Hypothalamic adipsic hypernatraemia syndrome

Orpha code: 443101OMIM code:

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.

Disease data
Classification

Disease

ORPHA code
443101
OMIM code
-
ICD10 code
E23.3
ICD11 code
-

No additional description.

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