Nephrogenic diabetes insipidus is a disorder in which a defect in the small tubes (tubules) in the kidneys causes a person to pass a large amount of urine and lose too much water.
Normally, the kidney tubules allow most water to be removed and returned to the blood.
Nephrogenic diabetes insipidus (NDI) occurs when the kidney tubules do not respond to a hormone in the body called antidiuretic hormone (ADH), also called vasopressin. ADH normally tells the kidneys to make the urine more concentrated.
As a result of the defect, the kidneys release too much water into the urine. This causes the body to produce a large quantity of very dilute urine.
NDI is rare. Congenital diabetes insipidus is present at birth as a result of defect passed down through families. Usually men are affected, though women can pass the gene on to their children.
Most commonly, NDI develops because of other reasons. This is called an acquired disorder. Factors that can trigger the acquired form of this condition include:
You may have intense or uncontrollable thirst, and crave ice water. You will produce large amounts of urine, usually more than 3 liters, and up to 15 liters per day.
If you do not drink enough fluids, dehydration can result. Symptoms may include:
Other symptoms that can occur due to lack of fluids include:
A physical exam may reveal:
Testing may reveal:
Other tests that may be done include:
The goal of treatment is to control the body's fluid levels. Patients will be given a large amount of fluids. The amount of fluids given should be about equal to the amount of water being lost in the urine.
If the condition is due to a certain medication, stopping the drug may improve symptoms. Never stop taking any medication without first talking to your doctor.
Medicines may be given to improve symptoms.
If a person drinks enough water, this condition will not have significant effects on the fluid or electrolyte balance of the body. Sometimes passing a lot of urine for a long time can cause other electrolyte problems.
NDI that is present at birth is a long-term condition requiring lifelong treatment.
Call your health care provider if you or your child has symptoms of this disorder.
Congenital NDI cannot be prevented.
Treating the disorders that can lead to the acquired form of the condition may prevent it from developing in some cases.
Nephrogenic diabetes insipidus; Acquired nephrogenic diabetes insipidus; Congenital diabetes insipidus
Brown D, Fenton RA. The cell biology of vasopressin action. In: Taal, MW, Chertow GM, Marsden PA, et al, eds. Brenner and Rector's The Kidney. 9th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 11.
Robinson AG, Verbalis JG. Posterior pituitary. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 10.
Updated by: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
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