Diabetes Insipidus in Children

Diabetes insipidus is an uncommon condition that causes excessive production of very diluted urine and, as a result, intense thirst.

In healthy individuals, antidiuretic hormone (ADH, also called vasopressin) helps the kidneys correctly regulate the amount of water in the body. ADH is secreted by the hypothalamus (a small gland located at the base of the brain), stored in the pituitary gland, and then released into the bloodstream.

The underlying problem in diabetes insipidus is either too little ADH reaching the kidneys or the failure of the kidneys to respond to it. For either reason, when a person has diabetes insipidus the kidneys are unable to concentrate the urine, and large amounts of dilute urine are released.

The disease is separated into two categories:

• Central diabetes insipidus results from an insufficient production or secretion of ADH.
• Nephrogenic diabetes insipidus results from a lack of kidney response to normal levels of ADH.

Note that diabetes insipidus is not the same as diabetes mellitus, a more common and better-known disorder in the production of a different hormone, insulin, that can also lead to frequent urination.

Central diabetes insipidus can be the result of damage to the hypothalamus or pituitary gland caused by:

• Brain surgery
• Brain malformation
• Tumor in or near the pituitary gland
• Inflammation of the pituitary gland (hypophysitis)
• Langerhan cell histiocytosis
• Some genetic disorders
• Head injury
• Interruption of the blood supply to the pituitary gland
• Encephalitis (inflammation of the brain)
• Meningitis (inflammation of the meninges, the membranes that cover the brain and spinal cord)
• Sarcoidosis (a rare inflammation of the lymph nodes and other tissues throughout the body)
• Tuberculosis

Nephrogenic diabetes insipidus is the result of a defect in the kidneys, which may be caused by:

• Certain medications, such as lithium
• Genetic disorder
• Kidney disease
• High level of calcium in the body (hypercalcemia)

Symptoms of diabetes insipidus in children and adolescents may include:

• Excessive thirst, often intense, with the need to drink large amounts of water
• Excessive urine production, with the need to urinate frequently, often every hour through the day and night
• New onset bedwetting or waking up several times at night to urinate
• Dehydration

Symptoms in infants may also include:

• Irritability
• Poor feeding
• Failure to grow
• High fevers

Diagnosis will usually begin with a complete medical history and physical examination. The doctor will ask about the child's daily fluid and food intake and about urination. 

If symptoms suggest the possibility of diabetes insipidus, tests may be ordered. These may include:

• Urine tests, including chemical analysis of the urine and measures of urine output
• Blood tests
• Water deprivation test, in the hospital, to establish the diagnosis and if it is present, determine the type of DI
• Magnetic resonance imaging (MRI), to check for pituitary abnormalities

Treatment will focus first on identifying the cause of the condition when possible.

Treatment for the symptoms of central diabetes insipidus begins with establishing the cause. Symptomatic management may include:

• Modified antidiuretic hormone medications, such as desmopressin (DDAVP), taken as a pill, injection or nasal spray
• Modifying the fluids that a child is receiving
• Diuretic medications

If nephrogenic diabetes insipidus is identified, referral to CHOP nephrologists (kidney specialists) is made. Treatment for this condition may include:

• Anti-inflammatory medications
• Diuretics (water pills)
• Stopping a medication, such as lithium, if it has been found to be a cause of the kidney problem

Treatment for both forms of diabetes insipidus also includes careful attention to fluid and sodium intake. Children and adolescents with diabetes insipidus need close monitoring to prevent dehydration and electrolyte imbalance.

Diabetes insipidus can be a temporary or a permanent condition, depending on what is causing the disease.

Children with central diabetes insipidus, with proper management, can lead full, healthy lives.

Children with nephrogenic diabetes insipidus can also lead relatively normal lives with proper medical care and management, especially if the medical care is started early.

The amount of follow-up required depends substantially on the underlying cause of the diabetes insipidus.

Regardless of the cause, children with DI require ongoing attention to the amount of urination and thirst. Serum electrolytes as well as assessment of other pituitary hormones are required periodically. 

The Neuroendocrine Center at Children’s Hospital of Philadelphia offers families a coordinated and multidisciplinary approach to treatment for neuroendocrine disorders like DI. Our team combines the expertise of many pediatric specialists to develop a treatment plan that is customized to meet your child’s needs.

Our endocrinologists have substantial clinical experience in the diagnosis and management of diabetes insipidus, and are academic leaders in educating other clinicians about this condition through publications and lectures.