Diabetes insipidus.,Nature Reviews Disease Primers

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Diabetes insipidus.
Nature Reviews Disease Primers ( IF 76.9 ) Pub Date : 2019-08-08 , DOI: 10.1038/s41572-019-0103-2
Mirjam Christ-Crain _{1,

2} , Daniel G Bichet _{3,

4} , Wiebke K Fenske ₅ , Morris B Goldman ₆ , Soren Rittig ₇ , Joseph G Verbalis ₈ , Alan S Verkman _{9,

10}

Affiliation

Division of Endocrinology, Diabetes and Metabolism, University Hospital Basel, Basel, Switzerland.
Department of Clinical Research, University of Basel, Basel, Switzerland.
Division of Nephrology, Hôpital du Sacré-Cœur de Montréal, Montréal, Québec, Canada.
Départements de Médecine, Pharmacologie et Physiologie, Faculté de Médecine, Université de Montréal, Montréal, Québec, Canada.
Medical Department III, Endocrinology, Nephrology, Rheumatology, University Hospital of Leipzig, Leipzig, Germany.
Department of Psychiatry and Behavioral Sciences, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Department of Pediatric and Adolescent Medicine, Aarhus University Hospital, Aarhus, Denmark.
Division of Endocrinology and Metabolism, Georgetown University Medical Center, Washington, DC, USA.
Department of Medicine, University of California, San Francisco, CA, USA.
Department of Physiology, University of California, San Francisco, CA, USA.

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.

中文翻译：

尿崩症。

尿崩症 (DI) 是一种以大量低渗尿液排泄为特征的疾病。中枢性 DI 是由于垂体或下丘脑精氨酸加压素 (AVP) 缺乏所致，而肾源性 DI 是由于肾脏对 AVP 的抵抗所致。中枢性和肾源性 DI 通常是获得性的，但必须评估遗传原因，尤其是在儿童早期出现症状时。中枢性或肾源性 DI 必须与原发性烦渴相鉴别，原发性烦渴涉及尽管 AVP 分泌和作用正常，但仍过量摄入大量水。原发性多饮在精神病患者和健康爱好者中最常见，但一小部分患者的多饮似乎是由于口渴阈值异常低，这种情况称为致渴性 DI。区分不同类型的 DI 可能具有挑战性，可以通过缺水试验或通过高渗盐水刺激以及和肽素（或 AVP）测量来完成。此外，需要详细的病史、体格检查和影像学研究来确保准确的 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。

更新日期：2019-08-08

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