Adipsic Diabetes Insipidus in Pediatric Patients

作者：Janus Dominika Malgorzata^*; Wojcik Malgorzata; Zygmunt Gorska Agata; Wyrobek Lukasz; Urbanik Andrzej; Starzyk Jerzy Bogdan

来源：Indian Journal of Pediatrics, 2014, 81(12): 1307-1314.

DOI：10.1007/s12098-014-1421-8

• Adipsic diabetes insipidus
• Hypernatraemia
• Hyperosmolar status
• Polyuria
• Polydipsia
• Adipsia

Objective To present symptoms, complications and proposition of management protocol in children diagnosed with adipsic diabetes insipidus (aDI). %26lt;br%26gt;Methods 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. %26lt;br%26gt;Results 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 In two patients in whom DDAVP (1-desamino-8-D-arginine vasopressin; Desmopressin) therapy was withdrawn based on lack of polyuria deep venous thrombosis developed. %26lt;br%26gt;Conclusions 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.

• 出版日期2014-12