Nephrogenic diabetes insipidus
Alerts and Notices
Synopsis
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
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Central DI - brain tumors, central nervous system autoimmune or infiltrative diseases, vascular malformations, brain metastases, cerebral hemorrhage, or post-neurosurgical injury
- Polygenic polydipsia (see diabetes insipidus)
- Diabetes mellitus type 1 or type 2
- Urinary tract infection
- Pyelonephritis
- Ureteral obstruction (see ureteral stricture, ureteropelvic junction obstruction)
- Metabolic disturbance – hypokalemia, hypercalcemia
Best Tests
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Management Pearls
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Therapy
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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
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Last Reviewed:12/13/2018
Last Updated:12/13/2018
Last Updated:12/13/2018
Nephrogenic diabetes insipidus