Chua Tzy Harn, Loh Wann Jia
Department of Endocrinology, Changi General Hospital, Singapore.
Endocrinol Diabetes Metab Case Rep. 2020 Aug 20;2020. doi: 10.1530/EDM-20-0039.
Severe hyponatremia and osmotic demyelination syndrome (ODS) are opposite ends of a spectrum of emergency disorders related to sodium concentrations. Management of severe hyponatremia is challenging because of the difficulty in balancing the risk of overcorrection leading to ODS as well as under-correction causing cerebral oedema, particularly in a patient with chronic hypocortisolism and hypothyroidism. We report a case of a patient with Noonan syndrome and untreated anterior hypopituitarism who presented with symptomatic hyponatremia and developed transient ODS.
Patients with severe anterior hypopituitarism with severe hyponatremia are susceptible to the rapid rise of sodium level with a small amount of fluid and hydrocortisone. These patients with chronic anterior hypopituitarism are at high risk of developing ODS and therefore, care should be taken to avoid a rise of more than 4-6 mmol/L per day. Early recognition and rescue desmopressin and i.v. dextrose 5% fluids to reduce serum sodium concentration may be helpful in treating acute ODS.
严重低钠血症和渗透性脱髓鞘综合征（ODS）是与钠浓度相关的一系列紧急病症的两端。严重低钠血症的管理具有挑战性，因为难以平衡过度纠正导致ODS的风险以及纠正不足导致脑水肿的风险，特别是在患有慢性皮质醇缺乏症和甲状腺功能减退症的患者中。我们报告了一例患有努南综合征且未经治疗的垂体前叶功能减退症患者，该患者出现症状性低钠血症并发展为短暂性ODS。
患有严重垂体前叶功能减退症和严重低钠血症的患者易因少量液体和氢化可的松导致钠水平迅速升高。这些患有慢性垂体前叶功能减退症的患者发生ODS的风险很高，因此，应注意避免每天钠水平升高超过4 - 6 mmol/L。早期识别并使用去氨加压素和静脉注射5%葡萄糖溶液降低血清钠浓度可能有助于治疗急性ODS。
