Ooi Hooi Leng, Maguire Ann M, Ambler Geoffrey R
J Pediatr Endocrinol Metab. 2013;26(11-12):1047-52. doi: 10.1515/jpem-2013-0078.
Central diabetes insipidus (DI) is a rare disorder in children caused by a deficiency of antidiuretic hormone arginine (vasopressin). Desmopressin is the first line agent in management of central DI. However, one of the side effects of desmopressin is water intoxication and hyponatraemia. This study reviews the patterns of desmopressin use and side effects in our institution.
Retrospective chart review of all patients with central DI followed up in one tertiary centre between 1 January 2008 and 31 December 2010.
Forty-one patients (22 males and 19 females) were included. Twelve patients (29.3%) had congenital and 29 patients (70.7%) had acquired DI, mostly as a result of intracranial tumours. Thirty-six (87.8%) patients were on oral desmopressin and the remaining on nasal formulation. The median oral dose was 9.5 (4.2-17.0) μg/kg/day with median frequency of 2.5 (2-3). The median nasal dose was 0.7 (0.4-1.4) μg/kg/day with median frequency of 2.0 (2-3.5). Fourteen patients (34.1%) were switched from nasal to oral desmopressin with the median dose conversion factor of 20.1 (10.7-31.8). Forty percent of patients on nasal desmopressin experienced hypo/hypernatraemia compared to 18.1% on oral, however, there were no significance difference between standardized hypo/hypernatraemia episodes per treatment year.
Oral desmopressin is used in the majority of our patients including infants and toddlers. There is wide inter-individual variation in dose requirement and dosing intervals. Management of central diabetes insipidus remains a challenge in adipsic patients and in young children during intercurrent illness regardless of the desmopressin formulation.
中枢性尿崩症（DI）是一种儿童罕见疾病，由抗利尿激素精氨酸（血管加压素）缺乏引起。去氨加压素是中枢性尿崩症治疗的一线药物。然而，去氨加压素的副作用之一是水中毒和低钠血症。本研究回顾了我院去氨加压素的使用模式和副作用。
对2008年1月1日至2010年12月31日在某三级中心随访的所有中枢性尿崩症患者进行回顾性病历审查。
纳入41例患者（男22例，女19例）。12例患者（29.3%）为先天性尿崩症，29例患者（70.7%）为获得性尿崩症，主要由颅内肿瘤引起。36例（87.8%）患者使用口服去氨加压素，其余使用鼻用制剂。口服剂量中位数为9.5（4.2 - 17.0）μg/kg/天，给药频率中位数为2.5（2 - 3）。鼻用剂量中位数为0.7（0.4 - 1.4）μg/kg/天，给药频率中位数为2.0（2 - 3.5）。14例患者（34.1%）从鼻用去氨加压素转换为口服，剂量转换系数中位数为20.1（10.7 - 31.8）。使用鼻用去氨加压素的患者中有40%发生低钠血症/高钠血症，而口服去氨加压素的患者中这一比例为18.1%，然而，每治疗年标准化的低钠血症/高钠血症发作次数之间无显著差异。
我院大多数患者包括婴幼儿使用口服去氨加压素。剂量需求和给药间隔存在广泛的个体差异。无论使用何种去氨加压素制剂，对于无渴感患者和并发疾病期间的幼儿，中枢性尿崩症的管理仍然是一项挑战。
