本文關(guān)鍵詞： 中樞性尿崩癥 垂體后葉高信號(hào)消失 多種垂體激素缺乏 腫瘤 垂體柄中斷綜合癥　出處：《山東大學(xué)》2014年碩士論文　論文類型：學(xué)位論文

【摘要】：研究背景和目的： 中樞性尿崩癥(central diabetes insipidus,CDI)是以煩渴、多尿、排出大量低滲、低比重尿?yàn)樘卣鞯呐R床綜合征,因抗利尿激素(antidiuretic hormone,ADH)合成和/或分泌減少而致病。本病嚴(yán)重危害兒童的身心健康,由于晚上不能正常睡眠,食欲差,如果長(zhǎng)期得不到正確治療,會(huì)導(dǎo)致患兒身材矮小,泌尿系統(tǒng)擴(kuò)張,甚至繼發(fā)腎功衰竭,影響患兒壽命。并且CDI也是下丘腦-垂體區(qū)腫瘤的早期表現(xiàn),因此磁共振成像(magnetic resonance imaging, MRI)對(duì)CDI診斷有重要價(jià)值。ADH為主的神經(jīng)內(nèi)分泌顆粒由下丘腦神經(jīng)核分泌,主要存儲(chǔ)于垂體后葉,并因此可在磁共振成像(MRI) T1WI圖像中形成高信號(hào),垂體后葉高信號(hào)消失或縮小對(duì)CDI的診治有重要意義。根據(jù)病因不同,CDI分為原發(fā)性和繼發(fā)性CDI,原發(fā)性包括特發(fā)性或遺傳性,由于不明原因的下丘腦神經(jīng)分泌細(xì)胞不能分泌ADH所致,繼發(fā)性由于多種原因破壞下丘腦或下丘腦垂體通路而致病,最常見(jiàn)為鞍區(qū)腫瘤,包括顱咽管瘤、生殖細(xì)胞瘤、垂體瘤等,其他如郎格罕細(xì)胞組織細(xì)胞增生癥、空蝶鞍、垂體發(fā)育不良、垂體柄中斷綜合癥(pituitary stalk interruption syndrome, PSIS)、手術(shù)、放療等也是CDI重要原因。其中,某些先天性因素、垂體占位、垂體柄的損傷、顱內(nèi)腫瘤或感染、顱腦創(chuàng)傷等可引起除CDI以外的一些垂體相應(yīng)靶腺功能減低的表現(xiàn),表現(xiàn)為除生長(zhǎng)激素缺乏外還存在1種或1種以上垂體前葉和*或后葉激素缺乏,即多種垂體激素缺乏癥(multiple pituitary hormone deficiency,MPHD)。大多數(shù)報(bào)道認(rèn)為垂體后葉高信號(hào)消失是CDI的特點(diǎn),但有報(bào)道認(rèn)為腎性尿崩癥也可有垂體后葉高信號(hào)消失,有的CDI也可有垂體后葉高信號(hào)不消失。本文分析了68例CDI患兒的MRI表現(xiàn),禁水加壓素試驗(yàn),垂體靶腺功能,及治療反應(yīng),探討下丘腦-垂體區(qū)MRI與CDI的相關(guān)性。 研究方法： 1.觀察對(duì)象：68例CDI患兒,年齡(10.63±3.18)歲,根據(jù)MRI,禁水加壓素試驗(yàn)及治療效果進(jìn)行病因分析。 2.MRI檢查：68例均采用Siemens3.0T核磁共振掃描儀,所有病例均行垂體矢狀位、冠狀位T1WI、T2WI平掃,層厚為3mm。對(duì)比劑使用釓噴酸葡胺(Gd-DTPA),劑量為0.1mmol/kg,'快速手推對(duì)比劑后即進(jìn)行增強(qiáng)掃描。主要觀察垂體的形態(tài)、垂體前葉高度,垂體后葉位置及大小,垂體柄的形態(tài),鞍區(qū)、鞍旁結(jié)構(gòu)有無(wú)其他異常表現(xiàn)。 3.垂體-靶腺功能及腫瘤標(biāo)志物檢測(cè)：所有尿崩癥患兒就診時(shí)均需清晨空腹抽血,分別測(cè)定垂體-甲狀腺軸功能：測(cè)血清促甲狀腺激素(TSH)、游離三典甲狀腺原氨酸(FT3)、游離甲狀腺素(FT4)；垂體-腎上腺軸功能：測(cè)促腎上腺皮質(zhì)激素(ACTH),皮質(zhì)醇(COR)；垂體-性腺軸功能：測(cè)卵泡刺激素(FSH)、黃體生成素(LH)、雌二醇(E2)、睪酮(TO)、泌乳素(PRL)及腫瘤標(biāo)志物絨毛膜促性腺激素(beta-HCG)及甲胎蛋白(AFP)；生長(zhǎng)激素-胰島素樣生長(zhǎng)因子軸測(cè)定(GH-IGF-1). 研究結(jié)果： 1.68例CDI患兒,其中18例(男14例)為顱咽管瘤術(shù)后,15例(男14例)為完全垂體柄阻斷并垂體后葉消失,15例(男13例)為PSIS并垂體后葉異位且接近消失(亮點(diǎn)非常小),18例為垂體柄增粗并垂體后葉消失(其中8例為生殖細(xì)胞瘤,10例為朗格罕細(xì)胞組織細(xì)胞增生癥),2例為CDI繼發(fā)腎性尿崩癥(NDI)。 2.其中50例伴有不同程度多種垂體激素缺乏。 3.其中2例病史較長(zhǎng)者均伴有智力低下,并已出現(xiàn)腎臟并發(fā)癥,如腎積水、輸尿管擴(kuò)張,膀胱擴(kuò)張等,且對(duì)單純彌凝治療不敏感,聯(lián)合氫氯噻嗪治療效果較好。 研究結(jié)論： 1.下丘腦-垂體區(qū)MRI與垂體后葉功能密切相關(guān),中樞性尿崩癥患兒MRI呈現(xiàn)垂體后葉高信號(hào)消失或減弱,結(jié)合禁水加壓素試驗(yàn)可以確診。 2.在繼發(fā)性CDI,MRI結(jié)合垂體激素和腫瘤標(biāo)志物檢查對(duì)病因診斷有重要意義。 3.MRI結(jié)合泌尿系B超、彌凝治療反應(yīng),可發(fā)現(xiàn)CDI并發(fā)NDI,應(yīng)聯(lián)合應(yīng)用彌凝、氫氯噻嗪及吲哚美辛治療。
[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.

【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2014
【分類號(hào)】：R725.8

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