ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (2)
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 (DI) 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 nephrogenic DI 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 nephrogenic DI. Other medical conditions that predispose to nephrogenic DI 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

Subscription Required

References

Subscription Required

Last Reviewed:12/14/2018
Last Updated:12/14/2018
Copyright © 2021 VisualDx®. All rights reserved.
Nephrogenic diabetes insipidus
Nephrogenic diabetes insipidus (Congenital) : Fever, Failure to thrive, Hypernatremia, Irritability, Polyuria, Polydipsia, Excessive crying
Copyright © 2021 VisualDx®. All rights reserved.