Abstract
A 38-year-old man was referred to the department of nephrology for the evaluation of bilateral hydronephrosis. The patient had a history of polyuria since childhood for which he had been using a desmopressin nasal spray for >20 years without a clear diagnosis. On physical examination, he had no costovertebral angle tenderness or peripheral edema. The family history revealed that his grandmother, father, aunt, uncle, and sister had similar manifestations. His laboratory findings included a serum sodium level of 140 mmol/l, a urine osmolality of 454 mOsm/kg, a serum creatinine level of 1.52 mg/dl, and basal pituitary hormone levels within the normal range.
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