title: ROHHADNET syndrome presenting as major behavioral changes in a 5-year-old obese girl.
abstract: Behavioral issues are a frequent problem in the pediatric population. Often, these are evaluated and considered to be psychiatric in origin. We report on a pediatric patient who presented with severe behavioral disturbance and developed organic symptoms including hypoventilation and dysautonomia and who was ultimately diagnosed with ROHHADNET syndrome, a syndrome of rapid-onset obesity, hypothalamic dysfunction, hypoventilation, and autonomic dysregulation associated with a neuroendocrine tumor. Autopsy findings revealed novel findings of the syndrome, including hypothalamic encephalitis.
authors: Division of Pediatric Critical Care, Department of Pediatrics, karen_sethi@urmc.rochester.edu.
authors: Karen
authors: K
authors: Sethi
authors: Division of Pediatric Surgery, Department of Surgery, and.
authors: Yi-Horng
authors: YH
authors: Lee
authors: Division of Pediatric Critical Care, Department of Pediatrics.
authors: L Eugene
authors: LE
authors: Daugherty
authors: Division of Pediatric Critical Care, Department of Pediatrics.
authors: Andrea
authors: A
authors: Hinkle
authors: Department of Pathology, and Laboratory Medicine University of Rochester School of Medicine and Dentistry, Rochester, New York.
authors: Mahlon D
authors: MD
authors: Johnson
authors: Department of Pathology, and Laboratory Medicine University of Rochester School of Medicine and Dentistry, Rochester, New York.
authors: Philip J
authors: PJ
authors: Katzman
authors: Division of Pediatric Critical Care, Department of Pediatrics.
authors: John S
authors: JS
authors: Sullivan
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000473:pathology; D001342:Autonomic Nervous System Diseases / Q000150:complications* / Q000473:pathology; D001921:Brain / Q000473:pathology; D002653:Child Behavior Disorders / Q000209:etiology*; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D018305:Ganglioneuroblastoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D006963:Hyperphagia / Q000150:complications; D007027:Hypothalamic Diseases / Q000150:complications* / Q000473:pathology; D007040:Hypoventilation / Q000150:complications*; D009765:Obesity; D013577:Syndrome
keywords: ROHHADNET