2003
DOI: 10.1297/cpe.12.7
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A Sudden Death Due to Central Hypoventilation in A 3-Year-Old Boy with Idiopathic Hypothalamic Dysfunction

Abstract: Abstract. Idiopathic hypothalamic dysfunction is a rare disorder associated with adipsia, obesity and other symptoms such as central hypoventilation without any hypothalamic structural lesion. We report the case of a 3-year-old boy who died suddenly due to central hypoventilation. His obesity index increased from 0% to 75% during the 7 months prior to 3 yr 0 mo. During this clinical course, adipsia, hyperthermia, sudoresis, mild central hypoventilation, blepharoptosia, a change of character, hypernatremia, and… Show more

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Cited by 4 publications
(2 citation statements)
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“…Even if such life‐threatening events can be avoided, there is an ongoing neurocognitive cost to recurrent hypoxemia and hypercarbia during wakefulness and sleep in ROHHAD. The risk of cardiorespiratory arrest and neurocognitive impairment demands that hypoventilation in ROHHAD be a central focus of clinical management . This feature of the disorder is thought to result from a failure of chemosensory drive in response to hypoxia and hypercapnia.…”
Section: Introductionmentioning
confidence: 99%
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“…Even if such life‐threatening events can be avoided, there is an ongoing neurocognitive cost to recurrent hypoxemia and hypercarbia during wakefulness and sleep in ROHHAD. The risk of cardiorespiratory arrest and neurocognitive impairment demands that hypoventilation in ROHHAD be a central focus of clinical management . This feature of the disorder is thought to result from a failure of chemosensory drive in response to hypoxia and hypercapnia.…”
Section: Introductionmentioning
confidence: 99%
“…The risk of cardiorespiratory arrest and neurocognitive impairment demands that hypoventilation in ROHHAD be a central focus of clinical management. 3,[6][7][8][9][10] This feature of the disorder is thought to result from a failure of chemosensory drive in response to hypoxia and hypercapnia. While especially prominent during sleep, 2,11 this blunted chemosensory response has also been identified in awake patients from the earliest clinical case and cohort reports.…”
Section: Introductionmentioning
confidence: 99%