Bronchiectasis, its causes,pathogenesis and treatment.

1. BRONCHIECTASIS IN
CHILDREN
By-Dr.YusufImran
j.n medicalcollege
amu-Aligarh(India)

2. INTRODUCTION
Bronchiectasis is the irreversible abnormal dilatation of
bronchi with destruction of elastic and muscular
components of their walls.

3. ETIOPATHOGENESIS
 Bronchiectasis generally results from obstruction and/or
inflammation of the airway.
 Chronic infection lead to recruitment of neutrophils, T
lymphocytes, and monocyte-derived cytokines.

4.  The release of inflammatory mediators, elastases, and
collagenases leads to inflammation and destruction of
elastic and muscular components of bronchial walls.
 Bronchiectasis associated with bronchial obstruction may
have a focal distribution distal to the site of obstruction
[ Foreign body] .
 Bronchiectasis associated with underlying disease is likely
to be diffuse [ Cystic Fibrosis].

5. ETIOLOGY
 Bronchiectasis may result from infection, congenital or
acquired disorders, or obstruction.
 All causes share the same pathophysiologic pathway:
ineffective pulmonary toilet and chronic or recurrent
infection and inflammation.

6. Common infectious causes includes:
1. Severe pneumonia, especially viral, [inadequately treated]
2. Measles, tuberculosis, pertussis, Adenovirus, Mycobacterium
avium, and Aspergillus fumigatus infections.
3. HIV infection

7. Congenital disorders associated with
bronchiectasis include the following:
1. CYSTIC FIBROSIS
2. Young syndrome
3. CILIARY DYSKINESIA
4. Marfan syndrome

8. DISORDERS ASSOCIATED WITH DEFECTIVE DEFENCE-
1. Bruton agammaglobulinemia.
2. Congenital absence of bronchial muscle (Mounier-Kuhn
syndrome) or cartilage (Williams-Campbell syndromes).
3. Immunoglobulin A (IgA) and G (IgG) deficiencies and IgG
subclass deficiencies, especially IgG2 deficiency.

9. Acquired disorders associated with bronchiectasis
include the following:
1. Intrinsic airway luminal obstruction by a bronchial foreign
body or extrinsic compression by mass.
2. Chronic aspiration, which is associated with swallowing
dysfunction, gastroesophageal reflux disease, or
tracheoesophageal fistula.

10. MISCELLANEOUS CAUSES :
1.Connective tissue disorders, including rheumatoid
arthritis and systemic lupus erythematosus.
2. Allergic bronchopulmonary aspergillosis
3. Tracheal stenosis with impaired mucociliary clearance
4. Fibrosing lung diseases associated with sarcoidosis or
idiopathic pulmonary fibrosis
5. Persistent atelectasis

11. CLINICAL PRESENTATION
 Recurrent cough often productive and longer than 6
weeks, related to posture.
 Purulent Sputum, Often large in amount.
 Recurrent respiratory infections.
 Hemoptysis.

12. EXAMINATION
 MALNUTRITION - Child is usually in poor health due to
poor appetite and recurrent infections.
 Clubbing may be present.
 CHEST –coarse leathery crepitations –
localized/generalized.

13. DIFFERENTIAL DIAGNOSIS
 Aspiration Pneumonia.
 Non/slowly resolving Bacterial Pneumonia.
 Chronic Obstructive Pulmonary Disease.
 Emphysema.

14. DIAGNOSTIC WORKUP
 CHEST X RAY: Honeycombing.
 CT SCAN: gold standard for diagnosis.
 SPUTUM CULTURE OR DEEP OROPHARYNGEAL SWAB.
 Serum IgG,IgM and IgA.
 Sweat chloride

15.  Ciliary biopsy.
 Antinuclear antibody and rheumatoid factor.
 Flexible fiberoptic bronchoscopy and bronchoalveolar
lavage.
 HIV and TB should be ruled out.

16. MEDICAL MANAGEMENT
 Acute exacerbation-
1. Antibiotics to cover both Gram+ ,Gram- as well as
anaerobes.
eg- Combination of Cephalosporins, aminoglycosides and
metronidazole.
2. Good chest physiotherapy- to drain the secretions.
3. Treatment related to underlying disorders
Eg. Mucolytics, ICS in Cystic fibrosis.

17.  Surgical treatment- Only when medical management
fails or there is a sequestrated lung.
 Pulmonary segmental resection.
 Transplantation.