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Restrictive Pulmonary Disease
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Restrictive Pulmonary Disease

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Questions and Answers

Which of the following is a characteristic of restrictive pulmonary disease?

  • Decreased elastic recoil
  • Increased lung volumes
  • Decreased vital capacity with low lung volumes (correct)
  • Increased FEV1

    • Which of the following is an example of a restrictive pulmonary disease?

  • Pneumonia
  • Asthma
  • Chronic obstructive pulmonary disease (COPD)
  • Sarcoidosis (correct)

    • What is the typical pattern of lung volumes in restrictive pulmonary disease?

  • TLC, FRC, and RV are decreased (correct)
  • TLC and FRC are increased
  • TLC and FRC are decreased, but RV is increased
  • TLC is increased, but FRC and RV are decreased

    • What is the hallmark of restrictive lung disease?

    <p>Low FVC with low FRC and RV</p> Signup and view all the answers

    What is the effect of aging on chest wall compliance?

    <p>It decreases due to rib calcification</p> Signup and view all the answers

    What is the characteristic pattern of FEV1 and FVC in restrictive pulmonary disease?

    <p>FEV1 is decreased, FVC is decreased</p> Signup and view all the answers

    What is the significance of a low FEV1/FVC ratio?

    <p>It indicates an obstructive pattern</p> Signup and view all the answers

    What is the effect of providing high-concentration supplemental oxygen to lung regions with relatively poor ventilation?

    <p>Reverse pulmonary vasoconstriction</p> Signup and view all the answers

    Which of the following is a distinguishing feature of restrictive pulmonary disease?

    <p>Low TLC</p> Signup and view all the answers

    What is the definition of a pulmonary shunt?

    <p>Blood passing through the pulmonary circulation without changing its chemical composition</p> Signup and view all the answers

    What is an example of a pulmonary shunt?

    <p>Regional atelectasis created by a pneumothorax</p> Signup and view all the answers

    What is the most significant characteristic of a pulmonary shunt?

    <p>Giving supplemental O2 raises PAO2, but there is no significant increase in PaO2</p> Signup and view all the answers

    What is the PO2 of blood entering the pulmonary circulation under resting conditions?

    <p>40 mmHg</p> Signup and view all the answers

    What is the purpose of alveolar ventilation in the pulmonary capillaries?

    <p>To add oxygen to the bloodstream</p> Signup and view all the answers

    What is the approximate amount of alveolar ventilation necessary to saturate hemoglobin?

    <p>4L/min</p> Signup and view all the answers

    What is the approximate pulmonary blood flow (Q) at rest?

    <p>5L/min</p> Signup and view all the answers

    What is the course of severe respiratory distress?

    <p>Cyanosis and RVH to death from decompensated cor pulmonale</p> Signup and view all the answers

    What is primary pulmonary hypertension commonly seen in?

    <p>Young adult females</p> Signup and view all the answers

    What is the genetic mutation associated with primary pulmonary hypertension?

    <p>Mutation of bone morphogenetic protein receptor type 2</p> Signup and view all the answers

    What is the effect of endothelin in primary pulmonary hypertension?

    <p>Vasoconstriction and increased pulmonary pressure</p> Signup and view all the answers

    What is a cause of secondary pulmonary hypertension?

    <p>Hypoxemia due to COPD</p> Signup and view all the answers

    What is a consequence of microvascular injury in pulmonary hypertension?

    <p>Narrowing of the lumen and increased pressure in pulmonary circulation</p> Signup and view all the answers

    What is a condition associated with the development of secondary pulmonary hypertension?

    <p>CREST syndrome</p> Signup and view all the answers

    What is a drug associated with the development of secondary pulmonary hypertension?

    <p>All of the above</p> Signup and view all the answers

    What is the reason for lower lung fields being affected more severely in A1AT deficiency?

    <p>They receive relatively greater perfusion</p> Signup and view all the answers

    What is the characteristic feature of biopsy in A1AT deficiency?

    <p>Pink-PAS-positive globules in hepatocytes</p> Signup and view all the answers

    What increases the risk of panacinar emphysema in patients with A1AT deficiency?

    <p>Smoking</p> Signup and view all the answers

    What is the purpose of pursed-lip breathing in patients with emphysema?

    <p>To increase airway pressure</p> Signup and view all the answers

    What is a late complication of emphysema?

    <p>Cor pulmonale</p> Signup and view all the answers

    What type of hypersensitivity is involved in the pathogenesis of asthma?

    <p>Type I hypersensitivity</p> Signup and view all the answers

    What is the primary cause of V/Q mismatch in the context of regional alveoli involvement?

    <p>Decreased ventilation with maintained perfusion</p> Signup and view all the answers

    What is an important preventive measure in patients with asthma?

    <p>Allergen avoidance</p> Signup and view all the answers

    What is a risk factor for developing asthma in children?

    <p>Exposure to second-hand smoke</p> Signup and view all the answers

    Which of the following is a direct cause of pulmonary epithelial and/or endothelial injury?

    <p>Pulmonary contusions</p> Signup and view all the answers

    What is the primary reason for hypoxemia and cyanosis in ARDS?

    <p>Thickened diffusion barrier and collapse of air sacs</p> Signup and view all the answers

    What is the diagnostic criterion for ARDS that indicates respiratory failure is not due to HF/fluid overload?

    <p>Symptoms of respiratory failure are not due to HF/fluid overload</p> Signup and view all the answers

    What is the primary goal of treatment in ARDS?

    <p>Addressing underlying cause</p> Signup and view all the answers

    What is the characteristic of the fluid that accumulates in cardiogenic acute pulmonary edema?

    <p>Transudate</p> Signup and view all the answers

    What is the consequence of left ventricle infarction that leads to the accumulation of fluid in the lung interstitium and alveoli?

    <p>Cardiogenic acute pulmonary edema</p> Signup and view all the answers

    What is the histological representation of cardiogenic acute pulmonary edema?

    <p>Increased filtration of plasma water and electrolytes into the lung interstitium and alveoli</p> Signup and view all the answers

    Study Notes

    Restrictive Pulmonary Disease

    • Characterized by increased elastic recoil, decreased lung compliance, and reduced vital capacity with low lung volumes
    • Examples include acute respiratory distress syndrome (ARDS) and interstitial lung diseases such as sarcoidosis and idiopathic pulmonary fibrosis (IPF)

    Lung Function Measurements

    • TLC (total lung capacity) is smaller than normal in restrictive pulmonary disease
    • FEV1 (forced expiratory volume in 1 second) is reduced, but FEV1/FVC (forced vital capacity) is often increased
    • Critical distinction is low FVC with low FRC (functional residual capacity) and RV (residual volume)

    Distinguishing between Obstructive and Restrictive Patterns

    • Obstructive pattern:
      • TLC and FRC increase
      • FEV1 and FEV1/FVC decrease
    • Restrictive pattern:
      • TLC and FRC decrease
      • FEV1 decreases, but FEV1/FVC increases or remains normal

    Pulmonary Shunt

    • Defined as blood passing through the pulmonary circulation without changing its chemical composition
    • Occurs in areas of regional atelectasis, such as pneumothorax
    • Characterized by PaO2 below pulmonary end-capillary PO2, widening A-a gradient, and no significant increase in PaO2 with supplemental oxygen

    Ventilation-Perfusion Mismatch

    • Alveolar ventilation (VA) necessary to saturate Hb with oxygen is 4L/min
    • Pulmonary blood flow (Q) is 5L/min at rest
    • Lower lung fields may be affected most severely due to greater perfusion

    Emphysema

    • Clinical features:
      • Dyspnea and cough with minimal sputum
      • Prolonged expiration with pursed lips to increase airway pressure and prevent airway collapse
    • Radiographic findings:
      • Increased anterior-posterior diameter of chest (barrel-chest)
      • Flattened diaphragm
      • Increased lung field lucency
    • Complications:
      • Hypoxemia and cor pulmonale are late complications

    Asthma

    • Pathogenesis:
      • Type I hypersensitivity to various stimuli, including physical, chemical, and allergenic irritants
      • lgE-bound to pulmonary mast cells
    • Clinical features:
      • Episodic and related to allergen exposure
      • Presents in childhood, often associated with allergic rhinitis, eczema, and family history of atopy
    • Causes:
      • Direct pulmonary trauma or indirect non-pulmonary insults
      • Pulmonary epithelial and/or endothelial injury

    Acute Respiratory Distress Syndrome (ARDS)

    • Clinical features:
      • Hypoxemia and cyanosis with respiratory distress
      • Respiratory failure within 1 week of alveolar insult
    • Diagnosis:
      • Abnormal chest X-ray (bilateral lung opacities)
      • Decreased Pao2/Fio2 ratio (<300)
      • Symptoms of respiratory failure are not due to heart failure or fluid overload
    • Treatment:
      • Address underlying cause
      • Mechanical ventilation with low tidal volumes and high PEEP
      • Recovery may be complicated by interstitial fibrosis

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    Description

    This quiz covers the characteristics and indicators of restrictive pulmonary disease, including decreased lung compliance, reduced vital capacity, and examples of diseases such as ARDS and interstitial lung diseases.

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