PANCE Blueprint Pulmonary (10%)

Other Pulmonary Disease (PEARLS)

The NCCPA™ PANCE  Pulmonary Content Blueprint other pulmonary diseases

Acute respiratory distress syndrome (ARDS) Acute respiratory distress syndrome (ARDS) is a type of respiratory failure characterized by fluid collecting in the lungs depriving organs of oxygen

  • ⇑ Permeability of alveolar-capillary membranes ⇒ development of protein-rich pulmonary edema (non-cardiogenic pulmonary edema)
  • ARDS can occur in those who are critically ill or who have significant injuriessepsis (most common), severe trauma, aspiration of gastric contents, near-drowning

People with ARDS have severe shortness of breath and often are unable to breathe on their own without support from a ventilator

    • Rapid onset of profound dyspnea occurring 12-24 hours after the precipitating event
    • Tachypnea, pink frothy sputum, crackles

Chest radiograph shows air bronchograms and bilaterally fluffy infiltrate

  • Normal BNP, pulmonary wedge pressure, left ventricle function and echocardiogram

Treatment involves identifying and managing underlying conditions

  • Tracheal intubation with the lowest level PEEP to maintain PaO2 >60mmHg or SaO2 >90
  • ARDS is often fatal, the risk increases with age and severity of illness
Asthma (ReelDx) Presentation: Most often young patients present with wheezing and dyspnea often associated with illness, exercise and allergic triggers

Diagnosis and monitor with peak flow. PFT's: Greater than 12% increase in FEV1 after bronchodilator therapy

  • FEV1 to FVC ratio < 80% (You would expect the amount of air exhaled during the first second (FEV1) to be the greatest amount
  • In asthma, since there is an obstruction (inflammation) you will have a decreased FEV1 and therefore a reduced FEV1 to FVC ratio

Treatment guidelines:

Mild Intermittent: Less than 2 times per week or 3-night symptoms per month

  • Step 1: Short-acting beta2 agonist (SABA) PRN

Mild Persistent: More than 2 times per week or 3-4 night symptoms per month

  • Step 2: Low-Dose inhaled corticosteroids (ICS) daily

Moderate Persistent: Daily symptoms or more than 1 nightly episode per week

  • Step 3: Low-Dose ICS + Long-acting beta2 agonist (LABA) daily
  • Step 4: Medium-Dose ICS +LABA daily

Severe Persistent: Symptoms several times per day and nightly

  • Step 5: High-Dose ICS +LABA daily
  • Step 6: High-Dose ICS +LABA +oral steroids daily

Acute treatment:

Oxygen, nebulized SABA, ipratropium bromide, and oral corticosteroids

Making Sense of Forced Vital Capacity

  • Forced expiratory volume (FEV) measures how much air a person can exhale during a forced breath. The amount of air exhaled may be measured during the first (FEV1), second (FEV2), and/or third seconds (FEV3) of the forced breath. Forced vital capacity (FVC) is the total amount of air exhaled during the FEV test
  • You would expect the amount of air exhaled during the first second to be the greatest amount. In asthma, since there is an obstruction (inflammation) you will have a decreased FEV1 and therefore a reduced FEV1 to FVC ratio.

Cystic fibrosis Etiology: Autosomal recessive mutation in the CFTR gene

  • Abnormally thick mucus, difficulty clearing mucus

Presentation: Recurrent respiratory infections (especially Pseudomonas), steatorrhea

Diagnosis: Quantitative sweat chloride test

CXR may reveal hyperinflation, mucus plugging, and focal atelectasis


  • Maintenance: Chest physiotherapy, high-fat diet, supplement fat-soluble vitamins (A, D, E, K)
  • Acute exacerbations: Antibiotics
Foreign body aspiration Foreign body aspiration occurs when a foreign body enters the airways and causes choking. Objects can enter the esophagus through the mouth, or enter the trachea through the mouth or nose

  • Most often food and can be life-threatening. 80% in mainstem or lobar bronchus right > left
  • Risk factors include institutionalization, advanced age, poor dentition, alcohol, sedative use

Presentation (depends on the location of obstruction)

  • Inspiratory stridor (if high in the airway) or wheezing and decreased breath sounds (if low in the airway)

CXR (expiratory radiograph) may reveal regional hyperinflation of the affected side

  • ABG - necessary for appropriately evaluating ventilation, may be useful for following the progression of respiratory failure when it is of concern

Treatment: Remove foreign body with a bronchoscope

  • Rigid bronchoscopy preferred in children while flexible is diagnostic and therapeutic in adults
  • Complications include pneumonia, acute respiratory distress syndrome, asphyxia
Hyaline membrane disease Etiology: Insufficient surfactant

  • Population: Preterm newborn
  • Chest radiograph: Ground glass appearance, air bronchograms, bilateral atelectasis

Treatment: Ventilation and steroids

Bronchiectasis A condition in which the lungs' airways become dilated and damaged, leading to inadequate clearance of mucus in airways

  • Mucus builds up and breeds bacteria, causing frequent infections
  • A common endpoint of disorders that cause chronic airway inflammation (CF, immune defects, recurrent pneumonia, aspiration, tumor)
  • Symptoms include a daily cough that occurs over months or years and production of copious foul-smelling sputum, frequent respiratory infections

DX: CXR = linear “tram track” lung markings, dilated and thickened airways – plate-like” atelectasis; CT chest = gold standard

    • Crackles, wheezes, purulent sputum

TX: ambulatory oxygen, aggressive antibiotics for acute exacerbations, CPT (chest physiotherapy = bang on the back); eventual lung transplant

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