Each student will research a disease of their choosing from the assigned list below. This assignment is intended to serve as a comprehensive review of each of the diseases/conditions discussed this se

Bronchiectasis: Clinical Presentation

Pathophysiology
- Chronic dilation and distortion of bronchial airways
- Excessive production of foul-smelling sputum
- Smooth muscle constriction of bronchial airways
- Hyperinflated alveoli
- Atelectasis
- Consolidation
- Parenchymal fibrosis
Anatomic Alterations
- Hyperinflation of the distal alveoli
  - Atelectasis
  - Consolidation
  - parenchymal fibrosis
Forms of Bronchiectasis
- Varicose
  - Bronchi are dilated and constricted in an irregular fashion
- Cylindrical
  - Bronchi are dilated and have regular outlines
    - Like a tube. The dilated bronchi fail to taper for six to ten generations
    - They will appear to end squarely in a bronchogram because of mucous obstruction
- Saccular
  - Bronchi progressively increase in diameter and end in large, cyst-like sacs in the lung parenchyma
  - This form causes the most damage to the tracheobronchial tree
  - The bronchial walls become fibrous. Cartilage, elastic tissue, and smooth muscle are absent
Etiology: Acquired or Congenital
- Acquired
  - Pulmonary Infection
    - Repeated and prolonged resp tract infections. Children who have frequent bouts of broncho-pneumonia-because of resp complications of measles, chickenpox, pertussis, or influenza, - may acquire bronchiectasis later in life.

- - Bronchial Obstruction
    - caused by tumor masses, enlarged hilar lymph nodes, or aspirated foreign bodies may result in bronchiectasis distal to the obstruction.
    - These conditions impair the mucociliary clearance mechanism, and this impairment, in turn, favor, the development of necrotizing bacterial infections.
  - Pulmonary TB
    - Because of the inflammatory process and the bronchial wall destruction associated with pulmonary TB, bronchiectasis is a common secondary complication
- Congenital
  - Kartagener’s Syndrome
    - A triad consisting of bronchiectasis, dextrocardia (having the heart on the right side of the body), and paranasal (alongside the nose) sinusitis
    - Accounts for about 20% of all congenital bronchiectasis.
  - Hypogammaglobulinemia
    - individuals who have inadequate regional or systemic defense mechanisms because of inherited or acquired immune deficiency disorders. These individuals have a high risk for recurrent episodes of respiratory infections.
  - Cystic Fibrosis
    - Because of impairment of the mucociliary clearance mechanism and the abundance of stagnant, thick mucus associated with CF, bronchial obstruction from mucus plugging and bronchial infections frequently result.
    - The necrotizing inflammation that develops under these conditions often leads to secondary bronchiectasis
Physical Examination
- Increased RR
  - Several pathophysiologic mechanisms operating simultaneously may lead to an increased RR:
  - Stimulation of Peripheral chemoreceptors
  - Decreased lung compliance/increased ventilatory rate relationship
  - Anxiety
- Increased HR, CO, and BP

- Accessory Muscle Use
  - On inspiration and expiration
- Pursed-lip breathing
- Increased AP diameter
  - Barrel Chest
- Digital Clubbing
  - Cause unknown (often seen with chronic hypoxemia)
- Peripheral edema and distention
  - Polycythemia and cor pulmonale are associated with severe emphysema: You may see:
    - Distended neck veins
    - Pitting edema
    - Enlarged and tender liver
- Cyanosis
Cough and Sputum Production
- Chronic productive cough of large quantities of foul-smelling sputum
- Settles into different layers
- Hemoptysis
  - Streaks of blood are seen frequently, presumably originating from necrosis of the bronchial walls and erosion of bronchial blood vessels
  - Frank hemoptysis may also occur from time to time but is rarely life threatening
- Bacterial colonization
  - Streptococcus pneumoniae
  - Hemophilus influenzae
  - Pseudomonas aeruginosa
    - Due to the excessive bronchial secretions
    - Secondary bacterial infections are frequent

Chest Assessment
- Obstructive Bronchiectasis
  - Air trapping
- Hyperresonant percussion note
  - Air trapping
- Decreased tactile and vocal fremitus
  - Air trapping
- Diminished breath sounds
  - Bronchospasm
- Wheezing, Crackles, and Rhonchi
  - when accompanied by acute or chronic bronchitis
- Restrictive Bronchiectasis
  - Dull percussion note
    - Over areas of atelectasis and consolidation
  - Increased tactile and vocal fremitus
  - Bronchial breath sounds
  - Whispered Pectoriloquy
    - When patient whispers “1, 2, 3” the sounds are much louder and more intelligible over the affected area
Laboratory Evaluation
- Increased H&H
  - Hemoglobin (Hb: each RBC contains app. 280 million Hb molecules, grams /100ml of blood. Normal healthy adult male = 14-16 g% normal healthy adult female = 12-15 g%)
  - Hematocrit (Hct: volume of RBC in 100 ml of blood. Normal healthy adult male = 45% normal healthy adult female = 42%
- Hypochloremia
  - With chronic ventilatory failure
- Haemophilus influenzae
- Pseudomonas aeruginosa
Chest Radiograph
- Signs of hyperinflation in Obstructive disorder
  - Translucent lung fields (dark)
  - Flattened diaphragms (depressed)
  - Long narrow heart (pulled down by diaphragm)
  - Enlarged Heart (lateral x-ray)

- Restrictive disorder
  - Increased opacity (white)
  - Atelectasis and consolidation
    - When atelectasis and consolidation develop as a result of bronchiectasis, an increased opacity is seen
Bronchogram
- Cylindrical bronchiectasis
  - Dilated, cylinder-shaped bronchioles
  - Increased bronchial markings and adjacent emphysema
- Saccular bronchiectasis
  - Large sac-like structures, fibrotic markings, associated atelectasis, and adjacent emphysema
- Varicose bronchiectasis
  - Bronchi are dilated and constricted in an irregular fashion
  - Bronchi terminate in a distorted, bulbous shape
- Computed Tomography (CT) of the chest has largely replaced this technique
CT Scan
- Bronchial wall thickness and opacity is often seen
- Dilated
- Characterized by ring lines or clusters
- Signet ring-shaped
  - Produced by the ring shadow of a dilated airway with its accompanying artery
- Flame-shaped
  - Airways are filled with secretions
PFT
- Obstructive bronchiectasis
  - Decreased expiratory flows
    - Decreased: FVC, FEV1, PEFR
  - Increased lung volumes
    - Increased VT, RV, FRC
- Restrictive bronchiectasis
  - Decreased lung volumes and capacities
    - Decreased: VT, RV, FRC, TLC, VC, IC, ERV

ABG
- Mild to Moderate bronchiectasis
  - Early hypoxemia with respiratory alkalosis
    - Acute alveolar hyperventilation with hypoxemia
    - pH increased
    - PaCO2 decreased
    - HCO3 slightly decreased
    - PaO2 decreased
- Severe bronchiectasis
  - Late respiratory acidosis with hypoxemia
    - Chronic ventilatory failure with hypoxemia
    - pH normal
    - PaCO2 increased
    - HCO3 increased significantly
    - PaO2 decreased
General Management
- Patient education
  - patient and family should be instructed on disease and the effects on the body, Instructed on home care therapy
  - Use of pulmonary rehab if needed.
- Behavioral management
  - Stop smoking
  - Avoid inhaled irritants such as
    - Dust
    - Fumes
    - Mist
    - toxic gases
- Mobilization of secretions
  - Bronchial hygiene therapy
- Medications
  - Sympathomimetic and parasympatholytic agents for bronchospasms
  - Xanthines to enhance bronchial smooth muscle relaxation
  - Expectorants and Antibiotics to treat infections

- Supplemental oxygen
  - Treat hypoxemia
  - Decrease myocardial work
  - Decrease WOB
  - (in late stages be careful not to over oxygenate)
- Mechanical ventilation
  - provide and support alveolar gas exchange
  - eventually return the patient to spontaneous breathing