title: Hypothalamic adipic hypernatraemia syndrome with normal osmoregulation of vasopressin.
abstract: Adipsic hypernatraemia is an uncommon disorder in childhood caused by a defect in the osmoregulation of thirst, leading to impairment of water homeostasis and chronic hyperosmolality of body fluids. Adipsia is often associated with an abnormality in osmoregulated vasopressin secretion due to the close proximity of the hypothalamic osmoreceptors that control thirst with those regulating vasopressin secretion. Hypothalamic lesions of diverse aetiology (vascular abnormalities, neoplasms, granulomatous diseases, trauma etc.) have been described in this syndrome. We report a 12-year-old boy with evident weight loss due to hypernatraemic dehydration with a selective defect in osmoregulation of thirst and normal vasopressin secretion with no demonstrable structural lesion. To date, only six paediatric patients with this condition have been described in the literature.
  
  
  CONCLUSIONS
  Hypothalamic adipsic hypernatraemia syndrome must be suspected when a dehydrated patient denies thirst. The study of antidiuretic function is necessary because the osmoregulation of vasopressin secretion could be altered.
authors.affiliation: Paediatric Endocrinology Unit, Ramón y Cajal Hospital, Alcalá University, Crta. Colmenar Km 9,1, 28034 Madrid, Spain.
authors.forename: Marta
authors.initials: M
authors.lastname: López-Capapé
authors.forename: Luz
authors.initials: L
authors.lastname: Golmayo
authors.forename: Gustavo
authors.initials: G
authors.lastname: Lorenzo
authors.forename: Nieves
authors.initials: N
authors.lastname: Gallego
authors.forename: Raquel
authors.initials: R
authors.lastname: Barrio
mesh_terms: D001921:Brain / Q000378:metabolism / Q000473:pathology; D002648:Child; D003681:Dehydration / Q000209:etiology; D004326:Drinking / Q000502:physiology; D006801:Humans; D006955:Hypernatremia / Q000150:complications / Q000378:metabolism*; D007027:Hypothalamic Diseases / Q000150:complications / Q000378:metabolism*; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D013577:Syndrome; D014667:Vasopressins / Q000378:metabolism*; D014882:Water-Electrolyte Balance
chemical_list: D014667:Vasopressins