Bronchiectasis

Beschreibung

pathology of bronchiectasis
Farah  Mansour
Mindmap von Farah Mansour, aktualisiert more than 1 year ago
Farah  Mansour
Erstellt von Farah Mansour vor etwa 8 Jahre
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Zusammenfassung der Ressource

Bronchiectasis
  1. Permanent DILATION of the bronchi and bronchioles causing repeated episodes of airway infection and inflammation << Due to destruction of cartilage and elastic tissue by chronic necrotizing infections
    1. Causes
      1. Cystic fibrosis
        1. Autosomal recessive disease
          1. Pathogenesis
            1. (1) Most common mutation is a three-nucleotide deletion on chromosome 7 that normally codes for phenylalanine (70% of cases).
              1. (2) Mutation causes defective protein folding in the cystic fibrosis transmembrane conductance regulator (CFTR).
                1. (3) Defective CFTR is degraded in the Golgi apparatus.
                  1. (4) Loss of CFTR causes decreased Cl– reabsorption in the sweat glands
                    1. Effect of loss of CFTR in other secretions:
                      1. (a) Increased Na+ and water reabsorption from luminal secretions
                        1. (b) Decreased Cl– secretion out of epithelial cells into luminal secretions
                          1. (c) Net effect of these electrolyte alterations is dehydration of body secretions due to lack of NaCl.
                      2. Infections
                        1. (a) TB is the most common cause worldwide.
                          1. (b) Mycobacterium avium-intracellulare (typically involves the right middle lobe and lingula), adenovirus, Staphylococcus aureus, Haemophilus influenzae
                          2. Bronchial Obstruction
                            1. Primary ciliary dyskinesia
                              1. (a) Dynein arm in cilia is absent.
                                1. (b) Dynein arm contains ATPase (adenosine triphosphatase) for movement of the cilia.
                                2. Allergic bronchopulmonary aspergillosis
                                3. Gross Findings
                                  1. a. Most commonly occurs in the lower lobes
                                    1. b. Dilated bronchi and bronchioles are filled with pus
                                      1. (1) Dilated airways extend to the lung periphery.
                                        1. (2) Dilations are tube-like and/or saccular.
                                      2. Clinical Findings
                                        1. a. Cough productive of copious sputum (often cupfuls)
                                          1. b. Hemoptysis that is sometimes massive
                                            1. c. Digital clubbing
                                              1. d. Cor pulmonale
                                                1. Chest cardiograph & CT scan Findings
                                                  1. Crowded bronchial markings extend to the lung periphery
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