Eisenberg Yuval, Frohman Lawrence A
Endocr Pract. 2016 Jan;22(1):76-83. doi: 10.4158/EP15940.RA. Epub 2015 Sep 24.
Adipsic diabetes insipidus (ADI) is a rare disorder consisting of central diabetes insipidus (CDI) and a deficient or absent thirst response to hyperosmolality. Patients with ADI experience marked morbidity and mortality. Diagnosis and management of these patients is quite challenging, even in expert hands. In this review, we aim to provide an updated overview of this difficult clinical scenario.
We conducted a PubMed search for articles related to ADI. The search terms "adipsia," "adipsic," "thirst," and "diabetes insipidus" were used to identify relevant literature.
ADI has been described in only approximately 100 patients. This rarity has limited the quality and quantity of literature to case reports, case series, and expert opinion. Diagnosis focuses on confirmation of CDI followed by documenting subnormal or completely absent thirst in response to a hypertonic stimulus. Among the described patients with ADI, the majority experience morbidity (e.g., severe hypernatremia, sleep apnea, venous thromboembolism [VTE], and obesity) and an increased mortality risk. Management focuses on frequent reassessment of daily prescribed water intake with fixed antidiuretic therapy (desmopressin) and comorbidity screening.
The complexity of patients with ADI provides a difficult challenge for clinicians. Prompt recognition of thirst disorders in patients with CDI should lead to appropriately regimented management strategies and can result in safe outpatient care for these unique patients.
失水性尿崩症(ADI)是一种罕见的疾病,由中枢性尿崩症(CDI)以及对高渗状态的口渴反应不足或缺失组成。ADI患者的发病率和死亡率都很高。即使是专家,对这些患者的诊断和管理也颇具挑战。在本综述中,我们旨在提供关于这一复杂临床情况的最新概述。
我们在PubMed上搜索与ADI相关的文章。使用“失水性”“失水性的”“口渴”和“尿崩症”等检索词来识别相关文献。
仅约100例患者被描述患有ADI。这种罕见性限制了文献的质量和数量,大多为病例报告、病例系列和专家意见。诊断重点在于确认CDI,然后记录在高渗刺激下口渴反应低于正常或完全缺失。在已描述的ADI患者中,大多数有发病情况(如严重高钠血症、睡眠呼吸暂停、静脉血栓栓塞症[VTE]和肥胖)且死亡风险增加。管理重点在于通过固定的抗利尿治疗(去氨加压素)和合并症筛查对每日规定饮水量进行频繁重新评估。
ADI患者情况复杂,给临床医生带来了严峻挑战。对CDI患者的口渴障碍进行及时识别应能带来合理的管理策略,并可为这些特殊患者提供安全的门诊护理。