Gu F, Jin Z, Zhang D
Department of Endocrinology, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Yi Xue Za Zhi. 2001 Oct;81(19):1166-71.
To study the etiology and clinical characteristics of central diabetes insipidus (CDI).
The clinical data of 408 cases with CDI treated in Peking Union Medical College Hospital between 1956 and 2000, including 113 cases caused by tumors in sella region, were analyzed retrospectively. Follow-up for three months to 16 years was made to 35 cases of CDI without etiological diagnosis during the first visit.
Most of the cases among children and youngsters fell ill at the age of 8-12 years, while most of the adult cases fell ill at the age of 25-35 years. Idiopathic CDI accounted for 52%. CDI caused by tumor in sella region accounted for about 33% among the child and youngster cases, among which germinoma made up about 65%. CDI caused by tumor in sella region accounted for about 22% in adult cases. Trauma-induced CDI accounted for 11% among adult cases, and about 5% among child and youngster cases. All the CDI cases caused by histiocytosis X, accounting for 3%, were children. A correct diagnosis was made within the period of two years' follow-up among 86% of the cases. For those with brain tumors, the long-term survival rate reached 80% with radiotherapy and/or operation performed without delay.
GH deficiency accompanied with growth failure is a potential indicator of CDI caused by space occupying lesion in sella region. A sudden alleviation of polydipsia may indicate the dysfunction of adrenal cortex. MRI is the most valuable examination means for tumor in sella region. Regular clinical follow-up with serial brain MRI may discover the covert tumor in sella region in cases diagnosed originally as idiopathic CDI. The cases without a correct diagnosis during the first visit should be followed-up every 3 to 6 months. The prognosis of the patients with CDI without tumor is satisfactory after ADH replacement. Radiotherapy is the most effective treatment of germinoma in sella region.
研究中枢性尿崩症（CDI）的病因及临床特点。
回顾性分析1956年至2000年在北京协和医院治疗的408例CDI患者的临床资料，其中113例由鞍区肿瘤引起。对初诊时未明确病因的35例CDI患者进行了3个月至16年的随访。
儿童及青少年患者多在8 - 12岁发病，成年患者多在25 - 35岁发病。特发性CDI占52%。鞍区肿瘤所致CDI在儿童及青少年患者中约占33%，其中生殖细胞瘤约占65%。鞍区肿瘤所致CDI在成年患者中约占22%。外伤所致CDI在成年患者中占11%，在儿童及青少年患者中约占5%。所有组织细胞增多症X所致CDI患者均为儿童，占3%。86%的病例在随访两年内得到正确诊断。对于脑肿瘤患者，及时进行放疗和/或手术，长期生存率可达80%。
生长激素缺乏伴生长发育迟缓是鞍区占位性病变所致CDI的潜在指标。烦渴突然缓解可能提示肾上腺皮质功能障碍。MRI是诊断鞍区肿瘤最有价值的检查手段。对于最初诊断为特发性CDI的患者，定期进行临床随访及头颅MRI检查可能发现隐匿的鞍区肿瘤。初诊未明确诊断的患者应每3至6个月随访一次。CDI无肿瘤患者经抗利尿激素替代治疗后预后良好。放疗是鞍区生殖细胞瘤最有效的治疗方法。
