Title of article :
Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and
Autonomic Dysregulation (ROHHAD) Syndrome: A Case Report
Author/Authors :
Bagheri، Bahador نويسنده Pulmonology Department, Labbafi Nejad Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran , , Pourbakhtyaran، Elham نويسنده Department of Surgery, Shariati Hospital, Tehran University of Medical Sciences, Tehran, IR Iran , , Talebi Kiasari، Fateme نويسنده Student Research Committee, School of Medicine, Semnan
University of Medical Sciences, Semnan, Iran , , Taherkhanchi، Bahar نويسنده Pulmonology Department, Labbafi Nejad Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran , , Salarian، Sara نويسنده Department of Anesthesiology, Shahid Beheshti University
of Medical Sciences, Tehran, IR Iran , , SADEGHI، AFSANEH نويسنده ,
Abstract :
Rapid-onset obesity with hypoventilation, hypothalamic dysfunction,
and autonomic dysregulation (ROHHAD) is a rare disease. To date, there
have been only few reported cases of ROHHAD syndrome. We report a
5-year-old- Iranian girl who had normal growth and development until her
4th year of life. At that time, the patient developed weight gain,
constipation, coldness in the extremities, and hyperhidrosis. She first
presented to our children’s hospital with a suspected mediastinal mass
on chest radiography. After surgery and resection of the mass (a
ganglioneuroblastoma), extubation was unsuccessful, with tachycardia and
hypertension occurring after each attempt. After 48 days in the ICU, we
were still unable to wean her from mechanical ventilation. Finally,
based on the rapid weight gain, hypoventilation, hypothalamic
dysfunction, and signs of autonomic dysregulation, the patient was
diagnosed with ROHHAD syndrome. Only a few case reports have described
ROHHAD syndrome, and it is therefore difficult to manage. In children
with rapid and early-onset obesity associated with
hypothalamic-pituitary endocrine dysfunction, ROHHAD syndrome should be
considered in the differential diagnosis.
