Is Idiopathic Central Diabetes Insipidus Really Idiopathic?

Is Idiopathic Central Diabetes Insipidus Really Idiopathic?

Information sourced from Journal Watch:

Is Idiopathic Central Diabetes Insipidus Really Idiopathic?

An underlying etiology was later identified in nearly 40% of children initially diagnosed with idiopathic diabetes insipidus.

Diabetes insipidus (DI) is an uncommon diagnosis and might be idiopathic or caused by Langerhans cell histiocytosis, brain tumors, congenital anomalies, inflammation, autoimmune conditions, or other disorders. To clarify the natural history of idiopathic DI, researchers reviewed records of two cohorts of pediatric patients diagnosed with DI at multiple referral centers in the U.S.

The first cohort included 105 patients from three centers who were diagnosed with central DI in 1980 €“1989. Ultimately, 89% of patients had underlying diagnoses identified (including central nervous system tumors, 34%; congenital anomalies, 20%; Langerhans histiocytosis, 19%; and infection, 4%). Eleven percent had idiopathic DI. The second cohort included 30 patients (mean age, 8.5 years; 18 girls) who received diagnoses of idiopathic DI at six centers and were followed for a mean of 7 years. Subsequent etiologies were identified in 37%. Histiocytosis was diagnosed in 5 patients at a median of 14 months after the original diagnosis, germinoma was diagnosed in 1 patient within 5 years, and genetic abnormalities of the vasopressin pathway (decreased or absent secretion) were identified in 5 patients. Hormonal deficiencies in addition to DI were subsequently found in 10 patients (8 had growth hormone deficiency). Among 20 patients with no other endocrine abnormalities, 5 had genetic abnormalities of the vasopressin pathway, and 4 (20%) had another treatable etiology for DI. Among patients with other endocrine deficiencies, 50% had another treatable etiology for DI. Health-related quality of life was similar in children with DI and healthy children.

Comment: When children with central DI are assigned the diagnosis of idiopathic DI, careful follow-up is critical to identify the more than one third of patients who later will be found to have a treatable diagnosis. When other endocrine abnormalities develop, the suspicion for another underlying etiology for DI should be especially high.

€” F. Bruder Stapleton, MD

Published in Journal Watch Pediatrics and Adolescent Medicine October 26, 2011


Richards GE et al. Natural history of idiopathic diabetes insipidus. J Pediatr 2011 Oct; 159:566. [Medline ® Abstract]

Copyright © 2011. Massachusetts Medical Society. All rights reserved.

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