycobacterial species may also be detected

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Imaging
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  • Chest radiographs

    • May be mildly abnormal with slightly increased bronchovascular markings or areas of atelectasis

    • May demonstrate cystic changes in one or more areas of the lung

  • High-resolution CT

    • Best method to define extent of bronchiectasis

    • Often reveals far wider involvement of lung than expected from the chest radiograph

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Diagnostic Studies
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  • Pulmonary function testing demonstrates airflow obstruction and air trapping

  • Evaluation of lung function after use of a bronchodilator is helpful in assessing the benefit from bronchodilators

  • Serial assessments of lung function help define the progression or resolution of the disease

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Treatment

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Medical
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  • Aggressive antibiotic therapy during pulmonary exacerbations and routine airway clearance is mainstay of treatmen

  • Prolonged antibiotic use, anti-inflammatory therapy, hyperosmolar agents (hypertonic saline), and bronchodilators have not proven effective in non–cystic fibrosis bronchiectasis, although individual patients may benefit

  • Prolonged azithromycin

    • Has been shown to reduce exacerbations in adults with non–cystic fibrosis bronchiectasis

    • Whether these results translate to children with idiopathic bronchiectasis is unknown

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Surgical
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  • Removal of an area of lung affected with severe bronchiectasis is considered when the response to medical therapy is poor

  • Other indications for surgery include

    • Severe localized disease

    • Repeated hemoptysis

    • Recurrent pneumonia in one area

  • If bronchiectasis is widespread, surgical resection offers little advantage

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Outcome

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Prognosis
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  • Depends on the underlying cause and severity of bronchiectasis, the extent of lung involvement, and the response to medical management

  • Good pulmonary hygiene and avoidance of infectious complications in the involved areas of lung may reverse cylindrical bronchiectasis

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References

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Salerno  T  et al: Bronchiectasis and severe respiratory insufficiency associated with a new surfactant protein C mutation. Acta Paediatr 2013 Jan;102(1):e1–e2
[PubMed: 23025826] .
CrossRef
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