Perkins Robert M, Yuan Christina M, Welch Paul G
Department of Medicine/Nephrology Service, Walter Reed Army Medical Center, 6900 Georgia Ave NW, Washington, DC 20307-5001, USA.
Clin Exp Nephrol. 2006 Mar;10(1):63-7. doi: 10.1007/s10157-005-0397-0.
Dipsogenic diabetes insipidus is a syndrome of disordered thirst, in patients without psychiatric disease, which may be confused with partial central diabetes insipidus. Distinguishing these entities involves monitored water testing. Therapy with antidiuretic hormone in patients with dipsogenic diabetes insipidus is thought to be contraindicated for fear of inducing water intoxication. We report a case of a 26-year-old woman without psychiatric illness referred for longstanding polyuria and polydipsia. Otherwise healthy, she complained of near-constant thirst and frequent urination, causing severe disruption of her personal and professional life. She had been consistently eunatremic and polyuric, with low urine osmolality. Results of extensive water testing revealed intact urinary concentrating and diluting capacity, physiologic though blunted antidiuretic hormone (ADH) release, and an abnormally low thirst threshold, consistent with the diagnosis of dipsogenic diabetes insipidus. To control her polyuria we initiated treatment with intermittent, low-dose, intranasal desmopressin and strict water restriction during drug dosing. In follow-up she reported excellent control of polyuria and significant functional improvement. The reviewed literature demonstrates a limited number of reports about dipsogenic diabetes insipidus, and no prior report of a similar treatment strategy. Dipsogenic diabetes insipidus is an uncommonly (and not universally) recognized disorder, requiring monitored testing in order to distinguish it from incomplete forms of central diabetes insipidus. Though therapy with desmopressin cannot be recommended based on the results of a single case, the outcome presented here is intriguing and suggests that larger studies in such patients is warranted to assess the broader application of such an intervention.
精神性烦渴性尿崩症是一种在无精神疾病患者中出现的口渴紊乱综合征,可能会与部分中枢性尿崩症相混淆。区分这些病症需要进行监测水试验。由于担心诱发水中毒,抗利尿激素治疗精神性烦渴性尿崩症患者被认为是禁忌的。我们报告一例26岁无精神疾病的女性,因长期多尿和烦渴前来就诊。她身体健康,主诉几乎持续口渴和频繁排尿,严重影响了她的个人和职业生活。她一直处于血钠正常和多尿状态,尿渗透压低。广泛的水试验结果显示尿浓缩和稀释能力正常,抗利尿激素(ADH)释放生理但迟钝,口渴阈值异常低,符合精神性烦渴性尿崩症的诊断。为了控制她的多尿,我们开始采用间歇性、低剂量鼻内去氨加压素治疗,并在给药期间严格限制饮水。随访时她报告多尿得到了很好的控制,功能有了显著改善。回顾的文献表明关于精神性烦渴性尿崩症的报道数量有限,且此前没有类似治疗策略的报告。精神性烦渴性尿崩症是一种不常见(且并非普遍)被认识的疾病,需要进行监测试验以将其与不完全形式的中枢性尿崩症区分开来。尽管基于单个病例的结果不能推荐使用去氨加压素治疗,但此处呈现的结果很有趣,表明有必要对这类患者进行更大规模的研究,以评估这种干预措施的更广泛应用。