Bronchiectasis

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Bronchiectasis

  1. 1. BRONCHIECTASIS DR.CSBR.PRASAD, M.D.
  2. 2. CSBRP-Dec-2012
  3. 3. CSBRP-Dec-2012
  4. 4. Definition: Is a chronic necrotising infection of the bronchi and bronchioles leading to or associated with abnormal dilation of these airways. Prevalence: * 1.5 per 1000 population * Has decreased in recent times CSBRP-Dec-2012
  5. 5. Clinical features Cough, fever, copious foul smelling sputum The dilation is permanent. Reversible dilation occurs in viral and bacterial pneumonia. CSBRP-Dec-2012
  6. 6. Causes: Bronchial obstruction Congenital / Hereditary conditions Necrotizing pneumonias CSBRP-Dec-2012
  7. 7. Causes:Bronchial obstruction – Tumor Foreign body Mucous impaction [complication of atopic asthma, chronic bronchitis] CSBRP-Dec-2012
  8. 8. CSBRP-Dec-2012
  9. 9. CSBRP-Dec-2012
  10. 10. CSBRP-Dec-2012
  11. 11.  The three layers are purulent sediment, clear middle liquid and top foamy layer.CSBRP-Dec-2012
  12. 12. Causes : cont….. Congenital / Hereditary conditions • Congenital bronchiectasis (developmental anomaly) • Cystic fibrosis • Immunodeficiency states • Immotile cilia & Kartagener syndromes Necrotizing pneumonia [Post infective bronchiectasis - TB, Staph, mixed infection] CSBRP-Dec-2012
  13. 13. Ciliary dyskinesia:[Immotile cilia syndrome] [Kartagener’s syndrome] CSBRP-Dec-2012
  14. 14. Ciliary dyskinesia: [Immotile cilia syndrome] [ Kartagener syndrome ] Ciliary beating Mucociliary clearance Increased susceptibility to infections CSBRP-Dec-2012
  15. 15. CSBRP-Dec-2012
  16. 16. CSBRP-Dec-2012
  17. 17. CSBRP-Dec-2012
  18. 18. CSBRP-Dec-2012
  19. 19. CSBRP-Dec-2012
  20. 20. NORMAL CILIARY DYSKINESIA DYNEIN ARM CSBRP-Dec-2012
  21. 21. Commonest abnormalities: Absence of either outer or inner dynein arms Defects in radial spokes etc. CSBRP-Dec-2012
  22. 22. Clinical features of Ciliary dyskinesia: Repeated bouts of otitis & sinusitis Recurrent chest infections Situs inversus - [ 50% ] [ Kartagener syndrome - triad ] Males --- Infertility CSBRP-Dec-2012
  23. 23. Kartagener’s syndrome1. Bronchiectasis2. Situs inversus3. Sinusitis4. Infertility in menCharacterised by ultrastructural changes in microtubules causing immotility of cilia of respiratory epithelium, sperms. CSBRP-Dec-2012
  24. 24. CSBRP-Dec-2012
  25. 25. CSBRP-Dec-2012
  26. 26. Kartagener’s syndrome Why “Situs inversus” ? CSBRP-Dec-2012
  27. 27. Cystic fibrosis (CF) [Mucoviscidosis] CSBRP-Dec-2012
  28. 28. Cystic fibrosis [Mucoviscidosis]  Systemic disease  Prevalence: 1 in 2500 live births  Common in western countries  Uncommon in Asians & Africans  Pulmonary involvement dominates  Inheritance - AR  95% deaths - pulmonary disease [ Bronchiectasis ] CSBRP-Dec-2012
  29. 29. Sterility Increased Na+ and Cl-Bronchiectasis Vas deferens in sweat seminal vesicles Sweat Lungs glands CYSTIC FIBROSIS Biliary Gut systemMeconium Pancreas Jaundice ileus cirrhosis Malabsorption of fat Fibrosis CSBRP-Dec-2012
  30. 30. Pathogenesis of CF:Thick viscous mucusImpaired mucociliary clearanceRepeated bouts of pulmonary infectionsParenchymal fibrosis [ Bronchiectasis ] CSBRP-Dec-2012
  31. 31. Molecular defect: Gene - CFTR Chromosome - 7q31 - 32 Function - Anion channel Defect in cystic fibrosis ( 550 mutations ) --- deletion of codon 508 [phenylalanine]CFTR: Cystic Fibrosis Transmembrane conductance Regulator CSBRP-Dec-2012
  32. 32. CSBRP-Dec-2012
  33. 33. Normal Cystic Fibrosis AIRWAY Na+ H20 Na+ H2O Cl Cl CSBRP-Dec-2012
  34. 34. CSBRP-Dec-2012
  35. 35. CSBRP-Dec-2012
  36. 36. Pathogenesis: Thick mucus Obstruction to the bronchus Infection CSBRP-Dec-2012
  37. 37. Morphology - Gross Affects lower lobes Bilateral Air passages that are vertical Tumor/FB aspiration – localized single segment Severe involvement - more distal bronchi Airways are 4x dilated than normal CSBRP-Dec-2012
  38. 38. Gross contd….1. Cylindrical – most common, tube like dilation2. Fusiform – spindle shaped dilation.3. Saccular – sac like distension.4. Varicose – irregular bronchial enlargement. Cut sections shows HONEY COOMBED appearance. CSBRP-Dec-2012
  39. 39. CSBRP-Dec-2012
  40. 40. CSBRP-Dec-2012
  41. 41. OBSTRUCTING LESION BRONCHIECTASISPOSSIBLE LIPID ORINFECTIVE PNEUMONIA CSBRP-Dec-2012
  42. 42. This is a bronchogram that demonstrates saccular bronchiectasis on the right in the lower lobe. The contrast media fills dilated bronchi, giving a saccular, dilated outline.CSBRP-Dec-2012
  43. 43. Dilatation of Bronchi & Bronchioles 2cms CSBRP-Dec-2012
  44. 44. Bronchiectasis, secondary to obstruction involving the right middle lobe.CSBRP-Dec-2012
  45. 45. A closer view of the lung demonstrates the focal area of CSBRP-Dec-2012 dilated bronchi typical of bronchiectasis.
  46. 46. Fig15-3 Bronchiectasis in a patient with cystic fibrosis, who underwent lung transplantation. Cut surface of lung shows markedly distended peripheral bronchi filled with mucopurulent secretions.CSBRP-Dec-2012
  47. 47. CSBRP-Dec-2012
  48. 48. Bronchiectasis is seen here. The repeated episodes of inflammation can result inscarring, which has resulted in fibrous adhesions between the lobes. Fibrous pleuraladhesions are common in persons who have had past episodes of inflammation of thelung that involve the pleura. With extensiveCSBRP-Dec-2012 the pleural space may be involvement,obliterated.
  49. 49. CSBRP-Dec-2012
  50. 50. CSBRP-Dec-2012
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  52. 52. Clinical course: Cough - severe, persistent Expectoration - copious, foul smelling Fever Dyspnoea Orthopnea Severe cases Cyanosis CSBRP-Dec-2012
  53. 53. Complications ofbronchiectasis:  Metastatic abscesses ( brain abscess)  Recurrent pulmonary infection  Right sided cardiac failure [ chronic cor pulmonale ]  Massive haemoptysis  Reactive systemic amyloidosis [ Nephrotic syndrome ] CSBRP-Dec-2012
  54. 54. E N D CSBRP-Dec-2012
  55. 55. DYNEIN ARM SCHEMATIC DIAGRAM OF A CILIUMCSBRP-Dec-2012

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