ROHHAD

ROHHAD stands for rapid-onset obesity with hypothalamic dysregulation, hypoventilation and autonomic dysregulation. It is a rare condition of unknown cause that mainly affects the endocrine system and the autonomic nervous system. The first sign is rapid weight gain between the ages of about one and a half and eleven years, usually followed by hypoventilation, or abnormally slow breathing. Patients show both alveolar hypoventilation and hypothalamic dysfunction, which separates ROHHAD from congenital central hypoventilation syndrome. Roughly 40 percent develop neuroendocrine tumors, and some children have behavioral disorders while others develop normally in thought and intelligence. About 100 cases have been reported worldwide. There is no cure and treatment manages each patient symptoms. Without diagnosis and treatment it is fatal in 50 to 60 percent of cases through cardiopulmonary arrest, so earlier diagnosis gives a better outlook.