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Key Features

  • Hypoventilation resulting from blunted ventilatory drive and increased mechanical load imposed on the chest

  • Obstructive sleep apnea in most

Clinical Findings

  • Obesity

  • Lethargy, headache, hypersomnolence

  • Loud snoring if accompanied by obstructive sleep apnea

  • Dyspnea often absent

  • Signs of cyanosis and cor pulmonale may be found

  • Many patients have erythrocytosis

Diagnosis

  • Arterial blood gas measurements confirm daytime hypoxemia and hypercapnia, which improve with voluntary hyperventilation

  • Nocturnal sleep study helpful to evaluate for obstructive sleep apnea

Treatment

  • Weight loss is key

  • Noninvasive positive pressure ventilation is helpful in some patients

  • Respiratory stimulants (medroxyprogesterone acetate, 10–20 mg every 8 hours orally, theophylline, acetazolamide) may help

  • If present, obstructive sleep apnea must be treated aggressively (eg, nasal CPAP)

  • Treatment of comorbid conditions

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