Diabetes insipidus
Diabetes insipidus
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DOI：
10.1038/s41572-019-0103-2
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发表时间：
2019-08-08
影响因子：
81.5
通讯作者：
Verkman, Alan S.
中科院分区：
文献类型：
--
作者：
Christ-Crain, Mirjam;Bichet, Daniel G.;Verkman, Alan S.
尿崩症（DI）是一种以大量低渗尿排泄为特征的疾病。中枢性DI是由于垂体或下丘脑中精氨酸加压素（AVP）缺乏所致，而肾源性DI是由于肾脏中AVP抵抗所致。中枢性和肾源性DI通常是获得性的，但必须评估遗传原因，特别是如果症状发生在儿童早期。中枢性或肾源性DI必须与原发性多饮相区别，原发性多饮涉及过量摄入大量水，尽管AVP分泌和作用正常。原发性烦渴在精神病患者和健康爱好者中最常见，但在一小部分患者中的烦渴似乎是由于异常低的口渴阈值，这种情况称为致渴DI。区分不同类型的DI可能是具有挑战性的，并且可以通过水剥夺试验或通过高渗盐水刺激连同和肽素（或AVP）测量来完成。此外，需要详细的病史、体格检查和影像学检查，以确保准确的DI诊断。DI或原发性烦渴的治疗取决于潜在的病因，并且在中枢性DI、肾源性DI和原发性烦渴中有所不同。
Diabetes insipidus (DI) is a disorder characterized by excretion of large amounts of hypotonic urine. Central DI results from a deficiency of the hormone arginine vasopressin (AVP) in the pituitary gland or the hypothalamus, whereas nephrogenic DI results from resistance to AVP in the kidneys. Central and nephrogenic DI are usually acquired, but genetic causes must be evaluated, especially if symptoms occur in early childhood. Central or nephrogenic DI must be differentiated from primary polydipsia, which involves excessive intake of large amounts of water despite normal AVP secretion and action. Primary polydipsia is most common in psychiatric patients and health enthusiasts but the polydipsia in a small subgroup of patients seems to be due to an abnormally low thirst threshold, a condition termed dipsogenic DI. Distinguishing between the different types of DI can be challenging and is done either by a water deprivation test or by hypertonic saline stimulation together with copeptin (or AVP) measurement. Furthermore, a detailed medical history, physical examination and imaging studies are needed to ensure an accurate DI diagnosis. Treatment of DI or primary polydipsia depends on the underlying aetiology and differs in central DI, nephrogenic DI and primary polydipsia.