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ROHHAD
Other Names for this Disease
- Rapid-onset obesity with hypothalamic dysfunction, hypoventilation and autonomic dysregulation
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Overview
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 features 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
- 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 (ROHHADNET) syndrome in early childhood. J Clin Endocrinol Metab. October 2008. http://jcem.endojournals.org/cgi/rapidpdf/jc.2008-0238v1.pdf. Accessed November 28, 2011.
- 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. July 2007. http://pediatrics.aappublications.org/cgi/content/full/120/1/e179. Accessed November 28, 2011.
Your Questions Answered
by the Genetic and Rare Diseases Information Center1 question(s) from the public on ROHHAD have been answered. See questions and answers. You can also submit a new question.
On this page
General Information
- The Children's Memorial Hospital has developed an information page on ROHHAD. Click on the link to access the information.
- The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.
- PubMed is a searchable database of medical literature and lists journal articles that discuss ROHHAD. Click on the link to view a sample search on this topic.
Selected Full-Text Journal Articles
- 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.
- 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.