Nephrogenic Diabetes Insipidus
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Nephrogenic Diabetes Insipidus is not the name you expected.
Nephrogenic diabetes insipidus (NDI) is a rare kidney disorder that may be inherited or acquired. NDI is not related to the more common diabetes mellitus (sugar diabetes), in which the body does not produce or properly use insulin. NDI is a distinct disorder caused by complete or partial resistance of the kidneys to arginine vasopressin (AVP). Vasopressin is an antidiuretic hormone used by the kidney to manage water balance in the body. NDI causes chronic excessive thirst (polydipsia), excessive urine production (polyuria), and potentially dehydration. If left untreated, repeated episodes of severe dehydration may develop, eventually resulting in serious complications. Most cases of hereditary NDI are inherited as X-linked recessive disorders. Rare cases are inherited as an autosomal recessive or dominant disorder. Two different genes have been identified that cause hereditary NDI. NDI may also be acquired during life as a result of drug use (e.g., lithium therapy), kidney disease, obstruction of the tubes that carry urine from the kidneys to the bladder (ureters), and prolonged metabolic imbalances such as low levels of potassium in the blood (hypokalemia) or high levels of calcium in the blood (hypercalcemia). NDI may also be a temporary complication associated with pregnancy.
The term nephrogenic diabetes insipidus was first used in the medical literature in 1947. In the past, the term diabetes insipidus renalis was used to denote this disorder. NDI is different from central diabetes insipidus, which is a rare disorder characterized by the inability of the body to produce vasopressin (rather than vasopressin resistance as in NDI).
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March of Dimes
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NDI (Nephrogenic Diabetes Insipidus) Foundation
- PO Box 1390
- Main Street
- Eastsound, WA 98245
- Fax: (888)376-6356
- Tel: (888)376-6343
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- Website: http://www.ndif.org
NIH/National Institute of Diabetes, Digestive & Kidney Diseases
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- Bldg 31, Rm 9A06
- Bethesda, MD 20892-2560
- Tel: (301)496-3583
- Email: NDDIC@info.niddk.nih.gov
- Website: http://www2.niddk.nih.gov/
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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Last Updated: 3/17/2016
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