title: Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation: review and update.
abstract: OBJECTIVE
  The focus of this review is to compare and contrast two orphan disorders of late-onset hypoventilation. Specifically, rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) and congenital central hypoventilation syndrome (CCHS) are distinct in presentation, pathophysiology, and etiology.
  
  
  RESULTS
  While limited new information is available, appreciation and understanding of rare disorders can be attained through case reports. Recent literature in ROHHAD has included case reports with new findings that may provide insight into pathophysiology involving possible aberrant immune process and dysregulation at the level of the orexinergic system.
  
  
  CONCLUSIONS
  The etiology of ROHHAD continues to be elusive. The hope is that, with growing recognition, discussion, and investigation into the overlap of ROHHAD with disorders outside congenital central hypoventilation syndrome, further advancement will be made.
authors.affiliation: Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.
authors.forename: Pallavi P
authors.initials: PP
authors.lastname: Patwari
authors.forename: Lisa F
authors.initials: LF
authors.lastname: Wolfe
mesh_terms: D001342:Autonomic Nervous System Diseases / Q000235:genetics / Q000503:physiopathology*; D014644:Genetic Variation; D006801:Humans; D007040:Hypoventilation / Q000151:congenital* / Q000235:genetics / Q000503:physiopathology*; D047908:Intracellular Signaling Peptides and Proteins / Q000378:metabolism*; D009479:Neuropeptides / Q000378:metabolism*; D018377:Neurotransmitter Agents / Q000378:metabolism*; D009765:Obesity / Q000235:genetics / Q000503:physiopathology*; D064446:Orexin Receptors / Q000378:metabolism; D000068797:Orexins; D010641:Phenotype; D035583:Rare Diseases / Q000503:physiopathology; D020182:Sleep Apnea, Central / Q000235:genetics / Q000503:physiopathology*
chemical_list: D047908:Intracellular Signaling Peptides and Proteins; D009479:Neuropeptides; D018377:Neurotransmitter Agents; D064446:Orexin Receptors; D000068797:Orexins