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Bronchiectasis
 

Bronchiectasis

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Target: UG medical students.

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    Bronchiectasis Bronchiectasis Presentation Transcript

    • BRONCHIECTASIS DR.CSBR.PRASAD, M.D.
    • CSBRP-Dec-2012
    • CSBRP-Dec-2012
    • 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
    • Clinical features Cough, fever, copious foul smelling sputum The dilation is permanent. Reversible dilation occurs in viral and bacterial pneumonia. CSBRP-Dec-2012
    • Causes: Bronchial obstruction Congenital / Hereditary conditions Necrotizing pneumonias CSBRP-Dec-2012
    • Causes:Bronchial obstruction – Tumor Foreign body Mucous impaction [complication of atopic asthma, chronic bronchitis] CSBRP-Dec-2012
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    •  The three layers are purulent sediment, clear middle liquid and top foamy layer.CSBRP-Dec-2012
    • 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
    • Ciliary dyskinesia:[Immotile cilia syndrome] [Kartagener’s syndrome] CSBRP-Dec-2012
    • Ciliary dyskinesia: [Immotile cilia syndrome] [ Kartagener syndrome ] Ciliary beating Mucociliary clearance Increased susceptibility to infections CSBRP-Dec-2012
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    • NORMAL CILIARY DYSKINESIA DYNEIN ARM CSBRP-Dec-2012
    • Commonest abnormalities: Absence of either outer or inner dynein arms Defects in radial spokes etc. CSBRP-Dec-2012
    • Clinical features of Ciliary dyskinesia: Repeated bouts of otitis & sinusitis Recurrent chest infections Situs inversus - [ 50% ] [ Kartagener syndrome - triad ] Males --- Infertility CSBRP-Dec-2012
    • 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
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    • Kartagener’s syndrome Why “Situs inversus” ? CSBRP-Dec-2012
    • Cystic fibrosis (CF) [Mucoviscidosis] CSBRP-Dec-2012
    • 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
    • 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
    • Pathogenesis of CF:Thick viscous mucusImpaired mucociliary clearanceRepeated bouts of pulmonary infectionsParenchymal fibrosis [ Bronchiectasis ] CSBRP-Dec-2012
    • 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
    • CSBRP-Dec-2012
    • Normal Cystic Fibrosis AIRWAY Na+ H20 Na+ H2O Cl Cl CSBRP-Dec-2012
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    • Pathogenesis: Thick mucus Obstruction to the bronchus Infection CSBRP-Dec-2012
    • 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
    • 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
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    • OBSTRUCTING LESION BRONCHIECTASISPOSSIBLE LIPID ORINFECTIVE PNEUMONIA CSBRP-Dec-2012
    • 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
    • Dilatation of Bronchi & Bronchioles 2cms CSBRP-Dec-2012
    • Bronchiectasis, secondary to obstruction involving the right middle lobe.CSBRP-Dec-2012
    • A closer view of the lung demonstrates the focal area of CSBRP-Dec-2012 dilated bronchi typical of bronchiectasis.
    • 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
    • CSBRP-Dec-2012
    • 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.
    • CSBRP-Dec-2012
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    • Clinical course: Cough - severe, persistent Expectoration - copious, foul smelling Fever Dyspnoea Orthopnea Severe cases Cyanosis CSBRP-Dec-2012
    • 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
    • E N D CSBRP-Dec-2012
    • DYNEIN ARM SCHEMATIC DIAGRAM OF A CILIUMCSBRP-Dec-2012