Nephrogenic Diabetes Insipidus - Diabetes Insipidus

What is it?
Nephrogenic diabetes insipidus is a disorder in which the kidneys produce large amounts of dilute urine. Dilute urine has a high water content, while urine that is lower in water is more concentrated. Normally, the kidneys control the concentration of the urine in response to the body’s need for water by absorbing water and returning it to the blood.
Who gets it?

Nephrogenic diabetes insipidus is hereditary in males. It can also occur in people who take drugs such as lithium, demeclocycline, and aminoglycosides.
What causes it?

The gene that causes nephrogenic diabetes insipidus is carried on the X chromosome. Women with this gene can pass the disease to their sons, and it is usually males who develop symptoms. The body naturally produces an antidiuretic hormone (ADH), which tells the kidneys to return more water to the blood and concentrate the urine. However, in people with nephrogenic diabetes insipidus, the kidneys ignore this hormone and continue to produce dilute urine. When nephrogenic diabetes insipidus is induced by a drug, the drug damages the kidneys so they are unable to respond to the ADH.
What are the symptoms?

The symptoms of nephrogenic diabetes insipidus are extreme thirst, called polydipsia, and the excretion of large amounts of dilute urine, called polyuria. When the condition is hereditary, these symptoms appear at birth. Infants who do not receive enough fluids to replace the water lost through the urine may become dehydrated. Prolonged dehydration affects all the body’s tissues, including the brain cells. Untreated, nephrogenic diabetes insipidus can cause brain damage and affect physical growth.
How is it diagnosed?

To diagnose nephrogenic diabetes insipidus, doctors evaluate the symptoms and test the blood and urine for levels of water and sodium. If you are an adult, your doctor will need to know if you take any medications that can cause nephrogenic diabetes insipidus. To confirm a diagnosis, your doctor will test the kidney’s response to the antidiuretic hormone (ADH).
What is the treatment?

There is no cure for nephrogenic diabetes insipidus. Treatment involves preventing dehydration by drinking water at the first signs of thirst. In infants and children who may not readily communicate their thirst, it is the responsibility of the adult to provide water frequently. Your doctor may also prescribe certain drugs that help with this disorder. With treatment, infants with nephrogenic diabetes insipidus usually develop normally.
Self-care tips

If you or your child has nephrogenic diabetes insipidus, always make sure you carry water with you when away from home.

This information has been designed as a comprehensive and quick reference guide written by our health care reviewers. The health information written by our authors is intended to be a supplement to the care provided by your physician. It is not intended nor implied to be a substitute for professional medical advice.
October 31, 2006
Penn State Milton S. Hershey Medical Center
1-800-243-1455