儿童尿崩症病因及临床特点分析

Analysis of the Clinical Features and Causes of Diabetes Insipidus in Children

目的分析小儿尿崩症病因、临床特点及诊断方法。方法采用回顾性病例研究方法,对郑州市儿童医院1995-2015收治的108例小儿尿崩症患者进行分析。结果肾性尿崩症14例,占13.0%,中枢性尿崩症94例,占87.0%。中枢性尿崩症中,特发性60例,占64.4%（60/94）,继发性34例,占35.6%（34/94）。结论儿童尿崩症以中枢性尿崩症最常见,中枢性尿崩症又以特发性为最多,继发性以下丘脑-垂体病变引起,应定期随访颅脑MRI;肾性尿崩症多以先天性,为精氨酸加压素受体2（AVPR2）基因突变引起,应完善基因检测。

Objective To analyze the etiology and clinical features of children with diabetes insipidus. Methods Retrospective analysis was used. The 108 cases of children with diabetes insipidus were analyzed in the hospital of children＇s Zhengzhou city from the 1995 to 2015. Results Nephrogenic diabetes insipidus is 14 cases,accounting for 13.0%. central diabetes insipidus is 94 cases, accounting for 87.0%. The idiopathic central diabetes insipidus is 60 cases. accounting for 64.4%（60/94）. 34 cases were secondary, accounting for 35.6%（34/94）. Conclusion Central diabetes insipidus is the more common in children＇Diabetes Insipidus,of which idiopathic central diabetes is the most common.Idiopathic central diabetes was often caused by hypothalamus pituitary＇lesion,which should be regularlly followed-up by brain MRI. Most nephrogenic diabetes insipidus were congenital and caused by mutations of arginine pressure hormone receptor 2（AVPR2）gene which should be detected for further test.