Nephrogenic diabetes insipidus (DI) is a disorder characterized by polydipsia and polyuria of dilute urine. It is caused by a failure of the kidney to respond to antidiuretic hormone (ADH). Patients will most commonly present with polyuria and polydipsia. Patients without access to free water will develop symptomatic hypernatremia. Other symptoms include waking at night to urinate and bed-wetting. Infants may present with inconsolable crying, irritability, growth retardation, hyperthermia, and weight loss. Children may present with fatigue, anorexia, enuresis, and growth defects.

In neonates, there is an X-linked genetic component to nephrogenic DI that perpetually modifies the kidneys' ability to concentrate urine. This more commonly affects males. Specifically, the AVPR2 gene (which codes vasopressin receptor) and AQP2 gene (which codes aquaporin) have been implicated in inherited nephrogenic DI. Other medical conditions that predispose to nephrogenic DI include polycystic kidney disease, sickle cell disease, acute or chronic renal failure, ureteral obstruction, chronic hypokalemia, chronic hypercalcemia, or medication side effects, with lithium and demeclocycline as common offending agents.

Related topics: Central diabetes insipidus, Diabetes insipidus