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Nephrogenic diabetes insipidus

Endocrine, Nutritional and Metabolic Diseases

Nephrogenic diabetes insipidus, also called arginine vasopressin resistance, is a form of diabetes insipidus caused by the kidneys failing to respond properly to the hormone vasopressin rather than by a shortage of the hormone itself. In a healthy kidney, vasopressin signals the collecting ducts to reabsorb water and concentrate urine; when the kidney's receptors or downstream signaling do not respond, that reabsorption fails even though vasopressin levels are normal or elevated, so the body loses large volumes of dilute urine. Patients typically present with polyuria, waking at night to urinate, and excessive thirst, and if fluid intake cannot keep pace the result is dehydration, electrolyte imbalance such as high blood sodium, and in severe cases seizures. The condition can be inherited, most often through mutations affecting the vasopressin receptor or the water channel it controls, or acquired later in life from certain medications, high blood calcium, or chronic kidney disease. It is managed across nephrology, endocrinology, urology and medical genetics because both the kidney mechanism and any underlying cause matter to treatment.

Facts
Classification
Body System
Urinary System 1
ICD-10 Chapter
XIV: Diseases of the Genitourinary System 2
Communicable or Non-communicable
Non-Communicable 1
Causative Agent
Causative Agent (category)
Genetic / Hereditary 3
Sources
1. Nephrogenic diabetes insipidus (Wikipedia)
  • Lead section
    Nephrogenic diabetes insipidus (NDI), also known as arginine vasopressin resistance (AVP-R) and previously known as renal diabetes insipidus, is a form of diabetes insipidus primarily due to pathology of the kidney.
  • Lead: kidney pathology
    Nephrogenic diabetes insipidus (NDI), also known as arginine vasopressin resistance (AVP-R) and previously known as renal diabetes insipidus, is a form of diabetes insipidus primarily due to pathology of the kidney.
  • Lead
    Nephrogenic diabetes insipidus (NDI), also known as arginine vasopressin resistance (AVP-R) and previously known as renal diabetes insipidus, is a form of diabetes insipidus primarily due to pathology of the kidney.
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2. Wikidata: Nephrogenic diabetes insipidus
Wikidata P494 (N25.1)View the Source
3. MedlinePlus Genetics, National Library of Medicine: Nephrogenic diabetes insipidus
MedlinePlus Genetics, National Library of MedicineCauses: gene variants
Quote, Causes: gene variants
Variants in the AVPR2 or AQP2 genes lead to the production of proteins that are unable to respond to AVP.
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