ROHHAD
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Rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation (ROHHAD) is a newly described syndrome that can cause cardiorespiratory arrests and death.[1] As the name suggests, the most characteristic manifestions include rapid-onset obesity in the first 10 years of life, followed by hypothalamic dysfunction and then onset of symptoms of autonomic dysregulation (impairment of the autonomic nervous system) with later onset of alveolar hypoventilation (reduced lung function). The cause of this syndrome, once known as late-onset central hypoventilation syndrome with hypothalamic dysfunction (LO-CHS/HD), is unknown. Although there is no cure for this condition at this time, early diagnosis and proper management may help to avoid catastrophic consequences.[2]
References
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Bougneres P, Pantalone L, Linglart A, Rothenbuhler A, Le Stunff C. . J Clin Endocrinol Metab. October 2008;:. Available at: http://jcem.endojournals.org/cgi/rapidpdf/jc.2008-0238v1.pdf. January 2, 2009.
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Ize-Ludlow D, Gray JA, Sperling MA, Berry-Kravis EM, Milunsky JM, Farooqi IS, Rand CM, Weese-Mayer DE. . Pediatrics. July 2007;:. Available at: http://pediatrics.aappublications.org/cgi/content/full/120/1/e179. January 2, 2009.
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PubMed lists journal articles that discuss ROHHAD. Click on the link to go to PubMed and review citations to these articles.
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The Children's Memorial Hospital has developed an information page on ROHHAD. Click on the link to access the information.
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Ize-Ludlow D, Gray JA, Sperling MA, Berry-Kravis EM, Milunsky JM, Farooqi IS, Rand CM, Weese-Mayer DE. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation Presenting in Childhood. Pediatrics 2007;120;e179-e188.
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Bougneres P, Pantalone L, Linglart A, Rothenbuhler A, Le Stunff C. Endocrine Manifestations of the Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neural Tumor Syndrome in Childhood. J Endocrinol Metab. 2008 Oct;93(10):3971-80. Epub 2008 Jul 15.
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