Medical Care
Do not rapidly correct chronic hypernatremia in patients with adipsia. Correct hypernatremia over 48-72 hours, with no more than half of the calculated water deficit replaced during the first 24 hours of therapy. Drop in serum sodium level should be at rate of approximately 0.5 mEq/L/hour or 10-12 mEq/L/day.
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Long-term therapy
The underlying damage to the hypothalamic area is often irreversible.
The goal of medical care is to teach the patient and parents how to maintain adequate fluid intake. [19]
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Treatment options
No pharmacological therapy is currently available.
Behavioral procedures are successful in increasing water intake in some patients.
Electroconvulsive therapy has been used, with mixed results, in patients in whom the underlying cause is psychogenic.
When behavioral therapy fails, the only remaining option is long-term administration of fluids by nasogastric tube or G-button.
Nasal desmopressin acetate (DDAVP) therapy to limit urine output is useful in patients with coexisting central diabetes insipidus. [20]
In adipsic diabetes insipidus, recovery of thirst function after removal of underlying cause can be assessed by a visual analog scale after hypertonic saline infusion. [21]
Surgical Care
Removal of tumors, hematomas, or cysts that compress the thirst center may be curative in selected cases.
Consultations
Obtain the opinion of an oncologist, a neurosurgeon, or both for patients with space-occupying lesions.
Obtain the opinion of an endocrinologist in patients with associated central diabetes insipidus.
Diet and Activity
Diet
No dietary restrictions are necessary.
Frequent and scheduled water intake has to be maintained.
Activity
No restrictions on activity are necessary.
Further Care
Further outpatient care
Monitor the serum electrolyte levels in order to ensure adequate fluid intake. The level of comprehension and compliance of the patient and parents determines the frequency of the visits.
In children with normal aqueous vasopressin (AVP) secretion, measuring urine osmolality may be sufficient for follow-up care. The goal of a urine osmolality is 400-600 mOsm/kg H2O.
Further inpatient care
Patients with adipsia must remain in the hospital until hypernatremia is diagnosed and corrected and until the patient is able to maintain fluid and electrolyte homeostasis.
Transfer
Patients who are unable to achieve an adequate fluid intake may be transferred to a chronic care facility where they can be kept under close supervision and receive behavioral therapy.
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Anatomic relationships between pituitary and hypothalamic areas of interest with respect to regulation of antidiuretic hormone (ADH) secretion and thirst sensation. AN = Anterior (hypothalamic) nucleus; AP = Anterior pituitary; OC = optic chiasm; OVLT = Organum vasculosum of lamina terminalis; PA = Preoptic (hypothalamic) area; PP = Posterior pituitary; PVN = Paraventricular (hypothalamic) nucleus; SON = Supraoptic (hypothalamic) nucleus.