Department of Endocrinology, La Rabta University Hospital, University of Tunis El Manar, Faculty of Medicine of Tunis, Tunisia.
J Int Med Res. 2024 Mar;52(3):3000605241235747. doi: 10.1177/03000605241235747.
Central diabetes insipidus (CDI) typically manifests as a polyuria-polydipsia syndrome, in which normonatremia is generally maintained through the polydipsia. A 53-year-old woman presented with diabetic ketosis and hyperosmolar hyperglycemic syndrome. Her medical history included herpes meningoencephalitis, which was associated with confusion and amnesia. On physical examination, she was apyretic, confused, and had signs of extracellular dehydration. Her capillary glucose concentration was high and her urine was positive for ketones. Laboratory investigations revealed severe hyperglycemia, hypernatremia (plasma hyperosmolarity of 393.6 mOsm/L), and mild acute renal failure. In addition, she had a paucisymptomatic COVID-19 infection. Intravenous rehydration with isotonic saline solution and insulin therapy were effective at controlling the ketosis and ameliorating the hyperglycemia, but failed to normalize the hypernatremia and hyperosmolarity. She was not thirsty and had a urine output of 1 L/day, with urinary hypotonicity. Desmopressin administration reduced the hypernatremia and hyperosmolarity to within their normal ranges, and the patient's urinary osmolarity increased to 743 mOsm/L. Therefore, adipsic CDI was diagnosed. Endocrine investigations revealed isolated central hypothyroidism. The results of pituitary magnetic resonance imaging were normal. Thus, patients with impaired thirst may have an atypical presentation of CDI. In addition, the diagnosis of adipsic CDI is particularly challenging.
中枢性尿崩症(CDI)通常表现为多尿多饮综合征,其中多饮通常通过维持正常血钠来实现。一位 53 岁女性因糖尿病酮症酸中毒和高渗性高血糖综合征就诊。她的病史包括疱疹性脑膜脑炎,这与意识混乱和遗忘有关。体格检查时,她发热、意识混乱,并有细胞外脱水的迹象。她的毛细血管血糖浓度很高,尿液酮体阳性。实验室检查显示严重高血糖、高钠血症(血浆高渗 393.6 mOsm/L)和轻度急性肾衰竭。此外,她患有无症状 COVID-19 感染。静脉输注生理盐水和胰岛素治疗有效控制了酮症酸中毒并改善了高血糖,但未能使高钠血症和高渗性正常化。她没有口渴,尿量为 1 L/天,尿液低渗。去氨加压素治疗使高钠血症和高渗性恢复到正常范围,患者的尿渗透压增加到 743 mOsm/L。因此,诊断为渴感缺失性 CDI。内分泌检查显示孤立性中枢性甲状腺功能减退。垂体磁共振成像结果正常。因此,口渴受损的患者可能会出现 CDI 的非典型表现。此外,渴感缺失性 CDI 的诊断特别具有挑战性。