Pediatric Diabetes Insipidus Differential Diagnoses

Updated: Sep 27, 2017
  • Author: Karl S Roth, MD; Chief Editor: Robert P Hoffman, MD  more...
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DDx

Diagnostic Considerations

Central diabetes insipidus (DI), nephrogenic DI, and primary polydipsia (PP) are all classifed 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 DI is challenging  because the kidney's maximum concentrating ability is often impaired due to a washout of the renal salt gradient. [16]

Wolfram syndrome (DIDMOAD) is a differential diagnosis of neonatal CDI, and signs include diabetes insipidus; sensorineural deafness; neurologic signs, including ataxia, autonomic neuropathy, and epilepsy; and neurogenic bladder in combination with diabetes mellitus or optic nerve atrophy. [17]  

Differential Diagnoses