Christ-Crain M, Winzeler B, Refardt J
From the, Clinic for Endocrinology, Diabetes and Metabolism, University Hospital Basel, University of Basel, Basel, Switzerland.
J Intern Med. 2021 Jul;290(1):73-87. doi: 10.1111/joim.13261. Epub 2021 Mar 13.
Diabetes insipidus is a disorder characterized by excretion of large amounts of hypotonic urine. Four entities have to be differentiated: central diabetes insipidus resulting from a deficiency of the hormone arginine vasopressin (AVP) in the pituitary gland or the hypothalamus, nephrogenic diabetes insipidus resulting from resistance to AVP in the kidneys, gestational diabetes insipidus resulting from an increase in placental vasopressinase and finally primary polydipsia, which involves excessive intake of large amounts of water despite normal AVP secretion and action. Distinguishing between the different types of diabetes insipidus can be challenging. A detailed medical history, physical examination and imaging studies are needed to detect the aetiology of diabetes insipidus. Differentiation between the various forms of hypotonic polyuria is then done by the classical water deprivation test or the more recently developed hypertonic saline or arginine stimulation together with copeptin (or AVP) measurement. In patients with idiopathic central DI, a close follow-up is needed since central DI can be the first sign of an underlying pathology. Treatment of diabetes insipidus or primary polydipsia depends on the underlying aetiology and differs in central diabetes insipidus, nephrogenic diabetes insipidus and primary polydipsia. This review will discuss issues and newest developments in diagnosis, differential diagnosis and treatment, with a focus on central diabetes insipidus.
尿崩症是一种以大量低渗性尿液排泄为特征的疾病。必须区分四种情况:中枢性尿崩症,由垂体或下丘脑抗利尿激素精氨酸加压素(AVP)缺乏引起;肾性尿崩症,由肾脏对AVP抵抗引起;妊娠期尿崩症,由胎盘血管加压素酶增加引起;以及原发性烦渴症,即尽管AVP分泌和作用正常,但仍过量摄入大量水分。区分不同类型的尿崩症可能具有挑战性。需要详细的病史、体格检查和影像学检查来检测尿崩症的病因。然后通过经典的禁水试验或最近开发的高渗盐水或精氨酸刺激试验以及copeptin(或AVP)测量来区分各种形式的低渗性多尿症。对于特发性中枢性尿崩症患者,由于中枢性尿崩症可能是潜在疾病的首发症状,因此需要密切随访。尿崩症或原发性烦渴症的治疗取决于潜在病因,在中枢性尿崩症、肾性尿崩症和原发性烦渴症中有所不同。本综述将讨论诊断、鉴别诊断和治疗方面的问题及最新进展,重点关注中枢性尿崩症。