Janus Dominika Malgorzata, Wojcik Malgorzata, Zygmunt-Górska Agata, Wyrobek Lukasz, Urbanik Andrzej, Starzyk Jerzy Bogdan
Department of Pediatric Endocrinology, Jagiellonian University, Krakow, Poland,
Indian J Pediatr. 2014 Dec;81(12):1307-14. doi: 10.1007/s12098-014-1421-8. Epub 2014 May 2.
To present symptoms, complications and proposition of management protocol in children diagnosed with adipsic diabetes insipidus (aDI).
Clinical and biochemical analysis of six pediatric patients diagnosed with aDI, four boys aged 5, 13, 16, and 17 y and two girls aged 2.5 and 10 y. The etiology of aDI was germinoma (n = 2), extensive surgery due to optic glioma (n = 1) and astrocytoma (n = 1), congenital brain malformations (n = 1) and complications secondary to bacterial meningitis (n = 1). Two patients had severely impaired vision and two had hemiparesis.
In all the patients, loss of thirst reflex was observed. The serum electrolytes in all patients showed sodium concentration from 159 to 176.6 mmol/L with plasma osmolality from above 297 mOsmol/kg. Polyuria was absent in three most severely dehydrated patients on admission. In two patients in whom DDAVP (1-desamino-8-D-arginine vasopressin; Desmopressin) therapy was withdrawn based on lack of polyuria deep venous thrombosis developed.
Lack of polydipsia and polyuria, the key symptoms of diabetes insipidus (DI), may delay the diagnosis of aDI and may lead to severe complications of chronic hyperosmolar status. The fluid intake in patients diagnosed with aDI need to be supervised daily based on calculated constant volume of oral fluids, daily measurements of fluid balance, body weight and sodium levels, especially in patients whose vision is compromised or who are physically unable to take care of themselves.
介绍诊断为失水性尿崩症（aDI）患儿的症状、并发症及管理方案建议。
对6例诊断为aDI的儿科患者进行临床和生化分析，其中4例男孩，年龄分别为5岁、13岁、16岁和17岁，2例女孩，年龄分别为2.5岁和10岁。aDI的病因包括生殖细胞瘤（n = 2）、视神经胶质瘤（n = 1）和星形细胞瘤（n = 1）导致的广泛手术、先天性脑畸形（n = 1）以及细菌性脑膜炎继发并发症（n = 1）。2例患者视力严重受损，2例有偏瘫。
所有患者均观察到口渴反射丧失。所有患者的血清电解质显示钠浓度为159至176.6 mmol/L，血浆渗透压高于297 mOsmol/kg。3例入院时脱水最严重的患者无多尿症状。2例因无多尿而停用去氨加压素（1-去氨基-8-D-精氨酸加压素；弥凝）治疗的患者发生了深静脉血栓形成。
尿崩症（DI）的关键症状多饮和多尿的缺乏可能会延迟aDI的诊断，并可能导致慢性高渗状态的严重并发症。对于诊断为aDI的患者，需要根据计算出的恒定口服液体量、每日液体平衡测量、体重和钠水平，每天监督液体摄入量，尤其是对于视力受损或身体无法自理的患者。
