Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation: Review and update

Pallavi P. Patwari*, Lisa F. Wolfe

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

19 Scopus citations

Abstract

PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.

Original languageEnglish (US)
Pages (from-to)487-492
Number of pages6
JournalCurrent opinion in pediatrics
Volume26
Issue number4
DOIs
StatePublished - Aug 2014

Keywords

  • genetic
  • hypothalamic dysfunction
  • hypoventilation
  • obesity
  • paraneoplastic

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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