Research Article

Rapid-Onset Obesity with Hypoventilation, Hypothalamic, Autonomic Dysregulation, and Neuroendocrine Tumors (ROHHADNET) Syndrome: A Systematic Review

Table 5

Treatment of case-reported patients with ROHHADNET syndrome (IPD).

TreatmentTotal number of patients (n = 48)
Number of patients ()

Respiratory support21 (43.8)
 Mechanical ventilation20 (41.7%)
 BIPAP7 (14.6%)
 CPAP1 (2.1%)
 Noninvasive mask1 (2.1%)
 Tracheostomy6 (12.5%)
Steroids7 (14.6)
 Methylprednisolone2 (4.2%)
 Steroid pulse therapy2 (4.2%)
 Prednisolone2 (2.1%)
 Dexamethasone1 (2.1%)
Fluid resuscitation4 (8.3)
Intravenous immunoglobulins7 (14.6)
Immunosuppressive agents7 (14.6)
 Rituximab5 (10.4%)
 Cyclophosphamide6 (12.5%)
Other agents
 Antipsychotics3 (6.3%)
 Desmopressin acetate2 (4.2%)
 GH replacement2 (4.2%)
 Anti-epileptics2 (4.2%)
 Levothyroxine4 (8.3%)
 Caffeine1 (2.1%)
 Hypertensive medication1 (2.1%)
Procedure2 (4.2)
 Brain hypothermia1 (2.1%)
 Tonsillectomy1 (2.1%)

BIPAP: bilevel positive airway pressure; CPAP: Continuous Positive Airway Pressure; GH: growth hormone.