Abstract

Adipsic arginine vasopressin deficiency (AAVP-D) is caused by hypothalamic dysfunction (HD) due to varied aetiologies, including craniopharyngiomas. Its management is extremely challenging because two of the three regulatory mechanisms (thirst, arginine vasopressin, and renal medullary tonicity) for maintaining plasma osmolality are impaired (thirst, arginine vasopressin). These individuals develop severe dehydration and hypernatraemia, necessitating multiple emergency visits despite the administration of arginine vasopressin analogue (desmopressin). Here, we describe a case with HD secondary to craniopharyngioma treatment manifesting as AAVP-D, short stature, central hypothyroidism, hypoadrenalism and probable hypogonadism. AAVP-D was managed by fixing the renal water losses (using desmopressin) and water intake in a predetermined range. The treatment monitoring was done using total body weight and weekly to fortnightly serum sodium. Using this approach, the serum sodium was maintained in the range of 140–150 mmol/L, and there were no emergency visits for hypernatraemia or hyponatraemia (dysnatraemia) at 3 months.