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Hypoventilation syndromes & Congenital Central Hypoventilation Syndrome (CCHS)

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Hypoventilation syndromes & Congenital Central Hypoventilation Syndrome (CCHS)

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Questions and Answers

What is the purpose of regulating minute ventilation?

To control heart rate

To maintain arterial blood gases and stabilize pH (correct)

To control digestion

To regulate body temperature

Minute ventilation refers to the:

Volume of air inhaled and exhaled with each breath

Rate at which the heart pumps blood

Amount of oxygen in the blood

Rate and depth of breathing (correct)

Chronic hypoventilation is characterized by:

Reduced tidal volume and rate

Hypercapnia

Normal pH as compensation mechanisms initiated - Increased in bicarbonate levels

All of the above (correct)

Congenital central alveolar hypoventilation syndrome is associated with a failure of:

Central respiratory drive Signup and view all the answers

Which gene is commonly mutated in Congenital central alveolar hypoventilation syndrome?

PHOX2B Signup and view all the answers

The mutations in the PHOX2B gene associated with Congenital central alveolar hypoventilation syndrome can be:

Autosomal dominant Signup and view all the answers

Which of the following best describes the breaths taken in Congenital central alveolar hypoventilation syndrome?

Shallow breaths Signup and view all the answers

How can individuals with Congenital central alveolar hypoventilation syndrome stay alive?

Through mechanical ventilation or diaphragm pacing during sleep Signup and view all the answers

Which of the following is the recommended management for Congenital Central Alveolar Hypoventilation Syndrome (CCHS)?

Chronic ventilatory support Signup and view all the answers

What type of ventilation is commonly used in younger children with CCHS?

Portable positive pressure ventilators Signup and view all the answers

What type of ventilation is commonly used in older children and adults with CCHS?

Mechanical ventilation Signup and view all the answers

How often is bronchoscopy typically performed for individuals with CCHS?

Every 12-24 months Signup and view all the answers