Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020440 (hypercapnia)
 7,939 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A wide variety of mechanisms can lead to the hypoventilation associated with various medical disorders, including derangements in central ventilatory control, mechanical impediments to breathing, and abnormalities in gas exchange leading to increased dead space ventilation. The pathogenesis of hypercapnia in obesity hypoventilation syndrome remains somewhat obscure, although in many patients comorbid obstructive sleep apnea appears to play an important role. Hypoventilation in neurologic or neuromuscular disorders is primarily explained by weakness of respiratory muscles, although some central nervous system diseases may affect control of breathing. In other chest wall disorders, obstructive airways disease, and cystic fibrosis, much of the pathogenesis is explained by mechanical impediments to breathing, but an element of increased dead space ventilation also often occurs. Central alveolar hypoventilation syndrome involves a genetically determined defect in central respiratory control. Treatment in all of these disorders involves coordinated management of the primary disorder (when possible) and, increasingly, the use of noninvasive positive pressure ventilation.
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PMID:Hypoventilation syndromes. 2048 85

Central alveolar hypoventilation syndrome (CAHS) is a rare and potentially fatal condition. However, respiratory care for patients with CAHS caused by lateral medullary infarction (CAHS-LMI) remains an important unsolved problem. We describe 2 patients with CAHS-LMI and review the case reports for 17 previously described patients. Patient 1 was a 78-year-old man who was referred to our hospital because of dizziness. After admission, Wallenberg syndrome developed. Magnetic resonance imaging showed left LMI. He had hypercapnia and respiratory acidosis the next afternoon and temporarily received mechanical ventilation. A tracheotomy was performed on the 12th hospital day, and the patient was weaned from the ventilator on the 18th hospital day. Patient 2 was 72-year-old man who was referred to our hospital because of dizziness and gait disturbance. Wallenberg syndrome was diagnosed after admission, and magnetic resonance imaging showed right LMI. His consciousness deteriorated, and hypercapnia developed on the ninth hospital day. The patient received ventilatory support, and a tracheotomy was performed on the 12th hospital day. He was weaned from the ventilator by the 16th hospital day. Consistent with our findings, most previously reported cases of CAHS-LMI were initially associated with mild symptoms, which subsequently worsened. Five of the 19 patients (26.3%) died within 1 month after onset, and 7 patients (36.8%) died within 1 year. Tracheotomy was performed in 12 patients, 2 of whom died 1 month after onset (16.7%); another patient died of chronic renal failure after 2 years. Tracheotomy seemed to be an effective procedure in patients with CAHS-LMI. We speculate that tracheotomy assists alveolar ventilation by reducing dead space ventilation. Closure of the tracheotomy should, therefore, be avoided in patients with CAHS-LMI, even if respiratory status is good.
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PMID:Efficacy of tracheostomy for central alveolar hypoventilation syndrome caused by lateral medullary infarction. 2518 82