胚胎脑垂体移植治疗席汉氏综合症的临床研究

采用胎龄为６个月左右水囊引产的胚胎脑垂体植入股四头肌内，并将血管束植入垂体内的颅外移植法治疗产后大出血所致的席汉氏综合症。结果：与术前相比，内分泌激素水平显著增高（Ｐ＜０．０５），临床症状明显改善。提示：移植的脑垂体存活，并具有分泌功能；颅外移植法治疗席汉氏综合症有一定临床价值。     

斜坡异位垂体腺瘤2例并文献复习

目的 探讨罕见的斜坡异位垂体腺瘤的临床和影像学特点、诊断和治疗.方法 对我院收治的2例经病理证实为斜坡异位垂体腺瘤进行观察,简要复习相关文献,总结其临床、神经影像、诊断和治疗等方面的特点.结果 临床表现为内分泌紊乱或/和神经功能障碍症状,影像检查均显示斜坡部位块影.1例术前诊断为异位垂体腺瘤;1例术前误诊为脊索瘤.均采用单鼻孔-蝶窦入路,显微手术切除肿瘤.1例镜下肿瘤全切,术后激素水平降至正常,随访1年无复发;1例部分切除,术后并发脑脊液鼻漏和颅内感染,治愈后失访.结论 斜坡异位垂体腺瘤主要表现为内分泌紊乱或/和神经功能障碍症状,颅脑MRI具有一定的诊断价值.尽管罕见,斜坡部位肿瘤的鉴别诊断也应考虑此类肿瘤;手术全切肿瘤可获得理想的疗效.     

功能性垂体腺瘤患者性功能障碍的研究进展

 功能性垂体腺瘤不仅会产生内分泌紊乱的症状,而且常引发性功能障碍。功能性垂体瘤与性功能之间的关系逐渐得到重视,其发生机制可能与垂体肿瘤影响下丘脑-垂体-性腺轴有关。治疗上应首先治疗垂体肿瘤原发病,在肿瘤被切除或控制后,则可选择补充睾酮或应用磷酸二酯酶5型抑制剂治疗性功能障碍。本文就功能性垂体腺瘤患者性功能障碍的发病率、临床表现、发病机制、诊断及治疗的最新研究进展作一综述。     

原发性甲状腺功能减退致垂体增生两例报告及文献复习

目的:探讨原发性甲状腺功能减退致垂体增生的临床特点、发病机制、诊断和处理原则。方法:结合文献回顾性分析2例原发性甲状腺功能减退致垂体增生的临床资料。结果:2例均表现为促甲状腺激素(TSH)及泌乳素(PRL)增高,FT3及FT4减少,MRI示垂体增大,甲状腺素替代治疗6个月后,垂体体积恢复正常,内分泌检查亦恢复正常。结论:原发性甲状腺功能减退致垂体增生具有影像学特征,结合患者的临床症状、病史及内分泌检查可明确诊断。治疗首选甲状腺素替代治疗,而不宜行手术治疗。     

免疫检查点抑制剂主要内分泌不良反应急症处理中国专家共识

免疫检查点抑制剂（immune checkpoint inhibitors,ICPis）是新兴的抗肿瘤药物,内分泌不良反应是其较为常见的药物不良反应,主要累及多个内分泌腺如甲状腺、垂体、肾上腺和胰腺等,导致内分泌功能紊乱。其急症如不能在早期得到正确的鉴别和防治,可能会危及生命。国内外虽已推出相关指南/共识多部,但国内尚无针对ICPis导致内分泌不良反应急症的诊治流程和共识。为规范和提高相关科室临床诊治水平,本共识回顾分析了多项国内外专家共识和临床研究,综合肿瘤学、内分泌学和护理学专家意见后制订本共识。     

Cerebral Histiocytosis-X with Endocrine Symptoms

A neuropathologically verified case of histiocytosis-X in a 21-year-old man with endocrine symptoms is presented. The granulomas were confined to the central nervous system and occupied mainly the pituitary stalk and hypothalamus, thus giving us the opportunity to observe the endocrine symptoms caused by such localized lesions.

Diabetes insipidus was one cardinal endocrine symptom and is considered to be caused by a hypothalamic lesion in the supraoptic nuclei or the pathways to the posterior pituitary. In this case the anterior pituitary was not involved by granulomas, but the posterior lobe was atrophic.

Severe hypogonadism and probably also a growth hormone defect were present, probably resulting from pituitary insufficiency secondary to the hypothalamic lesions.

Hypothyroidism was another symptom but in this case was probably due to thyroiditis. Such a finding has previously been observed by two other authors and may therefore be pathologically connected to histiocytosis-X even though no granulomas were present in the thyroid gland.

Repeated X-ray investigations from 9 years of age showed that the development of the sella turcica ceased about the same time as the patient got signs of diabetes insipidus. Increase in sellar volume normally reflects pituitary growth. The lesions in the pituitary stalk or the hypothalamus must therefore have been present from the time when diabetes insipidus started.     

干燥综合征神经系统病变的临床及病理特征

目的探讨原发性干燥综合征（pSS）神经系统病变的临床及病理特征。方法回顾性分析10例累及神经系统的pSS患者临床及病理特征。10例均行自身抗体、脑脊液、唇黏膜滤纸试验和唇黏膜活检。6例周围神经系统病变（PNS·pSS）患者行肌电图、神经传导速度检查及腓肠神经活检,4例中枢神经系统病变（CNS—pSS）患者行头颅MRI、脑或脊髓组织病理学检查。结果PNS—pSS患者表现为对称性感觉运动性周围神经病、对称性感觉性周围神经病,肌电图及神经传导速度检查提示不同程度的髓鞘及轴索损伤;腓肠神经活检示典型血管炎性改变及以轴索和髓鞘变性为特征的非血管炎性改变。CNS—pSS患者表现为多发性硬化、亚急性横贯性脊髓炎、脑炎、垂体卒中及急性脑膜炎,头颅MRI显示多发脑白质病变、脊髓病变及垂体异常信号,病理可见脑及脊髓白质脱髓鞘及静脉周围淋巴套、典型血管炎性改变及垂体炎伴出血坏死。结论pSS可累及中枢及周围神经系统,临床表现复杂,电生理、脑脊液检查及组织病理活检有助于诊断,以细胞免疫为主的血管或组织炎性反应及非血管炎性神经损伤可能是其主要的发病机制。     

C—erbB2和EGFR在垂体腺瘤中的表达及其意义

目的 探讨Ｃ－ｅｒｂＢ２和表皮生长因子（ＥＧＦＲ）蛋白表达与垂体腺瘤激素类型及侵裘性的关系。方法 用免疫组化ＡＢＣ法对６４例垂体腺瘤进行Ｃ－ｅｒｂＢ－２和ＥＧＦＲ免疫染色,结果 Ｃ－ｅｒｂＢ２和ＥＧＦＲ仅在大多数无激素型腺瘤中阳性表达,而在其它激素类型腺瘤病中极少阳性表达。ＥＧＦＲ在侵袭性腺瘤中的阳性表达率（４１．９３％）高于非侵袭组（１２．１２％）两者差异有显著性（Ｐ〈０．０５）。结论 Ｃ－ｅｒ     

Erdheim-Chester病累及内分泌腺体的特征分析

目的 探讨Erdheim-Chester病(ECD)累及内分泌腺体的临床和影像特征。方法 回顾性分析北京协和医院2014年1月至2020年10月经病理确诊的ECD患者48例,其中内分泌腺体受累22例,总结其临床特点、影像及病理特征。结果 垂体受累17例(17/48,35.4%),肾上腺受累8例(8/48,16.7%),垂体和肾上腺同时受累3例(3/48,6.25%)。累及垂体患者最常见的症状为中枢性尿崩症(13/17,76.5%),影像表现为T1加权成像垂体后叶高信号消失,垂体柄增粗,垂体异常强化结节;累及肾上腺患者最常见的症状为肾上腺功能减低(3/8,37.5%),影像表现为CT图像上肾上腺弥漫增粗。ECD累及内分泌腺体患者中BRAF V600E突变阳性13例(13/22,59.1%)。结论 垂体和肾上腺是ECD内分泌腺体受累最常见的部位,结合其临床特点、影像及病理特征可作出明确诊断,及早进行干预治疗。     

Spontaneous remission of acromegaly or gigantism due to subclinical apoplexy of pituitary growth hormone adenoma

Background  Subclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion. The typical presence of pituitary growth hormone (GH) adenoma is gigantism and/or acromegaly. We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma.

Methods  Six patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed.

Results  In these cases, the initial clinical presences were diabetes mellitus or hypogonadism. No abrupt headache, vomiting, visual function impairment, or conscious disturbance had ever been complained of. The base levels of GH and insulin growth factor-1 (IGF-1) were normal or higher, but nadir GH levels were all still >1 μg/L in 75 g oral glucose tolerance test. Magnetic resonance imaging detected enlarged sella, partial empty sella and compressed pituitary. The transsphenoidal surgery was performed in 2 cases, and the other patients were conservatively managed. All the patients were in clinical remission.

Conclusions  When the clinical presences, endocrine evaluation, biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly, the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed. To these patients, conservative therapy may be appropriate.

     

原发性甲状腺功能减低症与垂体腺瘤的鉴别诊断

目的探讨原发性甲状腺功能减低致垂体增生的临床特点和诊治方法,避免误诊。方法9例患者各经X线平片、CT、MRI等影像检查发现鞍内鞍上占位性病变,但因影像学及临床特点不典型,经内分泌检查、骨龄检查等确诊为原发性甲状腺功能减低导致垂体增生,遂采用激素替代治疗。结果9例患者均口服甲状腺片治疗,随访4~18个月,内分泌检查除TSH仍高于正常,其余均恢复正常,症状均有不同程度减轻,其中2例患者复查MRI发现病变明显缩小。结论原发甲状腺功能减低,易误诊为垂体腺瘤,应进行内分泌检查、骨龄检查等明确诊断,治疗采用激素替代治疗。     

Pituitary function with a solitary intrasellar plasmacytoma

A solitary intrasellar plasmacytoma with marked pituitary fossa destruction and yet near normal pituitary gland function is described. We suggest that a minimal disturbance of endocrine function together with a radiologically abnormal pituitary fossa indicates that the primary lesion may lie outside the pituitary fossa.     

空蝶鞍综合征20例的临床和MRI分析

分析２０例空蝶鞍综合征（ＥＳＳ）的ＭＲＩ表现和其中１６例的内分泌改变。结果１６例ＥＳＳ均有内分泌功能异常。ＭＲＩ表现有蝶鞍充满长Ｔ＿１和长Ｔ＿２的脑脊液;垂体受压至鞍底,冠状位呈“锚”状,矢状位呈“新月”状;垂体柄延长。结果表明ＥＳＳ常具有内分泌功能紊乱,ＭＲＩ对ＥＳＳ具有特征性诊断意义。     

Magnetic resonance spectroscopy in pituitary tuberculoma

Magnetic resonance spectroscopy (MRS) is a new, noninvasive method of diagnosing a lesion in cases where magnetic resonance (MR) imaging cannot reliably differentiate between two or more possible aetiologies. This case report describes a 20-year-old pregnant woman who developed sudden onset of left-sided hemiparesis. MR imaging of the brain revealed an infarct of the right middle cerebral artery and a suprasellar mass. The endocrine workup was normal. As she was 20 weeks pregnant, the option of a transsphenoidal biopsy of the pituitary lesion was rejected in favour of MRS . It demonstrated features characteristic of a tuberculoma. She showed marked clinical improvement after she was started on anti-tuberculous drugs. MRS is a rapidly-developing diagnostic modality, and may be a useful and safe option for investigating intracranial lesions in patients who cannot undergo invasive procedures.     

类固醇生成因子-1与代谢综合征关系的研究进展

类固醇生成因子-1(SF-1)是一种孤儿核受体,在人类下丘脑、垂体、肾上腺和性腺等组织中均有表达,对内分泌器官的分化和发育,以及内分泌系统的代谢调节发挥重要的作用。通过对SF-1基因敲除小鼠模型的研究以及对人类SF-1基因突变表现型的分析,逐步明确SF-1在下丘脑—垂体—肾上腺轴和下丘脑—垂体—性腺轴的各个环节均参与调控。最近有研究表明,SF-1基因的突变和缺失,将导致一系列内分泌系统的代谢紊乱,最终导致糖尿病、肥胖和高血压等一系列代谢综合征的症候群。文章就SF-1与代谢综合征关系的研究进展作一综述。     

垂体卵泡刺激素腺瘤

垂体卵泡刺激素腺瘤是一种特殊类型的内分泌肿瘤,该病发病率低,患者临床表现多样化,故容易误诊和漏诊。近年来国内外学者对其病因和发病机制进行了深入研究,了解垂体卵泡刺激素腺瘤的临床特点及鉴别诊断对此类患者的诊治具有重要意义。     

人参皂甙对大鼠离体腺垂体细胞促性腺激素分泌功能影响的研究

应用人参有效成份二醇组甙和三醇组甙及其单体Rb_1和Rg_1作用于预培养3天后的大鼠腺垂体细胞,观察其对促性腺激素分泌的影响。两种组甙在10~(-1)～10~(-10)mg/ml浓度作用下,对LH分泌均有促进作用,其相应单体也呈现相似效应,表现出双相特点,即在低浓度出现第二个效应峰。同时观察了对FSH分泌功能的影响,其效应基本与LH分泌变化趋势相一致。     

Aneurysms of the Internal Carotid Artery Simulating Pituitary Tumours: Report of Two Cases

Two patients suffering from aneurysms of the internal carotid artery simulating pituitary tumours are reported. One of these was a 17-year-old boy with a mycotic aneurysm of the right internal carotid artery causing failure of growth and pubertal maturation. The second was a 53-year-old woman with a left internal carotid artery aneurysm, probably of atherosclerotic origin, who had evidence of anterior pituitary insufficiency.

In both cases there was paresis of extraocular muscles and one patient had bitemporal hemianopsia. While lateral radiographs of the skull suggested intrasellar calcification in each case, further roentgenological investigation established that this calcification was actually extrasellar, and carotid arteriograms demonstrated the presence of an internal carotid aneurysm in each case. Investigations of the endocrine system confirmed the presence of anterior pituitary insufficiency in both patients, and diabetes insipidus became evident in one patient after cortisone therapy was commenced.

This lesion, while rare, may be difficult to differentiate from a pituitary tumour. It is likely that this syndrome depends on aneurysmal expansion to the region of the sella turcica with compression of the pituitary gland itself, as well as the optic fibres.

     

29例垂体腺瘤的临床分析

目的 探讨垂体腺瘤临床特征和单鼻腔蝶窦入路手术体会。方法 对因患此病入院29例患者的临床资料进行了回顾性分析。结果 本组29例患者中27例手术顺利完成,术后未见并发症,另外2例口服用药有效。结论 垂体腺瘤大于1cm以上可以考虑手术为主要治疗方式,诊断主要依赖病史、体征、内分泌检查及影像学检查。     

51例垂体腺瘤临床分析

目的：探讨垂体腺瘤的临床表现、诊断及治疗方法。方法：对51例垂体腺瘤患者的临床表现、影像资料及治疗方法和结果进行回顾性分析。结果：视力障碍、内分泌紊乱和头痛是垂体腺瘤最主要临床表现。51例者中,42例采用手术治疗,8例化疗,10例放疗。术后42例患者出现一过性水电解质紊乱,6例患者出现一过性脑脊液漏,4例出现垂体功能低下,4例复发,1例出现垂体危象。随访1月-1年以上,大部分病例临床症状和内分泌水平有明显改善。结论：大部分垂体腺瘤首选经鼻蝶手术治疗,其具有微创、简便和安全的特点,化疗和放疗也是有效的治疗手段和手术治疗的有益补充。