Rapid onset obesity with hypothalamic dysfunction

ROHHAD is an acronym for rapid-onset obesity (RO) with hypothalamic dysregulation (H), hypoventilation (H), and autonomic dysregulation (AD).

Obesity

Clinical features

It is a rare, life-threatening condition that affects the autonomic nervous system (which controls involuntary actions like breathing and your heartbeat) and the endocrine system. As the name suggests, the key features include dramatic weight gain over a six- to 12-month period in the first 10 years of life, followed by hypothalamic dysfunction, dysregulation of the autonomic nervous system, and alveolar hypoventilation. Children with ROHHAD appear to have normal growth, development, and general health prior to onset of symptoms. The cause of ROHHAD is currently unknown. Treatment varies based on the signs and symptoms present in each person.

Symptoms

The signs and symptoms of ROHHAD and the severity of the condition vary from person to person. Most children with ROHHAD begin to develop symptoms in the first ten years of life, which often include:

• Dramatic weight gain over a six- to twelve-month period
• Hypothalamic dysfunction such as inability to maintain normal water balance in the body; hypothyroidism; early or late puberty; growth hormone deficiency; and/or high prolactin levels
• Dysregulation of the autonomic nervous system such as inability to regulate body temperature, slow heartbeat, excessive sweating, altered pupil response to light,  (crossed eyes), and/or intestinal abnormalities
• Alveolar hypoventilation with very shallow breathing during sleep

Mild to severe behavioral problems may also be present. Children with ROHHAD are also at an increased risk for certain types of  including ganglioneuromas and ganglioneuroblastomas.

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Cause

The exact cause of ROHHAD is currently unknown. However, scientists suspect that it may be a genetic condition. A number of  have been studied as a possible cause of the condition, but thus far, a disease-causing gene has not been identified.

Inheritance

No  cause of ROHHAD has been identified. However, in rare cases, more than one family member may have the disease. This suggests that it may be inherited in some cases.

Diagnosis

A diagnosis of ROHHAD is typically made based on the presence of the following:

• Rapid-onset obesity and alveolar hypoventilation during sleep starting after the age of one and a half years
• Signs and symptoms of hypothalamic dysfunction
• Exclusion of other condition that cause similar features, such as congenital central hypoventilation syndrome

It can take several months to years for other associated health problems to develop after the onset of rapid weight gain. This means that a child may need to be monitored over an extended period of time before a diagnosis of ROHHAD can be made.

Treatment

There is currently no cure for ROHHAD. Treatment varies based on the signs and symptoms present in each person. Because ROHHAD can affect many parts of the body, children with ROHHAD are often cared for by a team of healthcare providers who specialize in a variety of medical fields. For example:

• Children may be referred to nutrition services to help prevent additional weight gain.
• Hypothalamic dysfunction is often managed by an  who may recommend replacement therapy (such as growth hormone), a strict fluid intake regimen, and/or other measures.
• Children with alveolar hypoventilation will be referred to a  (a doctor who specializes in the diagnosis and treatment of lung conditions) and/or a respiratory therapist who can determine if and when ventilators are needed.
• Due to an increased risk for certain types of  including ganglioneuromas and ganglioneuroblastomas, children with ROHHAD may also be screened periodically for these tumors.

Other healthcare providers who may help with the care of a child with ROHHAD include ,  (doctors who specialize in the diagnosis and treatment of gastrointestinal conditions), , nurses, social workers, speech and language therapists, and special education teachers.

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

• Follitropin alfa, recombinant (Brand name: Gonal-f) - Manufactured by EMD Serono, Inc. FDA-approved indication: For the induction of spermatogenesis in men with primary and secondary hypogonadotropic  in whom the cause of infertility is not due to primary testicular failure. National Library of Medicine Drug Information Portal
• Gonadorelin acetate (Brand name: Lutrepulse) - Manufactured by Ferring Laboratories, Inc. FDA-approved indication: For induction of ovulation in women with hypothalamic amenorrhea due to a deficiency or absence in the quantity or pulse pattern of endogenous GnRH secretion. National Library of Medicine Drug Information Portal

Prognosis

The long-term outlook for people with ROHHAD varies. Children who are diagnosed early and appropriately managed can have a good quality of life. However, if the diagnosis is delayed, the symptoms are not anticipated, and/or the condition is not adequately treated,children with ROHHAD are more likely to have significant behavior problems and are at an increased risk for sudden death. While the number of reported cases of ROHHAD is limited, 25% of children with ROHHAD are reported to die because of respiratory failure.

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NIH genetic and rare disease info

• NIH info on Rapid onset obesity with hypothalamic dysfunction

Rapid onset obesity with hypothalamic dysfunction is a rare disease.

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