Dr yusuf imran bronchiectasis

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Bronchiectasis, its causes,pathogenesis and treatment.

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Dr yusuf imran bronchiectasis

  1. 1. BRONCHIECTASIS IN CHILDREN By-Dr.YusufImran j.n medicalcollege amu-Aligarh(India)
  2. 2. INTRODUCTION Bronchiectasis is the irreversible abnormal dilatation of bronchi with destruction of elastic and muscular components of their walls.
  3. 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. 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. 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. 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. 7. Congenital disorders associated with bronchiectasis include the following: 1. CYSTIC FIBROSIS 2. Young syndrome 3. CILIARY DYSKINESIA 4. Marfan syndrome
  8. 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. 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. 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. 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. 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. 13. DIFFERENTIAL DIAGNOSIS  Aspiration Pneumonia.  Non/slowly resolving Bacterial Pneumonia.  Chronic Obstructive Pulmonary Disease.  Emphysema.
  14. 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. 15.  Ciliary biopsy.  Antinuclear antibody and rheumatoid factor.  Flexible fiberoptic bronchoscopy and bronchoalveolar lavage.  HIV and TB should be ruled out.
  16. 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. 17.  Surgical treatment- Only when medical management fails or there is a sequestrated lung.  Pulmonary segmental resection.  Transplantation.

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