Related Terms

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Background

• Nephrogenic diabetes insipidus (NDI) is a rare disorder that is caused by large amounts of dilute urine produced by the nephrons, which are found in the kidneys. Normally, the kidneys control the concentration of the urine by absorbing water and returning it to the blood in response to the body's need for water. In a healthy person, an antidiuretic (urine-limiting) hormone called arginine vasopressin (AVP) sends a signal to the kidneys to control the concentration of urine. In patients with NDI, the kidneys are unable to respond to AVP, which causes a problem with water retention. A substantial amount of water that is needed by the body is lost in large quantities as dilute urine. This leaves NDI patients continuously thirsty and in danger of dehydration.
• NDI may be either acquired or inherited. Acquired NDI is more common and is usually caused by certain drugs, severe dehydration, or an electrolyte imbalance, such as elevated sodium or chlorine levels in the blood. Inherited NDI is caused by defects in the AVPR2 or AQP2 genes. NDI can be inherited in an X-linked or an autosomal fashion. X-linked mutations are carried on the X chromosome, which is one of the two sex chromosomes. Females have two X chromosomes, whereas males have one X and one Y chromosome. Males are most likely to have symptoms of inherited X-linked NDI because they receive only one X chromosome, compared to females who inherit two X chromosomes. Autosomal mutations are located on one of the 22 non-sex chromosomes. Males and females are equally likely to display symptoms of the autosomal forms of inherited NDI because this form of the disorder is not linked to a sex chromosome.
• Symptoms of NDI include extreme thirst (polydipsia), excessive urination (polyuria), short stature, and a failure to develop during infancy. NDI patients require a steady supply of water to alleviate their thirst and to prevent dehydration. With early diagnosis and proper management, the quality and duration of life can be normal for patients with NDI.
• NDI is different from diabetes mellitus, which involves problems with insulin and blood sugar regulation. The symptoms can be similar and may include extreme thirst and frequent urination. However, NDI is related to how the kidneys handle fluids. Urine and blood tests can determine if a patient has NDI or diabetes mellitus.

Risk Factors

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Causes

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Signs and Symptoms

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Types of the Disease

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Diagnosis

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Complications

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Treatment

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Integrative Therapies

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Prevention

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Author Information

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References

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

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