Diabetes insipidus - nephrogenic

 

Alternative Names

Nephrogenic diabetes insipidus; Acquired nephrogenic diabetes insipidus; Congenital diabetes insipidus

Definition

Nephrogenic diabetes insipidus is a disorder in which a defect in the small tubes (tubules) in the kidneys causes a person to pass a large amount of urine and lose too much water.

Causes

Normally, the kidney tubules allow most water to be removed and returned to the blood.

Nephrogenic diabetes insipidus (NDI) occurs when the kidney tubules do not respond to a hormone in the body called antidiuretic hormone (ADH), also called vasopressin. ADH normally tells the kidneys to make the urine more concentrated.

As a result of the defect, the kidneys release too much water into the urine. This causes the body to produce a large quantity of very dilute urine.

NDI is rare. Congenital diabetes insipidus is present at birth as a result of defect passed down through families. Usually men are affected, though women can pass the gene on to their children.

Most commonly, NDI develops because of other reasons. This is called an acquired disorder. Factors that can trigger the acquired form of this condition include:

  • Blockage in the urinary tract
  • High calcium levels
  • Low potassium levels
  • Use of certain drugs (lithium, demeclocycline, amphotericin B)

Symptoms

You may have intense or uncontrollable thirst, and crave ice water. You will produce large amounts of urine, usually more than 3 liters, and up to 15 liters per day.

If you do not drink enough fluids, dehydration can result. Symptoms may include:

  • Dry mucus membranes
  • Dry skin
  • Sunken appearance to eyes
  • Sunken fontanelles (soft spot) in infants

Other symptoms that can occur due to lack of fluids include:

  • Fatigue, lethargy
  • Headache
  • Irritability
  • Low body temperature
  • Muscle pain
  • Rapid heart rate
  • Weight loss
  • A change in alertness, and even coma

Exams and Tests

A physical exam may reveal:

  • Low blood pressure
  • Rapid pulse
  • Shock
  • Signs of dehydration

Testing may reveal:

  • High serum osmolality
  • High urine output, regardless of how much fluid you drink
  • Kidneys do not concentrate urine when you are given ADH
  • Low urine osmolality
  • Normal or high ADH levels

Other tests that may be done include:

Treatment

The goal of treatment is to control the body's fluid levels. Patients will be given a large amount of fluids. The amount of fluids given should be about equal to the amount of water being lost in the urine.

If the condition is due to a certain medication, stopping the drug may improve symptoms. Never stop taking any medication without first talking to your doctor.

Medicines may be given to improve symptoms.

Outlook (Prognosis)

If a person drinks enough water, this condition will not have significant effects on the fluid or electrolyte balance of the body. Sometimes passing a lot of urine for a long time can cause other electrolyte problems.

If the person does not drink enough fluids, high urine output may cause dehydration and high levels of sodium in the blood.

NDI  that is present at birth is a long-term condition requiring lifelong treatment.

Possible Complications


  • Dilation of the ureters and bladder
  • High blood sodium (hypernatremia)
  • Severe dehydration
  • Shock

When to Contact a Medical Professional

Call your health care provider if you or your child has symptoms of this disorder.

Prevention

Congenital NDI cannot be prevented.

Treating the disorders that can lead to the acquired form of the condition may prevent it from developing in some cases.

References

Brown D, Fenton RA. The cell biology of vasopressin action. In: Taal, MW, Chertow GM, Marsden PA, et al, eds. Brenner and Rector's The Kidney. 9th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 11.

Robinson AG, Verbalis JG. Posterior pituitary. In: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Textbook of Endocrinology. 12th ed. Philadelphia, PA: Elsevier Saunders; 2011:chap 10.

Review Date: 5/10/2014
Reviewed By: Brent Wisse, MD, Associate Professor of Medicine, Division of Metabolism, Endocrinology & Nutrition, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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