Weight management in youth with rapid-onset obesity with hypothalamic dysregulation, hypoventilation, autonomic dysregulation, and neural crest tumor (ROHHAD-NET): literature search and case report

Author(s):  
Ian Marpuri ◽  
Esther Ra ◽  
Monica N. Naguib ◽  
Alaina P. Vidmar

Abstract Objectives Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation, and neural endocrine tumor (ROHHAD-NET) syndrome is a youth-onset constellation of symptoms including rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation. Despite growing understanding of the clinical classification of this syndrome there is limited investigation into treatment of the rapid-onset obesity which can be progressive and life-limiting. The purpose of this case report is to describe the clinical timeline and treatment of severe obesity in a patient with of ROHHAD-NET and propose recommendations for the treatment of associated obesity. Case presentation We present the case of a 10-year-old female with a clinical presentation consistent with ROHHAD-NET who achieved clinically meaningful weight loss with a combination of lifestyle modification and anti-obesity pharmacotherapies. We report on the use of three separate pharmacological agents and ultimately the referral for bariatric surgery. Conclusions Given that early-onset obesity and hypoventilation are life-limiting components of this condition, early recognition and treatment are essential to improve health outcomes.

2016 ◽  
Vol 5 (1) ◽  
Author(s):  
Bahador Bagheri ◽  
Elham Pourbakhtyaran ◽  
Fateme Talebi Kiasari ◽  
Bahar Taherkhanchi ◽  
Sara Salarian ◽  
...  

2020 ◽  
Vol 105 (7) ◽  
pp. 2119-2131 ◽  
Author(s):  
Julie Harvengt ◽  
Caroline Gernay ◽  
Meriem Mastouri ◽  
Nesrine Farhat ◽  
Marie-Christine Lebrethon ◽  
...  

Abstract Context Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, autonomic dysregulation and neural crest tumor (ROHHHAD[NET]) is a rare and potentially fatal disease. No specific diagnostic biomarker is currently available, making prompt diagnosis challenging. Since its first definition in 2007, a complete clinical analysis leading to specific diagnosis and follow-up recommendations is still missing. Objective The purpose of this work is to describe the clinical timeline of symptoms of ROHHAD(NET) and propose recommendations for diagnosis and follow-up. Design We conducted a systematic review of all ROHHAD(NET) case studies and report a new ROHHAD patient with early diagnosis and multidisciplinary care. Methods All the articles that meet the definition of ROHHAD(NET) and provide chronological clinical data were reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis individual patient data guidelines. The data were grouped into 7 categories: hypothalamic dysfunction, autonomic dysregulation, hypoventilation, NET, psychiatric symptoms, other clinical manifestations, and outcome. Results Forty-three individual patient data descriptions were analyzed. The timeline of the disease shows rapid-onset obesity followed shortly by hypothalamic dysfunction. Dysautonomia was reported at a median age of 4.95 years and hypoventilation at 5.33 years, or 2.2 years after the initial obesity. A NET was reported in 56% of the patients, and 70% of these tumors were diagnosed within 2 years after initial weight gain. Conclusion Because early diagnosis improves the clinical management and the prognosis in ROHHAD(NET), this diagnosis should be considered for any child with rapid and early obesity. We propose guidance for systematic follow-up and advise multidisciplinary management with the aim of improving prognosis and life expectancy.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
M. Z. van der Horst ◽  
F. van Houwelingen ◽  
J. J. Luykx

Abstract Background Clozapine is an atypical antipsychotic proven to be superior in the treatment of treatment-resistant schizophrenia. Myocarditis is a rare, but well-known complication of treatment with clozapine. Only few cases have been reported in which nausea and vomiting were prominent symptoms. This is the first described report in which nausea and vomiting were the only presenting symptoms of clozapine-induced myocarditis. Case presentation We report a case of a 58-year-old woman, suffering from schizoaffective disorder, who is being treated with clozapine. Two weeks after initiation of clozapine, she developed nausea and vomiting, in absence of any other clinical symptoms. Laboratory examination and magnetic resonance imaging confirmed the diagnosis of clozapine-induced myocarditis. Clozapine was discontinued and the patient recovered fully. Conclusions This case emphasizes the importance of recognizing myocarditis as a cause of isolated nausea and vomiting in patients treated with clozapine. Early recognition improves clinical outcome and reduces mortality.


PEDIATRICS ◽  
2007 ◽  
Vol 120 (1) ◽  
pp. e179-e188 ◽  
Author(s):  
D. Ize-Ludlow ◽  
J. A. Gray ◽  
M. A. Sperling ◽  
E. M. Berry-Kravis ◽  
J. M. Milunsky ◽  
...  

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