Hypothalamic adipsic hypernatraemia syndrome
ORPHA:443101· ICD-10 E23.3
Definition
A rare endocrine disease characterized by severe chronic hypernatremic dehydration caused by decreased intake of water based on impaired thirst perception, due to a selective defect in hypothalamic osmoregulation of thirst. Structural hypothalamic lesions are absent and arginine vasopressin secretion is normal.
- Inheritance
- Not applicable