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Idiopathic central diabetes insipidus is associated with abnormal blood supply to the posterior pituitary gland caused by vascular impairment of the inferior hypophyseal artery system

机译:特发性中枢性尿崩症与垂体后叶下动脉系统血管受损引起的垂体后叶血液供应异常有关

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摘要

Central diabetes insipidus (CDI) has been linked to vascular central nervous system damage, although the pathophysiology of the mechanism has never been perfectly understood. Indeed, the vascular system of human pituitary gland has rarely been the subject of rigorous investigation except at postmortem. Recently, studies of pituitary gland blood supply have been carried out by means of a time evaluation of pituitary gland enhancement with noninvasive dynamic magnetic resonance (MR) imaging after contrast medium injection. In the present study, we decided to investigate the status of posterior pituitary blood supply by evaluating vascular pituitary patterns in a group of 19 patients with idiopathic CDI in whom previous standard MR imaging had failed to identify causal specific lesions. The control group was composed of 55 subjects with a median age of 12 yr (range, 4.2-17 yr) who had idiopathic isolated GH deficiency and normal pituitary morphology and 15 young adults (18-25 yr) who had normal pituitary gland and no endocrine dysfunction. Nineteen patients (12 females and seven males), ranging in age at the time of diagnosis of CDI from 0.5-14.9 yr (median, 5 yr), were examined with dynamic MR imaging between 1990 and 1997 at a median age of 14.1 yr (range, 5.0-26.3 yr). CDI was diagnosed according to clinical findings of polyuria and polydipsia, water deprivation test, and desmopressin acetate therapeutic trial. All of the patients had permanent CDI and were being treated with satisfactory results with desmopressin, two to three times daily, either intranasally or orally. The previous MR imaging findings of the 19 CDI patients had shown the absence of posterior pituitary hyperintensity, normal pituitary stalk, and normal anterior pituitary size. Enhancement of the straight sinus, representing a temporal reference point and occurring in normal subjects simultaneously to that of the posterior pituitary gland, was observed in all subjects after iv gadopentetate dimeglumine administration, with no substantial differences between patients and controls. However, the enhancement of the posterior pituitary lobe occurred simultaneously with the enhancement of the straight sinus in all of the controls but in only 14 of the 19 patients with CDI. In the remaining five patients, the enhancement of the straight sinus was not associated with the expected contrast enhancement of the posterior pituitary gland, suggesting abnormal blood supply to the posterior pituitary lobe. This is in keeping with vascular impairment of the inferior hypophyseal artery system and suggests that abnormal blood supply to the posterior pituitary gland is associated with what, until now, has been considered idiopathic CDI.
机译:中枢性尿崩症(CDI)与血管中枢神经系统损害有关,尽管该机制的病理生理学尚未完全清楚。的确,除了死后,人垂体的血管系统很少成为严格研究的对象。近年来,已经通过对造影剂注射后利用无创动态磁共振(MR)成像对垂体增强的时间进行了评估,从而进行了垂体血液供应的研究。在本研究中,我们决定通过评估一组19例特发性CDI患者的垂体后叶供血状况,这些患者先前的标准MR成像未能确定因果关系。对照组由55名中位年龄为12岁(范围为4.2-17岁)的特发性孤立性GH缺乏且垂体形态正常的受试者和15名正常垂体且无垂体的年轻人(18-25岁)组成内分泌功能障碍。在1990年至1997年之间,通过动态MR成像检查了19例患者(12例女性和7例男性),其年龄在0.5-14.9岁(中位数为5岁),诊断为CDI,其年龄中位数为14.1岁(动态)。范围,5.0-26.3年)。根据多尿症和多饮症的临床发现,水分剥夺试验和醋酸去氨加压素治疗性试验诊断为CDI。所有患者均具有永久性CDI,并且每天接受2次至3次去氨加压素鼻内或口服治疗,均获得满意的效果。先前的19例CDI患者的MR影像学发现表明,没有垂体后叶高信号,垂体茎正常,垂体前叶大小正常。静脉注射g庚二酸二聚二甲酰胺后,所有受试者均观察到笔直窦的增强,这是一个暂时的参考点,在正常受试者中与垂体后叶同时发生,在患者和对照组之间没有实质性差异。然而,在所有对照中,垂体后叶的增强与笔直窦的增强同时发生,但在19例CDI患者中只有14例同时发生。在其余五名患者中,笔直窦的增强与垂体后叶的预期对比度增强无关,提示垂体后叶的血液供应异常。这与下垂体下动脉系统的血管损伤保持一致,并表明垂体后叶的异常血液供应与迄今为止被认为是特发性CDI的疾病有关。

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