title: Natural history of idiopathic diabetes insipidus.
abstract: OBJECTIVE
  To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI.
  
  
  METHODS
  We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life.
  
  
  RESULTS
  In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population.
  
  
  CONCLUSIONS
  Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population.
authors: Division of Endocrinology, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA. gail.richards@seattlechildrens.org
authors: Gail E
authors: GE
authors: Richards
authors: Michael J
authors: MJ
authors: Thomsett
authors: Bruce A
authors: BA
authors: Boston
authors: Linda A
authors: LA
authors: DiMeglio
authors: Dorothy I
authors: DI
authors: Shulman
authors: Martin
authors: M
authors: Draznin