IMPE Abstracts

Diabetes insipidus (DI) is characterized by polydipsia and polyuria with a dilute urine. Central DI (CDI) results from a deficiency of arginine vasopressin (AVP) and may be due to congenital or acquired causes, the latter include tumors affecting the hypothalamic-pituitary region. Thirst acts as a central biological backup mechanism in the absence of AVP, allowing plasma osmolarity to remain stable. We describe ADI patients with hypothalamic damage, who lose the ability to perceive thirst. A 12-year-old male patient diagnosed with craniopharyngioma at 2 years of age, with multiple surgical interventions and proton therapy. He developed hypopituitarism replaced with cortisol, levothyroxine, desmopressin and growth hormone supplementation, with maternal supervision and good adherence. He had two admissions because of hypernatremia during the last year. In the most recent admission he presented with confusion and heart rate 110, blood pressure 90/70 and was admitted With 162,2 de sodio. Thirst scale = 0 (does not feel thirsty). Management of hypernatremia was initiated by increasing the dose of desmopressin, ensuring free water intake by schedule and control of sodium, balance and daily weight for adjustment. The plasma sodium concentration was normal the third day, reaching less than 147.3mEq/L. The patient was discharged with desmopressin (total dose 0.375mg/day) and trained to drink 1500 cc/day of water. ADI is a rare complication part of hypothalamic syndrome associated with extensive hypothalamic damage that is more apparent in patients with absent baroregulation of AVP release as our patient. Management of ADI is complex and difficult and patients have a high rate of potentially fatal complications including hypernatremic dehydration. Close clinical and biochemical follow up and intensive patient education are essential to optimize patient outcomes.