Canadian respiratory journal : journal of the Canadian Thoracic Society
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Review Practice Guideline
Managing dyspnea in patients with advanced chronic obstructive pulmonary disease: a Canadian Thoracic Society clinical practice guideline.
Dyspnea is a cardinal symptom of chronic obstructive pulmonary disease (COPD), and its severity and magnitude increases as the disease progresses, leading to significant disability and a negative effect on quality of life. Refractory dyspnea is a common and difficult symptom to treat in patients with advanced COPD. There are many questions concerning optimal management and, specifically, whether various therapies are effective in this setting. ⋯ There is also no evidence to support the use of supplemental oxygen to reduce dyspnea in nonhypoxemic patients with advanced COPD. Recognizing the current unfamiliarity with prescribing and dosing of opioid therapy in this setting, a potential approach for their use is illustrated. The role of opioid and other effective therapies in the comprehensive management of refractory dyspnea in patients with advanced COPD is discussed.
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Case Reports
Presentation and treatment of monozygotic twins with congenital central hypoventilation syndrome.
Congenital central hypoventilation syndrome is a rare genetic disorder characterized by hypoventilation during sleep secondary to a blunted response to hypercapnia and hypoxia. The current case report describes developmentally normal four-year-old monozygotic twin boys who presented in infancy with variable presentations and clinical severity of congenital central hypoventilation syndrome. Both were managed with noninvasive positive pressure ventilation.
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Achalasia is characterized by obstruction of the distal esophagus and subsequent dilation of the proximal esophagus, and is considered to be a rare disorder in children. Patients commonly present with gastrointestinal (GI) symptoms such as dysphagia; however, pulmonary symptoms may also occur. Rare pulmonary symptoms due to achalasia are dyspnea and wheeze due to tracheal compression. ⋯ The presence of GI symptoms, in addition to abnormalities on chest radiograph and spirometry, suggested the presence of achalasia. The diagnosis was confirmed and the patient subsequently underwent surgical myotomy that relieved his GI and pulmonary symptoms, and normalized spirometry. The present article is an illustrative case report to remind pediatricians to consider other diagnoses when a patient does not respond to asthma medications.