Jaruratanasirikul Somchit, Janjindamai Supachai, Sriplung Hutcha, Patarakijvanich Narumon, Vasiknanonte Punnee
Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Thailand.
J Med Assoc Thai. 2002 Jul;85(7):765-71.
Central diabetes insipidus (DI) is a rare disease in children. The authors retrospectively reviewed the records of children with central DI identified at Songklanagarind Hospital from 1985 to 2000. Of the total 29 patients identified, 16 patients were males and 13 were females. All patients received computed tomography or magnetic resonance imaging of the brain to differentiate the etiologies of central DI. The median age at diagnosis was 6.6 years (range 1.5-14.9). The etiologies of central DI were intracranial tumors in 7 patients (24.1%), histiocytosis in 3 patients (10.3%), septooptic dysplasia in 1 patient (3.5%), empty-sella syndrome in 1 patient (3.5%), pituitary abscess in 1 patient (3.5%), and idiopathic in 16 patients (55.1%). All patients with idiopathic central DI were followed-up for a median duration of 4.5 years (range 1.3-15.5). Three of 16 patients (18.8%) were found to have intracranial tumors at 1.3, 2.3, and 3.5 years of follow-up. It was also observed that the patients whose age at presentation was less than 5 years (histiocytosis was excluded) were less likely to have intracranial tumors than those older than 5 years, (0% vs 55%), with significant statistical difference (p<0.01). It is concluded that: 1) the common etiologies of central DI are intracranial tumor and idiopathic, 2) patients initially diagnosed with idiopathic central DI need to have long-term follow-up by magnetic resonance imaging to identify any occult intracerebral tumor.
中枢性尿崩症（DI）在儿童中是一种罕见疾病。作者回顾性分析了1985年至2000年在宋卡纳加拉医院确诊的中枢性尿崩症患儿的病历。在总共确诊的29例患者中，男性16例，女性13例。所有患者均接受了脑部计算机断层扫描或磁共振成像检查，以鉴别中枢性尿崩症的病因。诊断时的中位年龄为6.6岁（范围1.5 - 14.9岁）。中枢性尿崩症的病因包括：7例（24.1%）为颅内肿瘤，3例（10.3%）为组织细胞增多症，1例（3.5%）为视隔发育不良，1例（3.5%）为空蝶鞍综合征，1例（3.5%）为垂体脓肿，16例（55.1%）为特发性。所有特发性中枢性尿崩症患者的中位随访时间为4.5年（范围1.3 - 15.5年）。16例患者中有3例（18.8%）在随访1.3年、2.3年和3.5年时被发现患有颅内肿瘤。还观察到，就诊时年龄小于5岁（排除组织细胞增多症）的患者比年龄大于5岁的患者发生颅内肿瘤的可能性更小（0%对55%），差异有统计学意义（p<0.01）。结论如下：1）中枢性尿崩症的常见病因是颅内肿瘤和特发性；2）最初诊断为特发性中枢性尿崩症的患者需要通过磁共振成像进行长期随访，以发现任何隐匿性脑肿瘤。
