Division of Endocrinology, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA.
J Pediatr. 2011 Oct;159(4):566-70. doi: 10.1016/j.jpeds.2011.03.044. Epub 2011 May 17.
To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI.
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.
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.
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.
确定儿童期尿崩症（DI）中有多少是特发性的，并评估特发性 DI 的自然病史。
我们对 1980 年至 1989 年期间在 3 家医疗中心出生或诊断出 DI 的 105 例 DI 患者进行了回顾性图表审查。第二组是来自 6 家医疗中心的 30 例患者，他们在 1990 年后被诊断为特发性 DI，我们对其进行了回顾性病因诊断以及其他下丘脑/垂体功能减退的评估，并前瞻性地评估了生活质量。
在第一组中，11%的患者为特发性 DI。在第二组中，有 33%的患者出现了其他下丘脑/垂体激素缺乏，37%的患者被诊断为 DI 的病因。所有特发性 DI 患者的健康相关生活质量与健康参考人群相当。
只有一小部分 DI 患者在首次检查后仍为特发性。三分之一的患者会在以后出现其他激素缺乏，略多于三分之一的患者会被诊断出 DI 的病因。长期监测很重要，因为肿瘤在 DI 发作后长达 21 年才被诊断出来。这些患者的生活质量与参考人群一样好。
