让垂体瘤的治疗更趋完善

垂体瘤患者术后进行垂体功能的评估可以及时地对垂体功能减退者进行合适的替代治疗.但我们当前的临床现实是能够接受恰当的术后评估的患者还不到50％.只有加强内分泌科与神经外科医师的技术互补协作以及三级医院医师和基层医院医师的密切联系,提供以患者为中心的治疗才能弥补当前垂体瘤治疗存在的缺陷.     

垂体瘤患者植入永久心脏起搏器后并发垂体危象一例

患者男性,75岁.主因阵发性头晕5年加重2天入院.5年来患者间断性头晕,3～5 min可以缓解,自觉大脑"空白感",发病时自摸脉搏有长间歇,曾经晕厥1次,外院诊断为"房室传导阻滞",最近5天患者头晕发作频繁就诊.既往患者高血压病史30年,冠心病史15年,2年前发现"垂体瘤伴纤维化",曾在外院查垂体功能正常,未做特殊处理.     

空泡蝶鞍伴垂体前叶功能减退23例

<正>空泡蝶鞍在临床并非少见,是指蛛网膜下腔疝入蝶鞍内,蝶鞍被蛛网膜下腔中的脑脊液占据使其扩大,垂体受压而产生一系列临床综合征〔1〕。除产生头痛、视力下降、视野损害等占位效应外,部分患者可伴发垂体前叶功能减退。在手术、外伤、感染等应激状态下甚至发生垂体危象。本文就我院确诊的空泡蝶鞍伴垂体前叶功能减退患者的临床资料进行临床分析。1临床资料1.1一般情况2011年1月至2013年12月在我院确诊的空     

垂体危象及垂体卒中

垂体危象是指垂体前叶功能减退时,肾上腺皮质激素和（或）甲状腺激素缺乏,使机体应激能力下降,在感染、呕吐、腹泻、脱水、寒冷、饥饿、缺血坏死等情况下及应用安眠药或麻醉剂时可诱发危象.若不及时诊断及治疗,常可危及生命.垂体卒中是垂体危象的病因之一,在临床上也并非少见,但并非所有的垂体卒中均可导致垂体危象.本文就导致垂体危象及垂体卒中的常见病因、临床表现及治疗作一介绍.     

垂体瘤术后并发垂体前叶功能减退致甲状腺功能减退性心脏病一例

正垂体瘤是常见的颅脑肿瘤,约占颅内肿瘤的5%~20%。根据能否合成和分泌有生物活性的激素,临床上将垂体瘤分为功能性瘤(包括生长激素瘤、泌乳素瘤、促肾上腺皮质激素瘤、促甲状腺激素瘤)和无功能瘤。除泌乳素瘤外,手术(经颅或者经蝶窦入路)是首选的治疗方式。据文献报道,垂体微腺瘤(直径1 cm)手术切除治疗的成功率是70%左右,而大腺瘤则≤30%~([1])。因此,对于大腺瘤,术后定向放疗,如伽玛刀是一种较为安全的辅助治疗方式。术后伽玛刀放疗     

垂体前叶功能减退患者经皮冠状动脉介入治疗后诱发垂体功能急性失代偿一例

患者男性,42岁。主因“突发全身酸痛伴恶心、呕吐、胸闷3天”于2006年11月27日入院。患者10天前曾于我院住院,行冠状动脉造影提示左主干开口70％狭窄,前降支近段50％～60％狭窄,右冠状动脉近段50％狭窄,于左主干置入Endeavor4．0mm×15mm支架1枚（美敦力公司）。并给予辛伐他汀（商品名：京必舒新）20mg每晚1次（连续10天）及其他抗冠心病治疗出院。2006年11月24日患者出现感冒,体温最高37．5℃左右,对症治疗后体温正常,但持续恶心、呕吐,呕吐物为胃内容物,连续3天未能进食,并感全身酸痛不适。11月26日再次转入我院,入院诊断：冠心病,自发性心绞痛,PTCA加支架术后,心包积液原因待查,怀疑横纹肌溶解症;颅咽管瘤术后、放疗后,低钠血症收住病房。1997年曾于外院行颅咽管瘤切除术。     

流行性出血热导致垂体前叶功能减退一例报道

垂体前叶功能减退症是多种病因引起的一种综合征。多由产后大出血和垂体瘤引起，流行性出血热导致垂体前叶功能减退在临床上报道少见，现报道一例如下：     

垂体危象

垂体危象是指慢性垂体前叶功能减退的患者在应激的情况下发生休克或昏迷等危象表现。这是一种较罕见的急危重症。若发生危象前，无明确患有慢性垂体前叶功能减退症者，则诊断就十分困难了，需要医生的敏锐的观察力和抓住重点地询问有关病史。此时任何激素的测定因不能及时报告对指导垂体危象抢救都没有现实意义。慢性垂体前叶功能减退症的病因主要是席汉综合征Gheehansvndrome）和垂体及垂体旁的肿瘤。席汉综合征是产后大失血所致，多见于偏僻农村、文化卫生知识较低的妇女，现在城市大医院已很少见。垂体及垂体旁的肿瘤因压迫正常的垂体组…     

糖尿病合并垂体前叶功能减退症一例

患者，女，54岁。10年前患者出现口渴、多饮、多尿、伴消瘦、视力下降，到医院检查发现血糖高、尿糖阳性、尿酮阴性，并行口服葡萄糖耐量试验，胰岛素测定，确诊为2型糖尿病，一直用口服降糖药(优降糖，二甲双胍)降血糖治疗，病人症状改善，血糖下降(空腹血糖5～7mmol／L)，眼底及尿微量白蛋白检查未见异常。     

垂体腺瘤与女性生育功能

育龄期女性垂体腺瘤的发生率越来越高,由于垂体的重要位置和功能,垂体腺瘤与育龄期女性的生育功能有密切关系.性腺功能的破坏、高催乳素血症及腺体本身的高分泌状态是各类型垂体腺瘤影响生育功能的共同机制.另外,垂体腺瘤本身或是手术治疗后引起垂体前叶激素缺乏也可影响生育功能.对有生育要求的育龄期垂体腺瘤女性,选择合适的治疗方法以及对垂体前叶激素缺乏患者早期正确替代治疗,对恢复生育功能有重要意义.     

Postoperative assessment of the patient after transsphenoidal pituitary surgery

While most transsphenoidal pituitary surgery is accomplished without complication, monitoring is required postoperatively for a set of disorders that are specific to this surgery. Postoperative assessments are tailored to the early and later postoperative periods. In the early period, which spans the first few weeks after surgery, both monitoring of anterior and posterior pituitary function and managing neurosurgical issues are the focus of care. Potential disruption of pituitary-adrenal function is covered with perioperative glucocorticoids. Various strategies exist for ensuring the integrity of this axis, but typically this is done by measuring a morning cortisol on the 2nd or 3rd postoperative days. Patients with levels <10 μg/l should continue therapy with reassessment in the later postoperative period. Monitoring for water imbalances, which are due to deficiency or excess of ADH (DI or SIADH, respectively), is accomplished by continuous accounting of fluid intake, urine output and specific gravities coupled with daily serum electrolyte measurements. DI is characterized by excess volumes of inappropriately dilute urine, which can lead to hypernatremia. Most patients maintain adequate fluid intake and euvolemia, but desmopressin therapy is required for some. SIADH, which peaks in incidence on 7th postoperative day, presents with hyponatremia that can be severe and symptomatic. Management consists of fluid restriction. Neurosurgical monitoring is primarily for disturbances in vision or neurological function, and although uncommon, for CSF leak and infections such as meningitis. In the later postoperative period, the adrenal, thyroid and gonadal axes are assessed. New persistent hypopituitarism is rare when transsphenoidal surgery is performed by an experienced surgeon. Various strategies are available for assessing each axis and for providing replacement therapy in patients with deficiencies. Long term monitoring with assessments of visual, neurological and pituitary function coupled with pituitary imaging is necessary for all patients who have undergone surgery, irrespective of the hormone status of their tumors.     

Nonfunctioning pituitary adenoma: incidence,causes of death and quality of life in relation to pituitary function

The incidence of clinically significant, nonfunctioning pituitary adenoma, requiring surgical treatment, has not been established. According to previous studies, both surgery type and subsequent radiotherapy may have an impact on quality of life (QOL), and some studies have shown increased cardiovascular mortality in patients with pituitary disease. We studied all patients with functionless, suprasellar pituitary adenoma who were operated on during the period 1985–1996 (N = 192; transsphenoidal surgery = 160, craniotomy = 32). QOL was evaluated from Short Form 36 and Major Depression Inventory questionnaires. Causes of death were obtained from the Danish Register of Causes of Death. Incidence was 5.6/mill/year. Postoperatively, 27% of the patients had normal pituitary function and 27% were panhypopituitary. Fifty three patients had died. Death from cardiovascular, cerebrovascular and malignant diseases was not increased. Overall, QOL was not different from QOL of an age- and sex matched background population. QOL was, however, impaired in patients who had undergone craniotomy as compared to patients undergoing transsphenoidal surgery. QOL was not reduced in hypopituitary patients or in patients receiving radiotherapy. The study was supported by grants from The Aarhus University Hospital Research Initiative and The Obel Family Foundation, Aalborg, Denmark     

Brief review and commentary: Diagnosis of pediatric pituitary disorders

Pediatric pituitary diagnosis is complicated by the unique developmental characteristics, secretory patterns and regulation of each pituitary hormone system. Although dynamic test procedures have been described, validation of each procedure is limited and universally agreed diagnostic criteria are not available. Clinical acumen, experience and judgment continue to be primary elements of pituitary diagnosis.     

Multidisciplinary protocol of preoperative and surgical management of patients with pituitary tumors candidates to pituitary surgery

The optimal planning of preoperative diagnosis, management and treatment of pituitary tumors (PT) candidates to pituitary surgery (PS) requires a multidisciplinary approach involving a team of endocrinologists, neurosurgeons, ENT, neuro-ophthalmologists and neuroradiologists with experience in pituitary diseases. Such teams improve surgical results, minimize complications and facilitate their correct treatment if occurring, and optimize the hormonal, ophthalmological and radiological preoperative and follow-up evaluation. We have developed a clinical practice protocol for patients with PT who are candidates to PS based on the most recent national and international guidelines and the relevant literature regarding PT published in the last years. The protocol has been elaborated by a multidisciplinary team of a Spanish Pituitary Tumor Center of Excellence (PTCE) that includes at least one neurosurgeon, ENT, neuroradiologist, neuro-ophthalmologist, endocrine pathologist and endocrinologist specialized in pituitary diseases. We elaborated this guideline with the aim of sharing our experience with other centers involved in the perioperative and surgical management of PT thereby facilitating the management of patients undergoing PS.     

Mortality following pituitary radiotherapy

External beam radiotherapy has been used in the management of pituitary adenomas for nearly a century, preventing tumor regrowth following surgery for non-functioning pituitary adenomas and suppressing functional hypersecretion in those which are hormonally active. However, it has been linked with a number of potentially significant complications including formation of secondary intracranial tumors, cognitive impairment, hypopituitarism and cerebrovascular disease, as well as increased mortality. Radiation may cause a variety of vascular injuries and hemodynamic changes to the cerebral vasculature, and several authors have reported cerebrovascular complications and an increase in cerebrovascular mortality in patients receiving radiotherapy for pituitary and other central nervous system tumors. Ten years following pituitary radiotherapy, over 50% of patients develop deficiencies in one or more anterior pituitary hormones. A number of studies have demonstrated increased mortality in patients with hypopituitarism, predominantly due to cerebrovascular and cardiovascular disease. However, no clear answer has emerged with regards to causation, and pituitary radiotherapy has only been linked directly to mortality in one of these studies. Questions remain unanswered, and the use of conventional external beam radiotherapy in the management of pituitary disease must involve a critical risk-benefit analysis in each case.     

Waxing and waning of a pituitary mass in a young woman with combined pituitary hormone deficiency (CPHD) due to a PROP-1 mutation

We describe a 23-yr old woman with congenital combined pituitary hormone deficiency (CPHD) diagnosed at 10 years of age and a large sellar mass discovered at the age of 19 years, when her first pituitary MRI was performed. The mass (height: 13 mm) extended to the suprasellar region, close to the optic chiasm, showed signal hyperintensity in T1- and hypointensity in T2-weighted images, with no enhancement after gadolinium injection. Although these MRI features were suggestive of Rathke’s cleft cyst, cystic craniopharyngioma or previous hemorrhage, no visual symptoms, diabetes insipidus and/or hyperprolactinemia were present. In addition, similar MRI findings had been previously described in a few cases of CPHD due to PROP-1 mutations, which prompted us to carry out a molecular study before any therapeutic decision was made. A 301302delAG PROP-1 mutation was found in her DNA and the patient was closely followed through ophthalmologic evaluation and pituitary MRI scans. During a 3.6-year follow-up, we were able to document a marked initial growth followed by shrinkage and recurrent growth of the PROP-1 sellar mass. The patient remains free of compressive neuro-ophthalmological signs, suggesting that surgical intervention is unnecessary in these cases. However, they must be followed closely with sellar MRIs and campimetry until the mass completely regresses.     

Nelson's syndrome: course of aggressive pituitary corticotroph adenoma

Nelson's syndrome was defined in 1958 as the association of an expanding pituitary tumor with high ACTH secretion after bilateral adrenalectomy for Cushing's disease. Pituitary MRI and ACTH measurements led to the definition of Nelson's syndrome as the proliferation of a corticotrophic microadenoma or an aggressive and highly proliferative tumor residue induced by the decreased glucocorticoid inhibition after bilateral adrenalectomy. Now, the problem is not the definition of Nelson's syndrome but rather the identification of markers predictive of tumor growth. Based on a typical case and a review of the literature, we point out some predictive markers of tumor growth after bilateral adrenalectomy: young age at diagnosis, presence of tumor residue on pituitary MRI before adrenalectomy, markers of tumor aggressiveness (Ki-67>3%, mitoses, nuclear PTTG) and increase of ACTH levels during the first months following adrenalectomy.     

垂体瘤切除术对垂体瘤并发心脏扩大患者心功能的影响

目的 回顾分析垂体瘤切除术对垂体瘤并发心脏扩大患者的心功能变化的影响.方法 收集解放军总医院2005 -2011年经垂体瘤切除术治疗的14例垂体腺瘤并发心脏扩大患者临床资料,对比分析手术前后患者心功能情况及术后恢复情况,利用超声心动图等无创检测手段检测患者术前及术后血清生长激素(GH)、左室舒张末期内径(LVEDD)、室间隔厚度(ST)、左室后壁厚度(LVPWT)和左室射血分数(LVEF),进行对比分析.结果 垂体瘤切除术后GH、ST、LVEF、LVPWT 平均值较术前均显著改善(分别为GH 93.89 μg/L比5.16 μg/L、ST 11.13 mm比10.64 mm、LVEF 43.92％比49.28％、LVPWT 10.53 mm比8.87 mm).手术前后GH差值与手术前后ST、LVEDD、LVEF差值呈线性关系,与手术前后LVEF差值呈正相关,与手术前后LVEDD和ST差值呈负相关.结论 垂体瘤切除术用于治疗垂体腺瘤并发心脏扩大可显著改善患者心功能,其心功能恢复程度和手术前后GH差值关系明显.     

五例垂体柄中断综合征的临床诊断

目的 提高对垂体柄中断综合征(PSIS)的认识和诊断水平.方法 回顾分析5例PSIS患者的临床表现、垂体前叶及各靶腺轴和垂体后叶功能、影像学表现.结果 PSIS患者5例,其中男性4例,年龄17～21岁,3例有难产史,1例有头颅外伤史,均表现为不同程度的生长发育障碍,1例有畏寒、反应迟钝等甲状腺功能减退症状,均无多饮和低比重尿.所有患者存在生长激素缺乏、低促性腺激素性性腺功能减退和肾上腺皮质储备功能不足,4例患者同时存在中枢性甲状腺功能减退,3例临床并无相应表现.磁共振检查显示所有患者垂体柄和垂体后叶正常高信号消失,而在视交叉下(4例)、蝶窦内(1例)见到点状异常高信号,伴部分空蝶鞍.结论 PSIS以生长发育迟缓为主要临床表现,部分性或完全性垂体前叶功能减退而垂体后叶功能正常,磁共振检查呈现的特征性改变为重要诊断依据.