lecture about broncheictasis

1. B Y D R H I S H A M A L R A B T Y P E D I A T R I C C O N S U L T A N T
P U L M O N O L O G I S T
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2. TOPICS:
1. Introduction.
2. Definition.
3. Etiology.
4. Pathophysiology.
5. Epidemiology.
6. Clinical presentation.
7. Diagnostic approach.
8. Treatment.
9. Prognosis.
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3. INTRODUCTION:
It is not disease but a complication or sequel of other lung diseases.
It is chronic condition.
René Laennec, inventor of the stethoscope, first described bronchiectasis in
1819 while observing patients with tuberculosis and the sequelae of
pneumonia in the pre-antibiotic era.
The term bronchiectasis is derived from the Greek bronchion, meaning
windpipe, and ektasis, meaning stretched.
It could be localized one or two lung lobes involved or generalized both lungs
affected.
It is a type of obstructive lung disease.
Mucolytic and chest physiotherapy both are part of treatment.
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4. DEFINITION:
Bronchiectasis is a pathologic term defined by the
dilatation of bronchi with destruction of elastic and
muscular components of their walls.
Classification:
Upto bronchogram
• Cylindrical (fusiform).
• Saccular.
• Varicose.
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7. ETIOLOGY
Causes of this condition could be classified into :
o Infectious.
o Congenital/genetic.
o Immune deficiency.
o Acquired.
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8. 1-INFECTIOUS CAUSES:
• Severe pneumonia, viral and bacterial.
• Mycobacterial and Fungal infections.
• Measles, tuberculosis, pertussis, adenovirus, Mycobacterium avium,
and Aspergillus fumigatus.
• HIV infection: Children who develop lymphocytic interstitial
pneumonitis seem at increased risk of subsequent bronchiectasis.
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9. 2-CONGENITAL/GENETIC:
• Cystic Fibrosis.
• Primary Ciliary dyskinesia.
• Marfan syndrome.
• Alpha-1 antitrypsin deficiency.
• Bruton agammaglobulinemia.
• Congenital absence of bronchial muscle (Mounier-Kuhn
syndrome) or cartilage (Williams-Campbell syndromes).
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10. 3-IMMUNE DEFICIENCY:
• immunoglobulin A (IgA) and G (IgG) deficiencies and IgG subclass
deficiencies, especially IgG2 deficiency.
• Allergic bronchopulmonary aspergillosis (ABPA)/ allergic
bronchopulmonary mycoses.
• Bruton agammaglobulinemia.
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11. 4-ACQUIRED:
• Intrinsic airway luminal obstruction by a retained bronchial foreign body or
extrinsic compression by mass.
• Chronic aspiration, which is associated with swallowing dysfunction,
gastroesophageal reflux disease, or tracheoesophageal fistula.
• Connective tissue disorders, including rheumatoid arthritis and systemic lupus
erythematous.
• Extrinsic airway narrowing (vascular ring, adenopathy, compressive mass).
• Endobronchial mass or tumor.
• Tracheal stenosis with impaired mucociliary clearance.
• Severe Tracheomalacia or bronchomalacia with impairment of mucociliary
clearance.
• Fibrosing lung diseases associated with sarcoidosis or idiopathic pulmonary
fibrosis.
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12. PATHOPHYSIOLOGY:
Bronchiectasis is more directly the product of obstruction and/or inflammation of
the airway, however it is generally the result of an intricate interaction between the
host, pathogens and the environment.
Bronchiectasis associated with bronchial obstruction may have a focal distribution
distal to the site of obstruction.
Bronchiectasis associated with underlying disease is more likely to be diffuse.
Regardless of the etiology, there is an impairment in the mucociliary clearance ability
of the lungs, which leads to a diminished ability to clear the airway of the purulent
and inflammatory material, which in turn leads to increased bacterial colonization
and infection.
The release of inflammatory mediators, elastases, and collagenases leads to
inflammation and destruction of elastic and muscular components of bronchial walls.
These changes may be accompanied by bronchial arterial proliferation, which
predisposes to hemoptysis.
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14. EPIDEMIOLOGY:
 In developed countries, the frequency is similar to that in the United
States with bronchiectasis being more common among indigenous
populations and disadvantaged groups.
 Similarly to the United States, the most clinically significant cause of
bronchiectasis in developed affluent countries is cystic fibrosis.
 Throughout the world, bronchiectasis is predominantly associated with
non-CF related conditions rather than CF.
 The frequency is higher in the developing world, where measles,
adenovirus infection, pneumonia, tuberculosis, and HIV infection are
all on the rise and are associated with bronchiectasis.
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15. CLINICAL PRESENTATION:
From history (symptoms):
 Cough and daily mucopurulent sputum production .
 Blood streaked sputum or hemoptysis associated with acute infection.
 Dyspnea,pleurtic pain, wheezing, fever, wt loss, fatigue.
 Rarely episodic hemoptysis with little to no sputum .
By clinical examination (signs):
• Failure to thrive.
• Nail clubbing.
• Central cyanosis.
• Tachypnea.
• Wheeze and or grunting.
• Recessions .
• Coarse crepitations, rhonchi, decreased air entry , bronchial breathing .
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16. DIAGNOSTIC APPROACH:
Laboratory evaluation of bronchiectasis may include the following tests:
o Sweat chloride test.
o evaluation for allergic bronchopulmonary aspergillosis should include
immunoglobulin E (IgE) and serum precipitins for Aspergillus species , sputum
culture for fungus, and an aspergillus skin test.
o Serum immunoglobulin G (IgG) with IgG subclasses, immunoglobulin M (IgM),
and IgA .
o HIV test.
o Sputum culture or deep oropharyngeal swab in younger children .
o Spirometry for children older than 6 years of age.
o Ciliary biopsy.
o Antinuclear antibody and rheumatoid factor .
o Vaccine antigens.
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17. SPECIFIC IMAGING:
Computed Tomography:
The diagnosis of bronchiectasis is usually established using high-resolution CT (HRCT) scanning, which has
a sensitivity and specificity of more than 90%.
Gastroesophageal Reflux Disease Assessment:
Studies may include barium esophagraphy, gastric scintiscanning, or intraesophageal pH or impedance
monitoring.
Flexible Bronchoscopy : (virtual bronchoscopy)
Flexible bronchoscopy may help assess the caliber and appearance of the airways and provide
bronchoalveolar lavage fluid for evidence of chronic aspiration or infection and or obstruction for any
cause.
Histologic Findings:
Examination of the bronchoalveolar fluid reveals inflammatory cells. Hemosiderin-laden macrophages
generally suggest nonacute bleeding. Lipid-laden macrophages suggest chronic aspiration but may also be
observed in other forms of severe airway disease associated with inflammation.
Spirometry:
Often spirometry may be normal early in the course of patients with bronchiectasis. As the disease
progresses, it is obstructive in the earlier stages and becomes mixed obstructive and restrictive disease
process later in the disease.
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18. Thick walls←
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20. TREATMENT:
Supportive:
• Antipyretics.
• Good hydration .
• Nutrition.
• Mucolytics.
• Chest physiotherapy ultrasonic.
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21. Specific treatment:
 Antibiotics iv and or oral or nebulized(tobramycin).
 Steroids inhalational and or oral.
 Bronchodilators inhalational or nebulized.
 Surgery.
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22. MUCOLYTIC THERAPY:
Mucolytic drugs are given with the intent of improving
tracheobronchial clearance via alteration of sputum consistency.
• Aerosolized recombinant DNase breaks down DNA released by
neutrophils, which accumulates in the airways in response to
chronic bacterial infection.
• Nebulized acetylcysteine and hypertonic saline are other agents
aimed at altering mucous consistency to facility mucus
clearance.
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23. AIRWAY CLEARANCE TECHNIQUES:
Manual and mechanical interventions such as chest percussion,
vibration, postural drainage, cough-assist devices, and airway
oscillation are used to facilitate sputum volume and mucous
expectoration.
The goal is to facilitate effective airway clearance.
These techniques or devices are often used daily in order to be most
effective and may be used in combination.
Patients can be trained to do huff coughing with airway clearance to
help expectorate loosened mucus.
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25. ANTIBIOTIC THERAPY:
• Intravenous antibiotic therapy and hospitalization may be necessary for
children experiencing exacerbations of disease.
• Antibiotics may be prescribed for long-term use in patients with
bronchiectasis to reduce frequency of exacerbations, improve quality of
life, and diminish lung function decline.
• Inhaled tobramycin was associated with decreased Pseudomonas
aeruginosa load in sputum, improved lung function, and fewer
hospitalizations.
• Aztreonam and Colistin (colistimethate) have come into frequent use as
an inhaled antibiotic in patients with cystic fibrosis, and it may find its
way into therapy for non-CF bronchiectasis.
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26. PROGNOSIS:
It is upto cause ranging from life compatible to
lethal.
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