Robertson Gary L
Feinberg Medical School of Northwestern University, Chicago, IL, USA.
Best Pract Res Clin Endocrinol Metab. 2016 Mar;30(2):205-18. doi: 10.1016/j.beem.2016.02.007. Epub 2016 Feb 18.
Diabetes insipidus (DI) is a syndrome characterized by the excretion of abnormally large volumes of dilute urine. It can be caused by any of 4 fundamentally different defects that must be distinguished for safe and effective management. They are: (1) pituitary DI, due to inadequate production and secretion of antidiuretic hormone, arginine-vasopressin (AVP); (2) gestational DI due to degradation of AVP by an enzyme made in placenta; (3) primary polydipsia, due to suppression of AVP secretion by excessive fluid intake; and (4) nephrogenic DI due to renal insensitivity to the antidiuretic effect of AVP. This review describes several methods of differential diagnosis, indicates the advantages and disadvantages of each and presents a new approach that is simpler and less costly but just as reliable as the best of the older methods. The various treatments for the different types of DI and recent findings on the genetic basis of the familial forms of DI are also discussed with emphasis on their contributions to improved diagnosis and management.
尿崩症（DI）是一种以排出大量异常稀释尿液为特征的综合征。它可由4种根本不同的缺陷中的任何一种引起，为了安全有效地进行治疗，必须对这些缺陷加以区分。它们是：（1）垂体性尿崩症，由于抗利尿激素精氨酸加压素（AVP）产生和分泌不足；（2）妊娠期尿崩症，由于胎盘产生的一种酶使AVP降解；（3）原发性烦渴，由于过量饮水抑制了AVP分泌；（4）肾性尿崩症，由于肾脏对AVP的抗利尿作用不敏感。本综述描述了几种鉴别诊断方法，指出了每种方法的优缺点，并提出了一种新方法，该方法更简单、成本更低，但与以往最好的方法一样可靠。还讨论了不同类型尿崩症的各种治疗方法以及家族性尿崩症遗传基础的最新研究结果，重点是它们对改善诊断和治疗的作用。
