中枢性尿崩症408例的病因及临床特点分析

The etiology and clinical characteristics of central diabetes insipidus, a retrospective study of 408 cases

目的 探讨中枢性尿崩症 (CDI)患者的病因及临床特点。方法 采用回顾性病例研究方法 ,对北京协和医院 195 6～ 2 0 0 0年所收治的 40 8例中枢性尿崩症患者进行分析 ,其中包括 113例鞍区肿瘤导致的CDI患者 ,并对初诊时不能明确病因诊断的 35例CDI患者进行了 3个月～ 16年的随访。结果 在儿童青少年时期CDI的发病多在 8～ 12岁 ,在成人CDI的发病多在 2 5～ 35岁之间 ;CDI病因中特发性尿崩症占 5 2 % ,儿童青少年时期鞍区肿瘤占 33% ,其中以生殖细胞瘤为主 ,占 6 5 % ;而成年人鞍区肿瘤占 2 2 % ,并且外伤导致的CDI占 11% ,明显高于儿童时期 (占 5 % ) ;组织细胞增生症均发生在儿童青少年时期 ,占 3% ;伴有生长迟缓或生长速度下降的GH缺乏 ,是诊断鞍区肿瘤导致的CDI的独立危险因素 ;CDI患者无明显诱因症状突然缓解 ,是CDI病情加重的提示性征象 ;鞍区薄层MRI检查是CDI病因诊断最有价值的检查手段 ,对于初诊不能明确病因的CDI患者 ,应该 3～ 6个月复查MRI。结论 有 86 %的患者在 2年随访期内得到明确的病因诊断 ;除肿瘤之外的CDI患者只要进行激素的替代治疗 ,预后良好 ,几乎不会因CDI而死亡 ;放射治疗是治疗鞍区生殖细胞瘤最有效的方法 ,采用正规的放射治疗或加手术均可使CDI患者的长期生存率达到 80 %以?

Objective To study the etiology and clinical characteristics of central diabetes insipidus (CDI). Methods 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. Results 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. Conclusion 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 befollowed 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.