Nephrogenic 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.
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