Nephrogenic Diabetes Insipidus

What is nephrogenic diabetes insipidus?

Nephrogenic diabetes insipidus is a medical disorder that occurs when your kidneys can’t properly balance bodily fluids. Your body fails to respond properly to a natural hormone called antidiuretic hormone (ADH, also called vasopressin). Therefore, it produces too much urine, which can cause rapid, sometimes dangerous dehydration.

How common is nephrogenic diabetes insipidus?

Scientists aren’t sure how many people have nephrogenic diabetes insipidus, but they believe it’s rare.

What are the different types of diabetes insipidus?

There are four types of diabetes insipidus:

• Central diabetes insipidus involves damage to your pituitary gland or hypothalamus. Because of the damage, your body can’t produce, store or release ADH normally.
• Nephrogenic diabetes insipidus makes your kidneys unable to respond properly to ADH.
• Dipsogenic diabetes insipidus (also called primary polydipsia) occurs when your hypothalamus has a defective thirst mechanism. It makes you feel excessively thirsty and drink too much liquid.
• Gestational diabetes insipidus occurs only during pregnancy.

What’s the difference between nephrogenic diabetes insipidus and diabetes mellitus?

Although nephrogenic diabetes insipidus and diabetes mellitus can have some similar symptoms, the two conditions are entirely different. Diabetes mellitus is much more common. It involves high blood sugar levels due to a lack of or improper use of the hormone insulin.

What causes nephrogenic diabetes insipidus?

Your kidneys are responsible for filtering blood to remove waste and excess fluid from your body, in the form of urine. ADH (vasopressin) is a hormone that helps your body maintain the balance between fluid intake and urine output.

In a healthy person, ADH helps the body understand when to make less urine. For example, when a person sweats a lot or doesn’t drink enough fluids, higher levels of vasopressin tell the kidneys to make less urine. When a person has enough fluid in their body, lower levels of ADH tell the kidneys to make urine.

But if your body doesn’t know how to respond properly to vasopressin, then your kidneys make too much urine. This is what happens with nephrogenic diabetes insipidus. You can inherit the condition or develop it at any point during your life.

The acquired form is more common and results when something damages your body’s ability to respond to ADH. Examples include conditions that affect your kidneys, such as:

• Amyloidosis.
• Certain types of cancer.
• Chronic kidney disease.
• Kidney infection (pyelonephritis).
• Polycystic kidney disease.
• Medullary sponge kidney.

Other things that can cause nephrogenic diabetes insipidus include:

• Certain medications (for example, lithium).
• Low levels of potassium in the blood (hypokalemia).
• High levels of calcium in the blood (hypercalcemia).
• Sickle cell anemia.
• Sjögren’s syndrome.
• Urinary tract obstruction.

The hereditary form involves mutations to the AVPR2 or AQP2 gene. Those genes provide your body with information about how much water to include in urine. The signs and symptoms of hereditary nephrogenic diabetes insipidus usually appear within the first few months of life.

What are the symptoms of nephrogenic diabetes insipidus?

A person with nephrogenic diabetes insipidus is likely to have the following symptoms:

• Symptoms of dehydration such as dry mouth, fatigue and dizziness.
• Excessive thirst.
• Large amounts of urine output (more than 3 liters a day for adults and 2 liters a day for children).

In an infant, signs of nephrogenic diabetes insipidus may include:

• Diarrhea.
• Failure to thrive and gain weight.
• Fever.
• Irritability.
• Poor appetite.
• Seizures.
• Vomiting.

How is nephrogenic diabetes insipidus diagnosed?

A healthcare provider can diagnose nephrogenic diabetes insipidus based on:

• Blood tests.
• Symptoms.
• Urine tests.
• Water deprivation test, which involves not drinking any liquid for several hours and then assessing urine output.
• Magnetic resonance imaging of your pituitary gland to look for abnormalities.
• Genetic testing if you have a family history of diabetes insipidus.

Is there a cure for nephrogenic diabetes insipidus?

There’s no cure for nephrogenic diabetes insipidus.

Your provider can try to reverse the condition by changing any medications that contribute to the issue. Similarly, treating any underlying conditions may help reverse nephrogenic diabetes insipidus.

How is nephrogenic diabetes insipidus treated?

For irreversible cases of nephrogenic diabetes insipidus, treatment aims to prevent dehydration. Strategies include:

• Changes in diet.
• Frequent drinks of water throughout the day, whenever you feel thirsty, and especially when you’re hot or sweating.
• Medications that decrease the volume of urine, such as thiazide diuretics and prescription nonsteroidal anti-inflammatory drugs.

What foods or drinks should I avoid with nephrogenic diabetes insipidus?

Your healthcare provider may recommend limiting alcohol, caffeine, salt and protein.

How can I prevent nephrogenic diabetes insipidus?

There are no proven strategies to prevent nephrogenic diabetes insipidus. But avoiding certain medications and promptly treating underlying health problems can reduce your risk.

What can I expect if I have nephrogenic diabetes insipidus?

With appropriate treatment, people with nephrogenic diabetes insipidus usually have few complications and live a normal lifespan.

But without proper management, nephrogenic diabetes insipidus can cause serious complications:

• Dehydration.
• Infections such as urinary tract infections and kidney infections.
• Developmental delays, brain damage and intellectual disability in infants.
• Kidney failure.
• Electrolyte disturbance.

When should I seek medical attention for nephrogenic diabetes insipidus?

If you have any concerns about urine output, talk to a healthcare provider.

The average amount of urine output for an adult varies widely, especially depending on how much you drink. But if you’re peeing more than 2 liters per day or very frequently (more than seven times a day), that may signify a problem.

Consider keeping a journal to track your fluid intake and urine output before you talk to a healthcare provider.

A note from Cleveland Clinic

Nephrogenic diabetes insipidus is a disorder involving urine output. When your body fails to respond properly to a natural hormone called antidiuretic hormone (vasopressin), it produces too much urine. That can cause rapid, sometimes dangerous dehydration. If you suspect this condition in yourself or your child, talk to a healthcare provider.