Pediatric Arginine Vasopressin Disorders (Diabetes Insipidus) Differential Diagnoses

Updated: Aug 24, 2023
  • Author: Karl S Roth, MD; Chief Editor: Robert P Hoffman, MD  more...
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Diagnostic Considerations

Arginine vasopressin deficiency (AVP-D) (central diabetes insipidus), AVP resistance (AVP-R) (nephrogenic diabetes insipidus), and primary polydipsia (PP) are all classified as polyuria-polydipsia syndromes. Differentiating these disorders is essential, as misdiagnosis and inappropriate treatment can lead to hyponatremia. The diagnostic gold standard is water deprivation testing; however, distinguishing PP from partial forms of AVP is challenging because the kidney's maximum concentrating ability is often impaired due to a washout of the renal salt gradient. [24]

Wolfram syndrome (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness [DIDMOAD]) is a differential diagnosis of neonatal AVP-D, and signs include arginine vasopressin deficiency; sensorineural deafness; neurologic signs, including ataxia, autonomic neuropathy, and epilepsy; and neurogenic bladder in combination with diabetes mellitus or optic nerve atrophy. [2, 25]

Differential Diagnoses