Perioperative Care of a Child With ROHHADNET Syndrome

Goykhman,

doi:10.4021/jmc1445w

Cited by 7 publications

(13 citation statements)

References 23 publications

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“…Ganglioneuroma and neuroblastoma have been reported in some ROHHAD cases, suggesting paraneoplastic involvement of the hypothalamus ( 2 ). Thus, there is a group of patients comprising 40% of all patients, the term “ROHHAD-neuroendocrine tumors” has been coined in recent years ( 15 ). We screened both cases for the presence of such tumors but no mass lesion was detected.…”

Section: Discussionmentioning

confidence: 99%

A Rare Cause of Hypothalamic Obesity, Rohhad Syndrome: 2 Cases

Şiraz

Ökdemir

Direk

et al. 2018

Jcrpe

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Rapid-onset obesity with hypoventilation, hypothalamic dysfunction and autonomic dysregulation (ROHHAD) syndrome is a rare disease that is difficult to diagnosis and distinguish from genetic obesity syndromes. The underlying causes of the disease have not been fully explained. Hypothalamic dysfunction causes endocrine problems, respiratory dysfunction and autonomic alterations. Currently there are around 80 reported patients although this is likely due to underdiagnosis due to lack of recognition. We present two female patients suspected of ROHHAD due to weight gain starting in early childhood. Clinical and biochemical findings such as respiratory and circulatory dysfunction, hypothalamic hypernatremia, central hypothyrodism, hyperprolactinemia and central early puberty in these patients matched the criteria for ROHHAD syndrome. ROHHAD syndrome should be considered in the differential diagnosis of monogenic obesity.

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Section: Discussionmentioning

confidence: 99%

A Rare Cause of Hypothalamic Obesity, Rohhad Syndrome: 2 Cases

Şiraz

Ökdemir

Direk

et al. 2018

Jcrpe

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“…A 3-year-old female child weighing 40 kg (height of 100 cm and body mass index [BMI] of 40 kg m -2 ), a suspected case of rapid-onset obesity, hypothalamic dysregulation, hypoventilation, autonomic dysregulation, neuroendocrine tumour (ROHHADNET) syndrome was posted for tumour excision. Rapid severe weight gain due to hypothalamic dysfunction is the harbinger of the disease followed by signs of autonomic dysfunction, hypoventilation, and behavioural symptoms.…”

Section: Introductionmentioning

confidence: 99%

Rapid-Onset Obesity, Hypothalamic Dysregulation, Hypoventilation, Autonomic Dysregulation, and Neuroendocrine Tumour: Rare Syndrome with Myriad Anaesthesia Implications

Karnik¹,

Dave²,

Sayyed³

et al. 2022

Turk J Anaesthesiol Reanim

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Anaesthesia management of a child with rapid-onset obesity, hyperphagia, and hypothalamic dysfunction syndrome is complex due to the multisystem involvement, the most important features being morbid obesity, autonomic dysfunction, and dyselectrolytemia due to hypothalamic dysfunction. The acronym of the disease was amended in 2008 to rapid-onset obesity, hyperphagia, hypothalamic dysfunction neural crest tumour to include the risk of ganglioneuroma or ganglioneuroblastoma. Patients usually require removal of tumour in the prone position. Obstructive sleep apnea, difficult airway and intravenous access, and haemodynamic lability all add to the trials faced by the paediatric anaesthesiologist. Invasive haemodynamic monitoring, ultrasonography, bispectral index monitoring, and meticulous calculation of drug dosages help in smoothening the course of anaesthesia in the presence of constant vigilance.

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“…From birth, children with ROHHAD-syndrome grow and develop physiologically, but at the age of 1.5-7 years, a sharp increase in body weight beginsrapidly progressing obesity, weight gain for 6-12 months, it can be from 10 to 20 kg-as a result of hypothalamic dysfunction and polyphagia [3,8,9]. After rapid weight gain, children develop respiratory disorders in the form of obstructive apnea and hypoventilation due to loss of vegetative control over the breathing process.…”

Section: Introduction and Literature Reviewmentioning

confidence: 99%

Issues of diagnosing ROHHAD Syndrome in a 2-year-old girl : A Case Report and Review of Literature

Pushko

Gutyrchik

Ovsyannikov

et al. 2020

MJZ

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We present a clinical case of ROHHAD syndrome. In this article we discuss the rare occurrence of this pathology and, as a result, the issues encountered in the diagnosis and treatment of such children. Thus, in highlighting the problem, the team of Authors set the goal of emphasizing the importance of timely diagnosis, as well as correctly selecting comprehensive treatment for children with ROHHAD syndrome.

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Perioperative Care of a Child With ROHHADNET Syndrome

Cited by 7 publications

References 23 publications

A Rare Cause of Hypothalamic Obesity, Rohhad Syndrome: 2 Cases

A Rare Cause of Hypothalamic Obesity, Rohhad Syndrome: 2 Cases

Rapid-Onset Obesity, Hypothalamic Dysregulation, Hypoventilation, Autonomic Dysregulation, and Neuroendocrine Tumour: Rare Syndrome with Myriad Anaesthesia Implications

Issues of diagnosing ROHHAD Syndrome in a 2-year-old girl : A Case Report and Review of Literature

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