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Nephrogenic diabetes insipidus
Other Resources UpToDate PubMed

Nephrogenic diabetes insipidus

Contributors: Michael W. Winter MD, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Nephrogenic diabetes insipidus (NDI), also known as arginine vasopressin resistance, is a disorder characterized by polydipsia and polyuria of dilute urine. It is caused by a failure of the kidney to respond to antidiuretic hormone (ADH). Patients will most commonly present with polyuria and polydipsia. Patients without access to free water will develop symptomatic hypernatremia. Other symptoms include waking at night to urinate and bed-wetting. Infants may present with inconsolable crying, irritability, growth retardation, hyperthermia, and weight loss. Children may present with fatigue, anorexia, enuresis, and growth defects.

In neonates, there is an X-linked genetic component to NDI that perpetually modifies the kidneys' ability to concentrate urine. This more commonly affects males. Specifically, the AVPR2 gene (which codes vasopressin receptor) and AQP2 gene (which codes aquaporin) have been implicated in inherited NDI. Other medical conditions that predispose to NDI include polycystic kidney disease, sickle cell disease, acute or chronic renal failure, ureteral obstruction, chronic hypokalemia, chronic hypercalcemia, or medication side effects, with lithium and demeclocycline as common offending agents.

Related topics: central diabetes insipidus, diabetes insipidus

Codes

ICD10CM:
N25.1 – Nephrogenic diabetes insipidus

SNOMEDCT:
111395007 – Nephrogenic diabetes insipidus

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Last Reviewed:12/13/2018
Last Updated:11/27/2023
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Nephrogenic diabetes insipidus
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A medical illustration showing key findings of Nephrogenic diabetes insipidus (Congenital) : Fever, Failure to thrive, Hypernatremia, Irritability, Polyuria, Polydipsia, Excessive crying
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