Diabetes Insipidus

Diabetes insipidus is a rare disorder that occurs when the kidneys release a large amount of fluid through the urine. Children with diabetes insipidus can pass between three and 20 quarts of urine in one day. By comparison, a healthy child typically passes one to two quarts of fluid a day.

Approximately 1 in 30,000 children has diabetes insipidus. These children either don’t have enough of the hormone vasopressin or their kidneys do not respond to it. As a result, too much water is released into their urine. Insipid means that the urine is diluted and has no odor.

Although the names are similar, diabetes insipidus is different from diabetes mellitus, which includes type 1 diabetes and type 2 diabetes. Both types of diabetes cause frequent urination and thirst, but children with type 1 or type 2 diabetes have high blood sugar. Children with diabetes insipidus have normal blood sugar levels.

Prompt diagnosis and treatment of diabetes insipidus in children is very important. If left untreated, the condition can lead to severe dehydration, which can cause brain damage or impaired mental function.

There are two basic types of diabetes insipidus: central diabetes insipidus and nephrogenic diabetes insipidus.

• Central diabetes insipidus occurs when the body doesn’t produce enough vasopressin. About 98 percent of children with diabetes insipidus have this type of the condition. It’s usually caused by a problem with the brain or central nervous system, and can be easily treated with medication.
• Nephrogenic diabetes insipidus occurs when the kidneys cannot respond to vasopressin. This type is rare and much more difficult to treat.

Each child may exhibit slightly different symptoms, but some of the most common symptoms of diabetes insipidus include:

• Excessive thirst
• Excessive urine production during the day and at night
• Dehydration

Infants with diabetes insipidus may also have the following symptoms:

• Irritability
• Poor feeding
• Stalled growth
• High fever

About 30 to 50 percent of cases of diabetes insipidus are idiopathic, meaning they have no known cause.

Both central and nephrogenic diabetes insipidus can be caused by several conditions, including:

• Tumor
• Damage to the hypothalamus or pituitary gland during surgery
• Too little vasopressin produced by hypothalmus
• Failure of the pituitary gland to release vasopressin into the bloodstream
• Brain injury
• Family heredity

Diabetes insipidus is not typically apparent at birth. Often it is diagnosed when parents bring an infant who is dehydrated in to their doctor.

In addition to taking a complete medical history and physical examination, your child's physician may ask about the following:

• How much fluid your child is drinking?
• How much and what kind of food your child is eating?
• How often and how regularly your child goes to the bathroom (both bowel and bladder)?

The following tests help diagnose diabetes insipidus:

• Urine tests allow doctors to compare how concentrated (or diluted) a child’s urine is.
• Blood tests to see if the child’s blood is more concentrated than their urine, which could indicate diabetes insipidus.
• Water deprivation tests withhold water from the child for a short period of time so their doctor can observe them. If the child gets dehydrated more quickly than normal, they may have diabetes insipidus. This test should only be performed at a hospital.
• Magnetic resonance imaging (MRI), an imaging study, is a way for clinicians to check for pituitary abnormalities.

Treatment for diabetes insipidus depends on the type and cause of the condition. If caused by surgery, the condition may be temporary and your child should be monitored and kept hydrated until the condition resolves on its own.

• Central diabetes insipidus can be effectively treated with a medication called desmopressin (DDAVP). Your child will take DDAVP twice a day, which should allow them to lead a healthy, active life.
• Nephrogrenic diabetes insipidus is not as easy to treat, but if they drink plenty of fluids and eat a diet low in salt, children with the condition can still lead relatively normal lives.

Sometimes, other medications can be used to help control the condition.

Central diabetes insipidus can be a temporary or a permanent condition, depending on what’s causing it. If the condition is permanent, it’s typically easily treated with medication. Almost all children with central diabetes insipidus lead full, healthy lives.

Children with nephrogenic diabetes insipidus can also lead relatively normal lives with proper medical care and management.

The Division of Endocrinology at Boston Children’s Hospital provides comprehensive diagnosis, treatment, and management of children with diabetes insipidus and other disorders associated with the endocrine system.

As one of the largest pediatric endocrinology programs in the world, we have 35 doctors who see more than 7,000 patients every year. We see many children with temporary or permanent diabetes insipidus brought on by surgery, and we know how to help patients and their families manage the condition and live healthy lives.