Nephrogenic syndrome of inappropriate…

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Nephrogenic syndrome of inappropriate antidiuresis

ORPHA:93606Disease

Also called NSIAD

What it is

Nephrogenic syndrome of inappropriate antidiuresis (NSIAD) is a rare genetic disorder of water balance, closely resembling the far more frequent syndrome of inappropriate antidiuretic secretion (SIAD), and characterized by euvolemic hypotonic hyponatremia due to impaired free water excretion and undetectable or low plasma arginine vasopressin (AVP) levels.

Key facts

Prevalence
<1 / 1 000 000
Age of onset
Childhood
Inheritance
X-linked recessive
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Gene

AVPR2Disease-causing germline mutation(s) (gain of function)

ICD-10 codes

E22.2filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 10306MESH C564491MONDO 0010356OMIM 300539UMLS C1845202

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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