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Severe Hydronephrosis in Nephrogenic Diabetes Insipidus

A 33-year-old woman was admitted to Peking Union Medical College hospital complaining of a 26-year history of polydipsia and polyuria. She had a family history of nephrogenic diabetes insipidus. Blood chemistry was remarkable for elevated sodium >150 mmol/L. A magnetic resonance imaging scan of the patient’s brain revealed no abnormalities in the sella turcica. Water deprivation test demonstrated the presence of vasopressin resistant nephrogenic diabetes insipidus (NDI). Massive bilateral hydronephrosis and megaureter were demonstrated by intravenous pyelography (figure 1A▶) and three-dimensional computed tomography (figure 1B▶) of the kidneys. The patient’s total urine volume decreased by more than 50% after treatment with hydrochlorothiazide 25 mg three times daily and spironolactone once daily. Hydronephrosis was relieved by double-J ureteral stent after ureteroscopy.