Intravenous Formulation of Desmopressin Delivered via Oral and Gastrostomy Tube Routes for the Treatment of Central Diabetes Insipidus: First Experience in Infants.

Diabetes insipidus (DI) is a rare disease, with a prevalence of 1:25,000¹ . Central diabetes insipidus (CDI) is the most common form, and neonatal CDI has an estimated incidence of 2 per 100,000 live births² . Congenital causes of CDI are midline cerebral and cranial malformations or genetic defects in arginine vasopressin synthesis. We present 3 infants with CDI due to congenital holoprosencephaly, a complex cerebral malformation with global neurologic involvement and potential hypothalamic/pituitary dysfunction³ . The hypothalamic dysfunction can cause varied severity of CDI among patients or even within the same patient over time.