NIH

Zebra of the Week: Central diabetes insipidus

March 4, 2026

Central diabetes insipidus (CDI) is a rare endocrine condition caused by deficient production or release of antidiuretic hormone (ADH/arginine vasopressin), resulting in the kidney’s inability to appropriately concentrate urine. Clinically, CDI manifests as hypotonic polyuria, nocturia, and marked polydipsia, reflecting excessive renal free‑water loss.

Because ADH normally reduces urine output at the collecting duct, inadequate hormone levels produce large‑volume, dilute urine and obligate water intake. Without sufficient fluid replacement, patients may be at risk for dehydration. CDI may arise from pathogenic genetic variants, or from structural or functional injury to the pituitary—including trauma, neurosurgery, or other disease processes affecting ADH-producing pathways. Symptom onset may occur in childhood, and presentations vary.

FDA recently approved Desmoda, the first oral desmopressin solution for treating central diabetes insipidus across all ages.

Source:

(Accessed 2026, March 3). NIH Genetic and Rare Diseases Information Center (GARD). Central diabetes insipidus. https://rarediseases.info.nih.gov/diseases/6015/central-diabetes-insipidus