中枢性尿崩症的病因谱、诊断和预后需要长期随访:单一中心经验

D. Sarma, Manoj Gedam
{"title":"中枢性尿崩症的病因谱、诊断和预后需要长期随访:单一中心经验","authors":"D. Sarma, Manoj Gedam","doi":"10.30564/JER.V2I1.2510","DOIUrl":null,"url":null,"abstract":"Introduction: Central Diabetes insipidus (CDI) is a rare disorder caused vasopressin deficiency characterized by the excretion of copious volumes of unconcentrated urine. Objective: To assess the etiological,clinical, biochemical and radiological spectrum of Central DI in our institute and long term follow up of these cases. Material and Methods:32 patients with Central DI admitted in Department of Endocrinology,Guwahati Medical College, Assam in the last 2.5 years were included.Detailed clinical assessment, biochemical evaluation and MRI (Magnetic Resonance imaging) brain were done in all the patients. Central DI without any identifiable cause was considered Idiopathic and those with structural lesion in hypothalamic pituitary region were considered organic. Result: Idiopathic CDI was present in 12(37.5%) patients and 20(62.5%) patients had organic CDI with acute onset of presentation.12(60%) patients with organic CDI present with neurological symptoms but 8(40%) patients had no neurological symptoms even with organic cause. Pituitary dysfunction was common in organic CDI as compared to idiopathic CDI. Paediatric patients commonly present with organic cause for CDI with low cortisol most common hormonal deficit. One patient of idiopathic CDI with normal stalk thickness at baseline presented with clinical and radiological features of  (Langerhans cell histiocytosis) on follow up. Conclusion: Organic CDI more likely to have acute onset of presentation than idiopathic CDI and even in absence of neurological features. Paediatric patients commonly have organic cause for CDI. We propose the paramount importance of long-term clinical follow-up and reassessment of endocrine function in patients with CDI for definitive diagnosis of autoimmune and inflammatory causes of idiopathic CDI and timely treatment of pituitary ypofunction.","PeriodicalId":92667,"journal":{"name":"Journal of endocrinology and thyroid research","volume":"27 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2021-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Etiological Spectrum with Diagnosis and Prognosis of Central Diabetes Insipidus needs Long Term Followup: A Single Centre Experience\",\"authors\":\"D. Sarma, Manoj Gedam\",\"doi\":\"10.30564/JER.V2I1.2510\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Introduction: Central Diabetes insipidus (CDI) is a rare disorder caused vasopressin deficiency characterized by the excretion of copious volumes of unconcentrated urine. Objective: To assess the etiological,clinical, biochemical and radiological spectrum of Central DI in our institute and long term follow up of these cases. Material and Methods:32 patients with Central DI admitted in Department of Endocrinology,Guwahati Medical College, Assam in the last 2.5 years were included.Detailed clinical assessment, biochemical evaluation and MRI (Magnetic Resonance imaging) brain were done in all the patients. Central DI without any identifiable cause was considered Idiopathic and those with structural lesion in hypothalamic pituitary region were considered organic. Result: Idiopathic CDI was present in 12(37.5%) patients and 20(62.5%) patients had organic CDI with acute onset of presentation.12(60%) patients with organic CDI present with neurological symptoms but 8(40%) patients had no neurological symptoms even with organic cause. Pituitary dysfunction was common in organic CDI as compared to idiopathic CDI. Paediatric patients commonly present with organic cause for CDI with low cortisol most common hormonal deficit. One patient of idiopathic CDI with normal stalk thickness at baseline presented with clinical and radiological features of  (Langerhans cell histiocytosis) on follow up. Conclusion: Organic CDI more likely to have acute onset of presentation than idiopathic CDI and even in absence of neurological features. Paediatric patients commonly have organic cause for CDI. We propose the paramount importance of long-term clinical follow-up and reassessment of endocrine function in patients with CDI for definitive diagnosis of autoimmune and inflammatory causes of idiopathic CDI and timely treatment of pituitary ypofunction.\",\"PeriodicalId\":92667,\"journal\":{\"name\":\"Journal of endocrinology and thyroid research\",\"volume\":\"27 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-02-06\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of endocrinology and thyroid research\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.30564/JER.V2I1.2510\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of endocrinology and thyroid research","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.30564/JER.V2I1.2510","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

中枢性尿崩症(CDI)是一种罕见的由抗利尿素缺乏引起的疾病,其特征是大量未浓缩尿液的排泄。目的:了解我院中枢性脑梗死的病因、临床、生化及影像学特点,并对其进行长期随访。材料与方法:选取阿萨姆邦古瓦哈提医学院内分泌科近2.5年收治的32例中枢性DI患者。对所有患者进行详细的临床评价、生化评价及脑磁共振成像检查。无明确病因的中枢性DI被认为是特发性的,下丘脑垂体区结构性病变被认为是器质性的。结果:原发性CDI 12例(37.5%),器质性CDI 20例(62.5%)有急性起病表现,器质性CDI 12例(60%)有神经系统症状,器质性CDI 8例(40%)无神经系统症状。与特发性CDI相比,有机性CDI常见垂体功能障碍。儿科患者通常表现为器质性CDI,低皮质醇是最常见的激素缺陷。1例特发性CDI患者在基线时茎粗正常,随访时表现为朗格汉斯细胞组织细胞增多症的临床和影像学特征。结论:有机CDI比特发性CDI更容易出现急性发病,甚至在没有神经系统特征的情况下。儿科患者通常有器质性病因CDI。我们建议对CDI患者进行长期临床随访和内分泌功能的重新评估,以明确诊断特发性CDI的自身免疫性和炎症性原因,并及时治疗垂体功能低下。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Etiological Spectrum with Diagnosis and Prognosis of Central Diabetes Insipidus needs Long Term Followup: A Single Centre Experience
Introduction: Central Diabetes insipidus (CDI) is a rare disorder caused vasopressin deficiency characterized by the excretion of copious volumes of unconcentrated urine. Objective: To assess the etiological,clinical, biochemical and radiological spectrum of Central DI in our institute and long term follow up of these cases. Material and Methods:32 patients with Central DI admitted in Department of Endocrinology,Guwahati Medical College, Assam in the last 2.5 years were included.Detailed clinical assessment, biochemical evaluation and MRI (Magnetic Resonance imaging) brain were done in all the patients. Central DI without any identifiable cause was considered Idiopathic and those with structural lesion in hypothalamic pituitary region were considered organic. Result: Idiopathic CDI was present in 12(37.5%) patients and 20(62.5%) patients had organic CDI with acute onset of presentation.12(60%) patients with organic CDI present with neurological symptoms but 8(40%) patients had no neurological symptoms even with organic cause. Pituitary dysfunction was common in organic CDI as compared to idiopathic CDI. Paediatric patients commonly present with organic cause for CDI with low cortisol most common hormonal deficit. One patient of idiopathic CDI with normal stalk thickness at baseline presented with clinical and radiological features of  (Langerhans cell histiocytosis) on follow up. Conclusion: Organic CDI more likely to have acute onset of presentation than idiopathic CDI and even in absence of neurological features. Paediatric patients commonly have organic cause for CDI. We propose the paramount importance of long-term clinical follow-up and reassessment of endocrine function in patients with CDI for definitive diagnosis of autoimmune and inflammatory causes of idiopathic CDI and timely treatment of pituitary ypofunction.
求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信