Health Encyclopedia

Diabetes insipidus - nephrogenic

Definition

Nephrogenic diabetes insipidus is a disorder in which a defect of the small tubes (tubules) in the kidneys results in the passage of large volumes of urine.

Alternative Names

Nephrogenic diabetes insipidus; Acquired nephrogenic diabetes insipidus

Causes, incidence, and risk factors

Nephrogenic diabetes insipidus involves a defect in the kidney tubules (the portion of the kidneys that causes water to be excreted or reabsorbed). The defect affects the ability of the kidneys to respond to antidiuretic hormone (ADH; vasopressin), which normally instructs the kidneys to make the urine more concentrated.

As a result, the kidneys excretes an excessive amount of water into the urine, producing a large quantity of very dilute urine.

Nephrogenic diabetes insipidus is a rare disorder. It may be present at birth as a result of an inherited defect that usually affects men, although women can pass the gene on to their children.

Most commonly, nephrogenic diabetes insipidus is an acquired disorder. Factors that can bring on the disorder include:

Drugs (lithium, demeclocycline, amphotericin B)
Electrolyte disorders (high calcium or low potassium levels)
Urinary blockage

Symptoms

If a person has normal thirst mechanisms and drinks enough fluids, this condition has no significant effects on the fluid and/or electrolyte balance of the body. If the person does not drink enough fluids, the high urine output may cause dehydration and high blood sodium.

Symptoms include:

Excessive thirst (may be intense or uncontrollable, with a craving for ice water)
Excessive urine volume (may exceed 3 - 15 liters per day)

Inadequate fluid consumption can result in:

Dehydration
- Dry mucus membranes
- Dry skin
- Sunken appearance to eyes
- Sunken fontanelles (soft spot) in infants
Fatigue, lethargy
Headache
Irritability
Low body temperature
Muscle pains
Rapid heart rate
Weight loss

Signs and tests

Examination may reveal:

Dehydration and/or shock
High urine output, regardless of fluid intake
Low blood pressure
Rapid pulse rate

Signs associated with high urine output are:

High serum osmolality
Kidneys don't concentrate urine when the person is given ADH
Low urine osmolality
Normal or high ADH levels

This disease may also affect the results of the following tests:

Treatment

The goal of treatment is to regulate fluid levels in the body. Treatment should involve high fluid intake. The volume of fluids consumed should be about equal to the volume of urine produced.

Reducing or stopping medications that can cause nephrogenic DI may improve symptoms.

Hydrochlorothiazide may improve symptoms. This may be used alone or in combination with other medications, including indomethacin. Although this medication is a diuretic (these medications are usually used to increase urine output), in certain cases hydrochlorothiazide can actually reduce urine output for people with nephrogenic diabetes insipidus.

Expectations (prognosis)

Congenital nephrogenic DI is a chronic condition requiring lifelong treatment. Acquired nephrogenic DI may be short-term or long-term.

Complications

Dilation of the ureters and bladder
High blood sodium (hypernatremia)
Severe dehydration, shock (if inadequate fluid intake)

Calling your health care provider

Call your health care provider if you have symptoms of nephrogenic diabetes insipidus.

Prevention

There is no known way to prevent nephrogenic diabetes insipidus that occurs at birth (congenital). Treating the disorders that cause it may prevent some cases of the acquired form of the condition. Medications should only be used under the supervision of the health care provider.

Review Date:11/12/2007
Reviewed By:Charles Silberberg, D.O., Private Practice specializing in Nephrology, Affiliated with NY Medical College, Division of Nephrology, Valhalla, NY. Review provided by VeriMed Healthcare Network.

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