下丘脑—垂体区MRI与中枢性尿崩症的相关性研究

发布时间：2018-02-01 19:32

本文关键词： 中枢性尿崩症垂体后叶高信号消失多种垂体激素缺乏肿瘤垂体柄中断综合症　出处：《山东大学》2014年硕士论文　论文类型：学位论文

【摘要】：研究背景和目的：中枢性尿崩症(central diabetes insipidus,CDI)是以烦渴、多尿、排出大量低渗、低比重尿为特征的临床综合征,因抗利尿激素(antidiuretic hormone,ADH)合成和/或分泌减少而致病。本病严重危害儿童的身心健康,由于晚上不能正常睡眠,食欲差,如果长期得不到正确治疗,会导致患儿身材矮小,泌尿系统扩张,甚至继发肾功衰竭,影响患儿寿命。并且CDI也是下丘脑-垂体区肿瘤的早期表现,因此磁共振成像(magnetic resonance imaging, MRI)对CDI诊断有重要价值。ADH为主的神经内分泌颗粒由下丘脑神经核分泌,主要存储于垂体后叶,并因此可在磁共振成像(MRI) T1WI图像中形成高信号,垂体后叶高信号消失或缩小对CDI的诊治有重要意义。根据病因不同,CDI分为原发性和继发性CDI,原发性包括特发性或遗传性,由于不明原因的下丘脑神经分泌细胞不能分泌ADH所致,继发性由于多种原因破坏下丘脑或下丘脑垂体通路而致病,最常见为鞍区肿瘤,包括颅咽管瘤、生殖细胞瘤、垂体瘤等,其他如郎格罕细胞组织细胞增生症、空蝶鞍、垂体发育不良、垂体柄中断综合症(pituitary stalk interruption syndrome, PSIS)、手术、放疗等也是CDI重要原因。其中,某些先天性因素、垂体占位、垂体柄的损伤、颅内肿瘤或感染、颅脑创伤等可引起除CDI以外的一些垂体相应靶腺功能减低的表现,表现为除生长激素缺乏外还存在1种或1种以上垂体前叶和*或后叶激素缺乏,即多种垂体激素缺乏症(multiple pituitary hormone deficiency,MPHD)。大多数报道认为垂体后叶高信号消失是CDI的特点,但有报道认为肾性尿崩症也可有垂体后叶高信号消失,有的CDI也可有垂体后叶高信号不消失。本文分析了68例CDI患儿的MRI表现,禁水加压素试验,垂体靶腺功能,及治疗反应,探讨下丘脑-垂体区MRI与CDI的相关性。研究方法： 1.观察对象：68例CDI患儿,年龄(10.63±3.18)岁,根据MRI,禁水加压素试验及治疗效果进行病因分析。 2.MRI检查：68例均采用Siemens3.0T核磁共振扫描仪,所有病例均行垂体矢状位、冠状位T1WI、T2WI平扫,层厚为3mm。对比剂使用钆喷酸葡胺(Gd-DTPA),剂量为0.1mmol/kg,'快速手推对比剂后即进行增强扫描。主要观察垂体的形态、垂体前叶高度,垂体后叶位置及大小,垂体柄的形态,鞍区、鞍旁结构有无其他异常表现。 3.垂体-靶腺功能及肿瘤标志物检测：所有尿崩症患儿就诊时均需清晨空腹抽血,分别测定垂体-甲状腺轴功能：测血清促甲状腺激素(TSH)、游离三典甲状腺原氨酸(FT3)、游离甲状腺素(FT4)；垂体-肾上腺轴功能：测促肾上腺皮质激素(ACTH),皮质醇(COR)；垂体-性腺轴功能：测卵泡刺激素(FSH)、黄体生成素(LH)、雌二醇(E2)、睾酮(TO)、泌乳素(PRL)及肿瘤标志物绒毛膜促性腺激素(beta-HCG)及甲胎蛋白(AFP)；生长激素-胰岛素样生长因子轴测定(GH-IGF-1). 研究结果： 1.68例CDI患儿,其中18例(男14例)为颅咽管瘤术后,15例(男14例)为完全垂体柄阻断并垂体后叶消失,15例(男13例)为PSIS并垂体后叶异位且接近消失(亮点非常小),18例为垂体柄增粗并垂体后叶消失(其中8例为生殖细胞瘤,10例为朗格罕细胞组织细胞增生症),2例为CDI继发肾性尿崩症(NDI)。 2.其中50例伴有不同程度多种垂体激素缺乏。 3.其中2例病史较长者均伴有智力低下,并已出现肾脏并发症,如肾积水、输尿管扩张,膀胱扩张等,且对单纯弥凝治疗不敏感,联合氢氯噻嗪治疗效果较好。研究结论： 1.下丘脑-垂体区MRI与垂体后叶功能密切相关,中枢性尿崩症患儿MRI呈现垂体后叶高信号消失或减弱,结合禁水加压素试验可以确诊。 2.在继发性CDI,MRI结合垂体激素和肿瘤标志物检查对病因诊断有重要意义。 3.MRI结合泌尿系B超、弥凝治疗反应,可发现CDI并发NDI,应联合应用弥凝、氢氯噻嗪及吲哚美辛治疗。
[Abstract]:Research background and purpose:
Central diabetes insipidus (central diabetes, insipidus, CDI) with polydipsia, polyuria, discharge a large amount of low permeability, low proportion of urine clinical syndrome that is characterized by antidiuretic hormone (antidiuretic, hormone, ADH) synthesis and / or reduce the secretion and disease. This disease seriously endangering children's health, because it is not the night of normal sleep, poor appetite, if not long-term treatment, will lead to children with short stature, urinary dilatation, and secondary renal failure, affects children life. Early performance and CDI is the hypothalamic pituitary region tumors, due to the magnetic resonance imaging (magnetic resonance, imaging, MRI) is of great value.ADH. The neuroendocrine granules by the hypothalamic nucleus on the secretion of CDI diagnosis, mainly stored in the posterior pituitary, and thus in the magnetic resonance imaging (MRI) to form a high signal in T1WI images, the posterior pituitary high signal disappeared or reduced to CDI Has an important significance in the diagnosis and treatment. According to different causes, CDI is divided into primary and secondary CDI, including primary idiopathic or hereditary, due to hypothalamic unexplained secretory cells can secrete ADH caused by secondary damage due to various reasons, the hypothalamus or hypothalamic pituitary pathway and disease, the most common for sellar region tumor, including craniopharyngioma, germ cell tumor, pituitary tumor, such as Langerhans cell histiocytosis, empty sella, pituitary hypoplasia, pituitary stalk interruption syndrome (pituitary stalk interruption syndrome, PSIS), surgery, radiotherapy is an important reason of CDI. Among them, some congenital factors. Pituitary occupying, pituitary stalk injury, intracranial tumor or infection, brain trauma can cause except CDI some pituitary gland function to reduce the corresponding target performance, performance in growth hormone deficiency is 1 or more than 1 kinds of vertical Before or after * leaves and leaf hormone deficiency, namely multiple pituitary hormone deficiency (multiple pituitary hormone deficiency, MPHD). Most reported that the posterior pituitary disappeared high signal is characteristic of CDI, but there are reports that nephrogenic diabetes insipidus may also have high posterior pituitary signal disappeared, some CDI also leaves high the signal is not disappeared after pituitary. This paper analyzed the MRI findings of 68 cases of children with CDI, water deprivation vasopressin test, pituitary target gland function, and response to treatment, to investigate the correlation between MRI and CDI in pituitary hypothalamic area.
Research methods:
1. subjects: 68 children with CDI, aged (10.63 + 3.18) years old, were analyzed by MRI, water free vasopressin test and treatment effect.
2.MRI examination: 68 patients underwent Siemens3.0T MRI, all patients underwent pituitary sagittal, coronal T1WI, plain T2WI, thickness of 3mm. contrast agent GD DTPA (Gd-DTPA), the dose of 0.1mmol/kg, 'the quick push after contrast enhanced scanning. The main observation of pituitary the morphology of the anterior pituitary, pituitary height, position and size, pituitary stalk morphology, sellar region, parasellar structures have no other abnormal findings.
3. pituitary target gland function and tumor marker detection: all the children had diabetes insipidus fasting blood, pituitary thyroid axis function were determined by measuring the serum thyroid stimulating hormone (TSH), free triiodothyronine three code (FT3), free thyroxine (FT4); pituitary adrenal axis function: measurement of adrenocorticotropic hormone (ACTH), cortisol (COR); pituitary gonadal axis function: measurement of follicle stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2), testosterone (TO), prolactin (PRL) and tumor markers of human chorionic gonadotropin (beta-HCG) and alpha fetoprotein (AFP); growth hormone - insulin-like growth factor axis determination (GH-IGF-1).
The results of the study:
1.68 cases of CDI patients, including 18 cases (male 14 cases) for craniopharyngioma after surgery, 15 patients (14 males) to complete posterior pituitary pituitary stalk interruption and disappeared in 15 cases (male 13 cases) PSIS and ectopic posterior pituitary and nearly disappeared (bright spot is very small), 18 cases pituitary stalk thickening and posterior pituitary disappeared (including 8 cases of germ cell tumors, 10 cases of Langerhans cell histiocytosis), 2 cases of CDI secondary diabetes insipidus (NDI).
2. of them were accompanied by a variety of different levels of pituitary hormone deficiency.
3., 2 of the patients with longer history were accompanied by mental retardation and renal complications, such as hydronephrosis, ureteral dilatation and bladder distention. They were not sensitive to simple coagulation therapy, and combined with hydrochlorothiazide treatment.
The conclusions are as follows:
1. hypothalamus pituitary area MRI is closely related to the function of posterior pituitary. Central lobe diabetes insipidus is characterized by MRI showing a high signal loss or weakening of posterior pituitary lobe. It can be diagnosed by combining with water blocking vasopressin test.
2. in secondary CDI, MRI combined with pituitary hormones and tumor markers is of great significance in the diagnosis of the cause of the disease.
3.MRI combined with the urinary system B ultrasound and the treatment of coagulation therapy, CDI can be found to be associated with NDI. It should be combined with mica, hydrochlorothiazide and indomethacin.

【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R725.8

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